Disorders of Adrenal Glands and Sex Development in Children: Insights from the Tropics

The adrenal gland is essential for survival and its function is compartmentalized into specific zones. Disorders of the adrenal gland can be classified as those affecting the adrenal cortex or medulla. Pediatric adrenal disorders can have distinct presentations and etiologies in comparison to adults, such as adrenal insufficiency associated with genetic syndromes or Cushing’s syndrome associated with adrenocortical tumors and primary pigmented nodular adrenocortical disease. Congenital adrenal hyperplasia (CAH) has been commonly reported from the tropics, and rare variants of CAH have also been recognized in populations where consanguinity is prevalent. Pheochromocytomas and paragangliomas (PPGL) have been reported from tropical countries, some with rare presentations. The frequent rate of heritability and mutations in PPGL highlights the importance of genetic studies among children. The role of functional imaging is evolving for PPGLs as data is emerging from cohort studies. Disorders of Sex Development (DSD) comprise a heterogeneous group of disorders that can present in any age group. DSDs in childhood usually present with ambiguous genitalia and a multidisciplinary approach is required for its management. The diagnosis of adrenal disorders can sometimes pose a challenge in tropical countries due to resource constraints, lack of awareness, and access to medical care. However, available data from cohort studies and case reports have highlighted differences in etiology and presentation as compared to other parts of the world and the need for further studies. For complete coverage of all related areas of Endocrinology, please visit our on-line FREE web-text, WWW.ENDOTEXT.ORG.