© Freund Publishing House Ltd., London Journal of Pediatric Endocrinology & Metabolism, 19, 1045-1048 (2006) Type 1 Diabetes Mellitus Associated with Nephrotic Syndrome Pinar Isik Agras, Sibel Tulgar Kinik, Nurcan Cengiz and Esra Baskin Department of Pediatric Nephrology, Baskent University, Ankara, Turkey ABSTRACT Nephrotic syndrome (NS) in a patient with diabetes mellitus (DM) first suggests the diagnosis of diabetic nephropathy. However, glomerular diseases other than diabetic nephro- pathy have been reported in patients with DM. We present a child with type 1 DM (DM1) associated with NS. A 3 year-old boy who was diagnosed with DM1 developed proteinuria in nephrotic range at the 10 lh month of follow-up. He had remission on steroid treatment without any problem in glycemic control as he was given tapered daily doses instead of an alternate day regimen. He relapsed at the 7 ,h month of follow- up, and cyclophosphamide treatment brought about remission. He had HLA A24, DR4 and DRS3 antigens in common with previously reported cases of DM-NS association. The immunological basis of these diseases may have a causal effect on the association, but the etiopathogenesis is still unclear. KEY WORDS type 1 diabetes mellitus, nephrotic syndrome Reprint address: Pinar Isik Agras, M.D. Baskent University Department of Pediatric Nephrology 6. Cadde No:72/3 06490, Bahcelievler, Ankara, Turkey e-mail: pinaris2001@yahoo.com INTRODUCTION Type 1 diabetes mellitus (DM1) has an estima- ted prevalence of 1.9/1,000 with a peak presentation at ages 5-7 years and puberty 1 . Renal disease is a frequent late complication of DM1, occurring almost entirely in adult patients 2 . Typical diabetic nephropathy (DN) is characterized by proteinuria, and by the histological lesions of mesangial expansion and basement membrane thickening 2 . However, glomerular pathologies other than DN have been reported in patients with DM1 2 ' 3 . Idiopathic nephrotic syndrome (NS) is the most frequent type of NS in children, and minimal change disease (MCD) accounts for more than 90% of steroid responsive NS 3 . There are limited reports of an association between DM1 and NS in child- hood. We report here a child with DM1 associated with NS. PATIENT REPORT A 3 year-old boy presented with complaints of polyuria and polydypsia. He was diagnosed to have diabetic ketosis and DM1. Further laboratory investigations showed normal renal and liver function tests and urinalysis with no evidence of proteinuria. Anti-insulin, antigliadin, antiendomy- sial and islet cell antibodies were all negative, while antiglutamic acid decarboxylase antibodies were positive. He was discharged on subcutaneous insulin treatment with multiple injections. For 10 months of uneventful follow up, urinary protein/ creatinine ratio and microalbumin were within normal limits. His HbA| C levels were 6.8%, 8.4%, 6.7%, and 6.9% at the 3 rd , 6 th , 8 th , and 10 th months, respectively. At the end of the 10 th month, he presented with complaints of swelling of the eyelids for the last 10 days with no change in urine color or volume. Family history revealed type 2 DM in the maternal grandfather and mother's uncle. Physical examination revealed body weight VOLUME 19, NO. 8, 2006 1045 Brought to you by | University of California - San Francisco Authenticated Download Date | 2/20/15 7:08 AM