Osteosclerosis - causes
Osteosclerosis - causes
Osteosclerosis is an abnormal compaction of the bone, which includes an increase in bone density with minimal disruption of its modeling. Osteopetrosis with late manifestation.
This type is inherited in an autosomal-pre-dominant type, is benign and late, manifested in children, adolescents or young people. This disease, which has a relatively wide geographical and ethnic distribution. Patients may not have clinical manifestations; overall health is not impaired. At the same time, facial nerve paresis, deafness and mild anemia may occur.
In newborns, the bones of the skeleton are not changed as a rule during radiography. At the same time, sclerosis of the bones becomes more and more evident in children, and the diagnosis is usually made on the basis of X-ray, for reasons not related to the disease. The defeat of bones is common, however changes are focal. The skull is sealed, the sinuses can be obliterated.Sclerosis of vertebral end plates forms the characteristic “rugby shirt”. Erythrocyte transfusion or splenectomy is required for some patients to treat anemia.
Osteopetrosis with early manifestations.This type of osteopetrosis is inherited in an autosomal recessive manner, its course is malignant, manifestations are congenital or develop in infancy. This uncommon, often lethal disease is often the result of osteoclast-as-associated mutation in the TCIRG1 gene. The growth of bone tissue causes bone marrow dysfunction. Initial symptoms include impaired weight gain, the appearance of spontaneous hematomas, abnormal bleeding and anemia. Paralysis of the 2nd, 3rd and 7th pairs of cranial nerves and hepatosplenomegaly develop later. Death as a result of bone marrow exhaustion usually occurs in the first year of life.
When X-ray prevails generalized increase in bone density. Penetrating X-ray of long bones shows transverse bands in the region of the metaphysis and longitudinal stripes in the region of the diaphysis. With the progression of the disease, the ends of the long bones, especially the proximal humerus and the distal femur, take the form of a flask.In the vertebrae, pelvic bones and tubular bones, characteristic bones are formed inside the bone. The skull becomes thickened, the spine acquires a characteristic form of "rugby shirt".
Bone marrow transplantation from an HLA-identical donor — a sibling — provides excellent results. At the same time, the forecast is worse when there are mismatches in the HLA system. In some cases, prednisolone, cal-citriol and interferon-gamma are effective.
Osteopetrosis with renal tubular acidosis. This type is inherited according to the auto-somno-dominant type. In this disease, weakness, growth retardation and impaired weight gain are noted. The bones on the roentgenogram look dense, the calcifications in the brain are visible; renal-tubular acidosis is noted, the activity of erythrocyte carbonic anhydrase decreases. A genetic defect involves a mutation of the carbonic anhydrase II gene. Bone marrow transplantation treats osteopetrosis, but it has no effect on PTA. Supportive therapy consists of additional administration of Na bicarbonate and electrolytes to correct renal losses.
Picodisostosis. In this case, an autosomal recessive disease at an early age becomes noticeable growth retardation; adult height less than or equal to 150 cm.Other manifestations, including an enlarged skull, short and wide arms and legs, dystrophic nails and blue sclera, are usually recognizable in infancy. Patients are very similar to each other; they have a small face, a cut chin and carious, misplaced teeth. The skull grows in size, the small spring remains open. End phalanges are short, fingernails are dysplastic. Complications are pathological fractures.
Bone sclerosis appears on the roentgenogram in childhood, but no transverse bands or bones inside the bones are visible. The bones of the face and the paranasal sinuses are hypoplastic, the mandibular angle is obtuse. The clavicles may be thin, and their lateral divisions are underdeveloped; distal phalanx rudimentary. Plastic surgery was used to correct pronounced deformities of the face and jaw.
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