Contents Page(s)
1. Introduction 2
2. Definition of Nephrotic Syndrome2-3
3. Initial Investigation 3
4. Referral to Paediatric Nephrology 3
5. Complications 4
5.1 Hypovolaemia 4
5.2 Infection 4
5.3 Thrombosis 4
4
6. Treatment of Initial Prentation of Nephrotic
Syndrome秦俑
6.1 Prednisolone4-5
6.2 Albumin 5
6.3 Penicillin Prophylaxis 5
6.4 Salt/Fluid Restriction 5
6.5 Vaccination 5
7. Relap of Nephrotic Syndrome 6
8. Treatment of Relap of Nephrotic Syndrome 6
8.1 Prednisolone 6
8.2 Albumin 6
8.3 Salt Restriction 6
8.4 Penicillin 6
8.5 Vaccination 6
9. Treatment of Frequent Relaps6-7
9.1 Low Do Alternate Day Prednisolone7
9.2 Levamisole7
9.3 Cyclophosphamide7
9.4 Cyclosporin7
9.5 Mycophenylate Mofitil (MMF)7
10. Follow Up8
11. Future Guideline Review8
1. Introduction
for u by clinicians. The guidelines are bad on previous recommendations reviewed in the light of recent literature and will be update regularly. They cover many aspects of the management of “typical” nephrotic syndrome, but they are not exhaustive, and many not be relevant for children with “atypical” nephrotic syndrome. There is always a paediatric nephrology consultant on call for the unit who will be happy to discuss difficult or unusual cas.
2. Definition of Nephrotic Syndrome
• Nephrotic range proteinuria (> 1g/m2/day)
安全小报模板• Hypoalbuminaemia (<25 g/l)
• Oedema
异乎寻常Classification
• Idiopathic (primary) nephrotic syndrome
o Minimal change (80-90%)
溶洞的形成
o Focal gmental glomerulosclerosis (FSGS) (10-20%) • Secondary nephrotic syndrome (HSP, SLE, MPGN)
木偶的英文
• Congenital nephrotic syndrome
This document relates only to the management of idiopathic nephrotic syndrome. Children who prent with the typical features of nephrotic syndrome (e below) are generally responsive to steroid treatment and a renal biopsy, were it performed, would be likely to show minimal change nephrotic syndrome. Tho with atypical features are more likely to be unresponsive to steroid treatment, and a biopsy more likely to show FSGS or one of the other forms of nephrotic syndrome.
Therefore children with typical features are started on steroids without recour to renal biopsy. Tho with atypical features should therefore undergo renal biopsy before receiving steroid treatment.
Nephrotic Syndrome
商务专员Typical Features Atypical Features
Age 1-10 years <1yr, >10years
Normotensive Hypertensive
Normal Adrenal Function Elevated Creatinine
+/- microscopic haematuria Macroscopic Haematuria
3. Initial Investigation
• Blood: F BC, U+E’s; Creatinine; LFT’s; ASOT; C3/C4; Varicella titres
• Urine: Urine culture andUrinary protein/creatinine ratio
• BP
• Urinalysis including gluco太阳是大家的
• A urinary sodium concentration can be helpful in tho at risk of hypovolaemia.
• Varicella status should be known in all children commencing steroids.
• Hepatitis B status may be appropriate in children at high risk.
4. Referral to Paediatric Nephrology
• Age < 1 yr
• Age > 10-12 yrs
• Persistent hypertension
• Macroscopic haematuria
• Low C3/C4
• Failure to respond to steroids within 4 weeks
5. Complications
thrombosis.
5.1 Hypovolaemia
The initial examination of children with nephrotic syndrome needs to include an asssment of their i
ntravascular volume. Whilst the children may be very oedematous, they may also be intravascularly depleted. Signs of intravascular depletion are cool peripheries (capillary refill time > 2 cs), a core-peripheral temperature gap of > 2o C, and tachycardia. Hypotension is a late sign of hypovolaemia, but paradoxical hypertension may be prent. A urinary sodium of < 10 mmol/l is a uful investigation to confirm hypovolaemia.
5.2 Infection
incread risk of infection. Consider antibiotic prophylaxis whilst patients have significant proteinuria.
鱼卡通图片5.3 Thrombosis
Loss of proteins such as anti-thrombin III contributes to a pro-coagulant state. This might be exacerbaterd by hypovolaemia.
6. Treatment of Initial Prentation of Nephrotic Syndrome
6.1 Prednisolone
treatment can be started in children with typical features. Children with atypical features should be referred to paediatric nephrology for consideration of renal biopsy.
There is increasing evidence that longer initial cours of prednisolone are associated with a lower incidence of relap, and therefore a 12-week initial cour is recommended. The do of prednisolone is bad on surface area.
• 60 mg/m2/day for 4 weeks (maximim 80 mg)
• 40 mg/m2/on alternate days for 4 weeks (maximum 60mg)
• Reduce do by 5-10mg/m2 each week for another 4 weeks then stop Prednisolone can be given as a single do in the morning with food, or as divided dos during the day. Patients should be issued with a steroid warning card, and they should be aware of the side effects and risks of steroid treatment. Varicella status should be documented clearly in the canotes and on HISS. If
prednisolone caus gastric irritation, start ranitidine 2mg/kg bid for the duration of steroid treatment.
6.2 Albumin
• Clinical hypovolaemia • Symptomatic oedema
A low rum albumin alone is not an indication for intravenous albumin.
If there is evidence of hypovolaemia, give 1 g/kg 20% albumin (5ml/kg) over 4 - 6 hours. Give 2mg/kg of iv frumide mid-infusion. If clinically shocked give 10ml/kg 4.5% albumin. Children should be cloly monitored during albumin infusions, and where possible they should be administered during working hours.
6.3 Penicillin Prophylaxis
Whilst nephrotic, children are at incread risk of infection, particularly with encapsulated organisms such as pneumococcus. There is no evidence that antibiotic prophylaxis is of benefit, and some centres do not u prophylaxis. Penicillin V can be given while there is proteinuria and discontinued when the child goes into remission. Grossly oedematous children are at risk of cellulitis and may benefit from antibiotic prophylaxis.
Do: Under 5 yrs 125 mg bid 5yrs or above 250 mg bid
6.4 Salt/Fluid Restriction
restriction may also be helpful. The restrictions are lifted once the child goes into remission.
6.5 Vaccination
at the time of diagnosis. Varicella vaccination is only available on a named patient basis.
