Pseudohypoparathyroidism
Contents:
Description:
Pseudohypoparathyroidism (Greek pseudzs false + hypoparathyrosis; synonym: a hereditary osteodystrophy of Albright, Albright's disease) - the rare hereditary disease of bone system imitating a hypoparathyrosis and which is characterized by disturbance of exchange of calcium and phosphorus; often is followed by a delay of intellectual and physical development.
Pseudohypoparathyroidism symptoms:
Clinical signs of a pseudohypoparathyroidism are similar to symptoms of an idiopathic hypoparathyrosis. The attacks of tonic spasms arising spontaneously or under the influence of any irritants are noted. Kaltsinata in hypodermic cellulose show a tendency to an ulceration. Hypodermic ossification is often expressed to such an extent that imitates an ossifying miositis. The delay of intellectual development, lag in growth, a crescent-shaped face, obesity and a brachydactyly, especially shortening of the first, fourth and fifth metacarpal and plusnevy bones are characteristic. Multiple exostoses, a dyschondroplasia, manifestations of a secondary hyperparathyreosis in the form of a subperiosteal resorption of bones of fingers of hands can be observed; changes in an epiphysis of bones same, as at a fibrous osteodysplasia. Often note vomiting, and also a hamaturia owing to formation of oxalic stones in uric ways, reveal a lentikulyarny cataract, a hypoplasia of an adamantine substance of tooth.
At patients spsevdogipoparatireozy along with decrease in sensitivity to parathormone of target organs resistance to other hormones dependent on adenilattsiklazny system, for example gonads to gonadotropic hormones, a thyroid gland to thyritropic hormone, target organs to a glucagon and antidiuretic hormone can be observed. The increased frequency of autoimmune diseases and a diabetes mellitus is noted, the hypothyroidism and a hyperthyroidism are observed.
Allocate also a pseudo-pseudohypoparathyroidism which is characterized by lack of a hypocalcemia, hyperphosphatemia, spasms and osteomalacy.
Pseudohypoparathyroidism reasons:
Inborn defect – nonsensitivity of peripheral fabrics to action of PTG is an origin of a pseudohypoparathyroidism.
Treatment of the Pseudohypoparathyroidism:
Treatment at a hypocalcemia consists in purpose of drugs of calcium in the doses sufficient for maintenance of normal concentration of calcium in blood. Therapy by vitamin D is of great importance. The initial dose is calculated from 2000 ME/kg of body weight a day, but by no more than 100 000 ME a day. In order to avoid overdose of drugs of vitamin D control of concentration of calcium in blood each 3-7 days within the first two weeks of treatment and every month within the next 2-3 months is necessary. On reaching stable concentration of calcium in blood 1 time in 2-3 months is enough to check of it. It is possible to apply Calcitrinum, Dihydrotachysterolum, oksidevit, and also other drugs of active forms of vitamin D. The diet with restriction of phosphorus helps to normalize concentration of calcium in blood and to eliminate symptoms of a secondary hyperparathyreosis. At insufficiency of other hemadens carry out replacement therapy by the corresponding hormones. Treatment by parathormone not effectively. For stopping of convulsive attacks solution of Calcii chloridum or calcium of a gluconate intravenously enter 10%; inside - 5-10% Calcii chloridum solution on 1 tablespoon 3-4 times a day: calcium a gluconate, calcium a lactate - to 10 g a day.
The forecast at rational therapy favorable. Considering hereditary character of a pseudohypoparathyroidism, medicogenetic consultation concerning a possibility of emergence of a pseudohypoparathyroidism in posterity is necessary.
