Overview.
Pituitary dwarfism is a growth disorder caused by a lack of growth hormone in the anterior pituitary gland before puberty. The only effective treatment for this condition is human growth hormone (hGH) replacement therapy.
Etiology
Clinically, it is divided into idiopathic and secondary, with the former being the most common, accounting for 60% to 70% of all pituitary dwarfism. The latter is caused by organic lesions of the hypothalamus-pituitary gland, mainly craniopharyngioma and pineal tumors.
Symptoms
1. Growth disorder
After 1 or 2 years of birth, the growth gradually slows down, the stature is short, well-proportioned, and the appearance of adults is still like that of children, with a lot of subcutaneous fat and underdeveloped muscles; the maturity of bones is delayed, the age of bones is smaller than that of age, and the intelligence is comparable to that of age.
2. Sexual development disorder
Small penis, cryptorchidism, no beard, child-like voice in male, no menstruation in female, underdeveloped breasts, small uterus, childish vulva, no axillary hair.
Examination
1. X-ray examination
Bone age is childish.
2. Serologic examination
Decreased serum growth hormone level.
3. Insulin hypoglycemia test
Arginine loading test is unresponsive.
4. Other tests
Except Turner syndrome, thyroid dwarfism, chondrodysplasia, somatic growth retardation or delayed puberty.
Diagnosis
Diagnosis is made on the basis of etiology, clinical manifestations and laboratory tests.
Differential diagnosis
It should be differentiated from Turner syndrome, thyroid dwarfism, chondrodysplasia, somatic growth retardation or delayed puberty by combining clinical symptoms, physical signs and physical and chemical examinations, and also from parasitic dwarfism:
1. Schistosoma japonicum
Schistosomiasis in our country is more widespread, serious harm, has been controlled in some areas, according to the previous survey data, a region of schistosomiasis epidemic serious, high infection rate of the population, the degree of infection is heavy, schistosomiasis pycnodysplasia incidence is also high.
2. Chinese branch testicular schistosomiasis
People are infected by eating undercooked fish and shrimp, the cystic larvae of this worm are ingested into the body by mistake, and the adult worms are parasitized in the bile ducts of human beings, which can cause obstructive xanthomas, cholestasis, cholelithiasis, hepatomegaly, hepatic parenchyma atrophy and cirrhosis, and other symptoms, and children with severe disease can suffer from stunted growth and pygmy, and Schistosomiasis is more common in our country, and it is scattered and endemic.
3. Other
Hookworms, Plasmodium, gingerbread worms, black fever protozoa, spinosad nematodes and so on. Heavy infections with these parasites have been documented to cause stunted growth in children, with a few cases of dwarfism.
Complications
Infections, trauma, stress such as severe vomiting, diarrhea, and even pituitary crisis.
Treatment
1. Growth hormone replacement therapy
Once the pituitary dwarf is diagnosed, it should be treated as soon as possible, and the only effective treatment is to replace human GH, and the recombinant technology has been applied to produce hGH. In medical units without growth hormone, synthetic anabolic steroid hormone can be used, or chorionic gonadotropin can be used alternatively.
2. Synthetic anabolic steroid therapy
3. Hormone replacement therapy
Hormone replacement therapy for other endocrine insufficiencies.
4. Etiologic treatment