Pediatric adrenal insufficiency: thirty years experience at a Portuguese hospital
Author:
Mosca Sara M. F. S.1ORCID, Santos Tiago S.2ORCID, Mendes Ana C. B.1, Ribeiro Luís F. M.1ORCID, Freitas Joana M. C.1, Oliveira Maria J. R.1ORCID, Rocha Carla A.1, Borges Teresa M. S.1ORCID
Affiliation:
1. Department of Pediatrics, Pediatric Endocrinology Unit , Centro Materno-Infantil do Norte, Centro Hospitalar Universitário do Porto (CMIN-CHUPorto) , Porto , Portugal 2. Department of Endocrinology, Diabetes and Metabolism , CHUPorto , Porto , Portugal
Abstract
Abstract
Background
Adrenal insufficiency (AI) is a life-threatening condition caused by an impaired secretion of the adrenal glucocorticoid and mineralocorticoid hormones. It comprises a heterogeneous group of primary, secondary and acquired disorders. Presentation differs according to the child’s age, but it usually presents with nonspecific and insidious symptoms and signs. The main purpose of this study was to describe and compare patients with primary or secondary AI.
Methods
Retrospective analysis of all patients with adrenal insufficiency followed at the Pediatric Endocrinology Unit in a tertiary care Portuguese hospital over the last 30 years. Data on family history, age at the first manifestation and at etiological diagnosis, and clinical presentation (symptoms, signs and laboratory evaluation) was gathered for all patients.
Results
Twenty-eight patients with AI were included; 67.9% were male, with a median (25th–75th percentile, P25–P75) age of 1 (0.5–36) month at the first presentation. The principal diagnostic categories were panhypopituitarism (42.9%) and congenital adrenal hyperplasia (25%). The most frequent manifestations (75%) were vomiting and weight loss. They were followed for a median (P25–P75) period of 3.5 (0.6–15.5) years. In respect to neurodevelopmental delay and learning difficulties, they were more common in the secondary AI group.
Conclusions
Despite medical advances, the diagnosis and management of AI remains a challenge, particularly in the pediatric population, and clinicians must have a high index of suspicion. An early identification of AI can prevent a potential lethal outcome, which may result from severe cardiovascular and hemodynamic instability.
Publisher
Walter de Gruyter GmbH
Subject
Endocrinology,Endocrinology, Diabetes and Metabolism,Pediatrics, Perinatology and Child Health
Reference23 articles.
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