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

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Presentation on theme: "Diagnosing nephrotic syndrome Principles of management Case study of a severely affected child Complications."— Presentation transcript:

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

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

4  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  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  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  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  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  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  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  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  Hypovolaemia (despite peripheral oedema)  Infection – urinary loss of immunoglobulin  Thrombosis  Hyperlipidaemia

13  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


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