From Big Medical Encyclopedia

AKATALAZIYA (the Greek negative a-prefix + a catalase) — the hereditary anomaly of exchange connected with lack of enzyme of a catalase in blood and fabrics. Main manifestation And. the alveolar pyorrhea is.

And. it is for the first time described in 1948 by the Japanese otorhinolaryngologist S. Takahara. Till 1950 messages on this disease arrived only from Japan and Korea, however in recent years cases And. are registered in Switzerland and Sweden. Mode of inheritance And. — autosomal and recessive. The disease meets seldom — 1:24000 [Abie (N. of Aebi), 1966].

The catalase participates in processes of destruction of the hydrogen peroxide which is formed as a result of fabric metabolism. At And. there is no protective role of enzyme in relation to hydrogen peroxide, to-ruyu produce microorganisms of an oral cavity (hl. obr. hemolitic streptococcus). As a result of impact of peroxide on mucous membranes of an oral cavity and possible local deficit of oxygen inflammatory processes and even gangrene develop in them. Lack of any manifestations And. from blood can speak the fact that missing functions of a catalase are compensated by other enzymes, in particular glutathione-reductase, edges as well as the catalase, protects hemoglobin from the destroying influence of hydrogen peroxide.

The disease is shown at teenage age by recurrent ulcerations of gums. In more expressed cases there is alveolar gangrene, an atrophy of gums, dedentition is observed. Malignant forms are characterized by spread of gangrene on soft tissues and bone structures of jaws.

Characteristic laboratory sign And. emergence of dark brown coloring of blood after addition to it of several drops of hydrogen peroxide is; at the same time there is no rough release of oxygen which is usually observed at the corresponding reaction with blood of healthy people. Approximately in half an hour dark brown coloring disappears and the white-yellow deposit representing the denatured protein drops out. In nadosadochny liquid by means of special methods the end product of disintegration of hemoglobin — pentdiopent is found.

Pathogenetic treatments And. does not exist; a symptomatic treatment — see Periodontosis. Respect for careful hygiene of an oral cavity, extraction of the affected teeth is recommended.

Forecast And. it is favorable: with approach of puberty the alveolar pyorrhea spontaneously disappears.

Bibliography: Aebi H. The investigation of inherited enzyme deficiencies with special reference to acatalasia, Proc. 3. Int. congr. human genet., p. 189, Baltimore, 1967, bibliogr.; Knapp A. Genetische Stoffwechselstörungen, S. 239, Jena, 1970; Wyngaarden J. B. a. Howell R. R. Acatalasia, in book: Metabolic basis of inherited disease, ed. by J. B. Stanbury, p. 1343, N. Y. a. o., 1966, bibliogr.

Yu. E. Veltishchev.