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Pancreatic pathology in hyperinsulinemic hypoglycemia of infancy
Insights
Idiopathic hyperinsulinemic hypoglycemia in infants is linked to two pancreatic abnormalities: islet cell adenomatosis and a newly identified "endocrine cell dysplasia." This dysplasia involves altered islet structure and cell distribution, not increased endocrine tissue.
Area of Science:
- Pediatric Endocrinology
- Pancreatic Pathology
- Developmental Biology
Background:
- Idiopathic hyperinsulinemic hypoglycemia (IHH) is a significant cause of persistent hypoglycemia in neonates and infants.
- The underlying pancreatic morphology in IHH is not fully understood, with previous studies focusing on nesidioblastosis and adenomas.
Purpose of the Study:
- To investigate the pancreatic histopathology in infants with IHH using advanced immunostaining techniques.
- To identify and characterize novel morphologic abnormalities beyond adenomatosis and nesidioblastosis.
Main Methods:
- Histochemical and immunostaining analysis of pancreatic tissue from 10 infants with IHH (newborn to 9 months).
- Quantitative assessment of islet size, cell-type distribution, and total endocrine tissue area.
- Comparison of findings with age-matched control pancreases.
Main Results:
- Four out of ten patients exhibited islet cell adenomatosis (focal or generalized).
- The remaining six patients presented with a novel condition termed 'endocrine cell dysplasia,' characterized by disrupted islet architecture, irregular contours, and dispersed endocrine cell clusters.
- Endocrine cell dysplasia was not associated with an increased total endocrine tissue area compared to controls; nesidioblastosis was present in both patients and controls and decreased with age.
Conclusions:
- Idiopathic hyperinsulinemic hypoglycemia in infants can be attributed to islet cell adenomatosis or a newly described endocrine cell dysplasia.
- Endocrine cell dysplasia represents a distinct morphologic substrate for IHH, characterized by architectural changes rather than hyperplasia.
- Nesidioblastosis is a common finding in infant pancreases and does not appear to be the primary cause of hyperinsulinism in this age group.
Abstract:
Pancreas from 10 children with idiopathic hyperinsulinemic hypoglycemia was examined using histochemical and immunostaining techniques. The children ranged from newborn to 9 months in age. Sections were studied with particular reference to islet cell distribution in patients and controls, and quantitative assessments were made of islet size, relative cell-type distribution, and total area of pancreas occupied by endocrine tissue. Four had islet cell adenomatosis, three of these focal and one generalized. The others had a subtle morphologic abnormality seen best on immunostained sections and characterized by loss of the usual centrilobular congregation, irregular islet contours, a generalized of small packets of endocrine cells throughout the acinar tissue, and islet cell hypertrophy. We have termed this constellation "endocrine cell dysplasia." The range of islet cell area found in the controls using immunostaining was substantially higher than previously reported. In addition, we found no increase in mean total endocrine area in the cases with endocrine dysplasia when compared to age-matched controls. Both classic and beta-cell nesidioblastosis were common to patients and controls alike, appeared to decrease with age, and thus could not be considered as the morphologic substrate of hyperinsulinism in this age group.