Abstract
A term, small for dates (1.7 Kg <3rd centile), female infant developed transient neonatal diabetes (TNDM), requiring insulin from 12 hours post-delivery until the age of 6 months. She then remained well, off insulin, until 13 years when diabetes mellitus was diagnosed with hyperglycaemia, dehydration and mild ketosis. An oral glucose tolerance test (OGTT) aged 15 years ( with insulin therapy withdrawn for test ) shows a raised fasting level of insulin 192 pmol/l (Normal range 30-60) with a blunted insulin response to glucose loading (maximum 385 pmol/l) and a frankly diabetic glucose curve. An OGTT performed on the 18 year old non-diabetic, mildly obese sister reveals an even higher level of fasting insulin (237 pmol/l) and an exaggerated insulin response to glucose loading with a maximum response at 30 minutes of 3024 pmol/l. An OGTT on mother (non-diabetic, grossly obese) shows a similar picture with a raised fasting insulin level (244 pmol/l) and a maximal level of 1156 pmol/l.
Conclusion: This family study indicates that transient neonatal diabetes may be an early manifestation of inherited insulin resistance. We hypothesise that insufficient beta cell reserve associated with intra-uterine growth retardation seen in the family member with TNDM causes an inability to mount the exaggerated insulin response needed to compensate for this inherited defect, especially at times of metabolic stress such as birth and puberty. Study of other cases of TNDM is warranted and in progress.
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Shield, J., O'Rahilly, S. & Baum, J. 208 TRANSIENT NEONATAL DIABETES AS AN EARLY MANIFESTATION OF AN INHERITED INSULIN RESISTANCE SYNDROME?. Pediatr Res 36, 37 (1994). https://doi.org/10.1203/00006450-199407000-00208
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DOI: https://doi.org/10.1203/00006450-199407000-00208