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Paediatric Cushing's syndrome: epidemiology, investigation and therapeutic advances
Helen L Storr1, Li F Chan, Ashley B Grossman
1Department of Endocrinology, William Harvey Research Institute, Barts and the London, Queen Mary's School of Medicine and Dentistry, John Vane Science Centre, Charterhouse Square, London, EC1M 6BQ, UK.
Insights
Cushing
Area of Science:
- Pediatric Endocrinology
- Endocrinology
- Metabolic Disorders
Background:
- Cushing's syndrome (CS) is rare in children, posing diagnostic and therapeutic challenges.
- Paediatric endocrinologists often lack extensive experience managing pediatric CS.
- Management requires protocols similar to adult CS, adapted for pediatric needs.
Purpose of the Study:
- To provide a comprehensive overview of pediatric Cushing's syndrome.
- To guide the diagnosis and treatment strategies for pediatric CS.
- To address long-term management challenges for growth, bone health, and reproduction.
Main Methods:
- Review of epidemiology and diagnostic approaches for pediatric CS.
- Critical appraisal of treatment strategies for ACTH-independent and ACTH-dependent CS.
- Discussion of post-cure management for optimizing long-term outcomes.
Main Results:
- CS in children requires specialized diagnostic protocols.
- Effective treatment strategies exist for both ACTH-independent and ACTH-dependent CS.
- Long-term follow-up is crucial for addressing growth, bone health, and reproductive issues.
Conclusions:
- Pediatric Cushing's syndrome necessitates tailored diagnostic and therapeutic protocols.
- Management requires a multidisciplinary approach, drawing from adult CS expertise.
- Optimizing long-term health outcomes in pediatric CS patients is essential.
Abstract:
Cushing's syndrome (CS), which is caused by excessive circulating glucocorticoid concentrations, is rare in the paediatric age range but presents a diagnostic and therapeutic challenge. Most paediatric endocrinologists have limited experience of managing children or adolescents with CS and thus benefit from close consultation with colleagues who treat adult patients. A protocol for investigation is required that broadly follows the model for adult patients. Here, the epidemiology and diagnosis of different causes of CS are discussed according to typical age of presentation. Treatment strategies for adrenocorticotrophic hormone (ACTH)-independent and ACTH-dependent CS are described and critically appraised. The management of paediatric CS patients after cure also presents challenges for optimizing growth, bone health, reproduction and body composition from childhood into and during adult life.
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