27 Apr 2010- Nephrotic Syndrome in Children

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Diagnosing nephrotic syndrome
Principles of management
Case study of a severely affected child
Complications
 Proteinuria
 Hypoalbuminaemia
 Oedema
(periorbital, ascites, pleural
effusions)
 Commoner
in boys and atopic families. Often
precipitated by respiratory infections.
 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.
 Cause
is largely unknown.
 Idiopathic – over 80% minimal change, 20%
FSGS
 Secondary to systemic diseases eg HSP, SLE,
infections eg malaria
 Congenital (rare, severe)
 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
 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.
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.
 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
 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
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

 Hypovolaemia
(despite peripheral oedema)
 Infection – urinary loss of immunoglobulin
 Thrombosis
 Hyperlipidaemia
 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
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