In the case of treatment, the disease proceeds more slowly, dragging on for many decades. In young children, the process, taking a generalized form, flows more sharply and heavily. The outlines of the defects are sharp, even (Fig.), sometimes finely serrated; in some cases, there is a zone of protonix pills of bone tissue around the defects, and between them there are foci of compaction. Depending on the course of the disease, defects increase or decrease,disappear. Of particular importance is the change in the Turkish saddle. The photographs show the demineralization of the back of the saddle, its expansion and flattening, the absence of the posterior process, and the deviation of the back.
In other bones, as in the bones of the skull, the size and degree of damage are different: from a slight, barely noticeable bone rarefaction to the formation of large rounded defects. In tubular bones, defects take the form of cystic formations or subperiosteal foci. On x-ray examination of the chest organs, the accumulation of xanthoma cells along the bronchi looks like peribronchitis, interstitial pneumonia, and pulmonary infiltrates. In some children, the disease is accompanied by periodic increases in t° up to 38-39°. A blood test reveals moderate anemia, moderate leukocytosis, eosinophilia up to 8-13%, accelerated ESR. Sometimes biochemical blood tests help in the diagnosis, in which lipoidemia and an increase in cholesterol are found.
In the initial phase, the disease is often diagnosed as osteomyelitis, tuberculosis, sarcoma, myeloma, osteochondropathy, etc.; cases of pantoprazole eosinophilic granuloma can present great difficulties for diagnosis (see). Systemic enlargement of the lymph nodes, liver and spleen requires the exclusion of inflammatory (specific and nonspecific) processes, tumors, diseases caused by impaired lipid metabolism (see Gaucher disease, Niemann - Peak disease), leukemia (see), lymphogranulomatosis (see).

Assign a diet poor in cholesterol, pituitary drugs, blood transfusions, antibiotics, steroid therapy and radiation treatment. X-ray therapy is not always accompanied by long-term success, since after the rapid resorption of irradiated lesions, new defects are formed. The debilitating effect of diabetes insipidus on the body has recently made it necessary to begin treatment with irradiation of the interstitial brain and epididymis in small doses - 30-50-100 r in 1-3 days or more, bringing the total dose to 200-400 r. Repeated irradiations are carried out after obtaining the effect of the first course.

The diagnosis of an illness of Hand — Schuller — Christian in classical cases is simple; in unclear cases, it can sometimes be put only on the basis of biopsy data or postmortem. X-ray therapy, applied in a timely manner, stops the development of local foci and delays the generalization of the process. The prognosis is poor; spontaneous remissions are possible.

Skull of a 3.5-year-old child suffering from Hand-Schuller-Christian disease: outlines of defects in the parietal and occipital bones with clear, even contours. Hand-Christian-Schuller disease - lipoidosis, disorder of the function of the reticuloendothelial system. Children usually get sick. In the bones, especially the skull, foci containing cholesterol and filled with soft tissue develop. As a result of changes in the bones of the eye sockets, bulging eyes develop.

Diabetes insipidus, adipose-genital-pituitary dystrophy, loosening and swelling of the gums, loosening of the teeth, small yellowish nodules in the skin are noted. Idiopathic fractures occur in long bones at sites of cholesterol deposition. X-ray in the bones are determined areas of osteoporosis with uneven contours of unequal intensity.

The liver and spleen are somewhat enlarged. There is a lot of cholesterol in the blood, slight hypochromic anemia. Bone marrow contains numerous reticular cells with cholesterol deposits. In the cells of parenchymal organs, there is a significant accumulation of cholesterol. The disease was first described by Hand in 1893; he observed lesions of pantoprazole, bones of the skull, enlargement of the liver and spleen in a three-year-old child. Schüller (1916) and Christian (1919) identified a triad of symptoms in this disease: flat bone defects, exophthalmos (see) and diabetes insipidus (see Diabetes insipidus).

Etiology and pathogenesis are not fully understood. A number of researchers attribute the changes in Hand-Schuller-Christian disease to reactive ones, and the disease itself to infectious diseases, possibly of viral etiology. Pao (Rao) et al. (1970) believe that an inadequate response of the body to any infection contributes to the onset of the disease; Nesbit and Krivit (M. Nesbit, W. Krivit, 1975) associate the development of the disease with immunological deficiency. Other researchers believe that the disease is based on a dysfunction of the mononuclear phagocyte system and ondestruction of fat metabolism; they refer it to xanthomatosis (see).

Macroscopic examination in the bones, especially in the bones of the skull, reveals multiple defects of various sizes filled with soft, sometimes crumbling tumor-like granulomatous growths of yellow-gray color. If the eye socket is damaged, exophthalmos occurs, if the bones of the base of the skull are damaged, the temporal bone can be destroyed, and in some cases the Turkish saddle, compression of protonix gland or the spread of granulomatous growths into it and the hypothalamic region. When the central unequal system is involved in the pathological process, thickening of the meninges and infiltration of the brain substance are observed.

In the lungs, nodules and strands of granulomatous growths are found, which are located subpleurally, around the vessels and bronchi, along the interalveolar septa, which is often accompanied by diffuse interstitial sclerosis and signs of pulmonary heart disease (see Cor pulmonale). There is an increase in the liver, spleen, lymph nodes. Gingival hypertrophy, tooth loss, focal thickening and ulceration of the mucous membrane of the gastrointestinal tract can be observed. Skin lesions are manifested by papular rashes, hemorrhages, ulcerations are possible.

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Microscopically tumor-like granulomatous growths consist of histiocytes with an admixture of scattered or lying in the form of clusters of lymphocytes, plasma cells, eosinophilic leukocytes, mainly segmented. Siderophages may occur (see Brown compaction of the lungs). The presence of a large number of xanthomous (often giant multinucleated) macrophage cells with foamy vacuolated cytoplasm due to the accumulation of cholesterol and cholesterol esters is characteristic (printing. Fig. 5 and 6). The ratio of cellular elements may vary, reflecting different stages of the process.

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Tubular bones swell from the inside, contain multiple foci of destruction of various sizes, the cortical (compact) substance is thinned, no sequesters are observed. At a subkortikalkhm an arrangement of the centers there can be periostitis (see).

The defeat of the vertebrae is more often single, occurs mainly in the middle and lower parts of the thoracic spine. Sometimes compression of the vertebral bodies develops, with the formation of platybrachyspondylia (see Spine) with intact intervertebral discs. In rare cases, a pathological vertebral fracture may be the first clinical sign of the disease.

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Destructive processes in the area of ​​the pyramid, mastoid process and other parts of the temporal bone are the cause of ear damage. The Turkish saddle, as a rule, is not changed. In some cases, a slight compaction is detected. Of the bones of the facial skull, changes are more often found in the jaws, especially in the upper. With the defeat of the alveolar processes, an expansion of the dental alveoli is observed.eveol. In the pelvic area, foci of destruction are localized mainly in the wings of the ilium.

Moderate anemia (see), pancytopenia is sometimes noted in blood (see. Hypoplastic anemia); in the bone marrow punctate - the safety of megakaryocytes, sometimes infiltration with histiocytic elements. In the bones of the cranial vault, multiple irregularly rounded or oval defects with clearly defined corroded contours are formed without signs of osteoporosis and reactive sclerotic changes along the periphery. The sizes of the centers vary, sometimes the centers occupy extensive sites: bones.

In the punctate of tumor formations of the skin and soft tissues, infiltration with atypical histiocytic cells with macrophage function, signs of erythrophagocytosis are detected; as the disease progresses, such infiltrates are found in the bone marrow.

In rare cases, pathological fractures are complications. So, damage to the spine can lead to a compression fracture of the vertebrae (see Spine), cause compression of the spinal cord and radicular symptoms (see Spinal cord). The diagnosis is established on the basis of the clinical picture, the results of cytological and histological examination of lesions, as well as X-ray examination of the bones.

In 50-60% of patients, skin lesions are observed in the form of xanthoma, painted in yellow-brown color (see Xanthoma), vesicles, ulcerations, hemorrhages. When the lungs are involved in the process, cough, shortness of breath are noted. The disease can be chronic and progress slowly. Dissemination of the process with damage to the lymph nodes, liver, spleen and other organs is possible.

The destruction of the jaw bones contributes to loosening of the teeth, their loss, exposure of the roots of the teeth, the development of secondary gingivitis (see), stomatitis (see).

When the bones of the skull and pelvis are affected, a common symptom of the disease is bone pain. When the temporal bone is affected, non-infectious otitis media (see), resistant to antibiotic therapy, can develop. Due to the destruction of the bones of the orbit, exophthalmos occurs (Fig. 1), with the destruction of the Turkish saddle, there is a violation of the function of the pituitary gland, the hypothalamus with symptoms of diabetes insipidus (see Diabetes insipidus).

The cytoplasm of histiocytes is characterized by sudanophilia, diffuse PAS-positive reaction (see. PAS-reaction), high activity of hydrolases. An electron microscopic examination in the cytoplasm of histiocytes reveals the so-called. X bodies are elongated inclusions associated with the cell membrane, similar to lysosome-like granules of Langerhans cells of the spiny layer of the epidermis.