Pediatric Obesity Reproductive Impotence Syndrome
Introduction
Introduction to obesity reproductive incompetence syndrome in children Obesity reproductive incompetence syndrome, also known as Frohlich syndrome (Frohlichsyndrome) Babinski-Frohlich syndrome, Leunnois-Cleret syndrome, obesity reproductive incompetent dystrophy, brain obesity, this disease is more common in young children, school-age boys, obesity Sexual organ dysplasia, urine collapse, etc. are characteristic. Most of the hypothalamic lesions caused by multiple diseases such as tumors of the hypothalamic pituitary or its adjacent parts, encephalitis and brain trauma are important causes of this syndrome. basic knowledge The proportion of children: the incidence rate of children is about 0.0004%-0.0006% Susceptible people: children Mode of infection: non-infectious Complications: Diabetes insipidus
Cause
The cause of obesity reproductive incompetence syndrome in children
(1) Causes of the disease
Pituitary tumors and craniopharyngioma compress the hypothalamus as one of the common causes. The following common tumors or inflammation in the thalamus are the most common causes. Encephalitis, meningitis, brain abscess, intracranial tuberculosis, and craniocerebral trauma can also be caused. Some patients have not been able to detect organic lesions after various examinations or even pathological anatomy, which may be primary hypothalamic-pituitary dysfunction.
(two) pathogenesis
The sexual function of obese reproductive incompetence syndrome is hypothalamic. Due to a variety of reasons, the hypothalamic luteinizing hormone releasing hormone (LHRH) secretion disorder, resulting in decreased secretion of luteinizing hormone (LH) and follicle stimulating hormone (FSH), and secondary hypogonadism, animal experiments confirmed that involving the middle of the bulge Gonadotropin-releasing hormone (GnRH) secretion is low, sexual dysfunction, can cause genital atrophy. As for the cause of obesity, it is not due to the lack of certain pituitary hormones, but due to damage to the hypothalamus. Animal experiments have shown that the ventromedial nucleus and the median bulge of the hypothalamus are damaged, and the patient's satiety is lost and eaten and obese. When the ventromedial nucleus is involved, insulin secretion is hyperactive, causing an appetite, eating more and becoming obese.
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
Prevention of obesity reproductive incompetence syndrome in children
Actively prevent and treat infectious diseases such as encephalitis, meningitis, brain abscess, tuberculosis, etc., and actively prevent brain trauma.
Complication
Complications of obesity reproductive allergy syndrome in children Complications diabetes insipidus
Diabetes insipidus is the main complication of this disease. It is characterized by a large amount of low specific gravity urine, the urine volume is more than 3L/d, and the specific gravity is less than 1.006. Thirsty and drink more, especially good cold drink, in addition to burnout, fatigue, affect sleep, generally does not affect growth and development. According to the severity of the disease, it can be divided into partial diabetes insipidus and complete diabetes insipidus. Tumors, trauma, and surgery caused by the saddle area, such as the simultaneous influence of the function of the anterior pituitary, may be accompanied by partial or complete anterior pituitary hypofunction. Congenital diabetes insipidus and trauma, surgery, and saddle area tumors can affect the thirst center, make the patient more urinary but not thirsty, easy to dehydrate and lead to hypernatremia, hyperosmotic state, which can be accompanied by fever. Convulsions and even cerebrovascular accidents.
Symptom
Pediatric obesity reproductive incompetence syndrome symptoms common symptoms urine collapse bone length delay drowsiness penis short male infertility female infertility no menstrual cramps development slow men sexual dysfunction female obesity
70% of patients with this condition are under the age of 20, and boys are more common.
Obesity is usually moderate, and most of them appear rapidly in a short period of time. They are unevenly distributed, and are particularly prominent in the breast, lower abdomen and waist, and near the external genitalia. The face and limbs are relatively thin, and the fingers and toes are thin and pointed.
Sexual developmental disorders or decline in sexual function are clinically significant features. Boys often have penis, scrotum and testicles very small, often have cryptorchidism, no genital development in adolescence, beard, pubic hair, mane are lacking, short stature, fine pitch, delicate skin, can also appear feminine breast. The girl's breast is particularly large and the mammary gland is atrophic. The internal and external genitalia are dysplastic, immature, with no menstrual cramps and secondary sexual characteristics appearing or postponing. In adulthood, the second sexual characteristics gradually decline, sexual function is low, and reproductive ability is lost.
Both sexes have delayed bone age and sometimes diabetes insipidus. Eat more, sleepiness, laziness, etc. are also more common. The intelligence is mostly normal.
In addition, the symptoms of increased intracranial pressure may occur from the primary disease, such as nausea, vomiting, headache, visual impairment, optic disc change, blindness and saddle enlargement, saddle back and erect, saddle sagging, sphenoid sinus narrowing, The posterior bed suddenly changes linearly or disappears, and the X-ray film is abnormal. Sometimes the syndrome can be used as a prominent clinical manifestation and the primary disease is often not obvious.
Examine
Examination of obesity reproductive incompetence syndrome in children
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 can find space-occupying lesions.
6. Fundus examination has optic nerve head edema.
7. Skull X-ray examination showed saddle injury and calcification, in patients with optic nerve cross glioma, in addition to obesity reproductive incompetence, X-ray examination showed signs of optic nerve hole enlargement.
Diagnosis
Diagnosis and diagnosis of obesity reproductive incompetence syndrome in children
diagnosis
The diagnosis of this syndrome is mainly based on the three characteristics of primary disease, obesity and sexual development. The diagnosis of primary disease is slightly difficult. The development of internal and external genital organs is difficult to diagnose in the prepubertal period. If there is obesity in the lower body, it should be Consider this disease.
Differential diagnosis
When there is no history of primary disease, it is differentiated from puberty retardation. The latter is better after multiple healing. There are no obvious abnormalities in laboratory tests, and this disease often has obesity and internal and external genital dysplasia.
