Presentation on theme: "Nephrotic Syndrome in Children Laura Cornish GPST2 Airedale VTS"— Presentation transcript:
1Nephrotic Syndrome in Children Laura Cornish GPST2 Airedale VTS
2Presentation Overview Diagnosing nephrotic syndrome Principles of management Case study of a severely affected child Complications
3Diagnosing Nephrotic Syndrome ProteinuriaHypoalbuminaemiaOedema (periorbital, ascites, pleural effusions)Commoner in boys and atopic families. Often precipitated by respiratory infections.
4Proteinuria 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.
5Nephrotic Syndrome Cause is largely unknown. Idiopathic – over 80% minimal change, 20% FSGSSecondary to systemic diseases eg HSP, SLE, infections eg malariaCongenital (rare, severe)
6Steroid sensitive nephrotic syndrome Age 1-10 yearsNormal BPNormal renal functionNormal complement levelsNo macroscopic haematuriaThese children normally respond to steroids. Atypical features or non-responders need consideration for renal biopsy
7InvestigationsCheck bloods – FBC, U+Es, creatinine, LFTs, ASOT, C3/C4, Varicella titresUrine protein/creatinine ratio and urine cultureUrine dip for blood/glucoseUrinary sodium concentrationBPVaricella status/hep B status.
8Case Study 9 year old boy Known nephrotic syndrome, steroid dependent Unwell for 1 week with cough and coldParents 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.
9Case Study Already taking long term daily prednisolone treatment Started mycophenylate a few days earlier in OPD, as relapses becoming more frequentDeveloped loose stool post admission – s/e of medication – exacerbating hypovoloaemiaBP stable, oliguricHgb 14.1 wcc 32 urea 10.5, creat 47, albumin <10
10Case study Went into remission after approx 1 week Prednisolone continued, mycophenylate stoppedIV albumin with furosemideProphylactic penicillinRanitidine to help gut s/es.Daily weights
11Management of nephrotic syndrome High dose prednisolone, weaned over several weeksAlbumin 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 cellulitisFluid restrictionRemission = trace or neg protein on dipMore specialised drugs – levamisole, cyclophosphamide, mycophenylate
12Complications of nephrotic syndrome Hypovolaemia (despite peripheral oedema)Infection – urinary loss of immunoglobulinThrombosisHyperlipidaemia
13PrognosisMost (>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.
14GENERAL CONSIDERATIONS DURING FOLLOW UP* For children on long-term steroids:-1) Monitor BP2) Monitor growth (including bone age and pubertal stage where appropriate)3) Monitor weight – dietetic review where appropriate4) Glycosuria / HbA1c5) Bone mineral density / calcium supplements6) Ophthalmology review7) VACCINATIONPneumococcal: 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