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Vinoedh Naidu @ nephrotic syndrome

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NEPHROTIC SYNDROME CLINICAL CASE STUDY

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Vinoedh Naidu @ nephrotic syndrome

  1. 1. clinical caSE STUDY PRESEnTaTiOn R.VINOEDH NAIDU @ PRP U41 HSB (2014/2015) PRECEPTOR : MISS JESSICA
  2. 2. OBJECTIVES : 1. Review –Epidemiology & histopathology –Signs and symptoms –Treatments 2. Discuss * Pharmacotherapy
  3. 3. SOCIAL HISTORY: No family history of known medical illness Eldest son and has 3 y.o brother REVIEW OF SYSTEM: BP : 114 /73 mmHg PR : 80 beats/min T : 37 ̊C O / E : Alert , conscious PATIENT DEMOGRAPHIC: Name : M.I.N Age : 5 y.o Gender : Male Race : Malay DOA : 25 /03/ 2015 DOD : 03 /04/ 2015 ALLERGY : NKMI / NKDA HISTORY OF PRESENTING ILLNESS: 1st incident of periorbital puffiness 3/12, Coughing and running nose 1/52 Bilateral periorbital swelling and facial puffiness 3/7 Usually happen after waking up in the morning and resolves in the evening. Father also noted child had bilateral pedal edema on admission day. Looks chubby than usual since day ago CHIEF COMPLAINT : Bilateral periorbital swelling Facial puffiness & mild pedal edema Referred from private GP
  4. 4. DIAGNOSIS: NEPHROTIC SYNDROME WITH SPONTANEOUS REMISSION
  5. 5. DEfiniTiOn • Manifestation of glomerular disease, characterized by nephrotic range proteinuria associated with large urinary losses of protein : hypoalbuminaemia , edema and hyperlipidemia - Nelson Textbook of Paediatrics, Vol 2, 19th Edition, page 1
  6. 6. EPiDEMiOlOGY • 2 – 7 cases per 100,000 children per year • Higher in underdeveloped countries • Occurs at all ages but is most prevalent in children between the ages 1-6 years. • It affects more boys than girls, 2:1 ratio http://www.kidney.org/site/107/pdf/NephroticSyndrome.pdf
  7. 7. ETiOlOGY • Genetic • Secondary • Idiopathic or Primary
  8. 8. Complex disturbances in immune system Genetic Mutations / Mutations in proteins Increased permeability of the glomerular capillary wall Massive proteinuria Hypoalbuminaemia Edema
  9. 9. DIAGNOSIS / SIGNS & SYMPTOMS • Edema (gut, Facial, pedal) • Proteinuria • Abdominal discomfort due to oedema • Bacterial peritonitis (pulmonary, cardiac) • Poor appetite
  10. 10. VITAL SIGNS NORMAL RANGE 17/3 18/3 19/3 20/3 21/3 22/3 25/3 31/3 1/4 2/4 3/4 BP <110/70 113/7 3 97/67 107/60 110/73 106/68 110/66 109/62 93/63 96/62 106/72 99/60 100/70 112/75 97/58 118/70 109/61 103/62 95/65 TEMP AFEBRILE RR 20-25 26 PR 76-106 104 94 75
  11. 11. REFERENCE RANGE CLINICAL VALUE 18/3/15 22/3/15 26/3/15 Hb 11.5-16.5g/100ml 14.9 14.4 WBC 4-11x 10/L 11.1 11.2 Platelet 150-410 X 10/L 409 401 T.Protein 66-87 g/L 61 Albumin 35-50 g/L 17 20 25 T. Bilirubin <20 umol/L 3 ALP 53-141 u/L 209 ALT <32u/L 15 Creatinine 64-122 umol/L 19 16 19 Blood Urea 1.7-8.3 mmol/L 4.6 4.0 5.1 Na 135-145 mmol/L 136 137 135 K 3.5-5.0 mmol/L 4.2 4.0 3.8 Cl 96-106 mmol/L 109 107 104 Cholestrol 11 12.4 Triglycerides 3.2 3.5 Urine protein 0.05 – 0.08 1.4 0.54 ASOT -VE C3 0.9-1.8 1.3 C4 0.1-0.4 0.27 PCI : 0.89
  12. 12. LAb INVeSTIGATIoNS • Urine Examination • Complete Blood Count & Blood picture • Renal parameters : – Urine Protein : Creatinine ratio / 24h urine protein – Urea & electrolytes • Liver Function Test – Albumin URINE DIPSTICK 18/3 19/3 20/3 21/3 22/3 23/3 27/3 28/3 1/4 2/4 3+ 2+ 2+ 1+ NIL 2+ 2+ - -
  13. 13. “When bubbles settle on the surface of the urine, it indicates disease of the kidneys and that the complaint will be protracted” Hippocrates
  14. 14. Additional TestsAdditional Tests • Antinuclear factor / anti-dsDNA* • C3 and C4 levels * • Antistreptolysin O (ASOT) * Ghai Essential Paediatrics,8th edition, page 478 Indications for BiopsyIndications for Biopsy • Age below 12 months • Gross or persistent microscopic hematuria • Hypertension • Impaired renal Function • Failure of steroid therapy *
  15. 15. Nutritional deficiencies - Kwashiorkor, brittle hair and nails, alopecia, stunted growth, demineralization of bone Spontaneous peritonitis may occur and opportunistic infections are prevalent. Hypertension with cardiac and cerebral complications in patients with diabetes or collagen vascular disease. Hypovolemia - oliguria, abd pain, anorexia, postural hypotension COMPLICATIONS 30
  16. 16. MEDICATION CHART DRUGS 18/3 19/3 20/3 21/3 22/3 23/3 25/3 26/3 27/3 28/3 29/3 30/3 31/3 1/4 2/4 3/4 IV C-Penicillin 960,000 u QID Sy. Penicillin V 125mg BD IV Frusemide 20mg STAT IV Frusemide 20mg BD T. Prednisolone 25mg OM, 20mg ON C-Penicillin - 30mg/kg QID Penicillin V – 125mg BD (1-5 years) 250mg BD (6-12 years) 500mg BD (>12 years) IV Frusemide – 1mg/kg/dose T. Prednisolone – 60mg/m2/day
  17. 17. INITIAl EpIsODE • High protein diet • Salt moderation • Treatment of infections • If significant edema – diuretics • Corticosteroid therapy* with Prednisolone – 60mg/m2/day* for 4weeks (-> fail : STEROID RESISTANT NS*) – 40mg/m2/EOD for 4weeks – ↓ 25% dose monthly over next 4 months PAEDIATRIC PROTOCOL, MOH 80% REMISSION !!! *
  18. 18. subsEquENT COuRsE • Relapse – Infrequent Relapsers : 3 or less relapses per year – Frequent Relapsers : 4 or more relapses per year* (0.1-0.5mg/kg/dose for 6 months) • Steroid therapy – Steroid dependant : relapse following dose reduction or discontinuation – Steroid resistant : Partial or no response to initial treatment • Steroid toxicity : » Cyclophosphamide (2-3mg/kg/day 8-12weeks) * »
  19. 19. ROlE OF pHARMACIsT Counseling on Steroids : 1) Indications, dose, frequency & duration 2) Side effects of steroids 3) Importance of compliance 4) Need of coming to hospital when relapse / infection 5) Ensure proper understanding
  20. 20. sIDE EFFECTs WITH lONg TERM usE OF sTEROIDs “sTEROID TOxICITy 1) Hyperglycemia & ↑ appetite (↑ weight) 2) Cushing Syndrome 3) ↑ GI symptoms 4) Osteoporosis 5) ↓ skin thickness (dermatitis) 6) Cataract & glaucoma 7) ↓ immunity (infection risk) 8) Gross / scrotal edema
  21. 21. HOME MONITORINg Home monitoring of urine protein and fluid status is important. Parents should be trained to monitor first morning urine by dipstick. Record of daily weight,urine protein and steroid dose should be kept in log book. Any increase in urine protein or daily weight should be reported as early as possible.
  22. 22. CoChrane meta-analysis: steroid • In children in their first episode, treatment with prednisone for at least three months results in fewer children relapsing by 12 to 24 months with an increase in benefit being demonstrated for up to seven months of treatment compared with two months therapy. In a population with a baseline risk for relapse of 60% with two months of prednisone, daily prednisone for four weeks followed by alternate-day therapy for six months would be expected to reduce the number of children experiencing a relapse by about 33%. • In comparison with 3 months of therapy, six months of therapy results in a reduced risk for relapse without increase in adverse effects. • The reduction in risk for relapse is associated with both an increase in duration and an increase in dose. • During daily therapy, prednisone is as effective when administered as a single daily dose compared with divided doses. • Alternate-day therapy is more effective than intermittent therapy (3 consecutive days of 7 days) in maintaining remission.
  23. 23. nephrotiC syndrome in Childhood response to 4 weeks of daily steroids ISKDC
  24. 24. referenCes • Paediatric protocol, MOH (2012) • Lau, Keith, et al. "Steroid Responsive Nephrotic Syndrome in IgA Nephropathy with FSGS." The • Internet Journal of Nephrology 4 (2008): n. pag. Print. • "Pediatric Nephrotic Syndrome." Pediatric Nephrotic Syndrome. N.p., n.d. Web. <http:// • emedicine.medscape.com>. • USA. NIH. NIDDK. Childhood Nephrotic Syndrome. N.p.: n.p., 2008. Print. • Trachtman, Howard. “Common Diseases: Minimal Change Nephrotic Syndrome.” Nephrology • Self Assessment Program 11 (2012) 19-20. Print. • Trachtman, Howard. “Common Diseases: Focal Segmental Glomerulosclerosis.” Nephrology

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