Long-Term Outcome and Treatment in Persistent and Transient Congenital Hyperinsulinism : A Finnish Population-Based Study

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Männistö , J M E , Jääskeläinen , J , Otonkoski , T & Huopio , H 2021 , ' Long-Term Outcome and Treatment in Persistent and Transient Congenital Hyperinsulinism : A Finnish Population-Based Study ' , Journal of Clinical Endocrinology and Metabolism , vol. 106 , no. 4 , pp. E1542-E1551 . https://doi.org/10.1210/clinem/dgab024

Title: Long-Term Outcome and Treatment in Persistent and Transient Congenital Hyperinsulinism : A Finnish Population-Based Study
Author: Männistö, Jonna M. E.; Jääskeläinen, Jarmo; Otonkoski, Timo; Huopio, Hanna
Contributor organization: Helsinki One Health (HOH)
STEMM - Stem Cells and Metabolism Research Program
Centre of Excellence in Stem Cell Metabolism
HUS Children and Adolescents
Clinicum
Research Programs Unit
Timo Pyry Juhani Otonkoski / Principal Investigator
Children's Hospital
University of Helsinki
Helsinki University Hospital Area
Date: 2021-04
Language: eng
Number of pages: 10
Belongs to series: Journal of Clinical Endocrinology and Metabolism
ISSN: 0021-972X
DOI: https://doi.org/10.1210/clinem/dgab024
URI: http://hdl.handle.net/10138/331880
Abstract: Context: The management of congenital hyperinsulinism (CHI) has improved. Objective: To examine the treatment and long-term outcome of Finnish patients with persistent and transient CHI (P-CHI and T-CHI). Design: A population-based retrospective study of CHI patients treated from 1972 to 2015. Patients: 106 patients with P-CHI and 132 patients with T-CHI (in total, 42 diagnosed before and 196 after year 2000) with median follow-up durations of 12.5 and 6.2 years, respectively. Main outcome measures: Recovery, diabetes, pancreatic exocrine dysfunction, neurodevelopment. Results: The overall incidence of CHI (n = 238) was 1:11 300 live births (1972-2015). From 2000 to 2015, the incidence of P-CHI (n = 69) was 1:13 500 and of T-CHI (n = 127) 1:7400 live births. In the 21st century P-CHI group, hyperinsulinemic medication was initiated and normoglycemia achieved faster relative to earlier. Of the 74 medically treated P-CHI patients, 68% had discontinued medication. Thirteen (12%) P-CHI patients had partial pancreatic resection and 19 (18%) underwent near-total pancreatectomy. Of these, 0% and 84% developed diabetes and 23% and 58% had clinical pancreatic exocrine dysfunction, respectively. Mild neurological difficulties (21% vs 16%, respectively) and intellectual disability (9% vs 5%, respectively) were as common in the P-CHI and T-CHI groups. However, the 21st century P-CHI patients had significantly more frequent normal neurodevelopment and significantly more infrequent diabetes and pancreatic exocrine dysfunction compared with those diagnosed earlier. Conclusions: Our results demonstrated improved treatment and long-term outcome in the 21st century P-CHI patients relative to earlier.
Subject: hypoglycemia
neurodevelopment
pancreatic exocrine dysfunction
diabetes
recovery
PANCREATIC EXOCRINE
CLINICAL-FEATURES
HYPOGLYCEMIA
CHILDREN
MUTATION
INFANCY
GENE
DIAGNOSIS
RISK
MANAGEMENT
3121 General medicine, internal medicine and other clinical medicine
Peer reviewed: Yes
Rights: cc_by_nc_nd
Usage restriction: openAccess
Self-archived version: acceptedVersion


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