Abstract
Familial, insulin-dependent diabetes (IDDM) is unusual in a newborn. A 27 year old Caucasian Class F diabetic whose onset was at 10 wks of age was delivered of a 2100 g male at 36 wks gestation. By 5 days plasma glucose was elevated (242 mg/dl) and thereafter ranged from 350-550 without ketonuria. Islet cell surface antibodies were negative (Chicago). At six weeks an I.V. tolbutamide tolerance test (20 mg/kg) produced a fall in plasma glucose and rise in C-peptide (max. 1.75 pmol/ml at 40 min.). P1. cortisol and GH were normal, while glucagon was low (<50 pg/ml). Glucose production was elevated to 10 mg/kg/min (nl: 3.8±0.1, M±SE) by the D-[U-13C] glucose primed infusion technique. Although growth was adequate, insulin therapy was initiated at 3 mos. when ketonuria was associated with a URI. A repeat tolbutamide test at 4 mos. had a minimal C-peptide response (0.35 pmol/ml). Insulin binding (NIH) on Rbc's, cultured B-lymphocytes and fibroblasts from mother and baby was normal. Rat adipocytes metabolized labelled glucose normally in the presence of infant serum. HLA lymphocyte typing (Boston) of the family revealed no antigens commonly associated with IDDM (Dr3 and Dr4 were absent). With insulin therapy, growth and development have continued normal to 18 mos. The etiology of this early onset familial diabetes does not appear to be HLA linked, nor due to an abnormality in insulin receptors or in insulin sensitivity.
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