P di t i
P
3
P di t i
P
4
Intusseception - Intermittent Pain - Mass in RLQ - Occult blood positive -> Air contrast enema
P di t i
P
5
!""#$%%&'()*+*#&,*-(./0%)*+*%1,,*2.'34526,*2&-2& 1,,*2.'72 ,*2&-2&
P di t i
P
6
9.-"72 :*;+ *2 '." <./"*<*&, )*"! =*"-:*' > ??@ /*A+&"2
P di t i
P
7
CONGENITAL CMV PRESENTATION 1) Majority are asymptomatic at birth 2) Periventricular calcifications 3) IUGR, developmental delay, microcephaly, sensorineural hearing loss, retinitis, jaundice, hepatosplenomegaly, thrombocytopenia, hypotonia, lethargy, poor suck 4) Preterm infants may appear septic – apnea, bradycardia, intestinal distension) 5) Postnatal infections are generally asymptomatic
Presenting symptoms — The presenting symptoms reflect the location of the primary tumor and the extent of metastatic disease, if present. Patients with localized disease can be asymptomatic, whereas children with advanced disease appear ill at presentation, usually with systemic symptoms. Signs and symptoms of neuroblastoma may include [ 5,6]: Abdominal mass (retroperitoneal or hepatic)
●
Abdominal pain or constipation
●
Proptosis
●
Periorbital ecchymoses ("raccoon eyes", from periorbital ecchymosis caused by orbital metastases) ●
Horner syndrome (miosis, ptosis, anhidrosis)
●
Localized back pain, weakness (from spinal cord compression) ●
Scoliosis, bladder dysfunction
●
Palpable nontender subcutaneous nodules
●
Opsoclonus myoclonus ataxia syndrome
●
Otherwise unexplained secretory diarrhea (from paraneoplastic production of vasoactive intestinal polypeptide [VIP]) ●
Hematogenous spread most often occur to bone, bone marrow, skin and liver. Bone—bone pain (esp. with ambulation) and in the younger child limp or irritability. Infiltration of periorbital bones may produce periorbital ecchymosis, ptosis and proptosis. Bone marrow—blood cunt abnormalities and fever Liver—hepatomegaly Skin—nontender papules and subcutaneous nodules
Systemic symptoms (fever, weight loss)
●
Bone pain
●
Anemia
●
Heterochromia iridis (different colors of the iris or portions of the iris) ●
Hypertension
●
Unilateral nasal obstruction
●
P di t i
P
8
The presenting manifestations of SLE in children are as diverse as they are in adults. The most common initial symptoms are the gradual onset of fever, malaise, and general deterioration over several months. Children also may have small joint arthritis and renal disease. The classic malar rash is absent in twothirds of individuals. In retrospective reviews from France and Canada, the onset of juvenile SLE is at a median age of 12 to 13 years, with the disease developing in the majority of patients after eight years of age. In these studies, the most common presenting manifestations were as follows: * Hematologic: anemia, cytopenia, and/or thrombocytopenia * Mucocutaneous: malar rash and/or oral ulcers * Musculoskeletal: arthritis or arthralgia * Fever * Renal abnormalities (ex, nephritis and nephrotic syndrome)
P di t i
P
9
B lymphocyte Function
Group B Strep HIB = Encapsulated
B-A"&/*-; *'<&A"*.'2 )*"! #.;C2-AA!-/*,& &'A-#2D;-"&, E-A"&/*-F '&D"/.#!*;*G-"*&'"2 )*"! B A&;; ,&<*A*&'A*&2 -/& !*0!;C 2D2A"*E;& ". &'A-#2D;-"&, E-A"&/*-; *'<&A"*.'2(
P di t i
P
10
A varicocele (from the Latin word, varix for dilated vein; and the Greek word, kele for tumor) is an enlargement of the veins in the scrotum and is similar to varicose veins that occur in the legs. In younger men and boys, an abnormal appearing or feeling scrotum is often the reason for coming to a doctor. In adult men, varicoceles most often present during an evaluation for infertility. The anatomic defect in varicocele is an abnormal enlargement of the pampiniform venous plexus that drains the testicle in the scrotum. The pampiniform plexus is a collection of veins that helps regulate the temperature of the testicle - as the testicle best makes sperm at temperature two degrees lower than body temperature. Varicoceles are graded on a scale from I-II I, based on physical examination findings and are often described as appearing "like a bag of worms" in the scrotum
The first sign of tinea versicolor is often spots on the skin. The other signs and symptoms are: - The spots are lighter (sometimes darker) than the surrounding skin. The color of the spots can be white, pink, salmon, red, tan, or brown. - The spots can appear anywhere on the body. - Spots can be dry and scaly. - Skin may itch where the spots appear. Tinea versicolor is caused by the dimorphic, lipophilic organisms in the genus Malassezia, formerly known as Pityrosporum. Eleven species are recognized within this classification of yeasts, of which Malassezia globosa and Malassezia furfur are the predominant species isolated in tinea versicolor Tx) Terbanifine, Ketoconazole
P di t i
P
11
Appropriate blood glucose targets can be established based on the pattern of blood glucose responses and the ability of the individual athlete to make appropriate adjustments to keep glucose in the target range. At the beginning of sports training, it is often necessary to adjust insulin therapy by decreasing the total insulin dose by 20–50%. Reductions may be needed in both short- or rapid-acting insulin and intermediate- or long-acting insulin. Athletes on pump therapy may need to decrease bolus doses by 20–50% and may choose to discontinue basal insulin during exercise and decrease the basal insulin by 25% after exercise is completed.
P di t i
P
12
Short Stature Delayed menses Androgen -> hair growth -> HPA activated Weak Femoral Pulses (Coarctation) THINK TURNER’S
Treponema pallidum is a spirochete that is responsible for syphilis. First stage: Presents as a painless ulcer (Chancre) at the site of inoculation 3 weeks after infection, and the lesion resolves. - The chancre of primary syphilis usually begins as a single, painless papule that rapidly becomes eroded and indurated, with a surrounding red areola. The edge and base of the ulcer have a cartilaginous (buttonlike) consistency on palpation. Second stage: The patient may present with fever malaise, headache, adenopathy, generalized body rash, and mucosal lesions (condylomata lata). The rash of secondary syphilis can be confused with pitiriasis rosea. The symptoms of secondary syphilis resolves spontaneously and the infection enters a latent stage. Third stage: Can present years later and may manifest as neurologic, cardiovascular, and skeletal disease. HPV: Condylomata are discrete, papillary, cauliflowerlike lesions that involve multiple sites on moist surfaces. Keratotic warts are often seen on dry surfaces like the labia majora. Warts vary in size and can form large, exophytic, cauliflowerlike masses (see the images below). Discrete papules 1-3 mm in size can present on the shaft of the penis. The growth can extend into the vagina, urethra, cervix, perirectal epithelium, anus, and rectum HSV: Tender Bilateral lymphadenopathy. Lesions itch. Vesicular Lesions on an erythematous base
P di t i
P
13
P di t i
P
14
An infant can experience symptoms either very quickly after feeding (rapid onset) or up to several days or even weeks after first consuming the cow's milk protein (slower onset). The most common symptoms are gastrointestinal (50-60%), skin (50-60%) and respiratory (20-30%). Many infants may experience two or more of these symptoms.3 IgE-associated cases of CMPA tend to show an early reaction even to a small quantity of CMP, with symptoms including irritability, urticaria, dermatitis, swelling, or vomiting occurring within minutes. Although anaphylaxis is more common in other food allergies, in rare cases, anaphylaxis can occur in conjunction with CMPA. In these cases, CMPA may be the first expression or predictor of future atopy and other food allergies in the infant, and there is frequently a family history of atopy.1
Laryngotracheobronchitis Croup manifests as hoarseness, a seal-like barking cough, inspiratory stridor, and a variable degree of respiratory distress. However, morbidity is secondary to narrowing of the larynx and trachea below the level of the glottis (subglottic region), causing the characteristic audible inspiratory stridor (see the image below). Inspiratory stridor suggests a laryngeal obstruction, whereas expiratory stridor suggests tracheobronchial obstruction. A reactive inflammatory response causes subglottic edema. Narrowing of the airway can be life threatening in infants and young children because of their small airway “Pencil Sign” on AP X-ray
P di t i
P
15
A!.;&;*"!*-2*2 ?? #-"*&'"2 )*"! 2#!&/.AC".2*2%!&:.;C2*2 !-H& !*0! /*2+ .< 0-;;2".'&2
Hereditary Spherocytosis (HS) is the most common hemolytic anemia due to a red blood cell (RBC) membrane disorder. HS is due to a mutation in spectrin, ankyrin, band 3 protein, or protein 4.2. These proteins normally maintain the biconcave disc shape of RBCs.With a defect in these proteins, the RBC typically takes on a spherical shape. - These RBCs are not as flexible as the regular RBC, and can undergo hemolysis when trying to pass through the small capillaries of the reticuloendothelial system (RES). - This then causes the spleen to become engorged, which can be palpable on physical exam. Cholelithiasis — due to severe hemolysis, patients can develop cholelithiasis and acute cholecystitis due to the formation of pigmented gallstones.
1,:*'*2"&/ 2D/<-A"-'" -', I= 1BJK
Congenital cardiac defects — these can occur in 40 to 60% of patients with Down’s syndrome, and include complete AV canal defects, VSD and tetralogy of Fallot. Because of this risk, ALL newborns with Down syndrome should have an echocardiogram and ECG to screen for heart defects. If found, the patient should be referred to a pediatric cardiologist.
P di t i
P
16
HEMOLYTIC DISEASE OF THE NEWBORN The diagnosis is made when the infant is A, B or AB and has a positive direct Coombs test and a positive indirect Coombs result for anti-A or anti-B. The mother will lack the A or B antigen which is positive in the indirect Coombs test. TX) Exchange Transfusion
Hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) fall into the broader category of thrombotic microangiopathies (TMA). Thrombotic microangiopathies are characterized by the involvement of widespread occlusive microvascular thromboses resulting in thrombocytopenia, microangiopathic hemolytic anemia, and variable signs and symptoms of end-organ ischemia. Specifically, E coli serotype O157:H7 has been associated with more than 80% of infections leading to hemolytic uremic syndrome. TRIAD of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure.
P di t i
P
17
L/&:-2"&/ /&<;&M ).D;, 2"*;; E& #/&2&'" *' *,*,C:*"*2K
Epididymitis is the most frequent cause of acute onset scrotal pain in adults. In contrast with men who have testicular torsion, the cremasteric reflex (elevation of the testicle in response to stroking the upper inner thigh) is not altered.
Status Epilepticus: Benzos to abort -> progress to Phenytoin -> Phenobarb if doesn’t work
P di t i
P
18
2;*" ;-:# &M-: ?? I ,*,'7" "!*'+ *" )-2 2"*A -/"!/*"*2 0*H&' "!-" 2!& <&&;2 E&""&/ )*"! D2-0&( 1N1O :-,& :& "!*'+ .< -D".*::D'& A-D2&2 .< P.*'" #-*'F )!*A! :-,& :& "!*'+ -E.D" -22.A*-"&, &C& <*',*'02( L-2& Q*;&2 2-C2 "!-" DH&*"*2 *2 -' *:#./"-'" "!*'0 ". A-"A! *' RI1 -', /&A.::&',2 ,.*'0 - 2;*" ;-:# &M-: .' &H&/C.'& 2D2#&A"&, ". !-H& RI1(
Nephroblastoma: Usually asymptomatic kid, with mass found by parents - Does NOT cross midline
P di t i
P
19
Leukocoria or a white pupillary reflex is an ominous finding in infants. It can be caused by opacification of any of the structures that lie in the visual axis including the cornea, the lens, the vitreous, and the retina. The majority of these conditions are significant for visual impairment that may not be appreciated until the child is older. However, retinal conditions such as retinoblastoma (RB) can be life-threatening. Therefore screening for and evaluation of leukocoria should be performed at every well-baby and well-child visit The most common presenting feature in the US is leukocoria (an absence of the red reflex of the eye). Strabismus (misalignment of the eyes) is the second most common presenting feature. Retinoblastoma confined within the eye has excellent prognosis with reported survival rates of above 90%. When this disease goes undetected, as is often the case in developing countries, tumor often spills from the sclera into the orbit or beyond the optic nerve leading to seeding in the vitreous cavity or extension into the subretinal space, thereby decreasing survival rates to around 50%.
S&:.;C"*A ,*2&-2& .< "!& '&)E./' ?? *" *2 '." #!C2*.;.0*A E&<./& 4T?UV!/ .< ;*<&(
P di t i
P
20
Hyperinflation: Obstructive lung disease => Asthma
Endoscopy should be performed within the first 12-48 hours following ingestion as endoscopy occurring more than 48 hours after ingestion increases the risk of perforation of the weakened tissue. An exception to this is children who ingested household bleach often do not require endoscopy due to the very low likelihood of tissue damage. The presence and extent of injury is documented using flexible endoscopy. Endoscopic ultrasonography has been demonstrated to more accurately show the depth of lesions and may be used more widely in the future to aid in diagnosis and treatment. Ingestions of alkaline substances cause tissue damage by liquefactive necrosis. This involves saponification of fats, denaturation of proteins, and disruption of cellular membranes, allowing the alkaline substance to diffuse into deeper layers of the mucosa. Small vessel thrombosis and heat production also occur. Agents with a pH greater than 11 cause the most damage.
P di t i
P
21
Henoch-Schönlein purpura (HSP) is an acute immunoglobulin A (IgA)–mediated disorder characterized by a generalized vasculitis involving the small vessels of the skin, the gastrointestinal (GI) tract, the kidneys, the joints, and, rarely, the lungs and the central nervous system (CNS). Subsequently, symptoms develop, of which the following are the most common: - Rash (95-100% of cases), especially involving the legs; this is the hallmark of the disease - Abdominal pain and vomiting (35-85%) - Joint pain (60-84%), especially involving the knees and ankles - Subcutaneous edema (20-50%) - Scrotal edema (2-35%) - Bloody stools
Reactive arthritis is a painful form of inflammatory arthritis (joint disease due to inflammation). It occurs in reaction to an infection by certain bacteria. Most often, these bacteria are in the genitals (Chlamydia trachomatis) or the bowel (Campylobacter, Salmonella, Shigella and Yersinia)
P di t i
P
22
122.A*-"&, )*"! ;.) LU W!C#.A.:#;&:&'"&:*-X$ YGZ[Y \ #.2"2"/".A.AA-; 0;.:&/D;.'!/*"*2F :&:E/-'.#/.;*<&/-"*H&F -', []^ W;D#D2 '!/*"*2X
Acute post-streptococcal glomerulonephritis (PSGN) can occur after an episode of strep throat or, in rare cases, impetigo (a skin infection). The Streptococcus bacteria do not attack the kidney directly, but an infection may stimulate the immune system to overproduce antibodies, which are circulated in the blood and finally deposited in the glomeruli, causing damage. PSGN can bring on sudden symptoms of swelling (edema), reduced urine output (oliguria), and blood in t he urine (hematuria). Tests will show large amounts of protein in the urine and elevated levels of creatinine and urea nitrogen in the blood, thus indicating reduced kidney function. High blood pressure frequently accompanies reduced kidney function in this disease. PSGN is most common in children between the ages of 3 and 7, although it can strike at any age, and it most often affects boys. It lasts only a brief time and usually allows the kidneys to recover.
BUN:Cr > 20
G/&/&'-; -AD"& /&'-; <-*;D/& % 1_I % -`."&:*-
P di t i
P
23
Biliary atresia is a disorder unique to the neonatal period. The fetal/perinatal form is evident within the first 2 weeks of life; the postnatal type presents in infants aged 2-8 weeks.
P di t i
P
24
Asplenic patients need prophylactic Penicillin
_*', .< ;*+& 2*A+;& A&;; #-"*&'"2
P di t i
P
25
Infantile hemangiomas (IH) are benign vascular neoplasms with a course marked by early proliferation and spontaneous involution - IH are the most common tumors of infancy - IH can be cutaneous or extracutaneous. Common extracutaneous sites include: Liver, Gastrointestinal tract, Larynx, CNS Kassabach-Merritt Syndrome- Thrombocytopenia and/or coagulopathy resulting from platelet trapping within a vascular tumor. Infantile hemangiomas are rarely responsible for this syndrome! More commonly tufted angioma or kaposiformhemangioendothelioma
Hirchsprung’s disease: Hirchsprung’s disease, or congenital aganglionic megacolon, is a motor disorder of the colon that causes a functional intestinal obstruction. It occurs in 1/5000 infants with a male to female predominance of 4:1. The pathogenesis of the disease is failure of migration of the neural crest cells that form t he colonic ganglion cells. Without parasympathetic innervation, the colon cannot relax or undergo peristalsis, resulting in a functional obstruction. The aganglionic segment is limited to the rectosigmoid in the majority of patients. 10% have full colonic involvement and in 10% more, there is lack of ganglion cells into the small bowel. Diagnosis of Hirchsprung’s disease can be made with barium enema, revealing a transition zone between the constricted aganglionic segment and t he proximal, normally dilated segment. Confirmation of the diagnosis is made with rectal suction biopsy, which will demonstrate absent ganglion cells, hypertrophied nerve fibers, and elevated acetylcholinesterase activity. The treatment of Hirchsprung’s disease is through surgical resection of the aganglionic bowel and approximation of normal, ganglionic tissue near the anus. The major complications of the disease, even after surgical resection, are bowel obstruction and enterocolitis, though most patients go on to normal or near-normal bowel function.
P di t i
P
26
Puberty in Females - Onset between 7-13 - Thelarche -> Adrenarche -> Menarche (Age 9-15)
Latent TB infection - Positive PPD - >5mm for those in close contact - >10mm younger than 4, endemic region, chronic medical condition - >15mm older than 4 with no other risk f actors - Negative physical and chest x-ray - Children rarely progress to to TB disease Tx) Isoniazid for 9 months. Older adolescents are given B6 to prevent neuro complications TB Disease Tx) INH, Rifampin, Pyrazinamide for 2 months, followed by INH and Rifampin for 4 months
P di t i
P
27
Kawasaki Treatment: IVIG and Aspirin to prevent Coronary Aneurysm
I' -,,*"*.' ". -2#*/*'K
]*+&;C - H*/-; abI ??@ 2D ##./"*H& "/&-":&'"F 2&;;*:*"*'0
P di t i
P
28
HHT - Nose Epistaxis from Telangiectasias - AVM in brain and large organs Neurofibromatosis Type 1 - Cafe au Lait - Neurofibromas - Bone Defects - Scoliosis - Lisch Nodules - Optic Nerve Gliomas
Tuberous Sclerosis - Hamartomas - Ash Leaf Macules - Benign tumors to grow in the brain and on other vital organs such as the kidneys, heart, eyes, lungs, and skin. A combination of symptoms may include seizures, intellectual disability, developmental delay, behavioral problems, skin abnormalities, lung and kidney disease.
Sturge Weber - Port Wine Stain - Calcifications of Brain - Weakness of body opposite of stain
Von Hippel Disease Clinical hallmarks include the development of retinal and central nervous system (CNS) hemangioblastomas (blood vessel tumors), pheochromocytomas, multiple cysts in the pancreas and kidneys, and an increased risk for malignant transformation of renal cysts into renal cell carcinoma.
About a week to 11 days after the MMR injection, some children get a very mild form of measles. This includes a rash, high temperature, loss of appetite and a general feeling of being unwell for about two or three days.
P di t i
P
29