Disusun oleh : Ang A ngel elin inaa Di Dian anan anth thar arii G / 40 406 6 12 1210 1002 02 Pembimbing : Dr.Dyani Dr .Dyani Kusumowardhani, Sp.A
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Sindrom nefrotik merupakan penyebab tersering yang ditemukan pada penyakit ginjal. Sindrom nefrotik (SN) → sindrom klinik yang mempunyai banyak penyebab → ditandai permeabilitas membran glomerulus yang meningkat dengan manifestasi proteinuri masif (>40mg/m2/jam); yang menyebabkan
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Sindrom nefrotik (SN) Sindrom penyakit ginjal, ditandai : - Edema - Hipoalbuminemia Hiperkolestero olesterolemia/hiperlipidemi lemia/hiperlipidemiaa - Hiperk - Proteinuria yang masif
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Sindrom nefrotik (SN) pada anak merupakan penyakit ginjal anak yang paling sering ditemukan → hampir 50% mulai sakit saat usia 1-4 tahun & 75% mempunyai onset sebelum usia 10 tahun. laki-laki : perempuan = 2 : 1 kebanyakan umur 2 - 6 tahun (60%) Indonesia : 6 kasus/100.000 anak usia <14 th / tahun (Alatas, 2002)
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Sindroma Nefrotik Primaryy (87%) Primar Idiopathic Minimal lession (85%)
Steroid responsive (90-95%)
Relapses (60-80%)
Secondaryy (13%) Secondar Non idipathic
Non minimal lession
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Edema Albumin plasma <2,5 mg% Proteinuria masif >40 mg/m 2/jam Protein : rasio kreatinin >200 mg/ mmol
Sindrom Nefrotik
• • • •
Frequent relapses
• relaps ≥ 2 x dalam 6 bulan pertama setelah respons awal atau ≥ 4 x dalam periode 1 tahun
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Sebab pasti belum jelas Diduga sebagai suatu penyakit autoimun (reaksi antigenantibodi) → akibat kelainan fungsi limfosit (asal dari thymus) →
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2. Sindrom nefrotik sekunder Disebabkan oleh : Malaria kuartana atau parasit lain Penyakit Peny akit kolagen seperti lupus lupu s eritomatosus diseminata, purpura anafilaktoid
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Kelinan minimal - mikroskop biasa : glomerulus normal - mikroskop mikros kop elektron : foot foo t processus sel epitel terpadu. - cara imunofluoresensi : tidak terdapat Ig G / imunoglobulin beta-1C pada dinding kapiler glomerulus.
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Glomerulonefritis proliferatif a. Glomerulonefritis prolifer proliferatif atif eksudatif difus - proliferasi sel mesangial & infiltrasi sel polimorfonukleus. - pembengkakan sitoplasma endotel endotel → kapiler tersumbat - ditemukan pada :-nefritis yang timbul setelah infeksi dengan
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c. Dengan bulan sabit (crescent)
- proliferasi sel mesangial & proliferasi sel epitel simpai (kapsular) & viseral. - prognosis : buruk. d. Glomerulone Glomerulonefritis fritis membranoproliferatif
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4. Glomeruloskleros Glomerulosklerosis is fokal segmental
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- stadium lanjut : ditemukan sel busa interstisial yaitu atrofi
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2. Hipoalbuminuria
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Skema pemberian diuretik untuk mengatasi edema : Furosemid 1 – 3 mg/kgbb/hari + spironolakton 2-4 mg/kgbb/hari Respon -
Berat badan tidak menurun atau tidak ada diuresis dalam 48 jam Respon -
Dosis furosemid dinaikkan 2 kali lipat (maksimum 4-6 mg/kgbb/hari) Respon -
Tambahkan hidroklorothiazid 1-2 mg/kgbb/hari Respon Bolus furosemid IV 1-3 1 -3 mg/kgbb/dosis atau per infus dengan kecepatan 0,1-1 mg/kgbb/jam
Respon -
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KORTIKOSTEROID Pengobatan pada SN idiopatik,kecuali bila ada kontraindikasi Jenis steroid adalah predn prednison ison atau predni prednisolon solon TERAPI INSIAL * Prednison 60 mg/m2 LPB/hari atau 2 mg/kgbb/hari (maksimal 80 mg/hari) dosis terbagi → untuk menginduksi remisi. * Dosis prednison dihitung sesuai dengan berat badan ideal (berat badan terhadap tinggi badan). * Prednison dosis penuh ( full ( full dose) dose) inisial diberikan selama 4 minggu. * Bila terjadi remisi dalam 4 minggu pertama → dilanjutkan dengan 4 minggu kedua dengan dosis 40 mg/m2 LPB (2/3 dosis awal) atau 1,5
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PENGOBATAN SN RELAPS PENGOBAT REL APS * Prednison 60 mg/m2 LPB/hari atau 2 mg/kgbb/hari (maksimal 80 mg/hari) dosis terbagi dalam 4mgg → dilanjutkan dengan 4 minggu kedua dengan dosis 40 mg/m2 LPB (2/3 dosis awal) atau 1,5 mg/kgbb/hari, secara alternating (selang sehari)
* Jika proteinuria ≥ 2+ tanpa edema dan terbukti terbukti infeksi : beri antibiotiK 57 hari * proteinuria ≥ 2+ dengan edema : beri pengobatan steroid PENGOBATAN SN RELAPS PENGOBAT REL APS SERING SER ING ATAU ATAU DEPENDEN STEROID Terdapat 4 opsi pengobatan SN relaps sering atau dependen steroid : 1. Pemberian steroid jangka panjang * Dicoba dahulu pemberian steroid jangka panjang dosis penuh, setelah mencapai remisi, diberi steroid selang sehari dengan dosis diturunkan
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Bila terjadi keadaan keadaan di bawah ini: 1. Relaps pada dosis rumat > 1 mg/kgbb, selang sehari atau 2. Dosis rumat < 1 mg/kgbb tetapi disertai: a. Efek samping steroid yang berat b. Pernah relaps dengan gejala berat antara lain hipovolemia trombosis, tromb osis, dan sepsiS → diberikan siklofosfamid (CPA) 2-3 mg/kgbb/hari selama 8-12 minggu.
2. Levamisol Pemakaian terbatas karena efek masih diragukan. Efek samping : mual dan muntah.
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3. Pengobatan dengan sitostatik siklofosfamid 2-3 mg/kgBB dosis tunggal P.O/IV CPA CP A IV diberikan dengan dosis 500-750 500- 750 mg/m2 LPB dilarutkan dengan NaCl 0,9% 250 ml diberikan selama 2 jam, pemberian sebanyak 7 dosis dengan interval 1 bulan (durasi pemberian 6 bulan). Efek toksisitas pada gonad gonad bila dosis total kumulatif ≥ 200 -300 mg/kgBB. Pemberian oral selama 3 bulan dengan dosis total 180 mg/kgBB masih aman untuk anak. ES : mual, muntah, depresi sumsum tulang, alopesia, alopesia, sistitis hemoragik, azospermia, dalam jangka panjang dapat menyebabkan keganasan.
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4. Siklosporin SN yang tidak responsive terhadap steroid atau sitostatik 4-5 mg/kgbb/hari (100-150 mg/m2 LPB)
5. Mikofenolat mofetil (mycophenolate ( mycophenolate mofetil = MMF)
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PENGOBATAN SN DENGAN KONTRAINDIKASI STEROID
Bila didapatkan gejala atau tanda yang yang merupakan mer upakan kontraindikasi steroid : seperti seper ti tekanan darah tinggi, peningkatan ureum dan atau kreatinin, infeksi berat → sitostatik CPA oral/iv. Siklofosfamid 2-3 mg/kg bb/hari dosis tunggal/PO, maupun iv.
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Pengobatan SN Resisten Steroid
Siklosporin dapat menimbulkan remisi total sebanyak 20% pada 60 pasien dan remisi parsial pada 13%. Efek samping : hipertensi, hiperkalemi, bersifat nefrotoksik Perlu pemantauan kadar CyA dalam serum (dipertahankan antara 150250 nanogram/mL), kreatinin darah berkala, biopsy ginjal setiap 2
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TATA LAKSANA KOMPLIKASI SINDROM NEFROTIK : 1. INFEKSI Pasien SN sangat rentan terhadap infeksi → antibiotik. Infeksi yang terutama → seluliti selulitiss dan peritonitis primer. primer. Bila terjadi peritonitis primer (biasanya (bias anya disebabkan oleh kuman ku man Gram negatif dan Streptococcus pneumoniae) → penisilin parenteral dikombinasi dengan sefalosporin sefal osporin generasi ketiga yaitu sefotaksim atau
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2. TROMBOSIS Bila diagnosis trombosis telah ditegakkan dengan pemeriksaan fisis &radiologis → diberikan heparin / SC → dilanjutkan dengan warfarin selama 6 bulan atau lebih. Pencegahan tromboemboli → aspirin dosis rendah → tidak dianjurkan.
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4. HIPOKALSEMIA
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