K12.3-GNA_RDS_333

Kuliah Senin Tgl 08-09-2014Pkl 07.30 09.20 WibPOKOK BAHASAN GlomerulopathySUB POKOK BHN GNA dan GNK Akut < dari 1 bln Kronik > dari 1 bln

Glomerulonephritis in Children Acute Post Streptococcal Glomerulonephritis (AGNPS /GNAPS, terbanyak ditemukan) Henoch Schnlein Purpura (HSP) Other types of glomerulonephritis 2.

Characteristic features (Gambaran klinis yg Karakteristik) Sudden onset of macroscopic haematuriaOedemaOliguriaHypertension

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PRIMER GLOMERULUS YG DIKENAI DIIKUTI GANGGUAN SISTEMIK ( RINGAN BERAT ) KLINIS DAN LAB (1-10)1/10. OLIGURIA ( 240 ML / M2 / 24 JAM )- BATAS MINIMAL PENGELUARAN SOLUTE / HASIL METABOLISME: BILA LEBIH OLIGURIC R.F 4.

2/10. EDEMA SEDIKIT ( JARANG SEPERTI NS )- PL. COLLOID OSMOTIC PRESS. TIDAK - PL. PROTEIN LEVEL BIASA NORMAL3/10. HIPERTENSI ( SERING )- DISANGKA: RENIN & VASOKONSTRIKSI PERIFER - PENTING: RETENSI AIR DAN NATRIUMKLINIS DAN LAB (1-10)........ 5.

4/10. CIRCULATORY CONGESTION ( BISA ADA ) - PULMONARY EDEMA- CARDIAC OVERLOAD / Decomp.Cordis = DC- HEPATOMEGALY- DISTENSI V. JUGULARIS EXT.- GALLOP RYTHME

5/10. HEMATURIA: GROSS HEMATURIA, DARK, RED, BROWNISH URINE, TEA COLORED URINE, COCA COLA URINE , Cucian Daging) - SEDIMENT URINE: tanda khas (patognomonik)RBC (+++)GRANULAR CAST +RBC CAST +MIXED 6.

RBC CAST IN URINE 7.

6/10. PROTEINURIA30 - 100 MG / DL 1000 MG / DLATAU > NEPHROTIC LEVEL7/10. ANEMIABIASANYA NORMOCHROMICHB 9 - 11 MG/DL OK HEMODILUSI

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8/10. AZOTEMIA AKIBAT GFR BUN , SERUM CREATININE , SERUM PHOS PHATE ( + / - ) SERUM URIC ACID ( + / - ) PLASMA Ca++ LEVEL SERUM PHOSPHATE 9/10. ELECTROLYTE & ACID BASE TERGANGGU HIPERKALEMIA, HIPONATREMIA ASIDOSIS DIPERKUAT OLEH HIPERKALEMIA 9.

PATHOLOGIC FINDINGS TERGANTUNG DARI SPECIFIC DISEASES UMUMNYA SAMA:- ADA INFILTRASI PMN LEUKOSIT- PROLIF. 1 LEBIH GLOMERULAR CELL TYPE: 1. ENDOTHELIAL2. MESANGIAL3. EPITHELIAL

- GLOMERULAR SIZE - MESANGIAL EDEMA MESANGIAL MATRIX (FINE FIBRILAR TYPE) JLH CAPILLARY LOOP YG TERBUKA BER(- )- PADA INTERSTITIAL BISA INFILTR. PMN MN.I1 2 3PMN10.

Ag-Ab Complex Trapped in endothel - Glom. Capillaries [Epithel, Mesangial] Endothel Edema. & Prolif ,+ Fibrin Occluded Olig and GFR decreased.Infiltration of PMN Proteolytic Enzyms PMN disrupt of GBM Hemat. & .Plasma constituent. Urine11.Pembentukan Ag-Ab Complex mengaktifkan system complemen (C1-C3-C2-C4-C5-C6-C7=C8=C0 lysis) Pada AGNPS C3 banyak dipakai C3 dlm darah Turun 10/10

Etiology Develops after latent period of 14-21 days after (Gejala muncul ssd Laten period 14-21 hari inf.Strep) group A Hemolytic Streptococcus infectionSpecific serotypes are associated with APSGN [Acute Post Streptoc.GN] Type 12: pharingitisType 49: skin infection (impetigo)

12.Makin Banyak C3 dipakai, untuk Ag-Ab Complex , Makin rendah kadarnya dalam darah

13.Ada Edema & Bercak perdarahan

Clinical features / Gej.klinis

Most commonly seen in child 4 to 10 years old Develops after latent period after pharingitis or impetigo Sudden onset of facial or generalized oedema Hypertension : headache, convulsions, disturbed vision (penglihatan) Acute left heart failure / pleural effusions

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Causes of Acute GlomerulonephritisInfectionsBacteria: Streptococci, pneumococci, staphylococci, Treponema pallidum, Salmonella typhiViruses: Hepatitis B, Echovirus, Ebstein B virus, HIV.Protozoa: MalariaCollagen-vascular disease: Vasculitis of Small VesselsHenoch Schnlein Purpura, SLE,Genetic Alports syndrome DrugsMethicillin15.Terbanyak, Kejang, Sesak, DCSakit perut hebat, Kencing darah / berak darah Bercak2 darah pd kaki Jumlah Trombosit N.

Incidence of APSGN Unknown due to sub-clinical presentation in some Linked to poor socio-economic conditions Epidemics occur in developing countries

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Pathogenesis of PSGNTwo theories how immunologic events lead to renal damage Antibodies elicited by nephritogenic streptococci cross react with renal antigens leading to antibody mediated glomerular damage

Immune complexes consisting of Streptococ antigen and antibodies are trapped in the glomeruli. The complement cascade is activated resulting in increased glomerular celluarity 17.0AbAg-Ab CompTrapped

Ada 4 Lapisan GAS. 1. Capsule (Hyaluronic acid) 2. Cel wall ( M - protein and M Asosiated protein ) 3. Group Carbohydrate (N-Acethyl Glucisamine Ribosome) 4. Protoplast membrane (Protein, Lipid and Glucose)1.2.3.4. Group A Streptococcus, Cardionegenic str. = [GABHS]Structur Antigen GAS sama dgn structur dari sel-sel: 1. Sendi (synovial membrane) 2. Sel-sel myocardium 3. Sel-sel Valvula / katup jantung 4. Sarcolema myocardium dan Sarcolema dari Subthalmic brain dan Nucleus caudatus Dr.Babu UthmanDone by: Samya : RF and RHD 2007.1.18.

GABHS Cardiogenik Rheumatic fever / RHD - pathogenesisJones Criteria Mayor CAPOCHES 19.

Pathogenesis of PSGN

Acute inflammaory response in the glomeruli attract polymorphonuclear leucocytesEndothelial cells swell, fibrin is deposited, and the lumen of capillaries are thereby occludedRelease of proteolytic enzymes from polymorphs disrupts the integrity of the glomerular basement membrane and allows the excessive escape of blood cells and plasma constituents in the urine Leads to decreased GFR and oliguria20.

Special investigations Urine (Oliguria)Macroscopic appearance : grey,cloudy, coke colour urine, tea colored urine Dipstix : 3+ haematuria ; 1-2 + proteinuria . Azotemia ( Ureum & Creatnin darah naik). Ada hiprtensi) Microscopy: Red blood cells, white blood cells, red cell and granular casts

21.Dlm buku ajar nefranak PHAROH

Glomerular versus extraglomerular bleeding

Urinary finding GlomerularExtraglomerlarRed cell castsMay be presentAbsent

Red cell morphologyDysmorphicUniformProteinutiaMay be presentAbsent

ClotsAbsentMay be presentColorMay be red or brown May be red

Investigations S-Urea, creatinine and electrolytes Usually mildly urea and creatinine Determination of Streptococcus serology: After throat infection ASTO titreAfter skin infection Anti-DNase B or Anti-hyaluronidase Complement levels: C3 ECG to monitor effects of hyperkalaemia Chest X-ray may show cardiomegaly / pleural effusions22.

Treatment of APSGNAdmit to hospital if acutely ill with oliguria, fluid overload or hypertensionBed rest for acute hypertension, left heart failure or CNS symptoms Oral Pen VK for 7-10 days Monitor fluid balance: weigh daily fluid intake and output Regular blood press. monitoring and daily urine testing.Monitor BP and treat hypertension - furosemide

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Treatment of APSGNIf oliguric restrict salt and water intake Monitor renal function : restrict potassium intakeIf acute renal failure develops refer for dialysisAcute peritoneal dialysis is indicated for Severe fluid overload and left heart failureHyperkalemia that does not respond to other measuresAnuria 24.

Indications for referralMixed nephritic-nephrotic presentationDelayed recovery- including persistent Hypertension (> 4 weeks) Macroscopic haematuria (> 4 weeks) Hypocomplimentaemia ( > 8 weeks) Family history of renal disease.26.

THANK YOUTERIMA KASIH37.

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