In appropriate cases, glucocorticoids (usually hydrocortisone in children) are administered to suppress secretion of corticotropin releasing hormone (CRH) produced by hypothalamus and of adrenocorticotropic hormone (ACTH) produced by pituitary gland. This suppression will reduce concentration in blood of sex steroids produced by adrenal glands. Some of the main considerations in treatment include the watchful waiting of symptom severity as well as adverse responses to glucocorticoids administered as drugs, seen in patient bone mineral density, height and weight. For women, an oral contraceptive pill and androgen blockers such as spironolactone or cyproterone acetate are alternatives to glucocorticoids for managing symptoms of androgen excess. Women with LOCAH being treated with hydrocortisone have a lower miscarriage risk. Prevalence
According to haplotype association studies, the prevalence of LOCAH in the general white population is estimated to be 1:500 to 1:1000, but in people with a high rate of marriage between relatives, the prevalence rate is as high as 1:50 to 1:100. A 2017 CYP21A2 genotype analysis predicted that the total frequency of LOCAH in the white population of the United States is about 1:200 (95% confidence level, from 1:100 to 1:280).According to a 2017 meta-analysis, the prevalence of LOCAH among women with signs and symptoms of androgen excess is 4.2% globally, and between 1% and 10% depending on the ethnicity of the population being studied.LOCAH-affected individuals account for 88% of Anne Fausto-Sterlings 1.7% prevalence estimate of intersex conditions, which is cited by a number of prominent intersex advocacy organizations.
From the clinical perspective, LOCAH is not an intersex condition and including LOCAH in intersex prevalence estimates has been cited as an example of dubious statistical practice. See also
Congenital adrenal hyperplasia due to 17α-hydroxylase deficiency
Lipoid congenital adrenal hyperplasia

References


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