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 Oedema (periorbital, ascites, pleural effusions) Commoner in boys and atopic families. Often precipitated by respiratory infections.

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.

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)

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

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.

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.

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

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

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

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

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.

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