UWorld notes Pediatrics
3/1/17
UW Peds Adolescence/misc Wednesday, February 15, 2017 12:58 AM
Emergency contraception
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Ulipristal (like mefipristone in some sense), an antiprogestin that delays follicular rupture, inhibits ovulation, and impairs implantation Danazol is a testosterone derivative with progestin-like effects. This medication, in addition to GnRH agonists and progestins, is used for the treatment of endometriosis Dysmenorrhea:
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Gonococcal conjunctivitis: o Prevention: Application of topical erythromycin w/in 1 hour of birth
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Primary amenorrhea: o Isolated amenorrhea with well-developed 2ry sexual characteristics can be considered normal up to the age of 16.
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If karyotype is normal but clinical suspicion for Turner $ is high → FISH to detect mosaicism (eg, 45,X0/46,XX) that is beyond the resolution of standard karyotyping. Buccal smear for Barr bodies (inactivated X chromosomes) is an outdated and unreliable screening test for turner.
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Measles: o Rash spares the palms and soles o Rx: is typically supportive only. However, immunocompromised, pregnant, at extremes of age (eg, infants or the elderly), or who are vitamin A deficient are at greater risk for complications such as pneumonia, encephalitis, or blindness. o Treatment with vitamin A reduces the morbidity and mortality rates for patients with severe measles (eg, those requiring hospitalization) through the promotion of antibody-producing cells and regeneration of epithelial cells (eg, in the gut, lungs, and retina). HSV: o Fingers can get infected as well (+ lesions on the lips) forming what's called herpetic whitlow (vesiculopustular lesion)
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Kaplan says don't incise/drain the lesion for fear of delayed resolution, bacterial superinfection and systemic dissemination of disease. Conjunctivitis DD: o Trachoma: Chlamydia trachomatis serotype A-C. Major cause of blindness worldwide. Active phase of the disease is characterized by follicular conjunctivitis and pannus (neovascularization) formation in the cornea. Concurrent infection occurs in the nasopharynx→ nasal discharge. Dx: Giemsa stain examination of conjunctival scrapings. Rx: Topical tetracycline or oral azithromycin. Repeated infections can lead to scarring of the cornea. o Herpes simplex keratitis: Dendritic ulcer is the most common presentation. + pain, photophobia, and ↓ vision o Viral conjunctivitis: Adenovirus type 3 Conjunctiva is red with copious, watery discharge Children are more commonly infected through contaminated swimming pools
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Osteomyelitis:
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Empirical Rx in sickle cell disease pt w/ osteomyelitis = third generation cephalosporin (ceftriaxone) + anti staphylococcal (oxacillin, vancomycin) Vaginal F.B.: o Vaginal foreign bodies in children often present with foul-smelling vaginal discharge and vaginal spotting or bleeding. o Bimanual exam should never be performed in a prepubertal child as this may result in significant discomfort and trauma as the prepubertal vaginal introitus is narrow and the hymenal tissue is very sensitive. o Sedation/General anesthesia may be required to remove large foreign bodies, but a bimanual examination is not. o Toilet paper is the most common foreign body. Removal with a calcium alginate swab or irrigation with warmed fluid should be attempted after a topical anesthetic has been applied. Most common risk factor for orbital cellulitis? Bacterial sinusitis. Breast fibroadenoma:
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Although simple cysts are indistinguishable on physical exam from fibroadenomas, a cyst is most common in women age 35-50. In an adolescent, this diagnosis is less likely than a fibroadenoma. Fibroadenoma is the most common cause of breast mass in an adolescent
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Cat bites: o Most cat bites will become infected w/o abx prophylaxis
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N.B. cat scratch disease is treated w/ azithromycin (has activity against bartonella henselae). However, no need for prophylaxis in immunocompetent pt like in cat bites o Minor human or dog bites that aren't located on hands, feet, or genetalia in an immunocompetent pt can be observed w/ close f/u w/o Abx prophylaxis. #Ig hands, feet, and genetalia bites introduce bacteria on the skin as these areas are commonly contaminated :/ Neonatal conjunctivitis: o Dacryostenosis is the most common cause of neonatal eye discharge due to poor drainage of tears through the nasolacrimal duct. Rx: gentle massage
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CDC recommends that all pregnant women should be screened for chlamydia at the first prenatal visit. Repeat testing in the third trimester in all high- risk women (eg, age <25, new or multiple sexual partners)
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Lymphadenopathy:
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Palpable supraclavicular lymph nodes should also be considered pathologic until proven otherwise. Androgen insensitivity: o Cryptorchid gonads have a 1%-5% risk of developing a dysgerminoma or gonadoblastoma after puberty. o Most 46,XY individuals with complete AIS identify with the female gender identity, but the decision for gonadectomy is based on concern for malignancy and not the patient's gender identity. Patients should receive age-appropriate disclosure and families should be offered psychosocial support o Benefits from gonad-stimulated puberty ( eg, attainment of adult height) outweigh the low risk of malignancy. Therefore, a gonadectomy can be deferred until completion of puberty o Gonadectomy is performed before or during puberty in cases of incarcerated or symptomatic inguinal masses or suspected malignancy (based on imaging or tumor markers) o In contrast, gonadal dysgenesis in Turner syndrome is associated with a higher risk of malignancy (15%-30%), requiring closer surveillance and earlier gonadectomy
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Congenital infections:
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Congenital syphilis: penicillin therapy is curative and prevents the development of late manifestations (eg, frontal bossing, saddle nose, Hutchinson teeth) Suspect HIV infection when an infant has failure to thrive, LN, and thrush in the setting of maternal IVDU. Pregnant pts with risky behavior should undergo HIV antibody testing in the 1st and 3rd trimesters as it can take up to 3 months to develop detectable antibody (giving initial false negative result). Dx in infants (from birth to 18 months): PCR
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Strabismus:
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Retinoblastoma: o Every case of leukocoria is considered a retinoblastoma, until proven otherwise. Rb is the most common intraocular tumor of childhood Prepubertal vaginal bleeding:
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The most common cause of vaginal bleeding and discharge in the neonatal period is withdrawal of maternal estrogen.
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Universal screening for dyslipidemia is recommended at age 9-11 and again at age 17-21 as lipid levels are relatively stable just prior to and after puberty. Screen visual development in every well-child examination. Observe fixation and tracking. Perform cover-uncover test in older infants and children to assess for strabismus. Test visual acuity at age 3 w/ the tumbling E or Snellen chart.
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Neonatal sepsis:
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Bacterial rhinosinusitis:
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Streptococcus pneumoniae (-30%), nontypeable Haemophifus influenzae (-30%), and Moraxefla catarrhatis (-10%). Pseudomonas aeruginosa is common in nosocomial sinusitis, especially in immunocompromised patients with nasal tubes or catheters.
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Bacterial meningitis:
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Neonates do not herniate after LP, as their open fontanelles serve to relieve intracranial pressure o Infants who are critically ill (eg, status epilepticus, septic shock) may require Abx before LP Entrobius vemicularis: all household contacts should be treated Granulosa cell tumor:
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Abnormal uterine bleeding: o Adolescents w/ heavy bleeding should also be evaluated for bleeding disorders if clinically appropriate. o Sexually active adolescents should be for pregnancy o Rx options: High-dose intravenous or oral estrogen High-dose combined OCP High-dose progestin pills if contraindication to estrogen or to treat anovulatory bleeding Tranexamic acid (antifibrinolytic) if CI to estrogen and progestin. 12
Cavernous sinus thrombosis (vs orbital cellulitis): o Headache is the most common early symptom. o There's also periorbital edema, exophthalmos, and chemosis, but fundoscopy typically reveals papilledema and dilated tortuous retinal veins. o Eye swelling in CST usually begins as a unilateral but quickly (24-48 hours) involves the other eye. o Cranial nerves 3,4,5,6 involvement (eg, ptosis) Croup: o Rx: Mild (no stridor at rest): single dose of steroids (dexa) to decrease airway edema. Moderate/severe (stridor at rest): steroids + nebulized epinephrine Rabies:
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UW Peds cardiology Tuesday, February 7, 2017 12:23 AM
Prolonged QT syndrome: o Nielsen has sensory neural deafness o
Inherited syndromes are due to defect in K+ channels. Mutation in the LQT1 gene.
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Rx: avoid electrolyte derangements and meds that block K+ channels. Beta blockers with pacemaker placement can prevent cardiac arrest.
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Sudden cardiac death: o HCOM is more prevalent than long QT $. A question stem w/o enough clues to differentiate, choose HCOM. o HCOM peaks during puberty (↑ myocyte hypertrophy and fibrosis)
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Congenital heart diseases: o Pearl: Large (symptomatic) VSD classically cause a pansystolic murmur that is loudest at the left lower sternal border and a diastolic rumble at the apex due to increased flow across the mitral valve.
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Cyanotic heart diseases
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Cyanosis in the first few days → administer PGE1 immediately (rule of thumb)
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Tricuspid atresia ECG
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Hypertension in children: o The most common cause of 2ry HTN in children is fibromuscular dysplasia (20% of all cases) (seen in premenopausal women). o Physical examination reveals a hum or bruit in the costovertebral angle due to welldeveloped collaterals. o Rt renal artery is affected > Lt renal artery. o Angiography shows a "string of beads" pattern to the renal artery. Viral myocarditis: o Viral injury → myocyte necrosis w/ systolic and diastolic dysfunction.
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Chest x-ray cardiomegaly (N.B. age <1 yo nl transverse cardiothoracic ratio is 56%. age >1 year and adults nl cardiothoracic ratio is 55%)
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Heart murmurs: benign vs pathologic o Maneuvers that ↓ VR (eg, standing, Valsalva maneuvers) typically ↓ innocent murmurs.
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Carotid pulse with dual upstroke on physical exam? HCOM, due to midsystolic obstruction during cardiac contraction Turner $ has aortic root dilatation w/ increased risk of dissection.
UW Peds dermatology Saturday, February 11, 2017 7:04 AM
Child abuse burns: o Deliberate scald injuries are characterized by: sharp lines of demarcation uniform burn depth spared flexor surfaces Delayed seeking medical advice o In contrast, splash marks, poorly defined wound margins. non-uniform burn depth. and immediate presentation after injury are all features of accidents.
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Atopic dermatitis:
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Eczema herpeticum
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Scabies: caused by Sarcoptes scabiei infestation. Small pruritic papules in a linear arrangement (burrows) favor the web spaces, wrists and ankles, genitals, nipples, and waistline. o
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Neonatal rashes: o N.B. neonatal varicella: post-exposure prophylaxis (exposed to infected mother) → VZIG but in neonatal varicella (actual infection with vesicles etc) → acyclovir o Erythema toxicum: spares palms and soles. Can change appearance
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SSSS: The toxins target desmoglein 1, which is responsible for keratinocyte adhesion in the superficial epidermis Prodrome of fever, irritability, and skin tenderness. Erythema starts on the face, and generalizes w/in 24-48 hrs. Subsequent desquamation for about 5 days, and the entire process usually resolves within 1-2 weeks. Cultures from intact bullae are usually sterile, because this is a toxin mediated process The goal of treatment is to eliminate any focus of infection with antistaphylococcal antibiotics, and supportive wound care. SSSS is primarily a disease of children, but adults with renal disease or immunocompromised may also be affected. The mortality rate is low in pediatric patients, but can be quite high in adults.
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Tinea o All tineas (corporis, cruris, mannum, pedis) are treated w/ local therapy except capitis (hair) and unguinum (nail) which need systemic therapy. Dx can be made by KOH prep of skin scraping.
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versicolor
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Sunburn o Facts: All skin colors are at risk of cancer when exposed to UV light Water washes off sunscreen, even those that are water-resistant No sunscreen for babies < 6 months. Due to ↑ exposure to chemicals in high surface area to body wt ratio No evidence that steroids are helpful in Rx of sunburn. Sunscreen protection factor SPF: ↑ SPF = more protection from UV rays. However, SPF > 50% adds very little value. UV rays are reflected off cement, water, and snow and can penetrate clouds. You can get burned even if standing in the shade. Tanning bed significantly increase risk of melanoma. Dark fabrics offer greater protection from UV rays.
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UW Peds endocrinology Wednesday, February 8, 2017 6:28 AM
Constitutional short stature vs familial short stature growth charts
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Constitutional growth delay is characterized by a delayed growth spurt, delayed puberty, and delayed bone age. Refeeding $: o Risk factors: Chronic starvation and acute refeeding are potentially lifethreatening in pts w/ anorexia nervosa. o
Pathophysiology: Phosphorus is the primary deficient electrolyte as it is required for energy (adenosine triphosphate). Deficiencies in potassium and magnesium potentiate cardiac arrhythmias in a heart that is already atrophic from prolonged malnutrition.
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Prevention: Avoid aggressive initiation of nutrition without adequate electrolyte repletion
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Precocious puberty:
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MRI w/ contrast is indicated in all pts w/ central precocious puberty even in absence of neurological/visual symptoms and despite the fact that most central precocious puberty is idiopathic premature activation in girls GnRH agonist therapy to prevent premature epiphyseal plate fusion and maximize adult height potential. Girls age <6 who undergo GnRH agonist treatment have the potential to grow an additional 10 cm; girls age 6-8 can grow an additional 4-7 cm Reassurance can be provided in cases of isolated premature thelarche or adrenarche when bone age is normal
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Isolated premature adrenarche: Caused by ↑ adrenal androgen secretion. More in black & hispanics Lab: may show slightly ↑ DHEA-S, but testosterone levels are usually low. Generally benign but is a significant risk factor for PCOS, type 2 DM, and metabolic syndrome, especially in obese pts.
Gynecomastia: o Pubertal gynecomastia: 2/3 of adolescent boys during puberty. Unilateral, bilateral, or painful. Due to transient ↑ in testicular production of estrogen over testosterone and peripheral conversion of prohormones to estrogen No workup or treatment is necessary as it usually resolves within a few months to 2 years
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Congenital hypothyroidism: o Most common is Thyroid dysgenesis (85%) then defect in hormone synthesis (10%) then maternal receptor blocking antibodies (5%) o Initially asymptomatic due to presence of moderate amount of maternal hormones o Symptoms may also include: abdominal bloating, resistant macrocytic anemia, noisy breathing
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Kallmann $ is an X linked recessive disorder yet it can manifest in females
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Turner $: estrogen replacement to nearly all pts to promote normal maturation and to ↓ risk of osteoporosis McCune-Albright $: o Characterized by precocious puberty (endocrine abnormalities), cafe au lait spots and multiple bone defects (polyostotic fibrous dysplasia)
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UW peds GIT Saturday, January 21, 2017 12:52 AM
Neonatal IO suspected (bilious vomiting). First step? cessation of enteral feeding + decompression + IVF. Xray to rule out perforation.
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Both have empty rectum on PR exam but the anal tone is increased in HS. Both can have narrow colon, but it's usually recto-sigmoid in HS and diffuse in meconium ileus o Neonatal IO/malrotation: barium swallow is the fastest and most accurate test to order: ligament of Tretiz on the right side of the abdomen (malrotation) or corkscrew (vovulus). US has high false -ve. Barium enema has unacceptable false +ve and -ve Beckwith Wiedemann $: o the hypoglycemia is usually temporary during neonatal period and kids grow out of it later on, hence, no need for ongoing glucose monitoring.
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Screening recommendation for BWS
Age
Test
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0-4 yr
Abdominal US a-fetoprotein
Every 3 m
4-8 yr
Abdominal US
Every 3 m
8-18 yr
Renal US
Patients with isolated hemihyperplasia are also at increased risk for Wilms tumor and hepatoblastoma. These patients should undergo frequent screening as in BWS. Constipation Rx in kids: Sorbitol-containing juices (eg, prune, pear, apple) can increase stool water content Juice is less effective than laxatives, and excessive consumption increases the risk for obesity.
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Laxatives: polyethylene glycol, mineral oil Enema is Last resort Bilious emesis in the neonate is an ominous sign of intestinal obstruction and requires immediate workup. Exclude pneumoperitonium first by abd x ray then proceed with enema or upper GI series.
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Breastfeeding failure jaundice can be caused by both maternal (eg, inadequate milk supply, cracked/clogged nipples, engorgement, infrequent feeding) and infant (eg, poor latch, ineffective suck, falling asleep) factors.
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Signs of dehydration are: decreased urine output, weight loss more than expected, brick-red urate crystals in baby diapers, delayed passage of yellow stools. Rx: in an otherwise healthy full-term newborns is to increase the frequency and duration of feeds to stimulate milk production, maintain adequate hydration, and promote bilirubin excretion. Monitor bili w/in 2 days and If continued to rise, supplement w/ formula but don't stop breastfeeding as that will cause further decrease in milk production.
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Biliary atresia presents with conjugated hyperbilirubinemia (direct bilirubin >20% of total bilirubin) in the first 2 months of life, clay-colored stools, dark urine, and an enlarged liver. Dubin-Johnson syndrome is usually asymptomatic with mild scleral icterus. Vomiting in babies
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Physiologic reflux Clinical Dx Rx: Small frequent feeds, Upright for 25 min after feeds Prone position while awake (not asleep). Avoid tight diapers, knee chest position. Improves @ 6 m when baby sits, resolves @ 1 yr Pathologic GERD Clinical Dx Don't use Thickened feeds (obesity) or PPI (pneumonia, diarrhea) for physiologic reflux. pH probe & endoscope only for severe cases Pyloric stenosis: o Formula feeding is thought to cause gradual hypertrophy of the pylorus until symptom onset at age 3-5 weeks. Compared to breastfed infants, formula-fed infants have slower gastric emptying and consume more volume in less time. The increased gastric burden may stimulate growth of the pylorus muscle. Pyloric stenosis presents with postprandial projectile vomiting followed by hunger (e.g., "hungry vomiter")
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Meconium ileus is virtually diagnostic for CF. Although only 20% of patients with CF develop meconium ileus, almost all newborns with meconium ileus have CF Celiac disease:
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Iron deficiency anemia: From malabsorption. Duodenal atrophy leads to decreased iron absorption. Rx: gluten free diet + iron supp Dermatitis herpetiformis: On knees, elbows, forearms, and buttocks Biliary cyst: choledocal cyst
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Can be congenital or aquired. Right upper quadrant mass o Slightly elevated amylase and lipase like any other biliary disease o Risk factor for cholangiocarcinoma, infectioin, and pancreatitis Radiolucent foreign body suspected and not seen on xray. Next step? CT Esophageal coins in asymptomatic patients can be observed for up to 24 hours after ingestion. If the patient is symptomatic (cough, vomiting, dysphagia) or the time of ingestion is unknown, the coin should be removed promptly by flexible endoscopy. Smooth F.B. located distal to the esophagus pass uneventfully in most cases and need only to be observed with stool examination and/or follow up x-rays to confirm excretion. Don't induce emesis or try to push it into the stomach (likely unsuccessful and causes more esophageal injury) o
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Milk protein allergy o The diagnosis is clinical and confirmed when bleeding ceases in response to dietary modifications. Visible bleeding should resolve within 3 days, but complete resolution of occult blood may take up to 2 weeks. Parents should be reassured that the prognosis is excellent and that almost all affected infants can tolerate dairy and soy products by age 1 year
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Painless hematochezia DD: milk protein allergy (v. common) vs meckle's diverticulum (rare)
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Ectopic gastric tissue secretes acid → mucosal ulceration → bleeding → can lead to severe anemia and hemorrhagic shock Cyclic vomiting syndrome: o Functional syndrome diagnosed after exclusion of all possible causes. Overlapping with migraine headache and abdominal migraine (cyclic functional abdominal pain with criteria similar to that of CMS. Family hx of migraine headache. Rx: hydration, antiemetic (odansetron), and reassurance; triptans can help pts w/ family hx of migraine. Resolves by age 5 -10 yo in 65% of cases. o
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Choanal atresia: first step is passing a catheter, next is to do a CT to reveal the bone structure. Next look for associated anomalies CHARGE.
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Cystic fibrosis
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Remember Vitamin deficiencies A K recurrent bleeding E D fractures/osteopenia
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Water soluble vitamins:
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Reye syndrome o In the setting of influenza B or varicella zoster infection aspirin is a mitochondrial toxin that can cause acute liver dysfunction in young ages
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Necrotizing enterocolitis
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Although neonates frequently are colonized with C difficile, symptomatic disease is rare in the first year of life, possibly due to an absence of intestinal receptors to the bacterial toxins. Jejunal atresia presents with bilious vomiting and abdominal distension. Abdominal xray shows a "triple bubble" sign and gasless colon. Risk factors include prenatal exposure to cocaine and other vasoconstrictive drugs.
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Intussusception: A pathological lead point should be suspected in older children with recurrent intussusception. Meckel's diverticulum is the most common congenital anomaly of the gastrointestinal tract and can be asymptomatic between episodes of telescoping Surgical resection is required to treat and prevent complications of obstruction.
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Gastroschisis:T2 sonography is > 95% sensitive for abdominal wall defects, and maternal serum a-fetoprotein is usually elevated. Gastroschisis results in the bowel being exposed to amniotic fluid → inflammation and edema of the bowel wall → ↑ risk of complications (eg, necrotizingenterocolitis, short bowel syndrome). Dysmotility (ileus, delayed gastric emptying, intolerance of feeds) occurs in > 50% of cases and may lead to prolonged reliance on TPN. Gastroschisis is an isolated defect >90% of the cases (no associated anomalies or neural tube defects). Rx: After delivery, the exposed bowel should be covered with sterile saline dressings andplastic wrap. Immediate surgery is required Suspect vitamin A deficiency in a 2 or 3-year-old child with impaired adaptation to darkness, photophobia, dry scaly skin, xerosis conjunctiva, xerosis cornea, keratomalacia, Bitot spots and follicular hyperkeratosis of the shoulders, buttocks, and extensor surfaces. Biliary atresia: Conjugated hyperbilirubinemia is defined as >2 mg/dl of direct bilirubin or a direct bilirubin fraction that is >20% of the total bilirubin level. Newborns with conjugated hyperbilirubinemia and hepatomegaly require immediate evaluation for biliary atresia. The first step in evaluation is abdominal ultrasound, which may show an absent or abnormal gallbladder Early treatment with a Kasai procedure dramatically improves outcomes.
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Kasai is a Temporary procedure: ↓ morbidity and mortality after liver transplantation
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Full-term infants are born with adequate iron stores to prevent anemia for the first 4-6 months of life regardless of dietary intake. Preterm infants are at significantly increased risk for iron deficiency anemia. Iron supplementation should be started at birth in exclusively breastfed preterm infants and continued until age 1 year All exclusively breastfed infants should also be started on vitamin D supplementation Crying infant: o Colic: Rx: Calming techniques include using an infant swing, swaddling, minimizing environmental stimuli (eg, quiet dark room), and holding and rocking the baby. Feeding patterns should also be reviewed to assess if there is overfeeding, underfeeding, or an inadequate burping technique
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Caustic ingestion: o Any intervention that could provoke vomiting should be avoided. This includes administration of milk, water, activated charcoal, vinegar, or nasogastric lavage, as vomiting can increase the extent of injury. 48
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Iron poisoning:
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The mechanism of iron poisoning is free radical production and lipid peroxidation, which impairs various cell processes, leading to systemic manifestations o Metabolic acidosis is due to lactic acidosis from shock and hypotension. o Dx is confirmed by measuring serum iron levels. Iron is radiopaque, and visualization of gastric tablets on abdominal x- ray further supports the diagnosis o Treatment depends on the severity of the poisoning. Whole-bowel irrigation is sometimes instituted, but other methods of decontamination (eg, activated charcoal, syrup of ipecac, gastric lavage) are not routinely recommended. Chelation therapy with intravenous deferoxamine is used in moderate-to-severe cases and would be indicated in this patient Lead poisoning: o Screening of high-risk populations is important, as most children with lead toxicity are initially asymptomatic but can have cognitive and behavioral problems that become apparent after school entry. 49
Capillary (fingerstick) blood specimens are widely used in screening for lead poisoning, but false-positive results are common. Confirmatory venous lead measurement is required if a screening capillary lead level is ≥5 ug/dL. Lead accumulates throughout the body, but measuring it in hair, teeth, bone, or urine is not recommended
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Other risk factors include lead piping living near a battery recycling plant, having a parent who works with batteries or pottery having a playmate or sibling with a history of lead poisoning Dimercaprol (BAL) + EDTA should be administered on an emergency basis for levels ≥70 or acute encephalopathy Rx of mild cases → remove from toxic environment
UW Peds ENT Saturday, February 11, 2017 7:12 AM Otalgia:
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Acute otitis media:
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Treatment RULES: Under 2 years → Abx. Delaying antibiotics in infants and young children could increase the risk of developing complications. Observation and analgesia is a reasonable option if the child is age >2 years, has a normal immune system, and symptoms are mild and unilateral. Recurrent AOM (eg, >3 w/in 6 months or ≥ 4 w/in 12 months) despite appropriate antibiotic treatment → Tympanocentesis and culture during myringotomy with tube placement Therapy with otic drops is appropriate for otorrhea from tympanostomy tubes, chronic suppurative otitis media, or external otitis, but not for AOM. Although viral URI often precede or occur w/ AOM, it would not change management, which consists of empiric antibiotic treatment. Cholesteatoma: o congenital or acquired secondary to chronic middle ear disease. o New-onset hearing loss or chronic ear drainage despite antibiotic o Granulation tissue and skin debris may be seen within retraction pockets of the tympanic membrane on otoscopy. o Complications of cholesteatomas include hearing loss, cranial nerve palsies, vertigo, brain abscesses or meningitis Laryngomalacia: o Dx: clinical. Laryngoscope only for moderate to severe cases
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Juvenile angiofibroma: o Any adolescent who presents with a nasal obstruction, visible nasal mass, and frequent nosebleeds (epistaxis) is considered to have a juvenile angiofibroma (JNA), unless proven otherwise. o Pathophysiology: benign growth, but is capable of eroding and locally invading (bone erosion). In the back of the nose or upper throat (nasopharynx) of adolescent boys. Potentially very dangerous because of high vascularity which may bleed readily + occur in areas difficult to access surgically 52
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Treatment: In some cases, no treatment is necessary. Surgical removal Indication: enlarging, obstructing the airway, or causing chronic nosebleeds. Often difficult because the tumor is unencapsulated and may be deeply invasive. Recurrence after resection is common. Bacterial rhinosinusitis:
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CT sinus scan/aspiration and Cx are not routine. X-ray sinus has low sensitivity (wrong answer) Indication for CT imaging: If the patient develops complications such as Periorbital edema Vision abnormalities Or altered mental status Indication for aspiration and Cx: persists or worsens after 3 days of Abx Rx: Augmentin® + intranasal steroid (for pt w/ hx of allergic rhinitis). Antihistamine is a wrong answer.
UW Peds Hem/onc Sunday, February 5, 2017 5:09 AM
Basophilic stippling are ribosomal precipitates which appear as blue granules of various sizes dispersed throughout the cytoplasm of the red cell. These are often seen with thalassemia, lead or heavy metal poisoning
Sickle cell disease: o Stroke is a common complication of sickle cell disease secondary to sludging and occlusion in the cerebral vasculature. Exchange transfusion is the recommended treatment acutely since it helps to decrease the percentage of sickle cells and prevent a second infarct from occurring. 53
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Periodic blood transfusions are used primarily for treatment (eg, acute stroke, acute chest syndrome, acute multi-organ failure, acute symptomatic anemia, aplastic crisis); transfusions are indicated for stroke prevention.
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Hydroxyurea is indicated in patients with frequent painful episodes; history of acute chest syndrome; and severe symptomatic anemia.
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Hydroxyurea has been shown to decrease pain crises, the need for transfusions, and episodes of acute chest syndrome. Side effects: bone marrow suppression (Leukopenia, anemia, and thrombocytopenia). These effects are generally temporary and reversible but may predispose the patient to infection.
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The high rate of heme catabolism and short red blood cell life span (-20 days) results in elevated lactate dehydrogenase, unconjugated hyperbilirubinemia, and compensatory reticulocytosis Excess hemoglobin (from extravascular and intravascular hemolysis) binds to haptoglobin ↓ haptoglobin
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Sickle trait: pts are at increased risk for renal issues, the most common of these is painless microscopic or gross hematuria that results from sickling in the renal 54
medulla. Isosthenuria (impairment in concentrating ability) is also common and can present as nocturia and polyuria. Spherocytosis: Dx is confirmed by acidified glycerol lysis test and eosin-5-maleimide binding test. It can present in neonates, however, they are unable to produce appropriate erythropoiesis in response to anemia ==> NORMAL reticulocyte count), thus predisposed to severe anemia and often require transfusions.
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Review Glycogen storage diseases Iron deficiency: o The most common nutritional deficiency in children o Often caused by excessive consumption of cow's milk (>24 ounces [700 ml] per day). The anemia is secondary to the low iron content of milk, poor bioavailability of iron from milk, and increased intestinal blood loss from cow's milk proteininduced colitis. o
RDW values >20% are suggestive of iron deficiency anemia; normal values of 12%-14% are seen in thalassemia.
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Iron deficiency in child < 2 yo Children with iron deficiency are often asymptomatic and may not have tell tale symptoms such as pica, fatigue, or pallor. Therefore, universal screening is recommended by age 1 year, and the diagnosis is typically based on a complete blood count. Further testing is not necessary in children with the classic presentation.
The most cost-effective approach to treatment is empiric oral iron therapy → recheck Hb in 4 wks → if Hb ↑ by 1g/dL → continue therapy for 2 - 3 months after Hb normalizes to replete iron stores.
If the anemia does not resolve w/ empiric iron therapy and appropriate dietary changes, other causes must be considered. Hemoglobin electrophoresis (for hereditary anemias), colonoscopy (for lower gastrointestinal tract bleeding), or serum creatinine measurement (for renal disease) may be indicated.
Blood transfusions are rarely necessary in the treatment of dietary iron deficiency anemia, even when the hemoglobin is as low as 4 g/dL Iron therapy should be sufficient.
ALL: Lymphoblasts lack peroxidase positive granules of AML but often contain cytoplasmic aggregates of periodic acid Schiff (PAS) positive material. lmmunostaining for terminal deoxynucleotidyltransferase (TdT) is positive in more than 95% of patients TdT is expressed only by pre B and pre T lymphoblasts
Wiskott-Aldrich syndrome: 56
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Dx can be made clinically, but gene testing allows screening of other family members. Rx: stem cell transplantation
Neonatal polycythemia:
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Erythrocyte CD55 and CD59 protein testing? Paroxysmal nocturnal hemoglobulinuria Hemophilia: Hemophilic arthropathy is a delayed consequence of recurrent hemarthrosis. Hemosiderin deposition leading to synovitis and fibrosis within the joint → chronic pain, limited range of motion. The risk of hemophilic arthropathy can be significantly reduced by prophylactic treatment with factor concentrates. 57
Bone tumors:
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osteosarcoma vs ewing sarcoma o Osteoid osteoma appears as a sclerotic, cortical lesion on imaging with a central nidus of lucency. It typically causes pain that is worse at night and unrelated to activity. However, the pain is quickly relieved by nonsteroidal anti-inflammatory medications. ITP: o BM biopsy is not required in well-appearing children with isolated thrombocytopenia and no other symptoms (eg, fever of unknown origin, leukopenia, anemia, unexplained weight loss, fatigue) o Splenectomy is a last resort for catastrophic bleeding or chronic ITP that is refractory to IVIG and glucocorticoids
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Vit K deficiency:
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Anemia evaluation:
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Pappenheimer bodies are iron-containing inclusion bodies found in peripheral red blood cells. They result from phagosomes that engulf excessive amounts of iron and are typically seen in sideroblastic anemia. Aplastic anemia: o Acquired aplastic anemia should be suspected in any patient with pancytopenia following drug intake, exposure to toxins or viral infections.
Both types of thalassemia minor are characterized by abnormally small RBCs. RDW is normal, and total RBC count is normal or elevated. This typically results in a Mentzer index (MCV/RBC) <13 (vs iron def it's > 13 due to ↓ RBCs). The hematocrit is usually >30% in patients with thalassemia minor. Henoch schonlein purpura: 60
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Usually follows an infection. Dx: clinical in children w/ classic signs and symptoms. Dx requires the presence of lower-extremity purpura or petechiae PLUS at least one of the following arthritis or arthralgia renal involvement abdominal pain positive histopathology Laboratory testing is not required but typically demonstrates a normal platelet count and mild ↑ creatinine. In children with atypical presentations, a renal biopsy to confirm the Dx(deposition of lgA in the mesangium will be seen.
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Purpura fulminans = purpura that happens w/ meningococcal meningitis. Henoch schonlein purpura: o ↑ risk for intussusception due to bowel wall edema and localized hemorrhage → act as lead-points for the intussusception o Unlike most cases of intussusception in children, which are ileo-colic, intussusceptions in HSP are more likely to be small-bowel or ilea-ileal (60% of cases). o Because of their location, small-bowel intussusceptions cannot be seen on contrast enema and are diagnosed by the presence of a "target" sign on ultrasound. o Ileocolic intussusceptions can be treated with air or contrast enema, but ilea-ileal intussusceptions that do not reduce spontaneously often require surgical management
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Osteosarcoma Monday, February 6, 2017 8:44 AM
Epidemiology: The most common 1ry bone tumor in children and young adults (13-16 yo have higher risk) Risk factors: Paget disease (old age), Retinoblastoma Li Fraumeni $ Presentation: Pain and swelling The most important physical exam finding is tender soft tissue mass. Normal range of motion, no constitutional symptoms (fever, wt loss, night sweats). Workup: lab: ↑ Alkaline phosphatase and LDH (high levels correlate w/ adverse prognosis) Imaging: x-ray: metaphyses of long bones such as the distal femur proximal tibia, and proximal humerus. Spiculated "sunburst" pattern (yellow arrow) and periosteal elevation known as the Cadman triangle (red arrow)
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Rx: excision and chemotherapy
Ewing sarcoma Monday, February 6, 2017 8:57 AM
Epidemiology: Although overall rare, Ewing sarcoma is the 2nd most common 1ry bone malignancy in adolescent males. Risk factors: Associated with t(11;22) translocation Presentation: Pain, mild fever, erythema and swelling, anemia (20% have systemic symptoms). Extremely aggressive with early metastases Workup: X-ray: Commonly appears in diaphysis of long bones, pelvis, scapula, ribs. Osteolytic lesion with a periosteal reaction that produces layers of reactive bone, giving the classic "onion skin" appearance Also read here
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UW Peds ID Sunday, January 29, 2017 6:02 PM
Malaria
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Cervical adenitis in children
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Lyme disease o Borrelia burgdorferi o Ixodes scapularis transmits Lyme disease (as well as anaplasmosis and babesiosis) o The rash is not particularly painful but may occasionally burn or itch. o Rx: Oral doxycycline, amoxicillin, and cefuroxime have equivalent efficacy for treating early localized Lyme disease. Doxycycline is used because it is effective in treating potential coexisting Anaplasma. IV ceftriaxone is reserved for Lyme meningitis and heart block, manifestations of early disseminated Lyme disease.
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Cystic fibrosis
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Pertussis:
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Dx: clinical, start Rx and don't wait for confirmatory tests. PCR used in uncertain cases and for epidemiological studies. Prevention: previous vaccination or infection can decrease disease severity but doesn't provide lifelong immunity. Adolescents who didn't get a booster @ 11-18 yr are at risk of waning immunity. Give Tdap booster w/ each pregnancy. 73
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Prophylaxis macrolide (azithro for <1 y/o bcz erythro → pyloric stenosis; and clarithro not well studied) to all contacts despite their vaccination status. Unvaccinated contacts should receive vaccines according to schedule. Respiratory isolation is necessary only during the first 5 days of antibiotics. Rx: 100 day cough but still, as a general rule; Over-the-counter antitussive medications should be avoided due to lack of proven efficacy and risk of toxicity, especially in children age <6 years Hospitalization if Infants <3 months (due to high risk of apnea) Severe paroxysms that impair feeding Complicated by pneumonia, seizures, or other comorbidities.
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Inflamed joint DD: o Bacterial joint infection that is often preceded by skin or upper respiratory tract infections (source of infection + hematogenous spread) → DD 1) transient synovitis (same local sings and symptoms but no systemic symptoms like fever or significant lab derangements; Rx: bed rest + NSAIDs), and 2) rheumatic fever (migratory, look for associated findings w/ RF; Rx: rest + NSAIDs). Suspect septic arthritis → arthrocentesis.
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Septic arthritis is often preceded by a seemingly mild infection (e.g. cellulitis) in which there may be intermittent bacteremic showers, allowing for hematogenous spread of bacteria into the synovial fluid. o Gram stains and cultures can be falsely negative and confounded in pts pretreated w/ antibiotics for his recent skin infection. (focus on WBCs in joint analysis) o Rx: Debridement and irrigation of the joint space is the most important intervention in preventing long-term disability. A delay of even 4-6 hours in treatment can lead to joint necrosis and requirement for a prosthetic. If no improvement w/ appropriate Abx and still febrile → MRI to look for concomitant osteomyelitis. Meningitis: The long-term neurologic sequelae associated with bacterial meningitis are: o Hearing loss o Loss of cognitive functions (due to the neuronal loss in the dentate gyrus of the hippocampus) o Seizures o Mental retardation o Spasticity or paresis Pharyngitis DD: 76
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Pearls: Note that Centor criteria includes only ANTERIOR cervical LN+ and tonsillar EXUDATES. Posterior LN+ and any tonsillar changes (ulcers, erythema..etc) other than exudates are not included.
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Herpangia also causes bilateral LN+ DD GAS & EPV (exudates on tonsils, not vesicles/ulcers)
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EBV
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Heterophile antibody Initial negative test does not rule out the IM diagnosis as it takes wks to develop. EBV-specific antibody testing can be ordered in those with more prolonged illness and negative heterophile antibody testing Atypical lymphocytes May also be present in pts w/ toxoplasmosis, rubella, roseola, viral hepatitis, mumps, CMV, acute HIV infection, and some drug reactions.
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o o o
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causes a milder pharyngitis and lymphadenopathy than EBV-induced IM. CMV lgM titers can be considered in patients with a negative heterophile antibody test Rapid influenza testing (high specificity and low to moderate sensitivity) can be performed in high-risk children in whom treatment would be indicated (eg, age <2, history of pulmonary/cardiac disease, need for hospitalization) Influenza typically presents with fever, cough, and rhinitis rather than pharyngitis. Retropharyngeal abscess: Dx by clinical + lateral X-ray. In pts with no signs of respiratory compromise, do CT w/ contrast to confirm presence and size of the abscess. scan with contrast should be performed to confirm the presence and size of the abscess
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Nonetheless, you have to cover pseudomonas in your empiric Abx
Epiglottitis: o Incidence was reduced due to widespread Hib vaccination, but Hib remains the most common cause, even in immunized children. o Symptoms often develop over several hours without a significant prodrome (eg, cough, congestion, rhinorrhea) vs croup that develops over days w/ prodrome. Signs of impending airway obstruction include restlessness, anxiety, worsening stridor, and a muffled "hot potato" voice
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HUS: o Vascular damage, microthrombi formation. Platelet consumption but normal PT/PTT. No fever. Abx are CI
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Acute rheumatic fever: o Rx: 83
Even in the absence of active pharyngitis, pts with ARF should still be treated with IM benzathine penicillin G until adulthood to eradicate bacterial carriage to prevent recurrent ARF and worsening rheumatic heart disease. Pts w/ severe chorea may require steroids, and those w/ pericarditis and arthritis may need NSAIDs
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Most common organism causing pericarditis or myocarditis? Coxsackievirus Impetigo: Risk factors: warm/humid climate, poverty/crowding, poor personal hygiene, and preexisting skin trauma/ inflammation (eg,insect bite, eczema) Colonization w/ staphylococci or streptococci.
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Varicella: o Vesicles are pruritic. o Potential complications include (bacterial superinfection in kids and pneumonia in adults. lmmunocompromised patients are at risk for potentially fatal disseminated disease. Patients are contagious from 2 days prior to rash onset until all vesicles are crusted (isolate the pt for this entire period)
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Immunocompetent pts who are asymptomatic and nonimmune should receive varicella vaccine for post-exposure prophylaxis within 3-5 days of exposure. lmmunocompromised (<1 yo & pregnant included) who are asymptomatic and nonimmune should receive VZIG w/in 10 days of exposure. Although VZIG may not prevent infection, it can reduce disease severity. Those who receive it require close monitoring for development of varicella infection as VZIG can prolong the incubation period beyond 1 month. Immune pts are ok, and symptomatic pts get acyclovir.
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Rashes o scarlet fever: Rash initially appears on neck, axillae, groin then generalizes. Towards the end of the 1st week, desquamation begins in the face, progresses down the trunk, and finally extends to the hands and feet o Kawasaki: can be confused w/ scarlet since it has strawberry tongue and other features. Key is to stick to Kawasaki criteria for Dx. Rapid response to penicillin confirms scarlet. Nikolsky sign -ve 88
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SSSS: exfoliative strains of S. aureus, characterized bullae followed by an exfoliation of the skin. Rare after 5 yo. Nikolsky sign +ve o Toxic epidermal necrolysis: hypersensitivity; considered to be a variant of Stevens-Johnson syndrome. 2ry to medications (sulfa drugs, anticonvulsants, and NSAIDs). Similar to SSSS, it presents with extensive erythema, tenderness, and blister formation, followed by exfoliation. Mucous membranes are severely affected, and shedding of the nails may occur. Nikolsky sign +ve o Stevens-Johnson $: severe variant of erythema multiforme (target lesions), followed by bullae of two or more mucous membranes. Sometimes, the Gl, respiratory, or GU tracts may also be involved. Sickle cell: o Strept pneumococci is the most common causes of sepsis (fever w/o origin) in those pts, despite vaccination (vaccine doesn't cover all serotypes; doesn't provide full immunity). On the other hand, H influenza and meningococcal vaccines are fully protective.
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Osteomyelitis: o Staphylococcus aureus is the most common organism in infants and children. Other commons are GBS and E coli in infants, and Streptococcus pyogenes in children. Staphylococcus epidermidis is a frequent cause of osteomyelitis associated with prosthetic devices. Rubella:
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Meningitis: o Bacterial meningitis: Neisseria meningitides is the most common cause of bacterial meningitis in children and young adults in the United States. The mortality rate of meningococcal meningitis is v. high (15%). Children age < 2 are at greatest risk for contracting this illness, with rates 10 times higher than those of the general population. Seventy-five percent of patients with Meningococcus meningitis present with a petechial rash that is prominent on the axilla, wrists, flanks and ankles. It appears within 24 hours of the infection. o Viral meningitis: The most common causes of viral meningitis are the non-polio enteroviruses, such as echoviruses and coxsackieviruses. Start w/ constitutional and upper respiratory symptoms w/ low-grade fever. Over the next 36-48 hours, the patient develops a high fever, headache, irritability, and nuchal rigidity. Other symptoms of enteroviral infection may also be seen such as pharyngitis, rash, or herpangina. self-limited Rx: supportive, resolves in 10 days
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Neonatal sepsis: o Early onset sepsis: < 7 days o Late onset sepsis: later o Pregnant women with listeriosis have nonspecific flulike symptoms such as fever. body aches. and fatigue prior to delivery
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Measles: o Measles (rubeola) is extremely contagious, spreads by droplets from respiratory secretions. The virus can remain airborne for several hours, and unvaccinated patients can contract it by entering a room that an infected person has exited.
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Patients are most contagious during the prodrome but can spread disease for several days even after the resolution of the rash. Patients with known or suspected measles should be isolated and placed on airborne precautions (negative pressure room, N95 facemask (not surgical masks) for health care personnel)
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Neonatal conjunctivitis: o Blood-stained eye discharge is highly characteristic of chlamydia! conjunctivitis
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STDs screening: o Screening for chlamydia trachomatis and Neisseria gonorrhoeae in the following pts: 93
All sexually active women age ≤24 Any person with a new partner in past 2 months Multiple partners History of sexually transmitted infections illicit drug use, incarceration, and contact with sex workers. o Best screening test: Nucleic acid amplification test (high sensitivity and specificity). The test can be performed on urine, endocervical, vaginal, or urethral specimens with similar accuracy. Immunizations: o All stable preterm infants should receive the first dose of the hepatitis B vaccine at birth ,unless the infant weighs <2 kg (4 lb 6 oz) → wait until they weigh 2 kg then vaccinate o All vaccines should be administered according to chronological rather than gestational age in preterm infants. o Laws concerning anti-vaccines people: All states allow medical exemption from vaccination (eg, allergy to vaccine components) Some states also allow for exemption based on a parent's religious and/or personal beliefs. If a child is unvaccinated and is not exempt, he/she may not be able to enroll in day care or school, depending on the state. The physician must respect the mother's decision but is obligated to inform her about the health-associated risks and benefits as well as the potential consequences (school enrollment) The discussion should be fully documented in the medical record. Physicians do not report cases of vaccination refusal.
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Rotavirus vaccine: live attenuated vaccine, typically administered 2-6 months of age
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Administration of multiple vaccinations in a single office visit is safe and increases vaccine compliance and optimal protection at a young age. The exception is livevirus vaccines, which should be administered 4 weeks apart due to possible interference of immune response. Vaccines can be administered safely during minor illnesses (eg, upper respiratory infections). They should be postponed until recovery from moderate or severe illnesses as manifestations of the underlying illness can be incorrectly attributed to the vaccine and vice versa. Contraindication and precaution to DTaP: Encephalopathy within a week of DTaP vaccine administration is a contraindication for further administration of the combination vaccine, and diphtheria and tetanus toxoids should be administered without pertussis. Infants with progressive neurologic disorders should also avoid pertussis immunization to prevent confusion about causality until treatment is established and the condition has stabilized. Febrile seizure after DTaP isn't a contraindication for further vaccination
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Immunizations Tuesday, February 28, 2017 7:39 AM
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UW Peds immunology Wednesday, February 8, 2017 12:18 AM
Do a quick step1 review here Bruton's agammaglobulinemia Defect in tyrosine kinase that prevents the development of mature B cells
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Pts w/ T cell disorders may have Humoral immunodeficiency due to defective T-cell help in B-cell activation for antibody production. All patients with Digeorge $ should receive routine killed or subcomponent vaccines. However, the safety of live vaccines depends on the degree of immunodeficiency. Decisions regarding administration should be tailored to the patient's immune status. Hereditary angioedema: Presents in late childhood. Episodes usually follow an infection, dental procedure, or trauma. Defect or deficiency of C1 inhibitor leads to ↑ edema-producing factors C2b and bradykinin Rapid onset of: Noninflammatory edema of the face, limbs, and genitalia Laryngeal edema Edema of the intestines resulting in colicky abdominal pain No evidence of urticaria
Hereditary angioedema
Acquired angioedema
C1 inhibitor deficiency
ACE-I → ↑ bradykinin
Normal Cq1 levels
↓ Cq1 levels
↓ C4 levels
↓ C4 levels
CGD: o CGD pts should receive lifelong antimicrobial prophylaxis, and interferon gamma can be used to boost intracellular killing in severe cases.
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Transient hypogammaglobulinemia of infancy: Pts present with increased sinopulmonary and gastrointestinal infections that are usually mild rather than life-threatening. The normal physiologic nadir of maternally-derived antibody is 3-6 months of age in term infants. Low serum lgG levels after age 6 months is termed transient hypogammaglobulinemia of infancy as it usually resolves by age 12 months. B and T lymphocyte counts and lgA and lgM levels are normal SCID:
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Humoral immune deficiencies:
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LAD
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Read that TERC thingy 99
UW Peds Misc Saturday, February 11, 2017 8:31 AM
Edward's $:
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Toilet training: o Most children are ready to begin toilet-training at age ≥ 2with the acquisition of the following skills: Voluntarily control sphincters Walk Remove pants Follow 2-step commands Communicate the need to urinate and stool Imitate actions of other people (e.g. sit on toilet) o Premature initiation of toilet-training can prolong the duration of training. Don't to rush the child, bedwetting is normal < 5. Most children master daytime continence within months, but nighttime continence can sometimes take years to accomplish.
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Chronic constipation can reduce bladder capacity and contribute to urinary incontinence. Stool burden can be assessed by abdominal x-ray but is unnecessary in the setting of soft, regular bowel movements. o Urinalysis performed only when children are > 5 to screen for urinary tract infection, diabetes mellitus, and diabetes insipidus o Alarm or desmopressin therapy are unwarranted for children <5. Infant development progresses from cephalic to caudal; from proximal to distal; and from stimulus-based reflexes to specific, goal-oriented reactions that become increasingly precise with age Child abuse:
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Ethics treatment of minors:
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Mental health services is also available for minors w/o parental notification. o Minors who are parents may consent for the treatment of their own minor children. o Parents are not allowed to refuse life-saving treatment for a child, including refusal for religious reasons. o If parents refuse to consent to treatment of their child for a non-emergency but fatal medical condition (chemotherapy for ALL), the physician should seek a court order mandating treatment o Only one parent is required to consent to the minor treatment. Even if the other parent disagrees. And remember that consent isn't required at all in emergency situations. Small for gestational age infants: o Small for gestational age infants have a weight under the 10th percentile for gestational age o SGA infants are at risk for several complications due to their size, including hypoxia, perinatal asphyxia, meconium aspiration, hypothermia, hypoglycemia (low glycogen stores, Rx: early and frequent feeds), hyperglycemia (infants w/ very low birth wt due to low insulin secretion), hypocalcemia (↓ Ca transfere across the placenta), and polycythemia (↑ erythropoietin secretion in response to fetal hypoxia) Neonatal displaced clavicular fracture:
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Prader willi $:
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Beckwith-Wiedemann $: o Disregulation of imprinted gene expression in chromosome 11 p15. Marfan: o Fibrillin-1 mutation. o Lens dislocation (ectopia lentis), iridodonesis (a rapid contraction and dilation of the iris), and myopia (from elongation of the globe) are typical o First-degree relatives should undergo genetic testing
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Ehlers-Danlos syndrome: o Collagen disorder characterized by scoliosis, joint laxity, and aortic dilation. o Pts do not have the disproportionately tall stature, lens dislocation, or pectus carinatum seen in Marfan syndrome Congenital contractural arachnodactyly: o Autosomal dominant condition resulting from mutations of the fibrillin-2 gene. o Tall stature, arachnodactyly, and multiple contractures involving large joints o Ocular and cardiovascular symptoms are not present in congenital contractural arachnodactyly. In addition, pts with Marfan $ do not have joint contractures.
UW Peds Neuro Thursday, January 26, 2017 2:11 AM
Congenital rubella is most dangerous during T1. Positive IgG later in pregnancy can't differentiate between infection in early pregnancy and immunization as IgG will appear a few weeks after infection. Neuroblastoma: The levels of serum and urine catecholamines and their metabolites (i.e., HVA and VMA) are usually elevated; however, patients do not present with fainting spells, sweating, palpitations and hypertension, as in pheochromocytoma Wilms tumor arise from metanephros, which is the embryologic precursor of the renal parenchyma 105
Fragile X chromosome pts may display features of ADHD and Autism spectrum disorder. Absence seizures:
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DD: Pts w/ inattentive subtype of ADHD often present with staring spells. However, these spells tend to occur with boredom or during sedentary activities and are typically responsive to vocal or tactile stimulation.
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ADHD is common w/ absence seizures or w/ other forms of epilepsy. These patients typically fall under the inattentive subtype of ADHD rather than the hyperactive subtype. The Dx of ADHD is important to make in children w/ epilepsy as effective behavioral (eg, minimization of distractions) and pharmacologic (eg, methylphenidate) therapies exist. Unrecognized or untreated ADHD leads to decreased health-related quality of life in children with epilepsy.
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Many children with absence seizures have a personal history of febrile seizures and/or a family history of a seizure disorder. o
Rx: First-line treatment is with ethosuximide. Valproic acid is a 2nd line Rx for absence seizures. Used less frequently than ethosuximide due to side effects (eg, hair loss, tremor, thrombocytopenia). Undetected hearing impairment: Undetected hearing impairment can lead to poor language skills and social isolation, easily confused with certain pervasive (autism) and behavioral (ADHD) disorders. Hearing tests should be routinely conducted in all children with any behavioral concerns. ADHD doesn't have problems w/ language skills. Selective mutism: characterized by failure to speak in one or more specific social situations. These children have normal language skills and speak normally in other situations or at home. PKU: 106
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Infant of diabetic mother
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SIDS: o Unexpected death that cannot be explained by history or postmortem examination (eg, investigation of the scene, autopsy). The leading cause of mortality in infants age 1 month to 1 year in the US
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Muscular dystrophy:
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Myotonic muscular dystrophy is characterized by grip myotonia (delayed muscle relaxation), facial weakness, foot drop, dysphagia (most dangerous smooth muscle involvement, ↑↑↑ risk of aspiration), and cardiac conduction anomalies.
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Niemann-pick vs tay-sachs: o You have to revise biochemistry and immunology chapters. 3 types of NPD, type A is the most severe; types B and C are milder. Types A and B are caused by sphingomyelinase deficiency.
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CT rule in Traumatic brain injury in kids: o head trauma is categorized into: minor, mild TBI, and serious TBI. Mild TBI: GCS score of 13-15, loss of consciousness for <5 minutes, Amnesia before/after injury for <24 hours can be present in mild TBI. If you chose to observe in the ER, do a CT if symptoms worsened. If the head CT scan is normal or the observation period is uneventful, the patient can be discharged home with a reliable guardian; detailed return precautions should also be provided Serious TBI: Admission and exam every 2 hours, CT, and neuroconsult. Minor TBI: can have mild headache, or scalp lacerations only. The potential risk of cancer due to CT-scan-associated radiation increases with decreasing age (eg, 1 in 1500 at age 1; 1 in 5000 at age 10) o MRI is more sensitive than CT in detecting minimal changes but it takes time and those minimal changes are unlikely to change management strategy. o CT should be done w/o contrast as contrast can confound the appearance of acute blood as they both appear white on CT.
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Febrile seizures
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Hydrocephalus suspected clinically + symptomatic pt = CT scan Hydrocephalus suspected clinically + Asymptomatic pt = MRI to ↓ radiation exposure Ultrasound is only helpful if ant. fontanelle is widely open in children < 6 months CP is usually caused by prenatal insults to brain development, with premature birth before 32 weeks gestation as the greatest risk factor.
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Neurofibromatosis: o NF1: Patients w/ holocranial headache (pain involving the whole head), vision changes, vomiting, and hypertension may indicate a symptomatic intracranial mass → MRI of the brain and orbits (more sensitive and specific than CT in showing soft-tissue contrast and anatomy) o NF2: Audiometry is the best initial screening laboratory test for the diagnosis of acoustic neuromas
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Differential diagnosis of intellectual disability
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Fetal alcohol syndrome can present w/ birth defects, behavioral problems (social withdrawal, ADHD, resembles autism), and cognitive impairment. Early Dx is crucial for children to benefit from aggressive speech and behavioral therapy. Lesh nyhan syndrome: o Pathophys X-linked recessive disorder deficiency in hypoxanthine-guanine phosphoribosyl transferase (HPRT) increased levels of uric acid o clinical Presents around the age of 6 months with hypotonia and persistent vomiting The clinical picture worsens thereafter with the progressive mental retardation, choreoathetosis, spasticity, dysarthric speech, dystonia and compulsive selfinjury. The uric acid excess deposits in different tissues, resulting in gouty arthritis, tophus formation and obstructive nephropathy o Rx Allopurinol Adequate intake of fluids Marfan vs homocystinuria 114
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Breath holding spells o Definition: an episode of apnea precipitated by frustration, anger, or pain. Most commonly in children age 6 months to 2 years o Presentation: The 2 types are cyanotic and pallid ) (شاحبand may represent a variant of vasovagal syncope due autonomic dysfunction Both are brief with subsequent confusion and sleepiness for a few minutes. o Dx: clinical
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DD:
Seizure disorder: the loss of consciousness isn't preceded by crying (or emotional trigger/minor trauma) such as in breath holding o Prognosis: usually benign. No effect on development Can recur but usually stop by age 5 Some pts develop vasovagal syncope later in life. o Rx: no treatment required If recurrent or family Hx suspect pathology and get cardiology workup for causes of syncope (ECG for arrhythmia, long QT & Echo if structural heart disease is suspected) Empty sella syndrome 116
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The sella turcica is often enlarged and contains no discernible pituitary gland. o Pts may be asymptomatic with an empty sella as an incidental finding on imaging or present with hypopituitarism Hypocalcemia is a common feature of DiGeorge and can cause tetany and seizures. It typically affects up to 60% of newborns with DiGeorge syndrome but is unusual later in life due to compensatory hyperplasia of existing parathyroid tissue. Brain abscess o The next step in management is CT w/ contrast or w/ MRI. o Images A and B above demonstrate a left temporal lobe lesion with a hypointense center and ring enhancement (red arrows) with surrounding edema (blue arrows). o CT is significantly faster in emergency cases and is better in depicting bone (ex. damage to mastoid process). o MRI is superior for soft-tissue details and does not use ionizing radiation. MRI is more sensitive for early cerebritis and is better at delineating the extent of ring enhancement and differentiating between edema and necrosis. o High doses of glucocorticoids should be used if imaging shows significant cerebral o edema and/or mass effect. The benefit of decreasing herniation risk would outweigh the possible disadvantage of suppressing antibiotic efficacy.
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Botulism: descending paralysis vs. guillain barre which is descending Medulloblastoma is the second most common posterior fossa tumor in children and typically arises from the cerebellar vermis, resulting in truncal and gait ataxia. Due to proximity to the fourth ventricle, obstructive hydrocephalus and signs/symptoms of increased intracranial pressure can also occur. Less commonly, medulloblastoma occur in the lateral cerebellar hemispheres similar to pilocytic astrocytoma, which affect fine motor planning and cause dysmetria, intention tremor. Differential for T-wave inversion: myocardial infarction, myocarditis, old pericarditis myocardial contusion and digoxin toxicity 118
3 years Copies ○ 4 years
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A diagnosis of seizures should be considered in any patient with an episode of loss of consciousness followed by a postictal state, or period of sleepiness and confusion. Cardiac syncope and vasovagal attacks regain their consciousness immediately after the episode (no postictal state) Child abuse
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Infant botulism: o Epidemiology: Incidence is highest in California, Pennsylvania, and Utah, which have the greatest concentrations of soil botulism spores. o Mode of transmission: Ingestion of inhaled botulism spores from environmental dust (e.g. construction sites)
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Or Ingestion of raw honey → colonic colonization of C botulinum → release of botulinum toxin → blocks acetylcholine release at presynaptic neuromuscular junctions Presentation: The diagnosis should be suspected in infants with bilateral bulbar palsies (eg, ptosis, sluggish pupillary response to light, poor suck and gag reflexes) followed by symmetric descending flaccid paralysis (hypotonia). Constipation and drooling due to autonomic dysfunction also occur. Treatment: Respiratory support, nasogastric tube feeding intravenous human-derived botulism immune globulin Botulism immune globulin should be administered as soon as possible, even before diagnostic confirmation of stool spores or toxin. DD: Spinal muscular atrophy (Werdnig-Hoffmann disease) is characterized by generalized symmetric proximal muscle weakness and hyporeflexia. In contrast to botulism, spinal muscular atrophy does not affect the pupils, and the weakness is greater in the lower than the upper extremities.
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Migraine headache: o The most common cause of acute and recurrent headaches in the pediatric population. o Presentation: Typically present with unilateral or bifrontal pain; photophobia; phonophobia; nausea; vomiting; and a visual, auditory, or linguistic aura. o Rx: First-line treatment in children includes acetaminophen, nonsteroidal antiinflammatory agents, and supportive management Triptans may be tried if these measures are not effective. o Diagnosis: Clinical and based on the presence of recurrent episodes of paroxysmal headaches. Unlike migraine headaches in adults, migraines in children are 123
often bifrontal and of shorter duration. Occipital headaches are extremely rare and, if present, should raise concern for a structural lesion Neuromuscular weakness:
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Recovery of Gullain Barre $ follows the inverse order of the initial disease progression Traumatic carotid injury: o Symptoms of hemiparesis and aphasia after injury to the posterior pharynx are suspicious for an injury to the cervical internal carotid artery, which is located directly lateral and posterior to the tonsillar pillars
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Hemiplegia in children o Hemiplegia with free brain imaging? Todd paralysis or hemiplegic migraine Todd paralysis: presentation Unwitnessed loss of consciousness, gradual return to baseline, and transient hemiplegia is most consistent with a first-time seizure with Todd paralysis.
Self-limited, focal weakness that occurs after a focal or generalized seizure in the postictal period with a partial or complete hemiplegia involving an ipsilateral upper and lower extremity, although one may be more affected than the other. Can also include aphasia and visual defects. Treatment Supportive Resolves within 36 hours Dx Clinical Other causes of paralysis (embolism, intracranial bleed) should be ruled out (by CT scan or MRI) if there is no clear history of a preceding seizure. Pathophysiology Hypothesized to involve neuronal exhaustion in the postictal period.
Syncope vs seizures: o Both seizures and syncope can cause sudden loss of consciousness, and it can sometimes be difficult to distinguish between them. Seizures typically have a preceding aura, delayed return to neurologic baseline, head deviation or unusual 125
body posturing, and tongue laceration. Syncope is associated with loss of postural tone and spontaneous return to baseline neurologic function.
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Febrile seizures: Multiple studies have shown that interventions such as imaging or lumbar puncture in the fully vaccinated child with a normal neurologic examination are low yield, unnecessarily invasive, and anxiety-provoking.
Brain imaging indications: o Child with a complex febrile seizure with a persistently abnormal neurologic examination o Macrocephaly o Or signs/symptoms of ↑ ICP
Lumbar puncture indications: 127
o o o
Pts with persistent neurologic abnormalities Those already on antibiotics that could mask symptoms/signs of meningitis Or infants age 6-12 months with incomplete/unknown immunization for Streptococcus pneumoniae or Hib.
Serum electrolytes indications: o Seizures in the setting of vomiting, diarrhea, and dehydration (can all cause ↓ Na, Glu)
Rectal, intramuscular, or intravenous abortive therapy indications: o Seizures ~5 minutes as prolonged seizures increase the risk of airway compromise o Daily use of oral anticonvulsants (eg, phenobarbital, valproate, levetiracetam) is not recommended after a first febrile seizure due to their potential for side effects and the good prognosis of feb rile seizures.
Caregivers should be reassured about the good prognosis and that hospitalization for observation is unnecessary.
Caregivers should be informed that antipyretics can make the child more comfortable, but there is no evidence that these will reduce the risk of future febrile seizures.
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Brain tumors: CNS tumors are the most common solid tumors and the second most common malignancies after leukemia
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Pineal gland mass:
Babinski reflex is normal < 2 yr Epilepsy classification: ILAE Classification of seizures Generalized seizures o Tonic-clonic (in any combination) o Absence Typical Atypical Absence with special features Myoclonic absence Eyelid myoclonia o Myoclonic Myoclonic 130
Myoclonic atonic Myoclonic tonic o Clonic o Tonic o Atonic Focal seizures Unknown o Epileptic spasms
Focal = partial seizure in the old classification. Simple = w/o impaired consciousness. Complex = w/ impairment of consciousness. Impairment of consciousness or awareness (conscious but unaware).
Juvenile Myoclonic Epilepsy (Janz Syndrome) • myoclonus particularly in morning; also presents as generalized tonic-clonic seizures or absence seizures • adolescent onset (12-16 yr of age); autosomal dominant with variable penetrance • typical EEG: 3.5-6 Hz irregular spike and wave, increased with photic stimulation • management: lifelong treatment (valproic acid); excellent prognosis
Lennox-Gastaut • characterized by triad of 1) multiple seizure types, 2) diffuse cognitive dysfunction, and 3) slow generalized spike and slow wave EEG • onset commonly 3-5 yr of age • seen with underlying encephalopathy and brain malformations • management: valproic acid, benzodiazepines, and ketogenic diet; however, response often poor Duchenne muscular dystrophy: o The gold standard for diagnosis is genetic testing, which would show deletion of the dystrophin gene on Xp21 o In DMD, electromyography demonstrates a myopathic pattern with normal nerve conduction velocities. However, these tests are supportive and not confirmatory o Muscle biopsy would show fibrosis and fatty infiltration and can support the diagnosis in this patient. Immunochemistry staining of muscle tissue would show absent dystrophin 131
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Serum creatine kinase and aldolase levels are elevated as early as infancy even before clinical manifestations. These elevations reflect muscle damage and release of these enzymes in the serum. As the disease progresses and more muscle is replaced by fat and fibrosis, these levels eventually drop. These tests are typically used for screening, as elevations can be seen in other myopathies DD Becker muscular dystrophy; BMD is a similar but milder version of DMD. In BMD, the dystrophin gene deletion preserves the reading frame for dystrophin, resulting in decreased (but not absent) dystrophin and muscle weakness later in childhood.
o
UW Peds pulmonology Wednesday, February 8, 2017
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DD stridor in infants
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Vascular rings result from abnormal development of the aortic arch causing tracheal, bronchial, or esophageal compression (e.g. right-sided aortic arch, double aortic arch, pulmonary sling, or anomalous left carotid or innominate artery). Dx by barium studies and confirmed by MRI angiography Severe coughing paroxysms may → ↑↑↑ intraalveolar pressure → subcutaneous emphysema (air leaks from the chest wall into the subcutaneous tissues). o Pneumothorax can occur by similar process → emergent chest x-rays to rule out pneumothorax in pts w/ apparent subcutaneous emphysema secondary to severe coughing paroxysms CF:
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>95% of men are infertile due to congenital bilateral absence of the vas deferens. The accumulation of inspissated mucus in the fetal genital tract obstructs the developing vas deferens o If the testes are descended, spermatogenesis should be normal. However, sperm cannot be ejaculated → transparent or no semen production (obstructive azoospermia). o In contrast to congenital bilateral absence of the vas deferens, congenital uterine and vaginal aplasia (Mayer-Rokitansky-KOster-Hauser syndrome) is uncommon. However, many women with CF are malnourished and are subfertile from pubertal delay and amenorrhea. In addition, viscous cervical mucus can obstruct sperm entry Ciliary dyskinesia vs CF o
Respiratory s/s
Ciliray dyskinesia
CF
Chronic sinopulmonary infections Nasal polyps Bronchiectasis Digital clubbing
Same
Situs inversus (50% of cases) Infertility due to immotile sperms Normal growth
Extrapulmonary s/s
Pancreatic insufficiency infertility due to absent vas deferens (azoospermia) FTT
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Ciliary dyskinesia is an autosomal recessive disease. Heart sounds displaced to the right is a clue in physical exam. APGAR score
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Neonates rarely earn the maximum score of 10 because acrocyanosis is very common, benign, and can last for 1-2 days. In contrast, cyanosis of the face or central body should raise concern for hypoxia from respiratory or cardiac problems. Most neonates have scores of 7-9 and require no intervention. Scores <7 may require further evaluation and resuscitation.
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Epiglottitis:
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Thymus gland: o Normal in age < 3 yr
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"sail sign" (Image) due to its triangular shape, scalloped border, and uniform density. However, the shape is actually quite variable with phases of respiration as it is a soft gland It can also shrink during times of stress or illness and rebound to a larger size after recovery (rebound hyperplasia) DD: Atelectasis: ass w/ mediastinal shift Middle lobe pneumonia: history of aspiration and picture of consolidation on xray Mediastinal mass: compression symptoms I guess Enlarged cardiac shadow: normal cardiothoracic index is <60 in 1st year and <50 later on
Bronchiolitis:
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Apnea could be the presenting symptoms o Infants age < 2 months tend to develop recurrent wheezing throughout childhood. o Bronchiolitis is associated w/ ↑ risk of acute otitis media (up to 10%), but the risk of bacterial pneumonia and sepsis are rare (<1%) unless the patient requires mechanical ventilation in the pediatric intensive care unit. Foreign body aspiration:
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If an FB causes partial obstruction, with air trapping during expiration, hyperinflated lungs are seen on imaging. o In contrast, complete obstruction can result in atelectasis, post-obstructive pneumonia, and/or localized bronchiectasis (late feature) Asthma: 138
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Moderate to severe exacerbations may benefit from noninvasive positive pressure ventilation (eg, bilevel positive airway pressure [BiPAP)) o Patients with signs of respiratory failure despite escalation of medical treatment (eg, BiPAP) require endotracheal intubation and mechanical ventilation. RDS: o The most important risk factor for RDS is prematurity o Other factors include male sex perinatal asphyxia maternal diabetes cesarean section without labor o Maternal DM ↑ the incidence of RDS by delaying the maturation of pulmonary surfactant production. Maternal hyperglycemia → fetal hyperglycemia → fetal hyperinsulinism → antagonize cortisol and block the maturation of sphingomyelin o Factors that ↓ the risk of RDS. Intrauterine growth restriction Maternal hypertension Chronic intrauterine stress from prolonged rupture of membranes Intrauterine stress is thought to stimulate early fetal lung maturity.
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Rx:
early CPAP Intubation and mechanical ventilation w/ exogenous surfactant for severe cases. Congenital diaphragmatic hernia:
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Clinical clues: right sided heart sounds and fair right lung areation Dx is clinical → immediate intubation. X-ray when the pt is stable to confirm the Dx o 1st step in Rx? Endotracheal Intubation then nasogastric tube w/ continuous suction to prevent bowel distension against the lungs. o Contraindications: Blow-by oxygen and bag and mask ventilation pumps air into the GIT and further compromise pulmonary functions. Neonatal respiratory distress: o
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UW Peds psych Sunday, February 26, 2017 12:55 PM
Autism spectrum disorder o Autism spectrum disorder is characterized by abnormal social development and extreme behavioral rigidity, with onset in early development. Higher-functioning individuals with normal language and intellectual ability may present later, when deficits become more apparent with increased social demands. o DD: Social (pragmatic) communication disorder; is characterized by impaired communication without evidence of restricted interests or repetitive behavioral patterns.
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OCD: o Rare subsets of OCD cases start abruptly and are referred to as pediatric acuteonset neuropsychiatric syndrome (PANS). If this onset is associated with group A streptococcal infection, it's called PANDAS (pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection) o Deep-brain stimulation has limited approval for severe, treatment-refractory OCD cases. It involves surgical implantation of electrodes in target brain areas (eg, nucleus accumbens) to achieve symptom relief
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ADHD: 143
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Dx is based on clinical evaluation in 2 different settings. Teacher evaluations are an important tool for assessing behavior in the school environment o Rx: Stimulants (methylphenidate, amphetamines) Non-stimulant (atomoxetine, alpha-2 adrenergic agonists) Behavioral therapy Selective mutism: o Dx requires consistent (≥1 month) failure to talk in situations in which it is expected (eg, school) despite speaking in other situations Tourette $:
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More frequent in males o The tics occur many times a day (frequently in bouts) nearly every day or at regular intervals for at least one year. Tics are usually preceded by irresistible urges and followed by feelings of relief. Pts experience them as involuntary, although they can be suppressed voluntarily for some time o Symptoms are exacerbated by stress and tend to subside during sleep o Comorbid conditions ADHD (60 percent) OCD (27 percent). OCD develops within 3-6 years after the tics first appeared. It may peak in late adolescence or in early adulthood at a time when the tics are waning. Less common comorbid conditions include anxiety, depression, and impulse control disorders. o Pharmacotherapy is indicated when severe symptoms interfere w/ social, academic or occupational functioning. Haloperidol and pimozide are FDA approved, but 2nd generation antipsychotics such as risperidone are well studied and are increasingly preferred due to their favorable side effect profile. Imaginary friends: are normal component of development for many young children, and are usually outgrown by the early elementary school years. Sexual behaviors in preadolescents:
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Thumb sucking: o Most young children engage in some form of rhythmic activity for soothing purposes (eg, thumb sucking, rocking, nail biting) These activities tend to decrease around age 3 or 4. Anorexia & Bulimia nervosa: o Risk factors for anorexia nervosa include high-achieving type-A personalities and being involved in activities that pressure participants to be slender (ballet, running) o Anorexic pts: to lose wt, they may fast and/or exercise excessively ( restricting subtype) or compensate for binge eating w/ laxatives or vomiting (purging subtype) o Rx: Hospitalization and acute stabilization for dehydration, electrolyte disturbances (eg, hypokalemia, hypophosphatemia), bradycardia, or severe weight loss. Meals should be supervised, and some pts require nasogastric tube feeding. During the onset of anabolism, patients require close monitoring for refeeding syndrome: electrolyte depletion, arrhythmias, and heart failure, which can result from fluid and electrolyte shifts
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Pyromania:
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DD: conduct disorder Pyromania is characterized by intentional and repeated fire setting with no obvious motive. Individuals with conduct disorder can also have a history of fire setting, but other features (eg, lying, theft, cruelty to others) are also present Trichotillomania:
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DSM-5 classifies trichotillomania as an OCD-related disorder to reflect increasing evidence of shared features with OCD and higher rates of comorbidity in patients with a personal or family history of OCD. Commonly affected sites include the scalp, eyebrows, and eyelids Hair pulling may be an automatic behavior (without full awareness) or be preceded by tension and followed by relief. Much more common in girls and women. Trichophagia (swallowing of hair) and subsequent formation of trichobezoars can lead to abdominal pain and bowel obstruction. The most effective treatment for trichotillomania is habit reversal training (a form of cognitive behavioral therapy).
UW Peds GU Monday, February 27, 2017 2:18 AM
Sickle cell trait:
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urinalysis with normal-appearing red blood cells (RBCs) suggest an extraglomerular etiology (vs dysmorphic RBCs, RBCs casts in glumerulonephritis) o Painless hematuria is likely due to renal papillary ischemia or necrosis o Other renal complications include inability to concentrate the urine (vasa rectae damage with inability to maintain concentrated medullary gradient) and distal renal tubular acidosis (tubular damage with impaired H+ secretion) Minimal change disease:
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Proteinuria is generally accomplished with a 24-hour urine collection, although a random level can be used in the appropriate clinical situation o Hypoalbuminemia is common in children with nephrotic syndrome. However, the inherent problem is increased glomerular permeability to albumin; infusing additional albumin will not correct it and may contribute to volume overload instead. o Empiric steroid therapy should be initiated upon suspicion of the diagnosis o Renal biopsy is indicated in children age >10 with nephrotic syndrome any child with nephritic syndrome minimal change disease that is unresponsive to steroids. UTI in infants:
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Urinary tract infection (UTI) most commonly arises by bacteria ascending into the bladder from the vaginal introitus. Recent sexual activity (eg, honeymoon cystitis) is an important risk factor for UTIs in women due to introduction of uropathogens into the urethra. During infancy, s/s of UTI are nonspecific and vague (eg, fever, fussiness, decreased urine output); abdomen/flank pain and dysuria can be difficult to recognize as infants are nonverbal. The presence of fever >39 C (102.2 F) in any child age <3 should prompt evaluation for occult UTI. Dx: Pyuria (≥5 white blood cells/hpf) and bacteriuria (50,000 colony-forming units/ml from a catheterized specimen) confirm the infection Rx: next step after Dx → 1-2 wks of Abx Indication for US: ideally performed after improvement of fever and symptoms to minimize false positive results from acute inflammation. Recurrent febrile UTIs in children of any age Age < 2 yr w/ a 1st febrile UTI UTI in any age w/ a family history of renal/urologic disease, HTN, or poor growth Children who do not respond to appropriate antibiotic treatment. Persistent or worsening symptoms may indicate renal abcess Indication for VCUG: Recurrent UTI First UTI with Abnormal US Age < 1 month (neonate) Age <2 yr + organism other than E coli Repeat blood work and urine culture should be performed only in children who fail to improve after 2-3 days of appropriate antibiotics. These studies are not indicated to prove a "cure" in children with obvious symptomatic improvement.
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Older children and adults generally do not need further evaluation of a 1st UTI due to lower likelihood of predisposing anatomic issues, lower risk of complications, and lower risk of recurrent UTI Vesicoureteral reflux: o The first UTI at age 2-24 months → US to evaluate for any anatomic defects. o Routine VCUGs after a first UTI are not usually recommended, as a normal US is generally reassuring. However, VCUGs are recommended in patients with recurrent UTIs as US is less sensitive in detecting VUR o Monitor pts for renal scarring (renal scan), developing renal insufficiency (serum creatinine), hypertension, or anemia.
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Constipation and UTI: o Distended rectum compresses the bladder and prevents complete voiding → residual urine/staisis → potential breeding ground for bacteria that ascend to the urethra from the perineum
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RTA:
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Presentation: Normal anion gap acidosis and failure to thrive. Calcium phosphate stones in type 1 due to alkaline urine. Normal anion gap = renal or git disorder (diarrhea) All types of RTA can present as growth failure {due to poor cellular growth and division in acidic conditions)
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Non AG acidosis no diarrhea
K+?
High K+ type 4
Low K+
Urine pH?
< 5.5 proximal type 2 ≥ 5.5 Distal type 1
o
Treatment consists of oral sodium bicarbonate to normalize the serum bicarbonate levels. Revise vasculitis from FA Henoch schonlein purpura: o Dx: Clinically in pediatric patients with a classic presentation Children with atypical presentations → renal biopsy to confirm → deposition of lgA in the mesangium o Arthralgias/arthritis: most commonly affect LL. Transient, no permanent damage to the joints Focal segmental glomerulosclerosis is the most common cause of nephrotic syndrome in adults in the United States. Revise nephritic and nephrotic from FA Wilms tumor:
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o o o
Some patients have abdominal pain, hypertension, hematuria, and fever. Less than 10% of patients have bilateral renal involvement (stage V disease). Although the lungs are the most common site of metastatic spread, children rarely present with pulmonary symptoms. US is the first step in imaging to differentiate Wilms tumor from other causes of abdominal masses. 155
o o
It should be followed by contrast CT of the abdomen to evaluate the nature and extent of the mass and of the chest to identify any pulmonary metastases DD of child w/ abdominal mass: Neuroblastoma 3rd most common pediatric cancer after leukemia and brain tumors but the most common cancer in the first year of life. Can arise anywhere in the sympathetic nervous system but typically involves the adrenal glands and presents as an abdominal mass that crosses the midline with systemic symptoms. Usually younger than pts. w/ wilms
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2ry enuresis:
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Type 1 DM has a bimodal onset, at age 4-6 years or at early puberty Nocturnal enuresis can be a presenting symptom in toddlers. o Nephrogenic diabetes insipidus is less common than DM. o Sleep apnea: disturbed arousal makes the pt unable to wake up to urinate. Electron Micorscopy of kidney biopsy showing alternating areas of thinned and thickened capillary loops w/ splitting of the glomerular basement membrane? Alport $ Thin basement membrane disease: familial disorder that presents in adulthood w/ microscopic hematuria w/o protenuria. Biopsy shows markedly thinned basement membrane Benign recurrent hematuria is asymptomatic. Renal biopsy is normal in most cases. Excellent prognosis. Isolated enuresis: 157
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Desmopressin: provides immediate improvement for frustrated families when behavior modification and alarm therapy (takes time) have failed The downsides risk of relapse (up to 70%) hyponatremia if too much fluid is consumed in the evening. Intranasal desmopressin is no longer used due to increased risk of hyponatremic seizures. o Presence of sacral hair tuft or dimple, abnormal reflexes → evaluate for neurogenic bladder o Oxybutynin is an anticholinergic medication that causes urinary retention. It is reserved for use in combination with desmopressin to increase bladder capacity in children with daytime incontinence. Transient proteinuria: o A urine dipstick can be positive in up to 10% of school-aged children. Proteinuria in children can be transient (intermittent), orthostatic, or persistent. o Transient proteinuria is the MCC of proteinuria and can be caused by fever, exercise, seizures, stress, or volume depletion. Usually discovered accidentally. If the UA shows no hematuria and is otherwise normal, the urine dipstick should be repeated on at least two additional specimens. If the proteinuria persists on the repeat sample or if any of the initial studies are abnormal → evaluate for underlying renal disease. Further investigation may include 24-hour urinary collection for protein, renal ultrasound, and, possibly, renal biopsy 158
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Orthostatic proteinuria is very common in adolescent boys and is defined as increased protein when the patient is in an upright position that returns to normal when the patient is recumbent Posterior urethral valves: o The MCC of urinary tract obstruction in newborn boys o Classic findings on prenatal ultrasonography include bladder distension, bilateral hydroureters, and bilateral hydronephrosis. o Potter sequence may develop
Nephritic vs nephrotic $:
o
Cryptorchidism o Most boys with unilateral cryptorchidism have no other anomalies, but bilateral cryptorchidism may be one of many manifestations of an endocrinopathy or genetic syndrome 159
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Testicles that have not descended by age 6 months are unlikely to descend spontaneously and require surgery. Early surgery can improve fertility, but sperm count and quality remain substandard. Prepubertal surgery also decreases but does not eliminate testicular cancer risk. Associated patent processus vaginal is that is repaired concurrently to prevent inguinal hernia
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Hemolytic uremic syndrome: o Approximately 10% of HUS cases are due to infection with Streptococcus pneumoniae; these patients have pneumonia or meningitis instead of diarrhea
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Hydrocele: 160
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Processus vaginalis fails to obliterate → peritoneal fluid accumulate within the processus vaginalis → communicating hydrocele. Collection of fluid within a tunica vaginalis that has properly obliterated its communication with the peritoneum is a noncommunicating hydrocele. Hydrocele can be differentiated from other testicular masses by transillumination; a hydrocele will transilluminate while other masses will not. NO NEED for ultrasound if the Dx is clear by physical exam. Most hydroceles, both communicating and noncommunicating, will resolve spontaneously by the age of 12 months and can be safely observed during that period. Surgical correction for persistent cases due to risk of inguinal hernia. Don't aspirate: Risks of aspiration include the potential for infection and damage to nearby structures. Risk > benefits
UW Peds musculoskeletal Tuesday, February 28, 2017 12:26 PM
Read here Acquired torticollis o Causes: MCC include Upper respiratory infections Minor trauma Cervical LN Retropharyngeal abscess Atlantoaxial subluxation o 1st step? Cervical spine x ray to exclude cervical spine fracture or dislocation. o Rx: Soft neck collar for musculoskeletal injury-related torticollis Botulinum toxin can be used to treat spastic torticollis in adults, but no evidence in children. Ewing's sarcoma: o LL>UL, metaphysis and diaphysis of the femur, tibia, and humerus. o Pain, swelling, erythema, warmth, fever, leukocytosis, anemia, increased ESR (Initially misdiagnosed as osteomyelitis) o X-ray Onion skin (periosteal reaction) Lytic, central, and associated w/ endosteal scalloping Onion skin is often followed by moth eaten or mottled appearance and extension into soft tissue. Glomus tumor: o Vascular benign tumor o 70% subungual o Triad of intermittent pain, tenderness, and sensitivity to touch o 4th decade. Females 161
Developmental dysplasia of the hips DDH: o Screening and early Dx and treatment is important before 6 months due to potential complications such as trendelenburg gait, scoliosis, arthritis, and avascular necrosis o Soft signs include: soft click, leg length discrepancy, asymmetric inguinal skin fold. They suggest possible hip laxity. o Hip laxity that is present at birth usually resolves by age 2 weeks. Therefore, imaging is not recommended until ≥2 weeks o Rx: Pt < 6 months: pavlik harness Pt > 6 months: surgical reduction and casting as harness is less successful in this age.
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Neonates w/ sacral dimple or tuft → screen w/ lumbosacral ultrasound → if positive → MRI to assess extent of defect Radial head subluxation:
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Supination + flexion is less successful and more painful than hyperpronation. Clinical suspicion → reduction (no x-ray).
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Legg-calve-perthes: o Antalgic gait (shorter time weight bearing on the affected side due to pain) o Dx requires high index of suspicion as initial x-rays may be negative (initial misdiagnosis of transient synovitis). Although transient synovitis typically follows a viral infection, symptoms should resolve within 1-4 weeks. This patient has had pain for >1 month. o Persistent symptoms → repeat imaging as it can take months for changes to appear on x-ray. o As the disease progresses, internal rotation and abduction at the hip joint can become markedly limited. Proximal thigh atrophy may also be present o MRI and bone scans can show subtle femoral head necrosis weeks to months earlier than x-rays and may be helpful to aid in early diagnosis Spondylolisthesis: o Back pain is very common in adults and usually benign in nature, in children it requires a careful search into the cause because it's usually due to organic causes o Mechanical causes of back pain like lumbosacral strain and herniated disk are uncommon in children o Spondylolisthesis is a developmental disorder characterized by a forward slip of vertebrae (usually L5 over S1 ) that usually manifests in preadolescent children. In the typical clinical scenario, back pain, neurologic dysfunction (e g., urinary incontinence), and a palpable "step-off' at the lumbosacral area are present if the disease is severe.
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Kawasaki disease:
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In patients with atypical presentation, supporting laboratory evidence can include the following ↑ CRP and ESR Leukocytosis with neutrophilia (as opposed to lymphocytosis in viral infections) Reactive thrombocytosis Sterile pyuria on urinalysis The disease usually resolves in 3 months w/o Rx but the risk of life-threatening complication necessitate early Rx Aneurysms are more prone to thrombotic occlusion and consequent MI. probably due to turbulent flow of blood. Echo: obtained at the time of Dx and repeated 6-8 wks to look for hcanges that may require closer monitoring. Aspirin and IVIG should be started within 10 days of fever onset to decrease the risk of complications 167
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Both aspirin and IVIG reduce systemic inflammation, and aspirin also has antithrombotic properties
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Nikolsky's sign is negative (v.s SSSS which has +ve sign)
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Atlantoaxial instability: o ↑ laxity in the posterior transverse ligament → ↑ mobility between the atlas (C1 ) and the axis (C2). 168
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Only 1-2% of Down $ pts w/ atlantoaxial instability are symptomatic o Symptoms usually progress over several weeks → compression of the spinal cord. o Symptoms include behavioral changes Torticollis Urinary incontinence vertebrobasilar symptoms (dizziness, vertigo, and diplopia) UMNL (spasticity, hyperreflexia, a positive Babinski sign, and clonus) Down syndrome are normally hypotonic, and they may remain hypotonic or have increased tone o Dx: lateral X-ray cervical spine (flexion, extension, neutral position, and open mouth) o Rx: surgical fusion of both vertebrae Intoeing:
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Both are diagnosed clinically Compartment syndrome: o severe pain, pallor, poikilothermia, paresthesia. Late s/s are absent pulse and paralysis.
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DD:
Transient neuropraxias (no increasing pain) Brachial a injury (intact pulse, no increasing pain)
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Lyme disease arthritis: o Migratory arthritis could be the presenting complaint of pts w/ lyme disease as early symptoms (eg, erythema migrans, fever) can be overlooked or misdiagnosed if history of travel to endemic areas is not obtained. o Untreated early localized disease can progress to an inflammatory monoarticular or asymmetric oligoarticular arthritis, most commonly involving the knee. o Synovial fluid shows Inflammatory profile w/ 25,000/mm3 leukocytes Gram stain and culture are usually negative PCR (done for investigative purposes) may demonstrate B burgdorferi DNA o Dx: can be confirmed with serum ELISA and Western blot testing o Rx: Oral doxycycline or amoxicillin is first-line treatment for Lyme arthritis in the absence of concurrent neurologic manifestations. SCFE: o Additional risk factors include endocrinopathies (eg, hypothyroidism, growth hormone deficiency) renal failure radiation o Pathophys: The physis (i.e , physical junction between the femoral head and neck) weakens during early adolescence because it is rapidly expanding and primarily composed of cartilage, which does not possess the strength of bone. When exposed to excessive shear stress, which is magnified by 170
obesity, the physis fractures and the femoral head slips posteriorly and medially relative to the femoral neck
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Serum sickness like reaction:
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Lab: nonspecific hypocomplementemia and elevated inflammatory markers (erythrocyte sedimentation rate, Creactive protein), which are consistent with any type Ill hypersensitivity reaction. Rx: Removal of the offending agent is generally sufficient to relieve symptoms within 48 hours. More severe cases may require glucocorticoid therapy
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Acute rheumtic fever: 172
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Growing pains: o Growing pains are a common complaint in children o 10%-30% of children age 2-12 years o The etiology of growing pains is unknown, but they are unrelated to growth, despite their name. o No need for lab or imaging
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Pearl: inflamed hip is typically flexed, slightly abducted, and externally rotated. This position maximizes the joint space, providing some pain relief. Transient synovitis. o MCC of hip pain in children. o Follows viral infection or mild trauma. 173
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Symptoms overlap w/ those of septic arthritis → you have to do a CBC, CRP, ESR to assess the severity of the inflammation. Pts who are ill appearing, febrile, or have >3 features of septic arthritis → immediate aspiration + IV Abx
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X-ray: to exclude bony lesions, fractures, and Legg-Calve-Perthes disease No additional workup unless symptoms persist or worsen → reconsider LeggCalve-Perthes → redo xray or do MRI Pauciarticular-onset juvenile idiopathic arthritis or pauciarthritis is the most common subgroup of juvenile idiopathic arthritis. Serum antinuclear antibodies are usually the only laboratory abnormality Lateral epicondylitis ("tennis elbow") is caused by repetitive contraction of the extensor muscles at the lateral epicondyle Tenderness is elicited over the lateral epicondyle, and pain is felt with passive wrist flexion and resisted wrist extension. Medial epicondylitis ("golfer elbow") manifests as localized tenderness over the medial epicondyle, pain with resisted wrist flexion, and pain with passive wrist extension. Lytic bone lesion o DD Infectious Brodie abscess from osteomyelitis endocrine hyperparathyroid osteitis fibrosa cystica neoplastic Ewing sarcoma Langerhans cell histiocytosis (histiocytosis X) metastases Idiopathic benign bone cyst aneurysmal bone cyst o hypercalcemia narrows the DD to: Hyperparathyroid Adenoma Old pts lytic bone neoplasm Young pts o Sarcoidosis can cause bone lesions and hypercalcemia, but Ages 20 and 35 years. Targets the hands and feet Multiple punched out cortical lesions and/or a reticulated pattern of cortical resorption o
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Osteoid osteoma: o benign bone-forming tumor that occurs most often in adolescence and early adulthood and is more common in males. o Rx: NSAIDs + serial examination and x-rays every 4-5 months Surgical excision reserved for pts w/ refractory s/s o Typically resolves on its own over several years o
Talipes equinovarus: o Might be congenital, teratologic, or positional Congenital cases are usually isolated, idiopathic cases. Teratologic cases are associated with a neuromuscular disorder or a complex syndrome → consider karyotyping to find out o The condition should be managed immediately (same day) Any delay would result in permanent damage Rickets: o The primary source of vitamin D is sunlight o Dark-skinned are at increased risk of vitamin D deficiency because melanin is a natural sunblock and prevents UV rays from reaching the skin for vitamin D synthesis. o However, infants of all ethnicities are typically shielded from direct sunlight due to the risk of sunburn and skin cancer, making dietary intake the chief source of vitamin D. o Prevention: breast milk alone does not have adequate vitamin D. Homemade baby food is also inadequate Infants who do not ingest fortified baby food or formula should receive vitamin D supplementation of 400 IU daily 175
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Rx: 1000-2000 IU daily vit D
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Gluteus muscle weakness: o Medius and minimus muscles
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Etiologies: Neuromuscular disease impingement of or trauma to the superior gluteal nerve inflammatory myopathies Osteogenesis imperfecta: o Autosomal dominant mutation of COL1A1 o The disorder has a varying spectrum of severity mild (type 1) fatal perinatal (type II) moderate (types III-IX), to disease o Patients with all types have osteopenia 176
o
Dx: should be suspected in any patient with blue sclerae. Other manifestations (recurrent fractures, easy bruisability, hypotonia, and hearing loss, dentinogenesis imperfecta - discoloration caused by discolored dentin shining through the translucent and weak enamel. Both primary and permanent teeth are affected.)
o
o
Osgood schlatters disease o 25% bilateral o
o
o
DD
Prepatellar bursitis 177
Chronic irritation of the anterior knee. pain with direct pressure and superficial swelling over the patella Patellar tendonitis An overuse syndrome resulting from repetitive jumping or kicking Anterior knee pain after exercise. Unlike Osgood-Schlatter disease, patients have point tenderness at the inferior pole of the patella. Tibial osteomyelitis Pain, swelling, tenderness, and erythema with refusal to bear weight on the affected extremity Systemic symptoms may also be present The pain from osteomyelitis does not remit with rest Patellofemoral stress syndrome An overuse injury commonly seen in runners. Anterior knee pain that worsens upon descending steps or hills. Pain is localized to the patella and radiographs do not demonstrate separation at the tibial tubercle.
178
