卵巢性闭经是怎么回事呢?What is ovarian amenorrhea?

陈凤林教授指出：卵巢性闭经是由于卵巢本身原因引起的闭经。Professor Chen Fenglin pointed out that ovarian amenorrhea is caused by ovarian itself.

卵巢性闭经时Gn水平升高，分为先天性性腺发育不全、酶缺陷、卵巢抵抗综合征及后天各种原因引起的卵巢功能减退。

The increase of Gn level during ovarian amenorrhea can be divided into congenital gonadal dysplasia, enzyme deficiency, ovarian resistance syndrome and ovarian dysfunction caused by acquired causes.

1.先天性性腺发育不全

患者性腺呈条索状，分为染色体异常和染色体正常两种类型。

(1)染色体异常型 45，X0综合征，染色体核型为45，X0及其嵌合体，如45，X0/46，XX或45，X0/47，XXX，也有45，X0/46，XY的嵌合型。45，X0女性除性征幼稚外，常伴面部多痣、身材矮小、蹼颈、盾胸、后发际低、腭高耳低、肘外翻等临床特征，称为Turner(特纳)综合征。

(2)染色体正常型 染色体核型为46，XX或46，XY，称XX型或XY型单纯性腺发育不全，可能与基因缺陷有关，患者为女性表型，性征幼稚。

1. Congenital gonadal dysplasia

The gonads of the patients were stranded and divided into two types: chromosomal abnormalities and normal chromosomes.

(1) Chromosome abnormality type 45, X0 syndrome, chromosome karyotype 45, X0 and its chimera, such as 45, X0/46, XX or 45, X0/47, XXX, there are also 45, X0/46, XY chimera. 45, X0 females are often accompanied by facial multiple nevus, short stature, webbed neck, shield chest, posterior hypothalamus, high palate, low ear, cubitus valgus and other clinical features, known as Turner syndrome.

(2) The karyotype of normal chromosome is 46, XX or 46, XY. It is called XX or XY simple gonadal dysplasia. It may be related to gene defect. The patient is a female phenotype with immature sexual characteristics.

2.酶缺陷

包括17α羟化酶或芳香酶缺乏。患者卵巢内有许多始基卵泡及窦前卵泡和极少数小窦腔卵泡，但由于上述酶缺陷，雌激素合成障碍，导致低雌激素血症及FSH反馈性升高;临床多表现为原发性闭经、性征幼稚。

2. enzyme deficiency

Including 17 alpha hydroxylase or aromatase deficiency. There are many primordial follicles, presinusoidal follicles and a few small sinusoidal follicles in the ovaries of the patients, but due to the above-mentioned enzyme deficiencies and estrogen synthesis disorders, resulting in hypoestrogenia and FSH feedback increase; clinical manifestations are mainly primary amenorrhea and immature sexual characteristics.

3.卵巢抵抗综合征

患者卵巢对Gn不敏感，又称卵巢不敏感综合征。Gn受体突变可能是发病原因之一。卵巢内多数为始基卵泡及初级卵泡，无卵泡发育和排卵。内源性Gn特别是FSH水平升高，可有女性第二性征发育。

3. Ovarian Resistance Syndrome

Patients'ovaries are not sensitive to Gn, also known as ovarian insensitivity syndrome. Mutation of Gn receptor may be one of the causes of the disease. Most of the ovaries are primordial follicles and primary follicles, without follicular development and ovulation. Increased levels of endogenous Gn, especially FSH, may lead to the development of female secondary sexual characteristics.

4.卵巢早衰

卵巢早衰(POF)指女性40岁以前由于卵巢功能减退引发的闭经，伴有雌激素缺乏症状。激素特征为高Gn水平，特别是FSH水平升高，FSH>40U/L，伴雌激素水平下降。与遗传因素、病毒感染、自身免疫性疾病、医源性损伤或特发性原因有关。

4. Premature ovarian failure

Premature ovarian failure (POF) refers to amenorrhea caused by ovarian dysfunction in women before 40 years of age, accompanied by estrogen deficiency symptoms. Hormone is characterized by high Gn level, especially increased FSH level, FSH > 40U/L, and decreased estrogen level. It is related to genetic factors, viral infection, autoimmune diseases, iatrogenic injury or idiopathic causes.