GARDNER SYNDROME

From Big Medical Encyclopedia

GARDNER SYNDROME (E. J. Gardner, the modern American doctor) — the hereditary symptom complex which is characterized by existence family diffusion a polypose of direct and colonic guts in combination with benign tumors of bones, skin and soft tissues. These tumors can develop at different times or at the same time. Separate elements of this syndrome in medical literature were described long ago.

In 1948 Gardner with sotr. paid attention to their interrelation and genetic dependence. More than 100 cases of this syndrome are known.

Of page is defined by a dominant autosomal gene. The parent possessing this gene irrespective of a floor transmits a disease to a half of descendants of firstgeneration (provided that the second parent is healthy).

Of page is characterized by existence of three symptoms: 1) family diffusion polipoz of direct and colonic guts; 2) osteomam of flat and tubular bones (see. Osteoma ); defiant deformations and a disfiguration of the person, shift of teeth are especially frequent an osteoma of bones of a facial skull. After achievement of a certain size growth ostey stops, the malignancy does not occur; 3) tumors of soft tissues from which are especially frequent the epithelial cyst which is superficially located lipoma (see) and fibroma (see. Fibroma , fibromatosis), neurofibroma (see).

As a rule, bone, skin tumors and tumors of soft tissues are found earlier, than polyposes. The diagnosis is based on a wedge, data. Early recognition of tumors (elements G. of page) allows to establish overseeing by a condition of intestines and to timely prevent developing of cancer against the background of a polypose (see. Intestines , tumors).

Treatment only surgical.


Bibliography: Rivkin V. L., etc. Polyps and diffusion polyposes of direct and thick guts, page 133, M., 1969, bibliogr.; G and-y of n e of E. J. Genetic and clinical study of intestinal polyposis, predisposing factor for carcinoma of colon and rectum, Amer. J. hum. Genet., v. 3, p. 167, 1951; Gard-nerE. J. a. R ichards R. C. Multiple cutaneous and subcutaneous lesions occu-ring simultaneously with hereditary polyposis and osteomatosis, ibid., v. 5, p. 139, 1953; J o n e s E. L. a. Cornell W. P. Gardner’s syndrome, Arch. Surg., v. 92, p. 287, 1966.

V. I. Astrakhan.

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