Long-Term Outcome and Treatment in Persistent and Transient Congenital Hyperinsulinism: A Finnish Population-Based Study.
J Clin Endocrinol Metab
; 106(4): e1542-e1551, 2021 03 25.
Article
em En
| MEDLINE
| ID: mdl-33475139
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 OUTCOMEMEASURES:
Recovery, diabetes, pancreatic exocrine dysfunction, neurodevelopment.RESULTS:
The overall incidence of CHI (nâ =â 238) was 111 300 live births (1972-2015). From 2000 to 2015, the incidence of P-CHI (nâ =â 69) was 113 500 and of T-CHI (nâ =â 127) 17400 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.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Hiperinsulinismo Congênito
Tipo de estudo:
Observational_studies
/
Risk_factors_studies
Limite:
Adolescent
/
Adult
/
Child
/
Child, preschool
/
Female
/
Humans
/
Infant
/
Male
País/Região como assunto:
Europa
Idioma:
En
Revista:
J Clin Endocrinol Metab
Ano de publicação:
2021
Tipo de documento:
Article
País de afiliação:
Finlândia