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correspondence

Indian Pediatr 2012;49: 844-845

Response to query


Aditi Sinha
,

Division of Nephrology, Department of Pediatrics, All India Institute of Medical Sciences,
New Delhi 110 029, India.



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/m2) 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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