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Premature ovarian failure 1(POF1)

MedGen UID:
1644269
Concept ID:
C4552079
Disease or Syndrome
Synonyms: FMR1-Related Primary Ovarian Insufficiency; Fragile x premature ovarian failure; HYPERGONADOTROPIC OVARIAN FAILURE, X-LINKED; POF1; PREMATURE OVARIAN FAILURE, X-LINKED; Primary ovarian insufficiency, fragile X-associated
 
Gene (location): FMR1 (Xq27.3)
 
Monarch Initiative: MONDO:0010706
OMIM®: 311360

Definition

Fragile X-associated primary ovarian insufficiency (FXPOI) is a condition that affects women and is characterized by reduced function of the ovaries. The ovaries are the female reproductive organs in which egg cells are produced. As a form of primary ovarian insufficiency, FXPOI can cause irregular menstrual cycles, early menopause, an inability to have children (infertility), and elevated levels of a hormone known as follicle stimulating hormone (FSH). FSH is produced in both males and females and helps regulate the development of reproductive cells (eggs in females and sperm in males). In females, the level of FSH rises and falls, but overall it increases as a woman ages. In younger women, elevated levels may indicate early menopause and fertility problems.

The severity of FXPOI is variable. The most severely affected women have overt POI (formerly called premature ovarian failure). These women have irregular or absent menstrual periods and elevated FSH levels before age 40. Overt POI often causes infertility. Other women have occult POI; they have normal menstrual periods but reduced fertility, and they may have elevated levels of FSH (in which case, it is called biochemical POI). The reduction in ovarian function caused by FXPOI results in low levels of the hormone estrogen, which leads to many of the common signs and symptoms of menopause, such as hot flashes, insomnia, and thinning of the bones (osteoporosis). Women with FXPOI undergo menopause an average of 5 years earlier than women without the condition. [from MedlinePlus Genetics]

Clinical features

From HPO
Premature ovarian insufficiency
MedGen UID:
9963
Concept ID:
C0025322
Disease or Syndrome
Amenorrhea due to loss of ovarian function before the age of 40. Primary ovarian inssuficiency (POI) is a state of female hypergonadotropic hypogonadism. It can manifest as primary amenorrhea with onset before menarche or secondary amenorrhea.
Irregular menstruation
MedGen UID:
56379
Concept ID:
C0156404
Finding
Abnormally high variation in the amount of time between periods.
Increased circulating gonadotropin level
MedGen UID:
400008
Concept ID:
C1862265
Finding
Overproduction of gonadotropins (FSH, LH) by the anterior pituitary gland.

Professional guidelines

PubMed

Reato G, Morlin L, Chen S, Furmaniak J, Smith BR, Masiero S, Albergoni MP, Cervato S, Zanchetta R, Betterle C
J Clin Endocrinol Metab 2011 Aug;96(8):E1255-61. Epub 2011 Jun 15 doi: 10.1210/jc.2011-0414. PMID: 21677034

Recent clinical studies

Etiology

Acharya S, Yasmin E, Balen AH
Hum Fertil (Camb) 2009 Dec;12(4):198-203. doi: 10.3109/14647270903377178. PMID: 19938908

Therapy

Acharya S, Yasmin E, Balen AH
Hum Fertil (Camb) 2009 Dec;12(4):198-203. doi: 10.3109/14647270903377178. PMID: 19938908

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