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Nephrotic Syndrome PowerPoint Presentation

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Description : Basic of nephrotic syndrome

Tags : Kidney

Published on : Feb 09, 2021 | Views : 10112 | Downloads : 78

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Nephrotic Syndrome By: HO NORAINI MENTOR: DR NG JL
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DIAGNOSIS Nephrotic syndrome is a clinical syndrome of massive proteinuria defined by Oedema Hypoalbuminaemia of < 25g/l Proteinuria > 40 mg/m²/hour (> 1g/m²/day)/early morning urine protein creatinine index of >200 mg/mmol (>3.5mg/mg) Hypercholesterolaemia
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Primary or Idiopathic (Unknown cause) Secondary cause of Nephrotic Syndrome Common Post Streptococcal Glomerulonephritis Systemic Lupus Erythematous HSP Minimal change lesion Focal segmental glomerulosclerosis (FSGS) Mesangial proliferative glomerulonephritis Membranoproliferative glomerulonephritis Membranous glomerulonephritis Congenital or Finnish type Nephrotic Syndrome Etiology
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Periorbital oedema (earliest sign) Scrotal, leg and ankle oedema Ascites Breathlessness CLINICAL PRESENTATION
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INVESTIGATIONS Blood Renal profile Serum cholesterol Liver function tests- Particularly serum albumin Urine Urine UFEME Urine culture Quantitative urinary protein excretion (spot urine protein: creatinine ratio or 24 hour urine protein) Others ANA Anti-dsDNA C3, C4 ASOT
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Renal biopsy Renal biopsy is Not needed prior to corticosteroid / cyclophosphamide therapy because 80% of children with idiopathic nephrotic syndrome have minimal change steroid responsive disease Main indication for renal biopsy: Steroid resistant nephrotic syndrome Defined as failure to achieve remission despite 4 weeks of adequate corticosteroid therapy. Features that suggest non-minimal change nephrotic syndrome: Persistent hypertension, renal impairment, and/or gross haematuria
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Management Diet ( normal protein, low salt, adequate calories) Antibiotics ( T. Penicillin V) – initial diagnosis/ relapse 125 mg BD (1-5 years age) 250 mg BD (6-12 years) 500 mg BD (>12 years) Assess haemodynamic status W/O for Hypovolaemia Abdominal pain, cold peripheries, poor capillary refill, poor pulse volume with or without low blood pressure W/O for Hypervolaemia Basal lung crepitations, rhonchi, hepatomegaly, hypertension.
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Diuretics (e.g. frusemide) – use with caution of hypovolaemia. Human albumin (20-25%) at 0.5 - 1.0 g/kg together with IV frusemide at 1-2 mg/kg to produce a diuresis (can be used in symptomatic grossly oedematous states). Fluid restriction - not recommended except in chronic oedematous states. Remember to monitor BP, hemodynamic status, urine output closely !!!
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General advice High probability (85-95%) of relapse. Home urine albumin monitoring (once daily, first morning urine). To consult the doctor if proteinuria ≥ 2+ for 3 consecutive days, or 3 out of 7 days. To consult the doctor if oedematous regardless of the urine dipstick result. Caution about contact with chickenpox and measles. Immunisation: only killed vaccines is safe while the child is on corticosteroid treatment and within 6 weeks after its cessation. Pneumococcal vaccine should be administered to all children with nephrotic syndrome (when the child is in remission)
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How to calculate BSA ?
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Corticosteroid therapy Oral Prednisolone should be started at: 60 mg/ m²/day ( maximum 80 mg / day ) for 4 weeks 40 mg/m²/every alternate morning (EOD) (maximum 60 mg) for 4 weeks then reduce Prednisolone dose by 25% monthly over next 4 months (Total 6 months duration of treatment) 80% of children will achieve remission (defined as urine dipstick trace or nil for 3 consecutive days) within 28 days. If fail to achieve response to an initial 4 weeks treatment with prednisolone at 60 mg/m²/ day -> Steroid resistant Nephrotic syndrome -> Refer Paeds nephrologist for renal biopsy
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Side effects of corticosteroids
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Nephrotic chart
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Complications Hypovolemia Clinical Features Abdominal pain, cold peripheries, poor pulse volume, hypotension, hemoconcentration Treatment Infuse Human Albumin 0.5 to 1.0g/kg/dose fast. Human plasma Infection Primary Peritonitis Clinical Features Fever, abdominal pain, tenderness in children within newly diagnosed or relapse nephrotic syndrome. Treatment Parenteral Penicillin and 3rd generations Cephalosporin Thrombosis Thorough investigation and adequate treatment with anticoagulation is usually needed. Consult nephrologist complications
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Treatment of relapses Relapse Nephrotic syndrome : Urine dipstick of ≥ 2+ for 3 consecutive days / edematous / urine albumin excretion > 40 mg/m²/hour Not all need admission unless they are grossly oedematous or have any of the complications of nephrotic syndrome Induction of relapse is with oral Prednisolone as follows: • 60 mg/m²/day ( maximum 80 mg / day ) until remission followed by • 40 mg/m²/EOD (maximum 60 mg) for 4 weeks only. Breakthrough proteinuria: Proteinuria which may occur with intercurrent infection and usually does not require corticosteroid induction if the child has no oedema, remains well and the proteinuria remits with resolution of the infection. However, if proteinuria persists, treat as a relapse.
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Treatment of frequent relapses Defined as ≥ 2 relapses within 6 months of initial diagnosis or ≥ 4 relapses within any 12 month period. Treatment Induction of relapse is with oral Prednisolone as follows: • 60 mg/m²/day ( maximum 80 mg/day ) until remission followed by • 40 mg/m²/EOD (maximum 60 mg) for 4 weeks only. • Taper Prednisolone dose every 2 weeks and keep on as low an alternate day dose as possible for 6 months. If the child relapse while on low dose alternate day Prednisolone, the child should be re-induced with Prednisolone as for relapse.
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Treatment of steroid dependent nephrotic syndrome Defined as ≥ 2 consecutive relapses occurring during steroid taper or within 14 days of the cessation of steroids. Treatment If the child is not steroid toxic, re-induce with steroids and maintain on as low a dose of alternate day prednisolone as possible If the child is steroid toxic (short stature, striae, cataracts, glaucoma, severe cushingoid features) consider cyclophosphamide therapy.
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Cyclophosphamide therapy Indicated in steroid dependent nephrotic syndrome with signs of steroid toxicity Parents should be counselled about the effectiveness and side effects of Cyclophosphamide therapy Leucopenia Alopecia Haemorrhagic cystitis Infertility Dose: 2-3 mg/kg/day for 8-12 weeks (cumulative dose 168 mg/kg) Monitor full blood count and urinalysis 2 weekly after started
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Relapses post Cyclophosphamide Consider : Cyclosporine Levamisole To do renal biopsy before initiation, to discuss with paediatric nephrologist
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Steroid resistant nephrotic syndrome Refer for renal biopsy. Specific treatment will depend on the histopathology. General management of the Nephrotic state: Control of edema Restriction of dietary sodium. Diuretics Monitor BP and renal profile 1-2 weeks after initiation of ACE inhibitor or AIIRB Control of hypertension (ACE inhibitor/AIIRB). Penicillin prophylaxis. Nutrition (normal dietary protein content, salt-restricted diet) Evaluate calcium and phosphate metabolism.
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Q&A Definition of nephrotic syndrome? Definition of relapse nephrotic syndrome? What is remission? How to calculate BSA ? Side effects of steroids? Complication of nephrotic syndrome?
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THANK YOU Reference: Malaysian Paediatric Protocols 4TH edition