Isolated hypogonadotropic hypogonadism, apropos of a case

Abstract

Introduction:Hypogonadism is a disorder caused by a deficient synthesis of sexual steroids by the testicle or ovary and which manifests itself in childhood generally by delayed puberty but also as arrested puberty or primary amenorrhea.

Objective:describe the isolated presentation of hypogonatropic hypogonadism in a female patient.

Case Presentation:Female patient aged 21 years with a history of intermittent bronchial asthma for which she is receiving treatment. At the age of 19 she was referred to the Endocrinology service of the “Hermanos Ameijeiras” Clinical-Surgical Hospital (HHA) from the “William Soler” Pediatric Hospital due to primary amenorrhea, poor development of secondary sexual characteristics and eunuchoidism, in the absence of anosmia or hyposmia. No family history of amenorrhea, delayed puberty or genetic disease was recorded. Her study was completed and isolated hypogonadotropic hypogonadism was confirmed from a clinical, hormonal and genetic point of view.

Conclusions:Congenital isolated hypogonadotropic hypogonadism is a rare pituitary hormone deficiency. A diagnostic exclusion exercise was carried out, in which a multidisciplinary team of specialists participated. This approach allowed the timely treatment of the patient, achieving the development of secondary sexual characteristics, stimulating the menstrual cycle, improving her quality of life, avoiding the appearance of cardiovascular and metabolic complications in the medium and long term, investigating the presence of other associated endocrine and genetic diseases, improving the psychosocial sphere, and laying the foundations for the future reproductive option.