Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0010200 (cough)
 23,843 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Histiocytosis X developed in five patients (one woman and four men) when aged between 15 and 44 years. The initial sign in four of them was eosinophilic granuloma of the bone, in one it was pulmonary involvement. In three patients the disease remained confined to bone, while in two it involved the lungs and central nervous system, respectively. Osteolysis regressed spontaneously in one of the men, while in the woman there has been no recurrence 8 years after resection of the focus. In another man an osteolytic focus in a rib was noted after a 9-year recurrence-free interval. The man with pulmonary and bone involvement received chemotherapy with vinblastine and prednisone: dyspnoea and cough disappeared, vital capacity improved and the interstitial lung changes regressed. The osteolytic foci were repeatedly irradiated in the man with bone and CNS involvement. This brought about considerable reduction in pain but no significant radiological changes of the foci. Two courses of chemotherapy were given over 12 years, once with vincristine and prednisone, afterwards with cyclophosphamide. This arrested the progression of the osteolytic foci, but each time they recurred when the drugs were stopped.
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PMID:[Histiocytosis X. Clinical aspects and course in 5 patients]. 193 52

A detailed retrospective analysis of 8 cases has been set up to obtain an update of the clinical, radiological, diagnostic and therapeutical aspects of pulmonary Langerhans' cell granulomatosis (PLCG), previously called Histiocytosis X. This disease represents 2.8% of interstitial lung diseases (ILD) in a registration by pneumologists in Flanders. Seventy five% of our patients were active smokers. Cough, dyspnoe and constitutional symptoms were the main presenting symptoms. There was a 37.5% frequency of pneumothorax during the whole disease evolution and all these patients had to be treated with chemical or surgical pleurodesis. Spirometric pattern was variable but CO-transferfactor (TLCO) was significantly impaired in all our patients. Radiologically nodules and/or cystic lesions were found with preserved volumes. Open lung biopsy led to the diagnosis in 6 of the 8 cases. Transbronchial biopsies (TBB) were found to be not sensitive. Systemic staging in our group showed 50% of the patients to have a second focus of organ involvement. Treatment with corticosteroids and possibly immunosuppressives was without clear effect. Since 25% of our patients ultimately became candidate for lung transplantation extrapulmonary staging is prerogative and the high frequency of pleurodesis procedures in the past medical history of our patients has important implications since bilateral pleurodesis is still a contraindication for lung transplantation in some but not all institutions.
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PMID:Pulmonary Langerhans' cell granulomatosis (histiocytosis X): clinical analysis of 8 cases. 986 60

Histiocytosis X (HX), also referred as Langerhans cell granulomatosis is a disorder characterized by the presence of destructive granulomas containing Langerhans cells, lymphocytes, eosinophils and fibroblastes in the involved organs. Three presentations are commonly observed: 1) nonproductive cough or effort dyspnea, 2) spontaneous pneumothorax 3) incidental pulmonary infiltrates on chest X-ray in asymptomatic patients. HRCT may be helpful in the initial diagnosis of pulmonary HX. HRCT scans show nodules, cysts and estimate the extent of disease. But the final diagnosis of histiocytosis X requires the histologic demonstration of specific histiocytosis X cell in biopsy specimens of the lung. The aim of this study was to define the importance of the detection of Langerhans cells in bronchoalveolar lavage fluid (BALF) for the diagnosis of HX. The searched cells express a specific CD1 antigen, recognized by the monoclonal antibody OKT-6. In our study the demonstration of more than 5% of CD1 positive cells was defined to confirm HX. We have studied the BALF in 21 patients with suspected histiocytosis X. In BALF of 4 patients more than 5% of CD1 positive cells were found. In 1 of them HX was confirmed with open lung biopsy. Two patients displayed 5% of CD1 positive cells. The final diagnosis of the first patient was hypersensitivity pneumonitis and of the second one was bronchitis chronica. In 5 patients out of 15 patients in whom less than 5% of CD1 positive cells were found histiocytosis X was histologically proven. In other 10 patients the following disorders were histologically recognised: pulmonary emphysema 3 cases, pneumoconiosis-3, LMA-BOOP-1, sarcoidosis-1 and pleuritis eosinophilica-1. The estimation of Langerhans cells in BALF can be a useful method among the diagnostic procedures for histiocytosis X. It is necessary to remember that demonstration of less than 5% of CD1 positive cells do not exclude histiocytosis X.
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PMID:[Usefulness of CD1 expression on surfaces of cells in bronchoalveolar fluid for diagnosis of histiocytosis X--our experience]. 1064 82

Histiocytic disorders are a group of rare diseases with systemic involvement and with multiple clinical manifestations. We present the case of a 51 years old patient investigated for dyspnea with orthopnea, dry cough, asthenia, muscular weakness and ataxia. The association of previous symptoms with skin lesions, diabetes insipidus, partial hypophyseal insufficiency and pericarditis induced many diagnostic debates. The diagnosis is Histiocytosis X must be sustained by tissue biopsy with immunohistochemical assay or genetic testing. The particularity of our patient is the presence of pericarditis, rarely associated with histiocytosis. Collaboration between medical specialties is mandatory in order to treat this disorder.
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PMID:Histiocytosis X and Pericarditis - A Rare Association and a Difficult Diagnosis. 3058 98