Congenital adrenal hyperplasia: transitional care.
Congenital adrenal hyperplasia (CAH) is a life-long disorder which poses management problems that are age- and sex-specific. The condition merits an organised, multi-disciplinary transitional care format similar to the kind that is now well established for Turner's syndrome in many centres. In the eyes of the paediatrician, achieving optimal growth is the primary target of CAH management during infancy and childhood. Fixation on this objective can be to the detriment of the patient because it may result in failure to appreciate the significance of metabolic disturbances that occur in later childhood, particularly in females, and which may be the progenitor of chronic problems with obesity, insulin resistance and infertility in adult life. Similarly, the care of the adult patient with CAH comprises more than just prescribing steroid replacement for primary adrenal insufficiency. The transition period between childhood and adulthood is an opportune time for review of the various management options and to assess the efficacy of steroid replacement, to consider alternative novel treatment modalities and to apply a checklist to the multi-faceted aspects of the medical, surgical and psychological needs of the patient.
Hughes
Ieuan A
IA
Department of Paediatrics, University of Cambridge, Addenbrooke's Hospital, P.O. Box 116, Hills Road, Cambridge CB2 2QQ, UK. iah1000@cam.ac.uk
D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital / Q000150:complications / Q000175:diagnosis / Q000628:therapy*; D000328:Adult; D000367:Age Factors; D002648:Child; D003266:Continuity of Patient Care; D005260:Female; D006801:Humans; D008297:Male