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Isolated follicle stimulating hormone deficiency

ORPHA:52901DiseaseAutosomal recessiveAdolescent, Adult

Ассоциированные гены (1)

FSHB
follicle stimulating hormone subunit beta
Disease-causing germline mutation(s) (loss of function) in
OMIM: 136530

Фенотипы (22)

Облигатный (100%)3
HP:0000026Male hypogonadism
HP:0000134Female hypogonadism
HP:0008213Gonadotropin deficiency
Очень частый (80–99%)10
HP:0000044Hypogonadotropic hypogonadism
HP:0000786Primary amenorrhea
HP:0000823Delayed puberty
HP:0002215Sparse axillary hair
HP:0002225Sparse pubic hair
HP:0002750Delayed skeletal maturation
HP:0008214Decreased serum estradiol
HP:0008734Decreased testicular size
HP:0012569Delayed menarche
HP:0040171Decreased serum testosterone concentration
Частый (30–79%)8
HP:0000027Azoospermia
HP:0000029Testicular atrophy
HP:0000798Oligozoospermia
HP:0000876Oligomenorrhea
HP:0010791Hyperplasia of the Leydig cells
HP:0012814Bilateral breast hypoplasia
HP:0012864Abnormal sperm morphology
HP:0030018Decreased female libido
Исключён (0%)1
HP:0000458Anosmia

Эпидемиология (1)

Point prevalence
Unknown
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы