PULSE NITROGEN MUSTARD TREATMENT OF CHILDHOOD IDIOPATHIC NEPHROTIC SYNDROME (INS.)

Abstract

As an alternative to long-term administration of oral cytotoxic agents, pulse intravenous nitrogen mustard (HN₂) has been used in 5 patients with steroid-resistant INS (all had “early resistance”) and one patient with steroid responsive INS. The dose of HN₂ was 0.1 mg/kg/day × 4 days. All patients had minimal-lesion histology. The patient with steroid-responsive INS had a frequently relapsing course (4.8 relapses/year) despite treatment with cyclophosphamide (2 courses) and chlorambucil. Since HN₂, she has had no further relapses. The current status of 5 patients with steroid-resistant INS is: 3 have persistent mild proteinuria, normal excretory function and absence of overt nephrotic syndrome; 2 have persistent INS despite HN₂ but subsequently remitted after cyclophosphamide and both have since had a steroid-responsive, relapsing course. No toxicity attributable to HN₂ was noted. This preliminary experience suggests that HN₂ may ameliorate INS in some patients with steroid-resistance. HN₂ may also be a useful adjunct in the management of some patients with steroid-responsive, frequently-relapsing INS, in whom oral cytotoxic therapy may not be warranted. A controlled therapeutic trial to better assess the usefulness of HN₂ is planned.

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