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Cushing's disease in children
1Division of Endocrinology, Albany Medical College, New York.
Insights
Diagnosing Cushing's disease in children presents unique challenges, impacting growth and development. Effective treatments, including transsphenoidal surgery (TSS), offer good outcomes for childhood Cushing's disease.
Area of Science:
- Pediatric Endocrinology
- Neuroendocrinology
- Pediatric Oncology
Background:
- Cushing's disease diagnosis in children is challenging, with distinct effects on growth and puberty.
- The pediatric presentation differs from adults, necessitating specific diagnostic and management approaches.
Purpose of the Study:
- To review the diagnostic and therapeutic challenges of Cushing's disease in pediatric patients.
- To highlight the effectiveness and safety of various treatment modalities in children.
Main Methods:
- Review of clinical presentations and diagnostic criteria for pediatric Cushing's disease.
- Analysis of treatment outcomes, including surgery, medical therapy, and radiation, in children.
- Comparison of pediatric and adult Cushing's disease progression and treatment response.
Main Results:
- Pediatric Cushing's disease exhibits a more aggressive clinical course than in adults, with higher rates of Nelson's syndrome and recurrence.
- Transsphenoidal surgery (TSS) is well-tolerated in children with fewer side effects compared to other treatments.
- Advanced techniques like proton knife therapy show promise for improved efficacy and reduced side effects in pediatric cases.
Conclusions:
- Cushing's disease in children requires tailored diagnostic and treatment strategies.
- TSS is a primary surgical option for pediatric Cushing's disease, with potential for satisfactory long-term psychosocial adjustment and fertility.
- Emerging therapies may offer enhanced outcomes for childhood Cushing's disease.
Abstract:
As with adults, the diagnosis of Cushing's disease in childhood can be difficult. The clinical presentation in children is distinctive in terms of the effects on growth and pubertal development. The biochemical diagnosis can usually be made with standard dexamethasone suppression testing, similar to such testing in adults. The clinical course of Cushing's disease in children tends to be more aggressive than in adults, as evidenced by higher rates of Nelson's syndrome after bilateral adrenalectomy and higher rates of recurrence after removal of corticotrope adenomas. TSS is well tolerated in children and has fewer side effects than other modalities. Currently, pituitary irradiation is best reserved as adjunctive therapy. Newer stereotactic computer assisted linear accelerator techniques (e.g., proton knife) may provide increased effectiveness with fewer side effects and be particularly useful in childhood Cushing's disease. Appropriate treatment of children with Cushing's disease can be compatible with satisfactory psychosocial adjustment and fertility in adulthood.