Pseudohypoparathyroidism

Pseudohypoparathyroidism( congenital Albright osteodystrophy) is a rare hereditary syndrome characterized by the body tissue's resistance to the parathyroid gland hormone, a decrease in the amount of calcium in the blood serum, an increase in parathyroid gland function, a short stature and skeletal anomalies( shortening of the metacarpal and metatarsal bones).Pseudohypoparathyroidism was the first endocrine disease, the example of which was the possibility of the existence of a phenomenon of tissue sensitivity disorders to the hormone with an unchanged mechanism of its formation and a normal level in the blood serum.

Pseudohypoparathyroidism of the 1 st and 2 nd types is distinguished. The type of inheritance of the disease remains unclear. The ratio of women and men is 2: 1.Among the pseudohypoparathyroidism of the 1st type, three subtypes are distinguished. In pseudohypoparathyroidism of type 1a, low stature, obesity, shortening of IV and V metatarsal and metacarpal bones, facial lunate, subcutaneous formation of calcareous stones and bone growths. Mental retardation is often noted.

Psevdopsevsegidopoparatireoz is an external copy of pseudohypoparathyroidism. In most cases, there are typical changes in appearance, characteristic of pseudohypoparathyroidism of type la, but the level of calcium in the blood remains normal, and the normal reaction of the body to the introduction of the hormone of the parathyroid glands remains. Some persons with pseudo pseudohypoparathyroidism are the closest relatives of patients with type 1a pseudohypo- parathyroidism.

The recognition of pseudohypoparathyroidism is based on the detection of a positive family history( the presence of the disease in the family) and the detection of malformations characteristic of pseudohypo- parathyreosis type 1a in combination with a decrease in serum calcium levels and an increase in phosphorus levels. For all types of pseudohypoparathyroidism, except for 1a and 1c, there are no characteristic external changes. In most cases, people with pseudohypoparathyroidism are diagnosed with an elevated level of the parathyroid hormone hormone, which makes it possible to distinguish pseudohypoparathyroidism from hypoparathyroidism.

Treatment of all types of pseudohypoparathyroidism involves prescribing vitamin D preparations in combination with calcium preparations.