Abstract
Two sibs of a consanguineous union were born with severe intrauterine growth retardation and agenesis of the pancreas confirmed at autopsy. We report the association between the growth failure and absence of fetal insulin and glucagon, and some of the changes in somatic and cell growth occurring in one of the infants at age 6 weeks. Birth weight 1280 gm at term; length 37 cm; head circ. 29 cm; (all <3rd centile). The brain weighed 214 gm (<3rd centile), and the liver weighed 95.7 gm (<25th centile). All other visceral organs were extremely small, particularly the adrenals whose combined weight of 1 gm was less than that of a 24-week old fetus. The total DNA in the cerebrum was 172 mg (N=258), in the cerebellum 95.6 mg (N=120), and in the liver 200 mg (N=320), respectively, indicating a reduction in the cell number in each of these organs. Protein/DNA ratio was normal or increased in brain and liver indicating a relatively greater reduction in DNA content than in protein. In muscle, the total DNA was reduced as calculated from muscle mass and the protein/DNA ratio of 58 was extremely low (N=100). The results in brain and muscle were similar to those seen in infants with severe postnatal marasmus, and suggest that insulin and glucagon are key hormones for normal fetal growth.
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Sherwood, W., Chance, G., Hill, D. et al. A NEW SYNDROME OF FAMILIAL PANCREATIC AGENESIS: THE ROLE OF INSULIN AND GLUCAGON IN SOMATIC AND CELL GROWTH. Pediatr Res 8, 360 (1974). https://doi.org/10.1203/00006450-197404000-00120
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DOI: https://doi.org/10.1203/00006450-197404000-00120
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