Metabolic disorders



histiocytosis photo histiocytosis - a complex of pathological conditions and independent entities that is to enhance the proliferative processes histiocytes and accompanied by the development of specific granulomas in various structural units of the human body.

specific cell histiocytosis of Largengansa develops as a result of a monoclonal proliferation of histiocytes localized in the pulmonary interstitial and airspace of the lung.

histiocytosis X in adults observed at a frequency of no more than 5 episodes per 1 000 000 population and not on gender.

reasons histiocytosis

Despite the rapid development of diagnostic technologies in medicine and many research, significant etiopathogenetic causes provoking the development of histiocytosis, have been identified.

Most researchers refer to the occurrence of histiocytosis autoimmune theory, according to which the excess infiltration of bronchioles and alveolar interstitial monoclonal CD1a-positive Langerhans cells and a limited accumulation of

excessive lymphocyte, plasma cells, neutrophils and eosinophils.

Pulmonary histiocytosis is the most common form of this disease and is at least 90% of all cases of disease histiocytosis.

Specific pathologic sign of histiocytosis is considered systemic failure of various organic structures in the form of granuloma formation, the substrate histiocytes which are produced by the bone marrow.

According to the research, development histiocytosis promotes long-term exposure to cigarette smoke.Pathogenetic mechanism is to excessive proliferation of histiocytes, which depends on the activity of cytokines, which are released by alveolar macrophages as a response to the process of intake of cigarette smoke.

Symptoms Histiocytosis

histiocytosis characteristic manifestation is the gradual emergence and progressive increase in respiratory disorders in the form of shortness of breath and cough with difficult expectoration.In some situations, the first symptoms of histiocytosis may make the clinical manifestations of spontaneous pneumothorax (sharp pain in the affected side of the chest, increasing shortness of breath and dyspnea).

In 15% of cases of histiocytosis X has no characteristic clinical symptom, and the establishment of the correct diagnosis depends on the qualifications of the expert in the field of radiation diagnosis.

extrapulmonary clinical symptoms develop in the late period of the disease and is the appearance of pain in the bones, skin rash and increase daily urine output.

acute form with torpid course of the clinical picture is observed mainly in patients pediatric age category up to three years and, as a rule, this pathology is unfavorable course and ends with death.

Persons younger, more likely to develop primary-chronic form of histiocytosis having latent for a long period of development, and developed clinical picture.At the onset of the disease, patients complain of malaise, rapid fatigue, shortness of breath associated with excessive physical activity and discomfort in the chest that has nothing to do with pain.In situations where the patient has acute intense pain in one long side of the chest, the restriction of the respiratory activity and a dry hacking cough without sputum, we must assume the existence of a patient spontaneous pneumothorax form that complicates histiocytosis.

At involving in pathological process of bone tissue, the patient concerned painful aching in the bones preferentially localized in the pelvic bones, ribs, and skull.Often the destructive processes affecting the bone structure of the sella, and therefore, the patient has signs of damage to the hypothalamic-pituitary system, manifested in violation of antidiuretic hormone secretion and the development of clinical symptom of diabetes insipidus.This category of patients the declared dryness in the mouth, a constant feeling of thirst, a significant increase in the frequency and amount of urination.

primary objective examination of the patient allows to suspect the presence of a chronic pulmonary disease in the form of acrocyanosis, deformation of nail phalanges in an "hour glass" and xanthelasma in the upper eyelid.Severe curvature of the thoracic spine can be visualized even without the use of X-rays.Percussion and palpation of the chest causing severe pain.When histiocytic infiltration of the orbit of the patient noted a significant proptosis, and this symptom is often one-sided.

During the percussion lung fields generally can not determine the pathological changes of pulmonary sound, and only in the case of spontaneous pneumothorax in a patient says tympanitis.Auscultation changes in the appearance of histiocytosis is a weakened vesicular breathing, and if there was a complete absence of pneumothorax breathing on the affected side.

defeat of the digestive tract in the form of histiocytosis is manifested in the emergence of pulling pain in the right upper quadrant and increase the size of the liver.

histiocytosis in children

This pathology in childhood is extremely rare and has an aggressive course and poor prognosis in relation to the patient's life.Under a diagnosis of "histiocytosis" in pediatrics is meant a group of diseases with different clinical picture.

as instigators of excess monoclonal proliferation of histiocytes in childhood are the activation of the herpes virus type VI, a violation of the regulation of humoral immunity.

eosinophilic granuloma, or Taratynova disease is the most favorable in regard to the alleged recovery, and in most cases has no clinical manifestations.Risk groups for this disease are children of school age.In severe cases of eosinophilic granuloma, histiocytosis as a manifestation of the child complains of weakness, loss of appetite, pain in bones and joints without a clear localization.

laboratory signs of disease have no specificity and are manifested in the form of eosinophilia, increased ESR, moderate anemia.Ray imaging techniques can diagnose the disease as a result of destructive detection of specific lesions in the bone structure without concomitant osteosclerosis.

extremely difficult for histiocytosis occurs in the presence of a child's illness Abt-Letterer-Siwa, which most often occurs in infancy.For this disease is characterized by acute debut of clinical manifestations in the form of hectic fever type, seborrhea, generalized vesicular rash, hepatosplenomegaly, and the rapid development of complications.To serious complications of this form of histiocytosis is purulent otitis with signs of perforation of the eardrum, severe pneumonia.Among the changes in the laboratory should pay attention to high leukocytosis, a significant increase in ESR.The defeat of the bone is to define multiple destructive foci of various localization using ray imaging techniques.

histiocytosis in the form xanthomatosis occur in any age period, and show signs of diabetes insipidus, progressive obesity, lymphadenopathy, seborrhea, and stomatitis.Children suffering from this disorder, are lagging behind in physical development and are prone to the development of secondary infection.Xanthomatosis accompanied by marked changes in the laboratory analysis of the circulating blood as hypoalbuminemia, leukocytosis, eosinophilia, increased ESR and hyperglobulinemia.

Sinus histiocytosis belongs to the category rarely diagnosed pathologies, as in most cases, this disease has a clinical presentation simulating other diseases.In this form of histiocytosis notes isolated lymph nodes as an excessive accumulation of them proliferating histiocytes.Favourite localization of the pathological process in this case is the lymph collectors in the neck and throat.A special feature of this disease is part of its association with hematologic malignancies.The only reliable diagnostic criterion sinus histiocytosis is the detection of macrophages, foamy protoplasm vacuolated nuclei and giant lymphocytes in the lymph node biopsy specimen obtained by the method of biopsy.

Treatment histiocytosis

In determining in respect of tactics and treatment of a patient suffering from histiocytosis, a fundamental form of this disease and the extent of its prevalence.Severe forms of histiocytosis subject to comprehensive treatment in a hospital hematology profile, while some cases of histiocytosis may be spontaneous samorazreshenie without medical intervention.

Child patients subject to compulsory admission and conduct of combined hormonal and cytostatic treatment in case of an acute course of the disease.The drugs of choice in this situation is prednisolone at a daily dose of 40 mg per Leykeran and 0.1 mg per 1 kg baby weight orally.Most experts recommend using a cyclic method of application of this drug combination consisting of ten cycles of 14 days with intervals of 2 weeks.For less aggressive course of the disease with no evidence of generalized lesions, is not justified the use of hormone therapy, and treatment involves the use of symptomatic drugs use (Hypothiazid 12.5 mg orally Dekaris 1 tablet 1 time / day.).

histiocytosis X, accompanied by a primary lesion of the respiratory system, the patient requires a mandatory refusal of smoking, which is the basis of non-drug preventive treatment.With existing symptoms of bronchial obstruction, symptomatic patients recommended the appointment of bronchodilators (theophylline), and severe chronic disease with the development of irreversible changes in the lung tissue subject to surgery and transplantation of lung injury.

histiocytosis with a primary lesion of bone respond well to treatment with local effects of ionizing radiation and the subsequent course glucocorticoid--cytostatic therapy to prevent relapse.In case of failure of radiation therapy or existing patient contraindications to its use, it is necessary to consider the option of surgical treatment, which consists in a work zone kyurretazha or resection of bone destruction in conjunction with the local administration of intraosseous glucocorticoid drugs.In the postoperative period is mandatory the use of calcium supplements (Calcemin 1 tablet 1 time / day.), And B vitamins

Recently, good results of treatment of sinus histiocytosis, consisting in the exchange application of drugs based on interferon, lasting at least 1 monthin a daily dose of 3 000000 IU parenterally.

histiocytosis, accompanied by diffuse lesions of the skin, is subject to a category of diseases requiring the use monochemotherapy (Vepeside a daily dose of 100 mg per 1 m 2).

Despite the expressed health problems of patients with histiocytosis, this pathology responds well to treatment with the use of various techniques, so 80% of cases there is complete recovery of the patient.

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