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Nephrotic & Nephritic Syndromes in Children.pptx
- 1. NEPHROTIC & NEPHRITICSYNDROME IBRAHIM SANDOKJI, MD, FAAP Pediatric Nephrologist, Assistant Professor Board Certified by the American Board of Pediatrics Taibah University isandokji@taibahu.edu.sa +966 50 632 5770
- 2. Ali isa previously-healthy 5-year-old boy who presents with puffy face and legs for the last three days. Lab Normal Hemoglobin 152 g/L 115–155 g/L White cell count 11.7 × 109/L 4.0–11.0 × 109/L Platelets 472 × 109/L 150–400 × 109/L Sodium 132 mmol/L 135–145 mmol/L Potassium 4.9 mmol/L 3.5–5.0 mmol/L Urea 6.3 mmol/L 1.8–6.4 mmol/L Creatinine 80 μmol/L 44–88 μmol/L Albumin 15 g/L 37–50 g/L Urine dipstick Blood Protein Leucocytes Nitrites 1+ 4+ - - Hemoconcentration Hypoalbuminemia Proteinuria
- 4. Nephrotic syndrome criteria ProteinuriaHypoalbuminemia Edema Hyperlipidemia
- 5. Remember Hallmark of nephroticsyndrome is Leakage of proteins in urine Remember Hallmark of nephritic syndrome is Inflammation
- 6. Most common causeof NS in children Most common cause of NS in adults
- 7. Clinical Manifestations Edema First → periorbital edema → generalized and massive (anasarca) Shortness of breath Abdominal pain? (be aware of SBP) Elevated blood pressure? Hematuria?
- 8. INVESTIGATIONS Urine: Urinemicroscopy First morning void to measure urinary protein to creatinine ratio Blood tests Electrolytes, creatinine, blood urea nitrogen, cholesterol, albumin Cholesterol and triglyceride levels → elevated in nephrotic syndrome C3/C4 → to investigate other glomerular diseases Antinuclear antibody (ANA) → vasculitides (e.g. SLE) A purified protein derivative (PPD) skin test (risk of TB flare with therapy)
- 9. COMPLICATIONS • Urinary lossof complements and immunoglobulins, T-cell dysfunction and immunosuppressive therapy Life-threatening infections • Urinary loss of AT3, Protein S and plasminogen, increased hepatic synthesis of procoagulant factors, hemoconcentration due to volume depletion and diuretic use Thromboembolism • Hypoalbuminemia and low oncotic pressure trigger hepatic lipoprotein synthesis Hyperlipidemia • Dehydration, medication nephrotoxicity and renal vein thrombosis Acute kidney injury • Urinary loss of vitamin-D binding protein Vitamin-D deficiency • Urinary loss of iron, transferrin and erythropoietin Anemia • Urinary loss of proteins, malabsorption due to edema of the gut, and steroids side effects Growth impairment • Growth impairment, hypertension, acne, cataract, weight gain, etc. Steroid-related side effects
- 10. MINIMAL CHANGE DISEASE Most common cause of pediatric NS Typical features for MCD include Age 2-8 years Normal blood pressures Normal renal function Normal complements No gross hematuria No findings to suggest other etiologies
- 11. Electron microscopy Light microscopyImmunofluorescenc
- 12. MINIMAL CHANGE DISEASE Diffusefoot process effacement on electron microscopy Normal histology on light microscopy
- 13. TREATMENT Steroid therapy Mainstay treatment for nephrotic syndrome in children Most patients respond well Some patients respond well to steroids initially, then become Steroid-dependent or frequently-relapsing Steroid-resistant These patients need further investigations and different immunosuppressive agents
- 14. Do you needa kidney biopsy before starting therapy in children? Answer: No Kidney biopsy is only indicated if Presented in ages <1 or >10 years Atypical clinical or laboratory features No appropriate response to steroid therapy Remember the typical features for MCD Age 2-8 years Normal blood pressures Normal renal function Normal complements No gross hematuria No findings to suggest other etiologies
- 15. MEMBRANOUS NEPHROPATHY Most commoncause of NS in adults Primary vs secondary (hepatitis B/C, HIV, malaria) Diffuse thickening of the basement membrane & immune complex deposits Treated with steroid + immunosuppressive agent(s)
- 16. FOCAL SEGMENTAL GLOMERULO- SCLEROSIS (FSGS) Focal =some glomeruli are affected Segmental = only some sections of the glomeruli are affected It is a histological diagnosis Usually idiopathic (primary) Can be secondary Hyperfiltration e.g. obesity Infections e.g. HIV Usually progress to ESRD Usually does not respond to steroids
- 17.
- 18. Salih isa previously-healthy 5-year-old boy who presents with puffy face and red urine for three days. His blood pressure is 125/85. Lab Normal Hemoglobin 140 g/L 115–155 g/L White cell count 11 × 109/L 4.0–11.0 × 109/L Platelets 390 × 109/L 150–400 × 109/L Sodium 134 mmol/L 135–145 mmol/L Potassium 4.8 mmol/L 3.5–5.0 mmol/L Urea 8.2 mmol/L 1.8–6.4 mmol/L Creatinine 120 μmol/L 44–88 μmol/L Albumin 40 g/L 37–50 g/L Urine dipstick Blood Protein Leucocytes Nitrites 4+ 4+ +1 - Elevated BUN/Cr Hematuria Proteinuria Hypertension
- 19. Remember Hallmark of nephroticsyndrome is Leakage of proteins in urine Remember Hallmark of nephritic syndrome is Inflammation
- 20. Nephritic syndrome (glomerulonephritis) criteria HematuriaProteinuria Edema Hypertension Glomerular hematuria
- 21. CAUSES OF GLOMERULONEPHRITISIN CHILDREN Primary glomerulonephritis IgA nephropathy* Membranous nephropathy Membranoproliferative glomerulonephritis Secondary glomerulonephritis Post-infectious glomerulonephritis* HSP nephritis Systemic lupus erythematosus nephritis *See the other lecture
- 22. HSP NEPHRITIS IgA-containingimmune complexes Typically occurs after systemic symptoms Glomerular hematuria and proteinuria Treatment: Mild → supportive Severe or persistent → steroids and/or immunosuppressive agents
- 23. SLE NEPHRITIS Variablepresentation Nephritic syndrome Nephrotic syndrome AKI or CKD Must biopsy Treatment is based on histological classification (not clinical presentation)
- 24. MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS (MPGN) Manifestwith gross hematuria, proteinuria, and low complement levels Can be mistaken for PIGN Both have low complements MPGN is usually a chronic condition that relapse and remit over time PIGN usually occurs after infections, resolve spontaneously, and rarely recur To definitely differentiate the two conditions, you need a kidney biopsy
- 25. RAPIDLY PROGRESSIVE GN Acute nephriticsyndrome with a progressive loss of kidney function over short period of time (days) Early diagnosis with kidney biopsy (crescent formation) Early initiation of therapy (pulse steroids + immunosuppression) Essential to minimize irreversible kidney injury
- 26. RPGN Immune Complex- mediated Crescentic GN PIGN HSP SLE Pauciimmune CrescenticGN ANCA-associated vasculitis (AAV) Microscopic polyangiitis pANCA (against MPO) Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) pANCA (against MPO) Granulomatosis with polyangiitis (Wegener granulomatosis) cANCA (against PR3) Anti-GBM antibody disease
- 27.
- 28. https://www.osmosis.org/learn/ High_Yield:_Nephritic_syndrome s https://www.osmosis.org/learn/ High_Yield:_Nephrotic_syndrome s Remember Hallmark of nephroticsyndrome is Leakage of proteins in urine Remember Hallmark of nephritic syndrome is Inflammation
- 30. HYPERTENSION IN CHILDREN Guidelines for normal blood pressures (BPs) in children are different from adults Normal BPs for children <13 y are based on gender and height. There are tables for normal BP percentiles. Normal BPs for children >13 y are similar to adults. Blood pressure cuffs are different for age and size of children. Hypertension in young children is more likely to be secondary (e.g. from renal, cardiac or endocrine cause) than adults. In adults, it is more likely to be primary hypertension. and adults