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A Novel Method: Super-selective Adrenal Venous Sampling
Published on: September 15, 2017
Pediatric adrenal insufficiency: thirty years experience at a Portuguese hospital
Sara M F S Mosca1, Tiago S Santos2, Ana C B Mendes1
1Department of Pediatrics, Pediatric Endocrinology Unit, Centro Materno-Infantil do Norte, Centro Hospitalar Universitário do Porto (CMIN-CHUPorto), Porto, Portugal.
Insights
Pediatric adrenal insufficiency (AI) diagnosis is challenging, often presenting with vague symptoms like vomiting and weight loss. Early identification is crucial to prevent life-threatening outcomes in children.
Area of Science:
- Pediatric Endocrinology
- Pediatric Critical Care
- Genetics and Genomics
Background:
- Adrenal insufficiency (AI) is a critical condition due to impaired adrenal hormone secretion.
- AI presents with varied symptoms, often insidious, differing by age in pediatric patients.
- This study aimed to characterize and contrast primary and secondary AI in children.
Purpose of the Study:
- To describe and compare pediatric patients with primary or secondary adrenal insufficiency.
- To analyze clinical presentation, diagnostic categories, and outcomes in pediatric AI.
- To highlight the diagnostic challenges and importance of early AI detection in children.
Main Methods:
- Retrospective analysis of pediatric AI patients over 30 years at a Portuguese tertiary hospital.
- Data collection included family history, age at onset and diagnosis, and clinical/laboratory findings.
- All patients followed in the Pediatric Endocrinology Unit were included.
Main Results:
- Twenty-eight pediatric patients with AI were analyzed; 67.9% were male.
- Common diagnoses included panhypopituitarism (42.9%) and congenital adrenal hyperplasia (25%).
- Vomiting and weight loss were the most frequent initial manifestations (75%); neurodevelopmental issues were more common in secondary AI.
Conclusions:
- Diagnosing and managing pediatric adrenal insufficiency remains a significant clinical challenge.
- A high index of suspicion is essential for early AI identification in children.
- Prompt diagnosis of AI can avert potentially fatal cardiovascular and hemodynamic instability.
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.
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