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MANAGEMENT OF ENDOCRINE DISEASE: Paediatric Cushing's disease
Helen L Storr1, Martin O Savage2
1Barts and the London School of Medicine and DentistryWilliam Harvey Research Institute, Centre for Endocrinology, Queen Mary University of London, 1st Floor, John Vane Science Centre, Charterhouse Square, London EC1M 6BQ, UK h.l.storr@qmul.ac.uk.
Insights
Cushing's disease (CD) in children is rare but serious, caused by pituitary tumors secreting ACTH. Early diagnosis and treatment are vital for better outcomes in pediatric patients.
Area of Science:
- Pediatric Endocrinology
- Neuroendocrinology
- Clinical Medicine
Background:
- Cushing's disease (CD) is the most frequent cause of ACTH-dependent Cushing's syndrome.
- It is a rare diagnosis in pediatric and adolescent populations.
- CD in children leads to significant morbidity, underscoring the need for timely intervention.
Purpose of the Study:
- To review the clinical and biochemical characteristics of pediatric CD.
- To evaluate current diagnostic and management strategies for pediatric CD.
- To emphasize the importance of early detection and treatment in children.
Main Methods:
- Literature review of clinical and biochemical features of pediatric Cushing's disease.
- Appraisal of current diagnostic protocols.
- Assessment of established and emerging management approaches.
Main Results:
- Key clinical signs and biochemical markers specific to pediatric CD are highlighted.
- Current diagnostic pathways are critically examined for their effectiveness in children.
- Management strategies, including surgical and medical options, are reviewed.
Conclusions:
- Pediatric Cushing's disease requires a high index of suspicion due to its rarity and significant health implications.
- Accurate diagnosis and prompt treatment are crucial for improving long-term outcomes in affected children.
- Collaboration with adult endocrinology specialists is recommended for optimal patient care, particularly in interpreting complex investigations and defining treatment plans.
Abstract:
Cushing's disease (CD) is the commonest form of ACTH-dependent Cushing's syndrome and is a rare clinical diagnosis in paediatric and adolescent patients. CD is caused by an ACTH-secreting pituitary corticotroph adenoma and is associated with significant morbidity in children; therefore, early diagnosis and treatment are critical for optimal therapeutic outcome. This review highlights the key clinical and biochemical features of paediatric CD and appraises current practices in diagnosis and management. A close liaison with adult endocrinology colleagues, particularly, for interpretation of investigations and definition of therapeutic strategy is strongly advised.
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