The basic structural lesion of persistent neonatal hypoglycaemia with hyperinsulinism: deficiency of pancreatic D cells or hyperactivity of B cells?

Rahier, Jacques;Fält, K;Müntefering, H;Becker, K.;Falkmer, S.;et.al.
(1984) Diabetologia : clinical and experimental diabetes and metabolism — Vol. 26, n° 4, p. 282-289 (1984)

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  • Rahier, JacquesUCLouvain
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  • Fält, K
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  • Müntefering, H
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  • Becker, K.
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  • Falkmer, S.
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Abstract
Pancreatic tissue obtained at subtotal pancreatectomy from 15 infants with persistent hypoglycaemia with hyperinsulinism, and autopsy specimens from 23 age-matched normoglycaemic controls, were studied with morphometric methods after immunocytochemical staining of the four main islet cell types (A, B, D and pancreatic polypeptide cells). In three cases, a focal lesion was detected by gross examination. Macroscopic or microscopic examination did not distinguish the 12 other cases from controls. As found previously, nesidioblastosis was not a specific feature of the pancreas in infantile hypoglycaemia, being observed in age-matched controls as well. In cases with hypoglycaemia the volume density of B cells was not significantly increased; that of the A cells was within normal range. The volume density of pancreatic polypeptide cells was markedly augmented and that of somatostatin cells was significantly decreased. The mean nuclear volume of the B cells was increased by 40% in cases with diffuse changes, but in cases with a focal lesion this increase was restricted to the abnormal area. This finding is of decisive importance for diagnosis and has therapeutic implications. The increase in B-cell nuclear size is thought to reflect an enhanced functional activity of these cells. On the other hand, the figures obtained for the volume density of B and D cells must be viewed with some reservation because degranulation may interfere with accurate detection of these cells.
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Rahier, J., Fält, K., Müntefering, H., Becker, K., Gepts, W., & Falkmer, S. (1984). The basic structural lesion of persistent neonatal hypoglycaemia with hyperinsulinism: deficiency of pancreatic D cells or hyperactivity of B cells? Diabetologia : clinical and experimental diabetes and metabolism, 26(4), 282-289. https://doi.org/10.1007/BF00283651 (Original work published 1984)