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Work-up and management of paediatric Cushing's syndrome
Martin O Savage1, Li F Chan, Ashley B Grossman
1Department of Endocrinology, William Harvey Research Institute, Barts and the London School of Medicine and Dentistry, London, UK. m.o.savage@qmul.ac.uk
Insights
Pediatric Cushing's syndrome is rare but serious. Early diagnosis and treatment are vital for good outcomes, though challenges in growth and body composition persist.
Area of Science:
- Pediatric Endocrinology
- Endocrinology
- Pediatric Cushing's Syndrome
Background:
- Cushing's syndrome in children and adolescents is uncommon but leads to significant morbidity.
- Management requires specialized expertise, often necessitating collaboration between pediatric endocrinologists and adult specialists.
Purpose of the Study:
- To present a diagnostic protocol for pediatric Cushing's syndrome.
- To outline management principles for this condition in young patients.
Main Methods:
- Utilizes classical endocrine studies alongside novel genetic and advanced imaging techniques.
- Reviews current therapeutic strategies, including surgical and radiological interventions.
Main Results:
- Trans-sphenoidal surgery has significantly improved outcomes for Cushing's disease, though pediatric surgical experience is limited.
- Pituitary radiotherapy serves as an effective second-line treatment option.
Conclusions:
- Prompt diagnosis and intervention are crucial for optimal long-term prognosis in pediatric Cushing's syndrome.
- Ensuring normal growth and body composition post-treatment remains a key challenge.
Purpose Of Review:
Paediatric Cushing's syndrome presents a diagnostic and therapeutic challenge. Most paediatric endocrinologists have limited experience in managing children or adolescents with Cushing's syndrome and thus benefit from close consultation with adult colleagues. A protocol for investigation of the child with suspected Cushing's syndrome is presented followed by principles of management.
Recent Findings:
Cushing's syndrome is rare in childhood, but causes serious morbidity. Investigations have evolved and now include new genetic and imaging techniques as well as classical endocrine studies. In Cushing's disease trans-sphenoidal surgery has transformed management, although only a few surgeons have experience in children. Pituitary radiotherapy is effective second-line therapy.
Summary:
Early diagnosis and treatment of Cushing's syndrome is vital for long-term outcome. The overall prognosis for Cushing's syndrome is good but challenges remain to ensure normal postcure growth and body composition.
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