Antenatal treatment for classic 21-hydroxylase forms of congenital adrenal hyperplasia and the issues
نویسندگان
چکیده
منابع مشابه
Bilateral laparoscopic adrenalectomy as a treatment for classic congenital adrenal hyperplasia attributable to 21-hydroxylase deficiency.
OBJECTIVE Current medical therapy for congenital adrenal hyperplasia (CAH) attributable to a complete 21-hydroxylase deficiency is not optimal. Difficulties in adequate adrenal androgen suppression are common, causing short adult stature, infertility, and hyperandrogenism. We report the use of laparoscopic bilateral adrenalectomy as a definitive therapy for this condition and argue that it is s...
متن کاملTreatment and Outcome of Congenital Adrenal Hyperplasia: 21-Hydroxylase Deficiency
The use of glucocorticoids to treat individuals with congenital adrenal hyperplasia (CAH) was first reported by both Wilkins and Bartter in 1950. Since that time, the care of these patients has improved dramatically, and through the efforts of dedicated medical researchers, it continues to improve today. With early detection by newborn screening, initiation of treatment in infants with salt-was...
متن کاملClassic congenital adrenal hyperplasia.
Congenital adrenal hyperplasia is attributed to inherited enzyme defects in the adrenal cortex. The classical form results in reduced production of cortisol and aldosterone, accompanied by an increase in production of adrenal cortical androgens. This causes virilisation in girls, adrenocortical failure and early puberty in both sexes. This article describes the genetics, clinical picture, diagn...
متن کاملObesity among children and adolescents with classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency.
OBJECTIVES Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is the most common inherited disorder of adrenal steroid biosynthesis. Patients with the classic form of CAH show androgen excess, with or without salt wasting. There are few studies reporting on higher rates of overweight and obesity among children with CAH. In addition to its role in the regulation of energy bala...
متن کاملAdrenal Hyperplasia, Congenital, due to 21-Hydroxylase Deficiency
Alternative Names Adrenal Hyperplasia III 21-@Hydroxylase Deficiency CYP21 Deficiency Congenital Adrenal Hyperplasia 1 CAH1 Cytochrome P450, Subfamily XXIA, Polypeptide 2 CYP21A2 Cytochrome P450, Subfamily XXI CYP21 Steroid Cytochrome P450 21-Hydroxylase P450c21 21-@Hydroxylase B, Included CYP21B CA21H Cytochrome P450, Subfamily XXIA, Polypeptide 1 Pseudogene CYP21A1P CYP21P CYP21A Hyperandroge...
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ژورنال
عنوان ژورنال: Genetics in Medicine
سال: 1999
ISSN: 1098-3600,1530-0366
DOI: 10.1097/00125817-199907000-00010