دورية أكاديمية

Recurrent focal segmental glomerulosclerosis in pediatric renal transplant recipients: successful treatment with oral cyclophosphamide.

التفاصيل البيبلوغرافية
العنوان: Recurrent focal segmental glomerulosclerosis in pediatric renal transplant recipients: successful treatment with oral cyclophosphamide.
المؤلفون: Kershaw DB; Department of Pediatrics, University of Michigan Medical Center, Ann Arbor., Sedman AB, Kelsch RC, Bunchman TE
المصدر: Clinical transplantation [Clin Transplant] 1994 Dec; Vol. 8 (6), pp. 546-9.
نوع المنشور: Case Reports; Journal Article; Research Support, U.S. Gov't, P.H.S.
اللغة: English
بيانات الدورية: Publisher: Munksgaard Country of Publication: Denmark NLM ID: 8710240 Publication Model: Print Cited Medium: Print ISSN: 0902-0063 (Print) Linking ISSN: 09020063 NLM ISO Abbreviation: Clin Transplant Subsets: MEDLINE
أسماء مطبوعة: Original Publication: Copenhagen : Munksgaard,
مواضيع طبية MeSH: Kidney Transplantation*, Cyclophosphamide/*administration & dosage , Glomerulosclerosis, Focal Segmental/*drug therapy , Glomerulosclerosis, Focal Segmental/*surgery, Administration, Oral ; Child ; Drug Administration Schedule ; Follow-Up Studies ; Glomerulosclerosis, Focal Segmental/epidemiology ; Humans ; Immunosuppression Therapy ; Male ; Recurrence ; Retrospective Studies ; Time Factors
مستخلص: Focal segmental glomerulosclerosis (FSGS) is the most common glomerulopathy leading to end-stage renal disease in children and transplantation is complicated by recurrent disease in a significant percentage of children. Treatment of recurrent FSGS has included high-dose steroids, high-dose cyclosporine (CSA), plasmapheresis, and ACE inhibitors with mixed results. We have had a consistent approach using oral cyclophosphamide (CTX) to treat recurrent FSGS since 1982. Three patients with ESRD secondary to nephrotic syndrome had recurrent disease. Biopsies in all 3 were consistent with recurrent FSGS. Patients were begun on a 8-12 week course of 1-2 mg/kg/day of CTX and dosage was adjusted for WBC count. Azathioprine was with held during CTX. Patients' dosage at the end of 12 weeks ranged from 0.89-1.75 mg/kg/day. All patients tolerated CTX well. After 8-12 weeks of treatment, 2 patients with nephrotic syndrome normalized their serum albumin and had negative to trace protein on urinary dipstick. One patient with proteinuria decreased his protein excretion from 770 to 340 mg/m2/day. At follow-up at 8, 38, and 125 months post-transplant, these 3 patients have stable graft function and negative to trace protein on urinalysis. The patient followed for 125 months has had 2 additional relapses at 51 and 82 months post-transplant that were treated successfully with pulse intravenous steroids. Three pediatric patients with recurrent focal segmental glomerulosclerosis post-renal transplant were treated with oral CTX and had significant improvement in proteinuria and preservation of graft function. This suggests that oral CTX is a potentially effective and well-tolerated treatment for recurrent FSGS in children.
معلومات مُعتمدة: DK7378-11A1 United States DK NIDDK NIH HHS
المشرفين على المادة: 8N3DW7272P (Cyclophosphamide)
تواريخ الأحداث: Date Created: 19941201 Date Completed: 19950330 Latest Revision: 20211203
رمز التحديث: 20221213
PMID: 7865917
قاعدة البيانات: MEDLINE