Treatment of Childhood Prednisone-Resistant Nephrotic Syndrome and Focal Segmental Glomerulosclerosis with Intravenous Methylprednisolone and Oral Alkylating Agents

Abstract

Patients with prednisone-resistant nephrotic syndrome and biopsy-proven focal segmental glomerulosclerosis were treated with intravenous methylprednisone. After the first 2 weeks of therapy, the average urine protein excretion decreased from 247 to 96 mg/m²/h (p < 0.04). Two of the 7 patients have had long-term, nearly complete remissions. The other patients relapsed. One relapsing patient was retreated with methylprednisolone and is now in remission. Four relapsing patients were treated with alkylating agents, in combination with methylprednisolone. All of these patients entered complete or partial remissions. Methylprednisolone causes a significant decrease in the proteinuria of children with focal segmental glomerulosclerosis. In addition, although the follow-up period is relatively short, it would appear that methylprednisolone, often in conjunction with an alkylating agent, has significantly improved the clinical status of these patients.

Ticlopidine Treatment in Idiopathic Plasmacytic Lymphadenopathy with Polyclonal Hyperimmunoglobulinemia Accompanied by Nephrotic Syndrome.

“…We speculated that these suppressor T cells were induced by normal immune reactions against the disordered immunoglobulin production and/or some cytokines such as interferon-y (14) , because the induction of these suppressor T cells is also observed in multiple myeloma patients with characteristic hyperimmuno globulinemia and synthesis of some cytokines (15 (16). In the present case, the renal function was thought to be deteriorating and the therapeutic problem appeared similar to the reported cases due to the evidence of focal glomerulosclerosis (16)(17)(18). However, the combination therapy of ticlopidine with small doses of prednisolone and cyclophosphamide was effective against nephrotic syndrome, and renal function has been kept stable for more than 3 years.…”

A Newer Approach to the Management of Membrano Proliferative Glomerulonephritis – Experience from A Tertiary Level Hospital, Bangladesh

“…Mendoza and co-workers reported a response rate of 65% in patient with SRNS when treated with a regimen of i/v methyl-prednisolone, with alternate day steroid with an oral alkylating agent 16 . Based upon this experience (Mendoza & Tune) we have treated our three patients.…”

Therapy of Idiopathic Nephrotic Syndrome in Adults

“…Nevertheless, oral or intravenous glucocor ticoids, usually given in high dosage (either daily or alter nate day) over prolonged periods of more than 3 months, have been associated with a significant increase in com plete and partial remission rates as compared to untreated patients [1,15,16,44,49,50], This is seen more often in children than in adults [1,15,16]. The overall remission rate with this aggressive glucocorticoid therapeutic ap proach has been in the range of 35-50% [44,49,50].…”