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Indian Pediatr 2012;49: 844-845
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Response to query
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Aditi Sinha,
Division of Nephrology, Department of
Pediatrics, All India Institute of Medical Sciences,
New Delhi 110 029, India.
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The present patient received inappropriately prolonged therapy with
daily prednisolone resulting in severe steroid toxicity. Following
stoppage of corticosteroid therapy, he showed a fast relapse, which is a
predictor of frequent relapses and thereby further steroid side effects.
The management of this patient therefore comprises of induction and
maintenance of remission, and prompt therapy for complications of the
nephrotic state. Since the disease is expected to resolve by adulthood
in most children with steroid sensitive nephrotic syndrome, limiting
adverse effects of medications is an important objective of management.
Oral corticosteroids are still the most effective
agents for inducing remission in patients with steroid sensitive
disease. The response to therapy with prednisolone is predictable and
rapid (8-14 days). Other corticosteroid preparations including IV
methylprednisolone and deflazacort are not recommended [1,2]. Similarly,
there is no experience with the use of cyclophosphamide, levamisole and
mycophenolate mofetil for inducing remission in such children. The other
class of agents that might be considered for inducing remission is
calcineurin inhibitors (cyclosporine, tacrolimus) [2], which reduce
proteinuria by 2-4 weeks and induce complete remission by 6-8 weeks.
However, the slower response to therapy and associated adverse effects
do not justify their use in patients with steroid sensitive nephrotic
syndrome. Indications where steroids are avoided for inducing remission,
and cyclosporine considered instead, are high blood sugar or steroid
psychosis [2].
Once remission is induced, patients with frequent
relapses, especially those having steroid toxicity should receive
treatment with steroid sparing agents [1-3]. For this purpose, therapy
with levamisole, cyclophos-phamide, mycophenolate mofetil and
calcineurin inhibitors is effective and safe [3]. However, there are few
comparative studies on the preferred second-line agent [3], and the
choice is determined chiefly by patient and physician preference.
Compared to others, treatment with alkylating agents or calcineurin
inhibitors offers the best prospect of medium-term steroid free
remission [2,3].
The present patient should be treated with
prednisolone in the standard dose until remission, and then on alternate
days for 4 weeks [1,2]. Subsequently, in order to maintain remission,
therapy with oral cyclophosphamide (2-2.5 mg/kg daily) and alternate day
prednisolone (20-30 mg/m 2)
is recommended. A 12-weeks’ course of such treatment is likely to result
in sustained remission in a significant proportion of patients. Therapy
with calcineurin inhibitors is considered if either steroid therapy is
contraindicated (see above) or if frequent relapses recur following
cyclophosphamide use. While tacrolimus and cyclosporine show similar
efficacy in reducing the frequency of relapses and need for
corticosteroids, the latter is preferred in patients with hyperglycemia.
Therapy with these agents is, however, initiated after confirming normal
renal functions and counseling parents regarding the need for close
monitoring.
Additional management includes screening for
infectious and other complications. An angiotensin converting enzyme
inhibitor is preferred for treating hypertension. The child should
consume a balanced diet and be physically active. Parents should be
counseled regarding the course of the illness, need for compliance with
therapy and adverse effects of medications.
References
1. Indian Pediatric Nephrology Group, Indian Academy
of Pediatrics, Bagga A, Ali U, Banerjee S, Kanitkar M, Phadke KD,
Senguttuvan P, Sethi S, Shah M. Management of steroid sensitive
nephrotic syndrome: Revised guidelines. Indian Pediatr. 2008;45:203-14.
2. Kidney Disease: Improving Global Outcomes (KDIGO)
Clinical Practice Guideline for Glomerulonephritis. Steroid sensitive
nephrotic syndrome in children. Kidney Int Supplements 2012;2:163-71.
3. Hodson EM, Willis NS, Craig JC. Corticosteroid
therapy for nephrotic syndrome in children. Cochrane Database Syst Rv.
2007;4:CD001533.
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