Abstract
A term newborn girl with FGR (1616 gm) and phenotypic features of Leprechaunism had hyperglycemia and marked persistent hyperinsulinism (232-3000 uU/ml). After sudden death at 7 weeks, postmortem exam revealed findings nearly identical to those described by Donahue and Uchida (J. Pediatr. 45:505, 1954). Studies were undertaken to distinguish between an abnormal insulin and an abnormal cellular response to insulin. Evidence that the patient's immunoreactive insulin (IRI) behaved chemically and biologically like normal insulin is as follows: (1)By gel chromatography, 92% of the IRI migrated as insulin; 8% as proinsulin. (2)Serum IRI reacted normally with insulin receptors in human placental cell membranes. (3)Serum IRI was degraded at a normal rate by a crude preparation of insulin glucagon protease. (4) Serum insulin-like activity in a rat fat pad bioassay was proportional to serum IRI. The patient apparently had normal insulin, receptors, since her skin fibroblasts could specifically bind 125I insulin as well as those of newborn controls (2-7%/1×10 cells). On the other hand, preliminary studies indicate that the patient's fibroblasts had a significantly reduced capacity to incorporate H-thymidine in response to either insulin or serum. These results infer that the explanation for this patient's hyperinsulinism, insulin resistance, and presumably FGR rests with a defective intracellular response to insulin and, possibly to other growth factors.
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D'ercole, A., Underwood, L., Groelke, J. et al. FETAL GROWTH RETARDATION (FGR) AND HYPERINSULINISM: EVIDENCE FOR AN ABERRANT INTRACELLULAR RESPONSE TO INSULIN. Pediatr Res 11, 513 (1977). https://doi.org/10.1203/00006450-197704000-00858
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DOI: https://doi.org/10.1203/00006450-197704000-00858