The DOI matches its Crossref record; 32 of the 32 references it cites were found in a registry or an index.
Hyperinsulinemic hypoglycemia of infancy (nesidioblastosis) in clinical remission: high incidence of diabetes mellitus and persistent beta-cell dysfunction at long-term follow-up
Open accessAbstract
In persistent hyperinsulinemic hypoglycemia of infancy (PHHI), the long term outcome of the disease is not well documented. Previous reports suggested that partial pancreatectomy in infants does not endanger future islet function. We evaluated endocrine pancreatic function in 14 PHHI patients 6.5-21 yr after diagnosis. Eight underwent early subtotal pancreatectomy, and 6 were medically treated; all were in clinical remission. Intravenous glucose tolerance and C-peptide suppression tests were performed, with multiple determinations of hormone levels. The insulin response to glucose was blunted in all pancreatectomized and in 2 conservatively treated patients. Glucose disposal was reduced in 6 pancreatectomized patients and in 2 medically treated subjects. Six of the pancreatectomized patients, including two with normal glucose disposal at first evaluation, developed overt diabetes during puberty. None in the medically treated group became diabetic; however, only 2 patients have reached puberty. C-Peptide suppression in response to hypoglycemia was inadequate in 4 of 5 pancreatectomized and 3 of 5 nonpancreatectomized patients studied. These results show that children with PHHI have impaired insulin responses to glucose and lack of suppressibility of endogenous insulin secretion several years after clinical remission. Thus, the beta-cell defect responsible for the disease in infancy is not corrected with time despite the disappearance of spontaneous hypoglycemia. Insulin secret…
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“While a low carbohydrate diet or α -glucosidase inhibitors are not effective in most patients, the potassium channel activator diazoxide has been shown to alleviate symptoms in some cases [58,59].”
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“While a low carbohydrate diet or α -glucosidase inhibitors are not effective in most patients, the potassium channel activator diazoxide has been shown to alleviate symptoms in some cases [58,59].”
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加齢に伴い組織学的に成熟化の変化を示した Nesidioblastosis の1例
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Congenital hyperinsulinemic hypoglycaemia in a neonate: a rare homozygous ABCC8 gene mutation
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Metadata
- Authors
- Leibowitz, G., Glaser, B., Higazi, A. A., Salameh, M., Cerasi, E., & Landau, H.
- Affiliations
- Hebrew University of Jerusalem (IL)
- Venue
- The Journal of Clinical Endocrinology & Metabolism
- Publisher
- The Endocrine Society
- Published
- 1995-02
- Volume
- 80
- Issue
- 2
- Pages
- 386-392
- ISSN
- 0021-972X (ISSN-L)
- 1945-7197
- OpenAlex
- W1967142752
- Cited by
- 156
- References
- 32
- Citations by year
- 2025: 1 · 2024: 3 · 2023: 5 · 2022: 2 · 2021: 1 · 2020: 2 · 2019: 1 · 2018: 2 · 2017: 2 · 2016: 3 · 2015: 7 · 2014: 5 · 2013: 2 · 2012: 6
- FWCI
- 6.90
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Licence
- cc-by
- Locations
- The Journal of Clinical Endocrinology & Metabolism (publishedVersion, cc-by)
- The Journal of Clinical Endocrinology & Metabolism (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Hadassah Medical Organization
- Topics
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Pancreatic function and diabetes
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Diabetes and associated disorders
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Diabetes Management and Research
- Keywords
- nesidioblastosis, diabetes mellitus, beta-cell dysfunction, subtotal pancreatectomy, glucose tolerance, glucose disposal, insulin secretion, long-term follow-up, puberty, medical treatment
- MeSH terms
- Adolescent, C-Peptide, Child, Diabetes Mellitus, Diazoxide, Female, Follow-Up Studies, Glucose Tolerance Test, Humans, Islets of Langerhans, Male, Pancreatectomy, Pancreatic Diseases, Prospective Studies, Time Factors, Octreotide
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