Challenges in management of transient hyperinsulinism - a retrospective analysis of 36 severely affected children.
congenital hyperinsulinism
diazoxide
hypoglycemia
transient hyperinsulinism
Journal
Journal of pediatric endocrinology & metabolism : JPEM
ISSN: 2191-0251
Titre abrégé: J Pediatr Endocrinol Metab
Pays: Germany
ID NLM: 9508900
Informations de publication
Date de publication:
27 Jul 2021
27 Jul 2021
Historique:
received:
06
11
2020
accepted:
12
02
2021
pubmed:
17
4
2021
medline:
16
12
2021
entrez:
16
4
2021
Statut:
epublish
Résumé
Transient hyperinsulinism (THI) is a hypoglycemia disorder which resolves spontaneously within the first weeks or months of life. The pathomechanism of THI is not elucidated yet; however, it is known that perinatal stress predisposes for THI. We aimed to characterize the clinical phenotype and treatment of children with THI, and to identify options for improved management. A retrospective analysis of 36 children with THI treated at the University Children's Hospital Düsseldorf between 2007 and 2019 was performed. All children had risk factors for neonatal hypoglycemia or indicators of perinatal stress. Eighty three percent were diagnosed with hypoglycemia on day of life (DOL)1. None of the six diagnosed later had routine blood glucose screening and showed significantly lower blood glucose levels at the time of first blood glucose measurement compared to the children diagnosed on DOL1. Ninety seven percent of all children received intravenous glucose, 42% received continuous glucagon and 81% were started on diazoxide. Diazoxide withdrawal and subsequent fasting tests lacked standardization and were based on clinical experience. Three patients had a subsequent episode of hypoglycemia, after fasting studies only demonstrated "clinical" remission without proving the ability to ketogenesis. Any kind of perinatal stress might pose a risk to develop THI, and postnatal monitoring for hypoglycemia still needs to be improved. Diazoxide is effective in children with THI; however, further studies are needed to guide the development of criteria and procedures for the initiation and discontinuation of treatment. Furthermore, establishing consensus diagnostic criteria/definitions for THI would improve comparability between studies.
Identifiants
pubmed: 33860651
pii: jpem-2020-0639
doi: 10.1515/jpem-2020-0639
doi:
Substances chimiques
Diazoxide
O5CB12L4FN
Octreotide
RWM8CCW8GP
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
867-875Informations de copyright
© 2021 Walter de Gruyter GmbH, Berlin/Boston.
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