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Prof. Dr. dr.Syarifuddin Rauf, SpA(K)Bagian Ilmu Kesehatan Anak FK - UNHASRS Dr. Wahidin Sudirohusodo MakassarNefrologi AnakNephrotic Syndrome in Children Syarifuddin Rauf

Department of Childhealth, Medical Faculty Hasanuddin University

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Pediatric NephrologyTabel. Sebaran penyakit ginjal anak yang dirawat inap di BIKA FK UNHAS RS Wahidin Sudirohusodo (2000 2004)Jenis penyakit ginjalJumlah%Sindrom Nefrotik9039,0Glomerulonefritis akut7130,7Infeksi saluran kemih3213,9Gagal ginjal akut41,7Gagl ginjal kronik20,9Tumor ginjal208,7Kelainan kongenital sal. Kemih20,9Batu saluran kemih41,7Nefritis Schoenlein Henoch41,7Nefritis Lupus10,4Proteinuria persistent10,4Jumlah23110051. Generalized oedema2. Heavy proteinuria(>50mg/kgbb)3. Hypoproteinemia(250mg/dl) (Hypercholestrolemia)NEPHROTIC SYNDROME

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1. Edema Masif2. Proteinuri Masif3. Hipoproteinemi (< 2,5 g/dl)4. Hiperkolesterolemi (>250 mg/dl)

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INCIDENCEWilawirya (1992): 6 cases/100.000 population < 14 yr old/yrSex ratio : : : = 1,5 2 : 1Children:Adult = 15 : 1Age incidence : - Highest Inc. = 2-5 years - Less common : > 5 yearsDepartment of Child Health, Hasanuddin University / General Hospital Wahidin Sudirohusodo : 1-2 cases/month

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ETIOLOGY

Unknown (idiopatik=primer)Acquired(sekunder): Diabetic MellitusGenetic factors : - Congenital NS (mutation on chromosom 19) - HLA antigens : HLA-DR7Predisposition: Allergy

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PATOMECHANISMSoluble antigen- antibody complexElectrochemic theory

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INCITING FACTOR (URIStreptococcus)IgG + Antigen complexes Deposite in glom. basement membrane (gbm)Activate complementImmune complexes in gbm (Antigen,IgG+C3)Renal symptomsPATHOPHYSIOLOGY

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CLINICAL MANIFESTATIONSCongenital NS (Finlandia type)Placenta enlargementMassif oedemaGenetic mutation on chromosome 19Steroid sensitive NSResponsif to cortikosteroidMinimal change NS (MCNS) : 70-80%Steroid resistantNo/minimal response to cortikosteroidFocal glomerulosclerosis (FSGS)

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SYMPTOMS & SIGNSOedema :Pitting oedemaGeneralized : starting in periorbital regions face abdomen (ascites) extremities Pleural effusionsMassive anasarca scrotal or vulval oedemaNo hypertension or hematuriaNormal renal function

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Abdominal striae and pitting edema8

MANAGEMENTHypoalbuminaemia (< 2 gr%)Salt-poor human albumin (plasbumin) 1 gr/kgBWFebrile / feels unwell / abdominal pain :AntibioticsDiuretic : Indications : severe oedema that causes dyspnoeSpecific treatment : corticosteroidProtocol : International Study of Kidney Disease in Children (ISKDC)

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PROTOCOL THERAPY OF NS

CD = 4 weeksAD/ID = 4 weeksTap. off1- 2 thn123 4 5 6 7 8remissionremissionISKDC: 1. ISKDC179

PROTOCOL THERAPY OF NS

CD = 6 weeksAD/ID = 6 weeksTap. off1 year1 2 3 45 67 8 9 10 11 12remissionremissionISKDC: 2. Arbeitsgemeinschaft fur Paediatrische Nephrologie (APN)

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PROTOCOL THERAPY OF RELAPS NS

CDAD/IDTap. off1 year123 4remissionremissionCD until remission( 1 - 4 minggu )19

Any Questions?21KESIMPULANSN pada anak bersifat idiopatik dan umumnya sensitif kortikosteroid. Pengobatan SN pada anak sebaiknya dimulai dengan prednison/ prednisolon yang setelah mencapai remisi, prednison / prednisolon dilanjutkan sampai mencapai dosis threshold selama 6 12 bulan.Bila terjadi relaps, apakah itu bersifat resistent atau dependen steroid maka dapat diberikan obat-obat immunosupresif lain, yang diberikan secara bersama-sama dengan steroid atau sebagai single tratment.Selain pemberian obat maka pada penatalaksanaan SN harus diperhatikan terapi penunjang..22Sindrom nefrotik relaps frekuen atau dependen steroidPrednison FD RemisiDiturunkan sampai dosis treshoid 0,1-0,5 mg/kgBB AD6-12 bulanPrednison AD + CPARelaps padaPrednison > 0,5 mg/kgBB ADLevamisol 2,5 mg/kgBB AD(4-12 bulan)Relaps pada prednison > 1 mg/kgBB ADatauEfek samping steroid meningkatCPA 2-3 mg/kgBB8-12 mingguRelaps prednison standarRelaps pada prednison > 0,5 mg/kgBB ADSiklosporin 5 mg/kgBB/hariselama 1 tahunGambar. Diagram pengobatan sindrom nefrotik relaps frekuen atau dependen steroid23MCNS(100%)Initial responder (93%)Initial nonresponder (7%)R/ 8 mgg I6 bln (sesudah R/ 8 mgg)Non relapser (36%)Infrequent relapser (18%)Frequent relapser (39%)Late responder (5%)Late non-responder (2%)Subsequent nonresponder (5%)Minimal Change NS(MCNS) after 8 weeks treatment of corticosteroid24GLOMERULONEFRITIS AKUT PASCA STREPTOKOKKUS (GNAPS)

ACUTE POST STREPTOCOCCAL GLOMERULONEPHRITISBEBERAPA ISTILAHGlomerulonefritis Akut (GNA) : Suatu bentuk proses proliferasi dan inflamasi glomeruli yang didahului oleh suatu proses infeksi dan terjadi secara akut.( contoh : GNAPS )

Sindrom Nefritik Akut (SNA ) :Suatu kumpulan gejala-gejala klinik berupa proteinuria, hematuria azotemia, redblood cast( torak eritrosit ), oligouria dan hipertensi (PHAROH)

ISTILAH GNA dan SNAGNA lebih bersifat histologik ( proliferasi dan inflamasi sel-sel glomerulus)

* SNA lebih bersifat klinik ( PHAROH)

Salah satu bentuk SNA/GNA adalah GNAPS

Proliferasi dan inflamasi glomeruli Sekunder oleh mekanisme imunologik Antigen: streptokokus B hemolitikus grup A

GNAPS

1. Angka kejadian Lebih sering umur 6-7 thn, jarang < 3 thn Laki laki > perempuan (2:1) 10- 12 % kasus infeksi strept. hemolitikus grup A Kaplan: 50% kasus asimtomatik pd epidemi GNAPS didahului ISPA atau piodermi

2. Etiologi Streptokokus hemolitikus grup A (tipe M) NEFRITOGENIK Faringitis (serotipe tersering 12, lalu 1,3,4,6,25) Piodermi (serotipe tersering 49, lalu 2,53,55, 56,57,58,60

Periode latent: 1 3 mingguEdema HematuriHipertensi OligouriaGejala-gejala lain: lelah, malaise, letargi & anoreksia g. Kelainan laboratoriumMANIFESTASI KLINIK

URIN:Hematuri, warna kemerah-merahan atau seperti air dagingProteinuri : kualitatif dan kuantitatif> 6 bulan proteinuri persisten biopsi ginjal

DARAH:Titer ASTO meningkatMenurunnya kadar C3LED meninggiHipoproteinemi ringanPemeriksaan bakteriologik

Bila memenuhi 4 gejala berikut Hematuri makroskopik atau mikroskopik Edema Hipertensi ASTO meningkat C3 menurun

Diagnosis GNAPS

PENGOBATANANTIBIOTIK :Bila dijumpai tanda-tanda infeksi

PENGOBATAN SIMTOMATIK : - Diuretik : + oligouri + edema pulmonum - AntihipertensiKOMPLIKASI YANG TERJADI PADA GNAPS DAN SN:

1.HIPERTENSI ENSEFALOPATI2. EDEMA PARU3. SYOK HIPOALBUMINEMI4. GAGAL GINJALPROGNOSISBAIK : Self limiting diseaseJELEK : Bila ada komplikasi yang tidak dapat diatasi : - gagal ginjal akut - edema pulmonum - ensefalopati hipertensi

BAIKURINARY TRACT INFECTION(UTI)

Infection from renal parenchyme orificium urethrae externaSignificant bacteriuriaWith or without symptoms DEFINITION

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Pathogenic bacteriaColony count : > 100.000/ml urine> 1x lab. examinations

Significant bacteriuria

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Relapsing UTI :Recurrent UTISame microorganismReinfection UTI :Recurrent UTIDifferent microrganism

ETIOLOGYBacteria :E. ColiKlebsiellaProteusPseudomonasOther microorganisms :ProtozoaVirus

CLASSIFICATION Clinically : 1. Symptomatic UTI 2. Asymptomatic UTIComplication :Simple UTI Complicated UTILocalization : 1. Upper UTI 2. Lower UTI

PATHOGENESISHematogenicPercontinuitatumLymphogenic

Clinically :Upper UTI (Pyelonephritis) :Fever, back/flank pain & with or without lower UTI symptomsLower UTI (Cystitis) :Suprapubic punction, dysuria,frequent voiding etc.DIAGNOSIS

PATHOGENESISNeonatesBaby & Child(>1 month)Hematogen(Septicemia)Percontinuitatum(Ascending)Bacteria enter to Urinary tractSymptomatic UTI Asymptomatic UTIColonization on GITCertain focus : Periurethra/Perineum: Subpreputium?

LAB. EXAMINATIONSURINE : Urinalysis :Leukocyte > 5-10/HPFErythrocyte : +/-Urine culture :Mid : stream urine : C.C. : > 100.000/ml urineCatheterization :C.C. : > 10.000/ml urineSuprapubic punction :C.C. : > 1000/ml urine

BLOOD :LeucocytosisIncreased BSR (> 30 mm/hour)Increased CRP (> 30 ug/ml)

Eradicate acute infectionDetection, prevention, & treatmentrecurrent infectionDetection & surgical correctionabnormality of anatomical structureMANAGEMENTEradicate acute infectionOral antibiotics : Amoksisilin,Sulfonamid,sefalosporin Parenteral antibiotics: ceftriakson, sefotaksim, seftazidim, gentamisin AmikasinCONGENITAL ANATOMY OF URINARY TRACT

KIDNEY :AGENESIS : BILATERAL RENAL AGENESIS = Potters SyndromeOligohydramnionPulmonary hypoplasiaLow-set earsRENAL HYPOPLASIA : The kidney is small Normal nephron

Renal agenesis (Potters sequence)

Potter syndromeRenal dysplasia

RERenal dysplasia

Horseshoe kidney : Fusion of the renal parenchymaJoined at the lower polePolycystic kidney :Infantile Polycystic Kidney (IPCK)Adults Polycystic Kidney (APCK)

Horseshoe kidny

HORSHOE KIDNEYIDNEYHOPolycystic kidneys

Polycystic Kidneys

BLADDER (VESICA URINARIA)AgnesiaBladder neck obstruction

Agnesia / atresia urethraCongenital posterior urethral valves

URETHRAPUV

Posterior Urethral Valves(PUV)Prune Belly Syndrome

Prune Belly Syndrome

URETER

Duplication of ureterUreteroceleEctopic ureter

Ureteric duplication

Ureteric DuplicationPUJ obstruction

Pelvic Ureteral Junction Obstruction

VESICO URETERAL REFLUXReflux of urine from the bladder into ureterDamage the upper urinary tract by bacterialInfectionCauses : Congenital anomalous developmentof the ureterovesical junctionBladder outlet obstruction

I P C K

Autosomal Recessive Polycystic KidneyEnlargement of distal tubulus & colligents ductusGlomerulus & proximal tubulus normalLiver enlargement

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