From Big Medical Encyclopedia

OCHRONOSIS (ochronosis; grech, ochros yellow + nosos a disease) — the defeat of cartilaginous tissue, sheaves, sinews, fastion, a firm meninx and other connective tissue educations which is characterized by their pathological pigmentation, a pain syndrome and an alkaptonuria. Virkhovom is for the first time described in 1866. Meets at men aged from 30 up to 50 years more often. Distinguish endogenous O. (inborn, hereditary and alkaptonurichesky) and exogenous, arising in nek-ry cases at poisoning carbolic to - that, phenol, resorcin.

Endogenous O. is connected with disturbance of interstitial exchange of tyrosine and phenylalanine. In the course of their oxidation it is formed homogentisic acid (see), the edge is not split as a result of lack of enzyme of a gomogentinaza, collects in a large number in blood serum and under the influence of a tyrosinase turns into a melaninopodobny oh-ronotichesky pigment. This pigment, connecting with hondromukoidy, causes elective coloring of cartilages and connecting fabric.

The Okhronotichesky pigment can be received in experimental conditions at immersion of a piece of a cartilage in solution homogentistic to - you. In an ultraviolet light the okhronotichesky pigment possesses bright fluorescence, coloring gistol, cuts nuclear fast red reveals bazofiljiye from a voistvo of a pigment.

Macroscopically at O. pigmentation from smoky-gray till ink-black color of intervertebral cartilages, synchondroses of haunch bones, cartilages of a grudinoklyuchichny joint, an auricle, a nose, a throat, a trachea and bronchial tubes is noted sharp diffusion patol. Patol, pigmentation of cartilages is combined with their porosity and fragility, a razvolokneniye and adjournment of salts of calcium. Sinews, sheaves, a fascia and joint surfaces usually get uneven spotty coloring. Quite often sites patol, pigmentation find in a firm meninx, an intima of an aorta, around atherosclerotic plaques, in a pericardium, an endocardium, sites of postinfarction fibrosis of a myocardium and in the sclerosed valves of heart. In some cases heavy O. patol, pigmentation meets in hemadens, smooth and cross-striped muscular tissue.

At gistol, a research of the struck cartilaginous tissue in cartilaginous cells and interstitial substance define a large number of an okhro-notichesky pigment. The cartilaginous cells loaded with it quite often are exposed to dystrophic and necrotic changes. On the periphery of accumulations of such cells inflammatory reaction with participation of colossal cells of foreign bodys can develop. The Okhronotichesky pigment in the form of cylinders is found in collective tubules of kidneys.

Depending on a wedge, pictures allocate limited and widespread forms O. The limited form O. is characterized by damage of large joints (humeral, knee, coxofemoral) with a picture of the deforming arthritis and the subsequent development of an anchylosis. Damage of kidneys, as a rule, secondary, can lead to a so-called okhronotichesky nephrosis. The common form of O. is characterized by emergence serovatobury patol, the coloring which is the most expressed on face skin around eyes and on hands. Sharp pigmentation of a tympanic membrane is noted, nails get bluish coloring, become fragile. Damage of intervertebral cartilages results in constraint, rigidity and the increasing morbidity in lumbar and sacral departments of a backbone. Radiological note straightening fiziol. bends of a backbone and calcification of intervertebral disks.

The diagnosis establish a wedge on the basis. data and detection in urine homogentistic to - you.

Treatment is directed to decrease alkaptopuriya (see). For this purpose appoint methylthiouracil, ascorbic to - that. The favorable effect is rendered by steroid hormones and corticotropin (AKTG).

Forecast, as a rule, adverse.

Bibliography: Lille R. Patogistologicheskaya of the technician and a practical histochemistry, the lane with English, page 380, M., 1969; Reynberg S.A. Radiodiagnosis of diseases of bones and joints, book 2, page 510, M., 1964; Gould L. and. lake of Cardiac manifestations of ochronosis, J. thorac. cardiovasc. Surg., v. 72, p. 788, 1976; Steiger U. Ochronose, Z. Rheumaforsch., Bd 22, S. 367, 1963.

G. M. Mogilevsky.