Learn About Hypogonadism Primary Partial Alopecia

What is the definition of Hypogonadism Primary Partial Alopecia?
Hypogonadism primary partial alopecia, also known as primary hypergonadotropic hypogonadism-partial alopecia syndrome, is very rare inherited (genetic) disorder characterized by delayed development of the sexual organs (gonads; ovaries and testes) and associated sexual characteristics, such as breast and sperm development, etc., combined with partial hair loss (alopecia). Hypogonadism primary partial alopecia is so rare that it has only yet been described in six females and one male from three families. Hypogonadism primary partial alopecia is due to having parents who are closely-related (consanguinity), in these cases, first cousins believed to have a recent common ancestor.
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What are the symptoms of Hypogonadism Primary Partial Alopecia?
Symptoms of hypogonadism primary partial alopecia in females may include partial scalp hair loss; sparse underarm and pubic hair; sparse eyebrows; thin upper lip; abnormally small head; flattened back of head; curvature of the spine; absent or small uterus, vagina, or ovaries; underdeveloped or absent breasts; absence of menstruation; sensorineural deafness; and mild intellectual disability. Symptoms of hypogonadism primary partial alopecia in males may include partial scalp hair loss; inability to produce sperm; and undeveloped, undescended, or absent testes.
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What are the current treatments for Hypogonadism Primary Partial Alopecia?
Treatment for hypogonadism primary partial alopecia involves long-term administration of human chorionic gonadotropin to support more normal sexual development. Alopecia (hair loss) associated with hypogonadism may possibly be treated with photochemotherapy.
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