HYPEROSTOSIS (hyperostosis; grech, hyper-+ost [eon] a bone + - osis) — pathological (excess) growth of a bone tissue. Can have character of independent process or be one of symptoms any patol, states. As the symptom is observed at a hypervitaminosis And, a syphilitic osteoperiostitis (see Syphilis). Limited in a look ostey and diffusion G. meet also at Bambergera-Mari periostosis (see), Leontiasis ossea (see) and other diseases of bone system.
The group of independent diseases of dysplastic character which main display are G. is heterogeneous. According to M. V. Volkov (1974), in it it is necessary to allocate the diseases connected with overdevelopment of correctly created bone tissue: inborn system diaphyseal G. (see. Kamurati-Engelmanna disease ), multiple cortical G., melocheostosis (see), osteopoikilosis (see) and the diseases connected with disharmony of development of bone and hemopoietic tissue as a result of growth of a bone tissue in the marrowy channel: marble disease (see), an osteomyelodisplasia (see. Osteomyelofibrosis ).
Morphologically in most cases G. represent .soby the growth of the condensed bone tissue extending both endostalno, and periostalno. In one cases (a marble disease, a melocheostosis, etc.) all elements of a bone tissue are surprised: thickening and consolidation of cortical and spongy substance, fibrous consolidation of a periosteum, disturbance of very tectonics of a bone; quite often a lot of unripe bone substance, an osteotabes due to substitution by its fibrous connecting fabric or endosteal distribution of bone growths is found. In others (e.g., an osteopoikilosis) — defeat is limited to accumulation of the sclerosed bone tissue only in spongy substance. At G.'s most the thickening and narrowing of a gleam or a zapustevaniye of the vessels feeding a bone is noted. Localization of defeats is various, but tubular bones are surprised more often. Inflammatory changes are found at multiple cortical G.
Multiple cortical hyperostoses at children (synonym: a syndrome of Kaffi — Silvermena, an infantile cortical hyperostosis). It is for the first time described in 1930 G. Roske; more detailed description is made in 1945 Kaffi and Silvermen (J. Caffey, W. Silverman).
The etiology and a pathogeny remain not clear. Some authors consider this disease virus, others — hereditary. Family cases — a disease of several children in one family are described; cases when one of parents of the sick child in the childhood had a similar disease are known. It was reported about the birth of children with multiple cortical G. at mothers who had respiratory infections to time of pregnancy. Delano and Butler (Delano, Butler) consider that this multiple bone inflammatory process represents the perverted reaction to smallpox vaccination. S. A. Reynberg adhered to the same point of view (1964). There are assumptions of communication of a disease with disturbance of vitamin or hormonal balance.
Morphologically hypostasis, periosteal stratifications and a hyperplasia of normally created bone are defined. In an early stage of a disease the insignificant centers of acute leukocytic and inflammatory reaction in a connective tissue stroma of the growing periosteum are found. In soft tissues necroses with fibrotichesky manifestations. Limf, nodes are not struck.
The disease occurs only at babies, is more often at the age of 3 — 4 months. The beginning acute with symptoms of the general intoxication — the high temperature, a leukocytosis, a relative lymphocytosis accelerated by ROE, loss of appetite; children are uneasy and irritable. On extremities and a face dense, sharply painful swellings of soft tissues develop, is more often without the inflammatory phenomena. Development of a swelling in the field of a mandible (a crescent-shaped face) is especially characteristic. Pseudopareses of the affected extremities are described. Biochemical in blood increase in activity of an alkaline phosphatase is defined; there is an observation, at Krom the considerable aminoaciduria is revealed.
Radiological — the expressed symmetric periosteal stratifications, a thickening compact and a sclerosis of spongy substance of a diaphysis of long and short tubular bones of top and bottom extremities (fig.), and also a clavicle and a mandible. Epi - and metafizarny departments of bones remain not struck. Periosteal stratifications are often sharply limited and sometimes have a lamellar appearance.
The arc-shaped curvatures of a tibial bone are noted.
The differential diagnosis is carried out with hematogenous osteomyelitis, bone and joint tuberculosis, inborn syphilis, Bambergera — Mari a periostosis, a hypervitaminosis And (a toxic osteopathy), inborn diaphyseal hyperostoses of Kamurati — Engelmanna.
Treatment fortifying; antibiotics and streptocides are not effective. There are messages on favorable effect of anabolic hormones and Prednisolonum.
The forecast for life and concerning recovery favorable. Most of authors report about spontaneous a wedge, and rentgenol, treatment in several months.
Bibliography: M. V wolves. Diseases of bones at children, M., 1974; Levin R. S. About a so-called cortical hyperostosis at children, Vestn, rentgenol, and radio-gramophones., No. 3, page 30, 1957; Potanin N. V. and Dobronravov A. V. Two observations of a children's cortical hyperostosis, Pediatrics, No. 9, page 83, 1967; Reynberg S.A. Radiodiagnosis of diseases of bones and joints, t. 1, page 341, M., 1964; Berio A., PelizzaA.e Di Stefa n about A. La sindrome di de Toni — Caffey — Sylverman, Minerva pediat., v. 23, p. 1567, 1971; Buchem F. S. P. Hyperostosis corticalis generalize ta, Acta med. scand., v. 189, p. 257, 1971, bibliogr.; With a f f e at J, Infantile cortical hyperostosis, J. Pediat., v. 29, p. 541, 1946; MaroteauxP. e. a. L’hyperostose corticale g6n6ralis6e, to transmission dominante (type Worth), Arch., franc-Pediat., t. 28, p. 685, 1971, bibliogr.; Toochinda P., Varavi-thyaW. Kashemsant G. Infantile cortical hyperostosis (Gaffey’s disease) of # J. med. Ass. Thailand, v. 55, p. 50, 1972
L. P. Kuzmina.