Paediatric Clinical Guidelines Renal 6.

1 November 2002

THE INVESTIGATION AND MANAGEMENT OF NEWLY PRESENTING CHILDHOOD NEPHROTIC SYNDROME
Revised Patient Group Author November 2002 Children with Nephrotic Syndrome Dr JHC Evans, Consultant Paediatric Nephrologist, Nottingham City Hospital NHST, Ext 47428

Background Nephrotic syndrome is characterised by heavy proteinuria (protein or albumin:creatinine ratio > 200mg/mmol), hypoalbuminaemia (serum albumin <25g/l) and oedema. It is an uncommon childhood condition with an annual incidence in the UK of only 2 per 100,000 children1. There are many different causes of nephrotic syndrome, but the majority of cases (over 90%) are primary and due to minimal change disease (MCD) 2. Approximately 80% of children will respond to prednisolone and this is the most important factor in terms of management and prognosis. Nephrotic syndrome is thought of as a relatively benign condition, however the mortality rate remains around 1%. There is significant acute and long term morbidity also, therefore it is appropriate to consider early referral of all patients to the Paediatric Renal Team at City hospital. 1. Clinical history To include history of…  Atopy  Immunisation  Natural childhood infections (particularly Varicella Zoster)  Family history (particularly renal disease and thrombophilia) 2. Clinical examination To include…  Height, Weight, Surface area  Blood pressure  Cardiovascular status and perfusion

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Cortico-steroids There is considerable debate as to the optimal regimen of prednisolone.Paediatric Clinical Guidelines Renal 6. 4. Blood for…  Urea. cases should be discussed with a Consultant Nephrologist where other investigations may be appropriate (C3. electrolytes. Management Nephrotic syndrome treatment aims to induce remission with steroids (most patients respond within 5-14 days). and therefore promote diuresis.1. Children who do not respond to prednisolone within 28 days will require a renal biopsy. ASOT. A standard regimen and an intensified regimen. Femoral stabs should never be performed as thrombosis is a described complication) If mixed nephrotic/nephritic picture with macroscopic haematuria and hypertension. but this may be at the expense of greater steroid induced adverse effects 3. Two regimens are therefore used. All other therapies are symptomatic and aimed at preventing complications.4. 4. Investigations In all new patients Urine for…  Dipstick urinalysis  Urine culture  Urine microscopy for casts if gross haematuria  Urine protein/albumin:creatinine ratio (early morning specimen) correlates with overnight protein loss and replaces the need for 24hr urine collection.1 November 2002 3. Page 2 of 7 . single venepuncture is ideal in children who may be difficult to bleed because of oedema. Prolonged courses (3-6 months) reduce the likelihood of relapse in the subsequent 2 years. C3d. ANF. The intensified regimen is used in children considered at high risk of relapse taking into account factors such as young age. Do not delay starting treatment in order to obtain. C4 complement. ANCA and immunoglobulins). ethnicity (south asian) severe illness on initial presentation and delay in achieving initial remission. creatinine and albumin  Full blood count  "Varicella Zoster immunity status" (Plan tests. The choice between the two regimens is an elective decision that should be made after discussion with the consultant paediatric nephrologist.

in the absence of hypovolaemia. Mild hypovolaemia + oedema = 20% albumin 5ml/kg (1g/kg) over 2-4 hrs with IV frusemide 1mg/kg halfway through the infusion provided signs of hypovolaemia have resolved. Suggested fluid intake <5yrs = 750mls. the dose of steroid is reduced to 40mg/m2 alternate days for the next 28 days and then stopped Week 1-4 (first 28 days) 5-8 (next 28 days) Prednisolone dosage 60 mg/m2/day (maximum dose 80 mg) 40 mg/m2/alternate day (maximum 40mg) Intensified Regimen The first 8 weeks are as in the standard regimen.Prednisolone 60mg/m2/day in a single morning dose (maximum dose 80mg) for 28 days even if proteinuria remits. 4. Severe circulatory failure = 4. followed by a further 8 weeks of tapering dosage. It should be administered with great caution with frequent monitoring of vital signs until at least two hours after the infusion is completed. After 28 days. Severe diuretic resistant oedema = 20% albumin 5ml/kg (1g/kg) over 2-4 hrs with IV frusemide 1mg/kg half way through infusion     Page 3 of 7 .2. Doses. Week 1-4 (first 28 days) 5-8 (next 28 days) 9-12 13-17 Prednisolone dosage 60 mg/m2/day (maximum dose 80 mg) 40 mg/m2/alternate day (maximum 40mg) 25 mg/m2/alternate day (maximum 25mg) 10mg/m2/alt day (maximum 10mg) A "steroid warning card" should be provided for the patient to carry.) Spirinolactone 2mg/kg/day in two divided doses Frusemide 1-2mg/kg/day in two divided doses Albumin infusions Albumin infusion is only indicated in symptomatic hypovolaemia or severe diuretic resistant oedema. Methyl prednisolone 60mg/m2/day can be used intra-venously in the vomiting child.5% albumin 20ml/kg over 30-60 mins repeated if necessary until volume status restored.Paediatric Clinical Guidelines Renal 6. >5yrs = 1 litre Diuretics (use only if severe and worsening oedema/ascites.    Oedema and ascites A balanced no added salt diet is recommended.1 November 2002 Standard Regimen Initial therapy .

4. if satisfactory… Anti-hypertensives Atenolol 0. Antibiotics  Oral Penicillin V (125mg BD if < 5yrs or 250mg BD <12yrs) should be prescribed to oedematous/ascitic patients to protect against pneumococcal infection.4.5. measles.3.1. subsequent doses should be administered as outlined in the BNF. Nb: If peritonitis is suspected then cover for Gram negative organisms is recommended until cultures are available. BCG). However. mumps. it seems reasonable that patients with a family history of thrombophilia or raised platelet count (>800) should receive low dose Aspirin as prophylaxis three times per week (if <30kg 37.5. Pneumococcal polysaccharide (7 valent) conjugate vaccine (Prevenar 0.   Hypertension Check volume status. Routine vaccinations should be given as outlined in the DoH handbook unless the child is "immunosuppressed"(as defined below). oral polio.5 ml) is recommended for children under 2 years.5-1mg/kg/day in single daily dose or Nifedipine SR 0. To decrease the risk of thrombosis.   Page 4 of 7 .Paediatric Clinical Guidelines Renal 6. >30kg 75mg).25-2mg/kg/day in two divided doses Infection prophylaxis 4. 4. Pneumococcal (23 valent) polysaccharide vaccine (Pneumovax II 0.  Risk of thrombosis Thrombosis either arterial or venous is rare (5% 5) in nephrotic syndrome.2.1 November 2002 4. rubella.5ml intramuscular) is recommended for all children with nephrotic syndrome over 2 years whether on steroids or overtly nephrotic.  Avoid hypovolaemia  Prevent sepsis  Encourage mobilisation and avoid bedrest  Low dose Aspirin* *Nb: There is no definite evidence (even in adults) that prophylactic anti-platelet or anticoagulation treatment is of benefit. It should be given during initial admission.5. The first dose should be given during the initial admission. "Immunosupressed" children should not receive live vaccines (ie. The consequences however can be devastating. Immunisation  4.5mg.

5. (d) long term lower dose immunosuppression. Prednisolone treatment usually delayed for at least 5 days unless the child is becoming oedematous. immunisation may be deferred. (a) Prednisolone 2mg/kg/day for > 1 week.3 "Chickenpox and immunosuppression therapy in renal children. "Varicella zoster immunity status" should be known (and documented) in each nephrotic patient. ZIG or Aciclovir may be required please refer to renal protocol 6.Paediatric Clinical Guidelines Renal 6. A balanced no added salt diet is recommended Psychosocial support Making adequate information available for the family is essential. Cotgrave.  Dietary advice A dietician should see the child and family. "Childhood Nephrotic Support" (contact Mrs Sandra Warhurst. (b) Prednisolone 1mg/kg/day or equivalent for 1 month (ie 40mg/ m 2 alternate days) (c) lower doses of prednisolone combined with cytotoxic drugs. In some instances where the risk of relapse is high. 0115 9892975).3.6. Childhood Nephrotic Syndrome booklets and a video are available from the Renal unit at City hospital. Relapse Relapse within the first year is common (86%3) and can occur years after the initial presentation.  4.    5. 4. A family support group exists. NG12 3QH.7. Tel.1 November 2002  "Immunosuppression" in the context of immunisation is defined as any child who is receiving or has received in the last 3 months. Referral to the paediatric community renal nurse and social worker is routine. 7 Bonnymead. Carers should be instructed on how to dipstick the child's early morning urine and record this in a daily diary. This requires discussion with the consultant. Duration Daily until urinary protein negative or trace for 3 days Next 28 days Subsequent therapy Prednisolone dosage 60 mg/m2/day (maximum dose 80 mg) 40 mg/m2/alternate day (maximum 40mg) Individualised (may / may not taper steroids) Page 5 of 7 . Relapse can follow immunisation or viral infection." 4. A relapse is defined as proteinuria of ++ or more for three consecutive days. Varicella Zoster    Chicken pox whilst immunosuppressed can be a very serious illness.

: Nephrotic syndrome in children.1 November 2002 Frequent relapsers are defined as having 2 or more relapses in 6 months and should receive second line treatments (not covered by this guideline). Herzog U. The Cochrane Library.: Hemostasis and thromboemblism in children with nephrotic syndrome: Differences from adults. 18: 197-251. Do patients receive Pneumovac? 2. Craig JC. Are patients managed according to the guidelines References 1 2 3 4 5 Kherr KK. Contact Numbers Consultant Paediatric Nephrologists Dr Jonathan Evans Dr Alan Watson Paediatric Renal Nurse – Liz Moore Paediatric Nephrology SpR 47428 or bleep via City Hospital switchboard 46169 or bleep via City Hospital switchboard 46488 46458 )(Lambley Ward) or bleep via City Hospital switchboard Audit Points As patient numbers per year are very low. a retrospective audit of case notes could be undertaken as there are only minor changes in the new version of the guidelines compared with the3 previous version. Knight JF. Issue 3. The International Study of Kidney disease in Children: The primary nephrotic syndrome in children. J Pediatr 1987:110:862-867 Page 6 of 7 . 98: 561-564. The Royal College of Paediatrics and Child Health/The British Association for Paediatric Nephrology. Oxford Update Mehls O. Ritz E. Makker SP. Koderisch J. 1999 Hodson EM. Corticosteroid Therapy for nephrotic syndrome in children (Cochrane Review). Andrassy K. Sweet M. Identification of patients with minimal change nephrotic syndrome from initial response to prednisolone. J Pediatr. Willis NS. 1. 1981.Paediatric Clinical Guidelines Renal 6. Evans J :National Audit of Childhood Nephrotic Syndrome. 2002. Curr Prob Pediatr.

1 November 2002 PAEDIATRIC CLINICAL GUIDELINES ISSUE: VERSION: FINAL Title: THE INVESTIGATION AND MANAGEMENT OF NEWLY PRESENTING CHILDHOOD NEPHROTIC SYNDROME Author: Job Title: Dr JHC Evans. clinical guidelines are ’guidelines’ only.e. However. If in doubt contact a senior colleague or expert. Ext 47428 First Issued: Date Revised: November 2002 Review Date: October 2004 Consultation Process: Paediatric Nephrologists Cross town Paediatric Clinical Guidelines Pharmacist Document Derivation: i. References: Group Included in document Ratified By: Paediatric Clinical Guidelines Committee Chaired By: Consultant with Responsibility: Dr Stephanie Smith Distribution: All wards QMC and CHN Training issues: Included in Induction Programme Audit: included in document This guideline has been registered with Nottingham City Hospital NHS Trust and QMC Clinical Guidelines Committee.Paediatric Clinical Guidelines Renal 6. The interpretation and application of clinical guidelines will remain the responsibility of the individual clinician. Nottingham City Hospital NHST. Caution is advised when using guidelines after the review date. Consultant Paediatric Nephrologist. MANUAL AMENDMENTS RECORD (please complete when making any hand-written changes/ amendments to guideline and not processed through guideline committee) Date Author Description Page 7 of 7 .