1. Nephrotic Syndrome in Children Laura 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