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Nephrotic Syndrome in Children Laura Cornish GPST2 Airedale VTS

Nephrotic Syndrome in Children Laura Cornish GPST2 Airedale VTS. Presentation Overview. Diagnosing nephrotic syndrome Principles of management Case study of a severely affected child Complications . Diagnosing Nephrotic Syndrome. Proteinuria Hypoalbuminaemia

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Nephrotic Syndrome in Children Laura Cornish GPST2 Airedale VTS

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  1. Nephrotic Syndrome in ChildrenLaura Cornish GPST2 Airedale VTS

  2. Presentation Overview Diagnosing nephrotic syndrome Principles of management Case study of a severely affected child Complications

  3. Diagnosing Nephrotic Syndrome • Proteinuria • Hypoalbuminaemia • Oedema (periorbital, ascites, pleural effusions) • Commoner in boys and atopic families. Often precipitated by respiratory infections.

  4. Proteinuria • Dipstick test positive Check urine protein/creatinine ratio in an early morning sample. Protein should not exceed 20mg/mmol of creatinine. Transient proteinuria can occur during a febrile illness or after exercise. Orthostatic proteinuria– check early morning and daytime urine P:C ratios.

  5. Nephrotic Syndrome • Cause is largely unknown. • Idiopathic – over 80% minimal change, 20% FSGS • Secondary to systemic diseases eg HSP, SLE, infections eg malaria • Congenital (rare, severe)

  6. Steroid sensitive nephrotic syndrome • Age 1-10 years • Normal BP • Normal renal function • Normal complement levels • No macroscopic haematuria • These children normally respond to steroids. Atypical features or non-responders need consideration for renal biopsy

  7. Investigations • Check bloods – FBC, U+Es, creatinine, LFTs, ASOT, C3/C4, Varicella titres • Urine protein/creatinine ratio and urine culture • Urine dip for blood/glucose • Urinary sodium concentration • BP • Varicella status/hep B status.

  8. Case Study • 9 year old boy • Known nephrotic syndrome, steroid dependent • Unwell for 1 week with cough and cold • Parents test urine dipstick for protein daily as frequent previous relapses – 4+ • Initial periorbital oedema, now distended abdomen and groin oedema, ankle oedema and breathlesness/wheeze on lying flat.

  9. Case Study • Already taking long term daily prednisolone treatment • Started mycophenylate a few days earlier in OPD, as relapses becoming more frequent • Developed loose stool post admission – s/e of medication – exacerbating hypovoloaemia • BP stable, oliguric • Hgb 14.1 wcc 32 urea 10.5, creat 47, albumin <10

  10. Case study • Went into remission after approx 1 week • Prednisolone continued, mycophenylate stopped • IV albumin with furosemide • Prophylactic penicillin • Ranitidine to help gut s/es. • Daily weights

  11. Management of nephrotic syndrome • High dose prednisolone, weaned over several weeks • Albumin if hypovolaemic (not just for low albumin) • Diuretics may be needed to help treat peripheral oedema – but caution if hypovolaemic. • Penicillin V tends to be given in relapses – increased risk of infection including cellulitis • Fluid restriction • Remission = trace or neg protein on dip • More specialised drugs – levamisole, cyclophosphamide, mycophenylate

  12. Complications of nephrotic syndrome • Hypovolaemia (despite peripheral oedema) • Infection – urinary loss of immunoglobulin • Thrombosis • Hyperlipidaemia

  13. Prognosis • Most (>90%) children respond to prednisolone within 2-4 weeks. • Can be an isolated episode, lead to infrequent or frequent relapses. • Most children grow out of nephrotic syndrome.

  14. GENERAL CONSIDERATIONS DURING FOLLOW UP* • For children on long-term steroids:- • 1) Monitor BP • 2) Monitor growth (including bone age and pubertal stage where appropriate) • 3) Monitor weight – dietetic review where appropriate • 4) Glycosuria / HbA1c • 5) Bone mineral density / calcium supplements • 6) Ophthalmology review • 7) VACCINATION • Pneumococcal: recommended for all children with NS. • Varicella: consider in varicella negative children with frequent relapses.Aim to administer vaccine when prednisolone dose is low. * Guideline for the management of nephrotic syndrome, Dr J Beattie, Royal Hospital for Sick Children, Yorkhill division, Greater Glasgow Oct 07 www.clinicalguidelines.scot.nhs.uk

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