Growth in patients with classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency. 2007

Helmuth G Dörr
Division of Pediatric Endocrinology, University Hospital for Children and Adolescents, Erlangen, Germany. helmuth-guenther.doerr@kinder.imed.uni-erlangen.de

BACKGROUND One important goal in the management of children with classic congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is to achieve normal growth. Reviewing available data published over the last few years on growth and height outcomes in CAH patients, it becomes evident that an acceptable height can be achieved by many CAH patients. However, linear growth and final adult height may be stunted in some patients due to factors related to the timing of diagnosis, the age at therapy onset, the start of therapy, the adequacy of metabolic control, the quality of therapy, patient compliance and the experience of the treating physician. In children with CAH who have a poor height prognosis, additional treatment options should be considered. CONCLUSIONS Treatment of children with CAH requires individualized approaches to prevent long-term growth failure.

UI MeSH Term Description Entries
D001827 Body Height The distance from the sole to the crown of the head with body standing on a flat surface and fully extended. Body Heights,Height, Body,Heights, Body
D002657 Child Development The continuous sequential physiological and psychological maturing of an individual from birth up to but not including ADOLESCENCE. Infant Development,Development, Child,Development, Infant
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D004334 Drug Administration Schedule Time schedule for administration of a drug in order to achieve optimum effectiveness and convenience. Administration Schedule, Drug,Administration Schedules, Drug,Drug Administration Schedules,Schedule, Drug Administration,Schedules, Drug Administration
D006128 Growth Gradual increase in the number, the size, and the complexity of cells of an individual. Growth generally results in increase in ORGAN WEIGHT; BODY WEIGHT; and BODY HEIGHT.
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000312 Adrenal Hyperplasia, Congenital A group of inherited disorders of the ADRENAL GLANDS, caused by enzyme defects in the synthesis of cortisol (HYDROCORTISONE) and/or ALDOSTERONE leading to accumulation of precursors for ANDROGENS. Depending on the hormone imbalance, congenital adrenal hyperplasia can be classified as salt-wasting, hypertensive, virilizing, or feminizing. Defects in STEROID 21-HYDROXYLASE; STEROID 11-BETA-HYDROXYLASE; STEROID 17-ALPHA-HYDROXYLASE; 3-beta-hydroxysteroid dehydrogenase (3-HYDROXYSTEROID DEHYDROGENASES); TESTOSTERONE 5-ALPHA-REDUCTASE; or steroidogenic acute regulatory protein; among others, underlie these disorders. Congenital Adrenal Hyperplasia,Hyperplasia, Congenital Adrenal,Adrenal Hyperplasias, Congenital,Congenital Adrenal Hyperplasias,Hyperplasias, Congenital Adrenal
D013256 Steroids A group of polycyclic compounds closely related biochemically to TERPENES. They include cholesterol, numerous hormones, precursors of certain vitamins, bile acids, alcohols (STEROLS), and certain natural drugs and poisons. Steroids have a common nucleus, a fused, reduced 17-carbon atom ring system, cyclopentanoperhydrophenanthrene. Most steroids also have two methyl groups and an aliphatic side-chain attached to the nucleus. (From Hawley's Condensed Chemical Dictionary, 11th ed) Steroid,Catatoxic Steroids,Steroids, Catatoxic
D035703 Therapies, Investigational Treatments which are undergoing clinical trials or for which there is insufficient evidence to determine their effects on health outcomes; coverage for such treatments is often denied by health insurers. Investigational Therapies,Experimental Therapies,Innovative Therapies,Investigational Treatments,Experimental Therapy,Innovative Therapy,Investigational Therapy,Investigational Treatment,Therapies, Experimental,Therapies, Innovative,Therapy, Experimental,Therapy, Innovative,Therapy, Investigational,Treatment, Investigational,Treatments, Investigational

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