Obese reproductive impotence syndrome

Introduction

Introduction to obesity reproductive incompetence syndrome Obesity reproductive incompetence syndrome, also known as Fröhlich syndrome (Fröhlichsyndrome), Babinski-Fröhlich syndrome, Leunois-Cleret syndrome, obesity reproductive incompetent malnutrition, cerebral obesity, this disease is more than children, school-age boys See, obesity, sexual organ dysplasia, urine collapse and so on. Most of them are caused by many diseases such as tumors, encephalitis and brain trauma in the hypothalamus, pituitary or its adjacent parts. Hypothalamic lesions are an important cause of this syndrome. basic knowledge The proportion of illness: 0.001% Susceptible people: more common in young children, school-age boys Mode of infection: non-infectious Complications: hypernatremia, diabetes insipidus

Cause

Etiology of obesity reproductive incompetence syndrome

Pituitary tumor, craniopharyngioma compression of the hypothalamus (35%):

Pituitary tumors, craniopharyngioma compression of the hypothalamus is one of the common causes, the following thalamic tumor or inflammation is the most common cause, encephalitis, meningitis, brain abscess, intracranial tuberculosis, craniocerebral trauma can also cause, some patients Although organic diseases have not been found through various examinations and even pathological anatomy, it may be a primary hypothalamic-pituitary dysfunction.

Hypothalamic luteinizing hormone releasing hormone (LHRH) secretion disorder (25%):

The sexual dysfunction of obesity reproductive incompetence syndrome is hypothalamic-derived, causing secretion of luteinizing hormone (LHRH) in the hypothalamus for various reasons, leading to secretion of luteinizing hormone (LH) and follicle stimulating hormone (FSH). Reduction, and secondary hypogonadism, animal experiments confirmed that when the middle ridge is involved, gonadotropin releasing hormone (GnRH) secretion is low, sexual dysfunction, can cause genital atrophy, as for the cause of obesity, it is not due to lack of certain Pituitary hormone, but due to damage to the hypothalamus, animal experiments have shown: damage to the hypothalamic ventromedial nucleus and median uplift, the patient's satiety loss and more food, obesity, involving the ventromedial nucleus, insulin secretion hyperthyroidism, resulting in appetite hyperthyroidism More food and obesity.

Insufficient secretion of pituitary growth hormone (15%):

The disease may sometimes be associated with skeletal growth disorders and is associated with hypothalamic growth hormone releasing factor and/or insufficient secretion of pituitary growth hormone.

Prevention

Obesity reproductive incompetence syndrome prevention

Actively prevent and treat infectious diseases such as encephalitis, meningitis, brain abscess, tuberculosis, etc., and actively prevent brain trauma.

Complication

Complications of obesity reproductive incompetence syndrome Complications, hypernatremia, diabetes insipidus

Diabetes insipidus is the main complication of this disease. It is characterized by a large amount of low specific gravity urine, a urine volume of more than 3L/d, a specific gravity of less than 1.006, polydipsia and polydipsia, especially good cold drinks, except for fatigue, fatigue, affecting sleep, generally not Affecting growth and development, according to the severity of the disease, can be divided into partial diabetes insipidus and complete diabetes insipidus, caused by tumors in the sellar region, trauma, surgery, such as affecting the function of the anterior pituitary, can be accompanied by Or complete anterior pituitary dysfunction, congenital diabetes insipidus and trauma, surgery, saddle area tumors, can affect the thirst center, so that patients with polyuria but not with thirst, easy to dehydrate leading to hypernatremia, Hyperosmotic state, this time may be accompanied by fever, convulsions and even cerebrovascular accidents.

Symptom

Symptoms of obesity reproductive incompetence syndrome Common symptoms Male type obesity visceral obesity no beard amenorrhea excess fat no menstrual cramps drowsiness severe obesity

The incidence of menstruation in men and women is similar, more than pre-puberty, with the following characteristics:

1. Obesity: The patient's body is unevenly obese, with the neck, trunk and limbs being most prominent at the proximal end, especially in the breast area, around the pelvis and the pubic symphysis. Due to the abnormal distribution of fat, the pelvis appears wide and the limbs are relatively large. Small, boys often have breast development, so male patients are female.

2. Physical characteristics: The nose, mouth and hands of the patient are often small, the fingers, the toes are slender, the nails are small, and there are different degrees of knee valgus, and the muscle tension is reduced, so the joints are excessively stretched and the skin is more Pale, soft, dry, after age, the skin can be fine wrinkles, white atrophic streaks on both sides of the abdomen and around the hips, height growth can be normal, delayed or rather normal than normal growth, into The bone action is delayed, and the epiphysis is not fused, so when the growth hormone secretion is normal, the body can be higher than normal.

3. Sexual hypoplasia: During the developmental period, the male penis, scrotum and testicles are still not developing, sometimes the testicles are falling, the boy is a small testicle, a small penis or cryptorchidism, and the second sex is absent, because the genitals are trapped in the pubic area. In fat, it is even more small. Before the developmental stage, it is difficult to determine whether there is sexual dysplasia, because normal boys have not developed genitals before development, but sometimes the development of genitals is particularly slow. Girls are not found before development. Sexual hypoplasia, no development of menstrual period until the developmental period, the vagina and uterus are not developed, no matter boys and girls, pubic hair and mane do not grow, boys have no beard growth, tone does not change, such as in adult onset, pubic hair Shedding, male patients with sexual dysfunction, female patients with amenorrhea.

4. Primary disease manifestations: If the primary disease is a tumor, it may cause hemianopia on both sides due to compression of the optic chiasm. There may be headache, and the intracranial pressure may increase in the late stage, and the fundus may change. X-ray examination The saddle can be damaged or enlarged.

5. With hypothalamic syndrome: due to damage to the hypothalamus, may be associated with diabetes insipidus, temperature instability and lethargy, intelligence is mostly normal, but also mental decline.

Examine

Examination of obesity reproductive incompetence syndrome

1. Hormone examination: urinary gonadotropin concentration and sex hormone concentration decreased.

2. Glucose tolerance test: It is often shown that glucose tolerance is reduced.

3. Pathological examination: Testicular biopsy showed that the seminiferous tubules were obviously atrophied, interstitial fibrosis, and no mature sperm, which were helpful for diagnosis.

4. Chromosome examination: There is no abnormality in the chromosome.

5. CT and other examinations: space-occupying lesions can be found.

6. Fundus examination: There is optic nerve head edema.

7. Skull X-ray examination: showing saddle injury and calcification, optic nerve cross glioma patients, in addition to obesity reproductive incompetence, X-ray examination showed signs of optic nerve hole expansion.

Diagnosis

Diagnosis and identification of obesity reproductive incompetence syndrome

diagnosis

The diagnosis of obesity reproductive incompetence syndrome is often abused by obesity and slower sexual development, and the body is like a female boy. Most of these children are fully developed (delayed puberty).

1. Generally there is unevenness in obesity and sexual dysfunction.

2. Blood, gonadotropin in the urine decreases or disappears.

3. Head X-ray imaging and CT, MRI can show tumors, and those with symptoms of intracranial disease can meet the diagnostic criteria of this disease.

Differential diagnosis

The diseases to be identified with this syndrome are several kinds of gonadal lesions caused by several kinds of gonadal diseases with obesity, the main identification is blood, urinary gonadotropin is higher than normal, the blood of this syndrome, urinary gonadotropin is very low It is differentiated from sexual function naive caused by insufficient secretion of pituitary gland gonadotropin (also known as selective pituitary naive). LHRH stimulation test has no response when simple gonadotropin deficiency.

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