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Nephrotic syndrome in children

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Nephrotic Syndrome in Children

Nephrotic Syndrome in Children

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  • 1. Nephrotic Syndrome Child Health II Speaker : Shriyans jain
  • 2. Definition • Manifestation of glomerular disease, characterized by nephrotic range proteinuria and a triad of clinical findings associated with large urinary losses of protein : hypoalbuminaemia , edema and hyperlipidemia - Nelson Textbook of Paediatrics, Vol 2, 19th Edition, page 1801
  • 3. Why ‘nephrotic range’ • Defined as – protein excretion of > 40 mg/m2/hr – First morning protein : creatinine ratio of > 2-3 : 1 Other causes of proteinuria - Nelson Textbook of Paediatrics, Vol 2, 19th Edition, page 1801
  • 4. Incidence ( paediatric ) ? • 2 – 7 cases per 100,000 children per year • Higher in underdeveloped countries ( South east Asia ) • Occurs at all ages but is most prevalent in children between the ages 1.5-6 years. • It affects more boys than girls, 2:1 ratio http://www.kidney.org/site/107/pdf/NephroticSyndrome.pdf
  • 5. Etiology • Genetic • Secondary • Idiopathic or Primary
  • 6. Genetic causes • Finnish type Congenital Nephrotic Syndrome • Focal Segmental Glomerulosclerosis • Diffuse Mesangial Sclerosis • Denys-Drash Syndrome • Nail – Patella Syndrome • Alport Syndrome • Charcot-Marie-tooth disease • Cockayne syndrome • Laurence-Moon-Beidl-Bardet Syndrome • Galloway-Mowat Syndrome - Nelson Textbook of Paediatrics, Vol 2, 19th edition, page 1802, table 521-1
  • 7. Secondary causes • Congenital – Oligomeganephronia • Infectious – Hepatitis (B,C) , HIV-1, Malaria, Syphilis, Toxoplasmosis • Inflammatory – Glomerulonephritis • Immunological – Castleman Disease, Kimura Disease, Bee sting, Food allergens • Neoplastic – Lymphoma, Leukemia • Traumatic ( Drug induced ) – Penicillamine, Gold, NSAIDS, Pamidronate, Mercury, Lithium - Nelson Textbook of Paediatrics, Vol 2,19th edition, page 1802, table 521-1
  • 8. Idiopathic • Minimal Change disease ( >80 % ) • Mesangial proliferation • Focal segmental Glomerulosclerosis • Membranous Nephropathy • Membranoproliferative glomerulonephritis - Nelson Textbook of Paediatrics, Vol 2, 19th Edition, page 1804
  • 9. Pathophysiology
  • 10. http://www.highlands.edu/academics/divisions/scipe/biology/faculty/harnden/2122/images/renalcorpuscle.jpg
  • 11. Complex disturbances in immune system Genetic Mutations / Mutations in proteins Extensive effacement of podocyte foot processes Increased permeability of the glomerular capillary wall Massive proteinuria Hypoalbuminaemia Edema
  • 12. Clinical Features
  • 13. • Edema – Mild to start with – peri orbital puffiness, lower extremities – Progression to generalized edema, ascites, pleural effusion, genital edema • Decreased urine output • Anorexia, Irritability, Abdominal pain and diarrhoea • Absence of – Hypertension – Gross hematuria - Nelson Textbook of Paediatrics, Vol 2, 19th Edition, page 1802
  • 14. CLINICAL FEATURES Minimal Change Nephrotic Syndrome Focal Segmental Glomerulosclerosis Membranous Nephropathy Age ( yr ) 2 - 6 2 - 10 40 - 50 Sex ( M : F ) 2 : 1 1.3 : 1 2 : 1 Nephrotic Syndrome 100 % 90 % 80 % Asymptomatic proteinuria 0 10 % 20 % Hematuria 10 – 20 % 60 – 80 % 60 % Hypertension 10 % 20 % early infrequent Rate of progression to renal failure Non progressive 10 yrs 50 % in 10 – 20 yrs Associated Conditions Usually none None Renal vein thrombosis, SLE, Hepatitis B - Nelson Textbook of Paediatrics, Vol 2 : page 1803, table 521-2
  • 15. DIFFERENTIALS • Protein losing enteropathy • Hepatic failure • Heart failure • Acute/Chronic Glomerulonephritis • Protein Malnutrition • < 1 year old • Family history of nephrotic Syndrome • Hypertension • Pulmonary edema • Gross hematuria • Extrarenal findings
  • 16. Lab Investigations • Urine Examination • Complete Blood Count & Blood picture • Renal parameters : – Spot Urine Protein : Creatinine ratio – Urinary protein excretion – protein selectivity ratio • Liver Function Test • Renal Biopsy ???
  • 17. • Urinalysis - 3+ to 4+ proteinuria • Renal Function –Spot UPC ratio > 2.0 –UPE > 40 mg/m2/hr • Serum Creatinine – normal or elevated • Serum albumin - < 2.5 gm/dl • Serum Cholesterol/ TGA levels – elevated • Serum Complement levels – Normal or low - Nelson Textbook of Paediatrics, Vol 2, 19th Edition, page 1804
  • 18. Additional Tests • C3 and antistreptolysin O • Chest X ray and tuberculin test • ANA • Hepatitis B surface antigen Ghai Essential Paediatrics,8th edition, page 478 Indications for Biopsy • Age below 12 months • Gross or persistent microscopic hematuria • Low blood C3 • Hypertension • Impaired renal Function • Failure of steroid therapy
  • 19. Idiopathic Lab Findings Minimal Change Nephrotic Syndrome Raised BUN in 15 – 30 % Highly Selective proteinuria Focal Segmental Glomerulosclerosis Raised BUN in 20 – 40 % Membranous Nephropathy Membranoproliferative Glomerulonephritis Type I Low C1, C4 , C3 – C9 Type II Normal C1, C4 , Low C3 – C9 - Nelson Textbook of Paediatrics, Vol 2 : page 1803, table 521-2
  • 20. Cause Light microscopy Immunoflorescence Electron Microscopy Minimal Change Nephrotic Syndrome Normal Negative Foot process fusion Focal Segmental Glomerulosclerosis Focal sclerotic lesions IgM, C3 in lesions Foot process fusion Membranous Nephropathy Thickened GBM Fine Granular IgG Sub epithelial deposits Membranoprolifer ative Glomerulonephriti s Type I Thickened GBM, proliferation Granular IgG, C3 Mesangial and subendothelial deposits Type II Lobulation C3 only Dense deposits - Nelson Textbook of Paediatrics, Vol 2 : page 1803, table 521-2
  • 21. Management
  • 22. Initial Episode • High protein diet • Salt moderation • Treatment of infections • If significant edema – diuretics Aldosterone antagonist ( Fursemide, spironolactone ) • Corticosteroid therapy with Prednisolone or prednisone – ( 2mg/kg per day for 6 weeks followed by 1.5 mg/kg single morning dose on alternate days for 6 weeks ) Ghai Essential Paediatrics,8th edition, page 476, 477
  • 23. Subsequent course • Relapse – Infrequent Relapsers : 3 or less relapses per year – Frequent Relapsers : 4 or more relapses per year • Steroid therapy – Steroid dependant : relapse following dose reduction or discontinuation – Steroid resistant : Partial or no response to initial treatment Ghai Essential Paediatrics,8th edition, page 479
  • 24. Management of Relapse • Parent Education • Symptomatic therapy for infections in case of low grade proteinuria • Persistent proteinuria ( 3 - 4+ ) – – Prednisolone ( 2mg/kg/day until protein is negative for 3 days ) 1.5 mg/kg on alternate days for 4 weeks ) Ghai Essential Paediatrics,8th edition, page 479
  • 25. Frequent Relapses • Alternate Day prednisolone • Steroid sparing agents – Levamisole ( 2 – 2.5 mg/kg ) – Cyclophosphamide ( 2 – 2.5 mg/kg/day) – Mycophenolate Mofetil ( 20 – 25 mg/kg/day ) – Cyclosporin ( 4 – 5 mg/kg/day ) – Tacrolimus (0.1 – 0.2 mg/kg/day ) – Rituximab ( 375mg/m2 IV once a week ) Ghai Essential Paediatrics,8th edition, page 479, 480
  • 26. Complications • Edema • Infections • Thrombotic complications • Hypovolaemia and Acute renal Failure • Steroid Toxicity Ghai Essential Paediatrics,8th edition, page 480, 481
  • 27. Steroid Resistant Nephrotic Syndrome • Diagnosis – Lack of response to prednisolone therapy for 4 weeks • Indication for renal biopsy , BBVS • Etiology – 10 – 20 % - Genetic ( Mutations in genes encoding podocyte proteins ) • Indications for mutational analysis : – Congenital Nephrotic Syndrome – Family History of SRNS – Sporadic resistance to steroids – Girls with steroid resistant FSGS Ghai Essential Paediatrics,8th edition, page 481
  • 28. Management of SRNS • Steroids + calcineurin inhibitors + ACE inhibitors / ARBs’ + HMG coenzyme-A + Diuretics Ghai Essential Paediatrics,8th edition, page 481, 482
  • 29. Prognosis • Steroid Responsive NS : Good prognosis ( MCNS ) • Steroid Resistant NS : Poor prognosis ( FSGS ) - Nelson Textbook of Paediatrics, Vol 2, 19th Edition, page 1806
  • 30. Congenital Nephrotic Syndrome • Presents in first 3 months of life • Anasarca, hypoalbuminaemia, oliguria ‘Finnish’ Type Nephrotic Syndrome • Antenatally detectable : – Raised AFP in maternal serum and amniotic fluid • Complications – Failure o thrive – Infections – Hypothyroidism – Renal Failure ( 2 – 3 yrs ) Ghai Essential Paediatrics,8th edition, page 482

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