pediatrics.Nephrotic syndrome.(dr.adnan hamawandi)


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pediatrics.Nephrotic syndrome.(dr.adnan hamawandi)

  1. 1. Nephrotic syndrome Dr. Adnan MH Hamawandi Professor of Pediatrics College of Medicine University of Sulaimany
  2. 2. Definition and Pathogenesis <ul><li>Nephrotic syndrome is characterized by Heavy proteinuria ( > 960 mg/m 2 /day). This leads to hypoalbuminemia , edema , and hyperlipidemia . </li></ul><ul><li>Nephrotic syndrome is not a single disease entity, it may accompany any glomerular disease or injury. It develops when the glomerular basement membrane show a marked prolonged increase in permeability to anionically charged plasma proteins, mainly Albumin the underlying pathogenesis is unknown, but evidence strongly supports the importance of immune mechanisms. </li></ul>
  3. 3. Idiopathic nephrotic syndrome Pathology <ul><li>Minimal change (MCNS) 85% of total NS. The light microscopy appear normal or show minimal increase in mesangeal cells and matrix. Immunofluorescence is negative, and electron microscopy reveal effacement of epithelial cell foot processes. More than 95% respond to corticosteroid therapy. </li></ul><ul><li>Mesangeal proliferative constitute about 5%, characterized by diffuse increase in mesangeal cells and matrix. Approximately 50% are steroid responsive. </li></ul><ul><li>Focal segmental glomerulosclerosis constitute 10%, the glomeruli show segmental scarring. Only 20% respond to steroids, and/or cytotoxic drugs. The disease is frequently progressive, ultimately involving all glomeruli and leads to end stage renal disease inmost patients. </li></ul>
  4. 4. Secondary Nephrotic syndrome <ul><li>With Glomerulonephritis - membranoproloiferative glomerulonephritis, membranous glomerulonephritis acute postinfectious glomerulonephritis </li></ul><ul><li>Infections - viral( AIDS, hepatitis B, CMV,EB virus), bacterial( SBE, shunt nephritis), parasitic( malaria) </li></ul><ul><li>Inflammatory - SLE, Henoch Shonlein purpura </li></ul><ul><li>Malignant - lymphoma </li></ul><ul><li>Metabolic - diabetes mellitus </li></ul><ul><li>Toxins - heavy metals </li></ul><ul><li>Drugs - captopril, penicillamine </li></ul><ul><li>Other disorders - sickle cell anemia, renal vein thrombosis, Hemolytic uremic syndrome </li></ul><ul><li>Congenital nephrotic - Fennish type. </li></ul>
  5. 5. Clinical manifestations <ul><li>More common in boys than in girls 2:1. </li></ul><ul><li>Common age group at diagnosis 2-6 years. </li></ul><ul><li>Edema is the usual presentation, which may be generalized and severe, starting in the face, gradually increase to involve the abdomen, scrotum, and lower limbs eventually resulting in ascitis, and even pleural effusion. Anorexia, abdominal pain, diarrhea, infection, and intravascular volume depletion may be present. </li></ul><ul><li>Blood pressure is usually normal, although there may be oliguria. </li></ul><ul><li>Minimal change disease usually has a relapsing course. Acute infections frequently trigger relapses. </li></ul>
  6. 6. Diagnosis <ul><li>Urinalysis shows +3 or +4 proteinuria. </li></ul><ul><li>24 hour urine excretion exceeds 960 mg./M 2 . </li></ul><ul><li>Serum cholesterol is elevated. </li></ul><ul><li>Serum Albumin is less than 2 gm./dL. </li></ul><ul><li>Blood urea and s. creatinine are usually normal, but they can be elevated in presence of oliguria. </li></ul><ul><li>C 3 complement is normal. </li></ul><ul><li>Microscopic hematuria may be present BUT gross hematuria is rare. </li></ul><ul><li>Full Response to a trial of steroid therapy makes a presumptive diagnosis of MCNS .( Response is defined as urine trace or negative for protein for three consecutive days). </li></ul>
  7. 7. Diagnosis – cont. <ul><li>Indication of Renal Biopsy: 1. Age <1 year> 8year. 2. Hypertension. 3. Raised s. creatinine. 4. Low C 3 serum level. 5. Persistent microscopic or gross hematuria. 6. No response to 6 weeks daily divided dose </li></ul><ul><li>of steroid therapy. </li></ul>
  8. 8. Complications <ul><li>Infection. Peritonitis is the most frequent type of infection. Pneumonia, Cellulitis, urinary tract infection and sepsis are also encountered. </li></ul><ul><li>Increased tendency to arterial and venous thrombosis. </li></ul><ul><li>Side effects of prolonged steroid therapy. </li></ul><ul><li>Hyperlipidemia. </li></ul>
  9. 9. Treatment <ul><li>Relief of edema . 1. salt restriction. 2. Fluid intake. 3. mild diuresis.4. Dietary protein. </li></ul><ul><li>Remission can be induced by Prednisolone </li></ul><ul><li>60mg/m 2 / day divided into 3 or 4 doses. The average time needed for response is about two weeks. </li></ul><ul><li>After the initial 6 weeks course, prednisolone is tapered to 40mg/m 2 /day given every other day as a single morning dose. The alternate day dose is then slowly tapered and discontinued over the next 2-3 months. </li></ul>
  10. 10. Treatment – cont. <ul><li>Steroid dependent and frequent relapsers may have prolonged remissions on alternative drugs like cyclophosphamide, cyclosporin A, tacrolmus and mycophenolate </li></ul><ul><li>Angiotensin converting enzyme inhibitors and angiotensin II blockers may be helpful as adjunct therapy to reduce proteinuria in steroid-resistant cases. </li></ul><ul><li>Polyvalent Pneumococcal vaccine should be given idealy while in Remission and not taking steroids. </li></ul>