acute nephritic syndrome piip 8 11 09

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Acute Nephritis in children-Is it always benign? : 

Acute Nephritis in children-Is it always benign? Susan Uthup MD DCH, DNB (Paed), D M , DNB (Nephro) Assoc. Professor of Paediatric Nephrology SAT Hospital Govt. Medical College Trivandrum

Introduction : 

Introduction Definition Aetio pathogenesis Clinical Features Management Case Scenarios Discussion Conclusions

Slide 5: 

Richard Bright 1927 Acute glomerulonephritis Abrupt onset Hematuria Oliguria Oedema Salt and water retention Hypertension Reduced GFR Prototype -Post infectious (eg. GABS)

The ‘Nephritic Syndrome ’ : 

The ‘Nephritic Syndrome ’ Acute, self limiting, reversible condition Post Infectious – Epidemic / Sporadic First presentation of chronic disease IgA nephropathy Membranoproliferative glomerulonephritis (MPGN) Alport’s Syndrome FSGS Systemic Diseases SLE and other chronic vasculitis

Acute Post infectious glomerulonephritis : 

Acute Post infectious glomerulonephritis Latent period : 2-3 weeks Other infections ……………. Histologic pattern and clinical syndrome similar Deposition of immune complexes and complement in glomerular capillary bed

Nephritogenic strains : 

Nephritogenic strains M types 1,2, 4,12, 18, 25, 49, 55, 57, 60 Proposed mechanisms : Molecular mimicry Autoimmune mechanism Polyclonal activation of B lymphocytes Immune Complex mediated (insitu /in circulation) Antigen load, Charge, Size

Effects of glomerular injury : 

Effects of glomerular injury Endothelium and subendothelial aspect of GBM Recruitment of leukocytes ----inflammatory injury, Hematuria, proteinuria Mesangium --Asymptomatic abnormalities of urinary sediment ; mild renal insufficiency Parietal epithelial cells crescent formation (acute or subacute renal failure)

Nature of injury : 

Nature of injury Rapid, extensive, immune complex deposition (poststreptococcal glomerulonephritis) Complement activation Leukocyte recruitment Lysosomal enzyme release Free radical generation Purturbation of vascular tone & permeability “Acute diffuse proliferative GN” Acute renal failure

Nature of injury : 

Nature of injury Slow , but sustained IgA-immune complex formation (IgA nephropathy) Less active inflammation Relatively well maintained GFR or Slowly progressive renal insufficiency over decades

Post streptococcal GNClinical Features : 

Post streptococcal GNClinical Features Mild disease usual Classically, overt nephritic syndrome . oliguric ARF Gross hematuria (100% microscopic) Edema (80-90%) Hypertension (60-80%) Nephritic urinary sediment dysmorphic RBCs, red cell casts, leukocytes Proteinuria - Subnephrotic range Nephrotic-range proteinuria- Uncommon Full recovery typically within weeks

INVESTIGATIONS : 

INVESTIGATIONS Urinalysis Serum Creatinine C3 decreased,C4 normal (complement level usually normal within 6-8 weeks) ASO, anti-DNAse B USS abdomen

Slide 14: 

Proliferative GN Post infective PSGN- granular bumpy pattern of immune deposits on immunofluorescence

Immunofluorescence Microscopy : 

Immunofluorescence Microscopy Deposition of IgG and C3

Electron Microscopy : 

Electron Microscopy Large electron – dense immune deposits subendothelial subepithelial mesangial

Treatment : 

Treatment Restriction of salt Restriction of Fluid intake Control of Hypertension with diuretics and anti hypertensives Dialysis Anuric renal failure Symptomatic Azotemia Diuretic resistant pulmonary oedema Intractable Acidosis Severe hyperkalemia

Indications for referral : 

Indications for referral Onset- Anuric renal failure,RPRF Mixed nephritic-nephrotic presentation Delayed recovery- including persistent Hypertension (> 4 weeks) Macroscopic hematuria (> 4 weeks) Hypocomplimentaemia ( > 8 weeks) Growth Failure Family history of renal disease .

Slide 19: 

Acute Glomerulo Nephritis Case Scenarios

Case 1 : 

Case 1 5 yr Girl Sudden onset Facial puffiness Oliguria & Cola colored urine Generalized oedema Headache Preceeding infected skin lesion following trauma No h/o skin rash, arthritis, bleeding manifestations or use of drugs Physical Examination - Generalized Oedema, Hypertension Growth Normal

On Evaluation : 

On Evaluation Urine coke colored 1-2 + proteinuria Red blood cells, white blood cells, red cell and granular casts Haemogram & Peripheral smear normal B. Urea 68-102-145mgm/dl S. Creatinine 0.8—1.6—3-4 mgm/dl S.Sodium / Potassium 128/5.8—132/6.4meq/L ASO Positive Serum Complement C3 – 0.3 mgm/dl (0.9- 1.2 mgm/dl) Sonography : Bulky kidneys with increased Echoes

Slide 23: 

RBC Cast

Acute Nephritic Syndrome - Possibilities : 

Acute Nephritic Syndrome - Possibilities Acute Post Streptococcal Glomerulonephritis Primary glomerulonephritis – IgAN, FSGS Henoch Schönlein Purpura Rapidly progressive glomerulonephritis Vasculitis like SLE HUS Acute interstitial Nephritis

Slide 26: 

Diffuse Endocapillary Exudative Proliferative GlomeruloNephritis

Rapidly progressive glomerulonephritis (RPGN) : 

Rapidly progressive glomerulonephritis (RPGN) crescemt = accumulation and proliferation of extracapillary cells. The glomerular capillaries collapse and are bloodless, and fibrin can be identified within the capsule  it can stimulate proliferation of parietal epithelial cells  deposits of fibrin compress the glomerular capillary tuft ( GFR and destruction of glomerulus)

Slide 28: 

crescent

Types of Crescentic GN : 

Types of Crescentic GN Type 1: Anti-GBM antibody disease Goodpasture’s syndrome Type 2: Immune-complex Lupus nephritis Post-infectious Mixed cryoglobulinemia IgA Type 3: Pauci-immune RPGN Anti-neutrophil cytoplasmic antibodies (ANCA) Wegner’s granulomatosis(cANCA); MPA; renal limited vasculitis(pANCA)

Case 2 : 

Case 2 14 year-old boy Intermittent hematuria x 1 year Intermittent proteinuria x 1 year History of recurrent pharyngitis Physical examination : Normotensive & Euvolemic

Is this Glomerular Bleeding? : 

Is this Glomerular Bleeding? Urinalysis: 3 + protein , RBC/HPF, Rare RBC cast Many hyaline casts Urine Culture sterile Urine Spot PC ratio 1.8 Hb 11 gms, TC 7800 P42 L52 E6; ESR -12 mm/hr Blood urea-52 mgm/dl. S.Creatinine-1 mgm/dl Ultrasound abdomen: Normal Renal size, echogenicity CMD maintained

Slide 33: 

Serum Complement C3 -- - Normal If C3 were reduced ?? Possibilities !! Renal biopsy is indicated to make a histological diagnosis

What are the possibilities? : 

What are the possibilities? IgA Nephropathy Henoch Schönlein Purpura (“allergic vasculitis”) Alport Syndrome Thin Basement Membrane Disease Focal segmental glomerulosclerosis Unlikely: Membrano proliferative glomerulonephritis Vasculitis-- Lupus Nephritis

IgA nephropathy (Bergers disease) : 

IgA nephropathy (Bergers disease) Etiology: - unknown Syn Pharyngitic Nephritis -latent period 2-3 days association with HLA (DQ, DP) Mesangioproliferative GN with deposits of IgA, C3 T-lymphocytes produce  levels of IL-2 (+  IR-2R) They constantly stimulate production of IgA by B-lymphocytes

Hereditary nephropathies : 

Hereditary nephropathies Alport syndrome Hereditary nephritis with deafness (X chromosome) Pathogenesis: congenital defect of collag synthesis  GMB very slight or with more layers GN focal (diffuse) proliferation with segmental sclerosis  hematuria, proteinuria or renal failure (males)

Case 3 : 

Case 3 15 yr girl April 05 - Hematuria,Oedema,hypertension Bilateral Knee Arthritis,Palpable purpura October 05 – Recurrence of hematuria,Rash March 07 - Persistent Proteinuria& microscopic hematuria Growth normal Euvolemic BP 140/90 mm Hg

Investigations : 

Investigations Hb 12 gm;TC 6000 P46L53 E1 PLT 3.5 Lakhs ESR 35mm/hr Urine 3 + Protein, Spot PC Ratio:2.2 RBC plenty Pus Cells 20-40/hpf B.urea 27 gm/dl S.Cr 0.6 mgm/dl C3 Complement-150 mgm/dl Ultrasound abdomen: Normal Renal size, increased echogenicity

What are the possibilities? : 

What are the possibilities? Henoch Schönlein Purpura IgA Nephropathy Alport Syndrome Thin Basement Membrane Disease Focal segmental glomerulosclerosis Unlikely: Membrano proliferative glomerulonephritis Vasculitis-- Lupus Nephritis

Henoch Schönlein Purpura systemic vasculitis affecting medium-sized vessels : 

Henoch Schönlein Purpura systemic vasculitis affecting medium-sized vessels Abdominal pain Maculo-papular purpuric rash Melena / intususception Arthralgia/ Arthritis Glomerulonephritis in 30% (similar to IgA N) Clinical diagnosis No specific diagnostic test

Case 4 : 

Case 4 15 yrs girl 2006 - Facial puffiness, Oliguria Hematuria & renal Failure C3 Complement- 80 mgm/dl Treated as AGN 2007- Recurrence of hematuria Renal failure Asymptomatic in between BP 110/70 mm Hg

Investigations : 

Investigations Hb 11 gm; TC 8000 P36 L63 E1 PLT 1.5 Lakhs ESR 75mm/hr Urine 2 + Protein RBC plenty Pus Cells 30-40/hpf B.urea 71 gm/dl S.Cr 3.5 mgm/dl C3 Complement-30 mgm/dl Possibilites: MPGN, SLE----- Renal Biopsy Ultrasound abdomen: Normal Renal size increased echogenicity

Slide 45: 

Fibro cellular Crescents Class IV Lupus Nephritis With Chronic Changes

Lupus Nephritis : 

Lupus Nephritis Many different tissues and organs involved in Lupus Renal involvement - most significant in terms of outcome

Lupus NephritisWHO classification : 

Lupus NephritisWHO classification Minimal mesangial lupus nephritis (class I) Mesangioproliferative lupus nephritis(class II) Focal lupus nephritis (class III) Diffuse lupus nephritis (class IV) Membranous lupus nephritis (class V) Advanced sclerosing lupus nephritis (class VI)

Slide 48: 

Crescentic Glomerulonephritis

Case 6 : 

Case 6 10 yr old boy facial puffiness & Oliguria x 1 week Pedal edema Abdominal distension No macrohematuria. No rash/joint pain Previously well child Had pyoderma 3 weeks back No renal disease in family

Slide 50: 

Grade 1 PEM; 2º Stunting Generalised oedema Pyderma scar Pallor BP – 150/100 mmHg CVS/RS/CNS : NAD P/A : Ascites Physical Examination

Investigations : 

Investigations Hb 7.9 gm; TC:12,300; P84L16 ESR: 35mm/hr Urine: Alb 2+ RBC 40-50/hpf Urine c/s : sterile Urea : 96 mg/dl; S.Cr : 9 mg/dl Na: 129 meq/l; K: 6.2 meq/l ASO : 221U/L C3 : 0.27mgm/dl Ca: 9 mg/dl P:5.4 mg/dl ALP: 182 S.Cholesterol:210 mg/dl S.TP: 4.4gm/dl S.Alb: 2.2gm/dl SGOT/PT:30/15

HEMODIALYSIS via double lumen femoral cannula F3 dialyser : 

HEMODIALYSIS via double lumen femoral cannula F3 dialyser Child with HEMATURIA HYPERTENSION RENAL FAILURE HYPOCOMPLEMENTEMIA HYPERKALEMIA ANEMIA GROWTH FAILURE

Slide 53: 

RAPIDLY PROGRESSIVE GLOMERULO NEPHRITIS ACUTE POST INFECTIVE GLOMERULONEPHRITIS BUT……..

PROBLEMS : 

PROBLEMS ACUTE…? CHRONIC..? ACUTE ON CHRONIC…?

Features of Underlying CRF : 

Features of Underlying CRF GROWTH FAILURE ANEMIA HYPERPHOSPHATEMIA (Renal Osteodystrophy) Confirmation…………

USG ABDOMEN : 

USG ABDOMEN B/L HYPERECHOIC POOR CMD Rt 5.7x2.5; Lt 5.8x2.8 MULTIPLE CYSTS IN BOTH KIDNEYS 7-8mm in the cortex Acute on Chronic Kidney Failure

Course of PIGN : 

Course of PIGN Typically Full recovery within weeks Resolution quick. Plasma Cr usually returns to previous levels by 3-4 weeks Hematuria resolves usually within 3-6 months Irreversible Renal Failure rare – less than 1 % in children, slightly higher in adults Some patients experience hypertension , recurrent proteinuria, and renal insufficiency 10-40 yrs after More than 20% of adults have some degree of persistent proteinuria and or compromise of GFR at 1 year

Few Final Points : 

Few Final Points Correct and prompt diagnosis of AGN Detect underlying renal or systemic Diseases early Compliment the C3 always…… Distinguish acute from CKD Follow Up always indicated as recovery may reveal the story better

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