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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A retrospective study was undertaken of 1005 normal contrast-enhanced head computed tomographic (CT) scans in children to determine normal standards for pituitary stalk diameter, basilar artery diameter, and their ratio for age and gender. The pituitary stalk enlarges with age, and girls have larger stalks than do boys after age 8. The pituitary stalk-to-basilar artery ratio is easily estimated visually. Ratios greater than or equal to 1 are unusual in normal children. A ratio greater than or equal to 1 should prompt direct measurement of the stalk and comparison with age-matched normal values. If the stalk measurement is greater than 2 SD above the age-matched mean, it is presumably abnormal and deserves further evaluation for numerous possible pathologic conditions including histiocytosis X, pituitary adenoma, hypothalamic lesions, Rathke cleft cysts, metastases, sarcoidosis, hypothyroidism, and infection.
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PMID:Normal pituitary stalk size in children: CT study. 387 41

Bronchoalveolar lavage has emerged as a useful technique for the study of pulmonary interstitial disorders. Several types of information are provided by the evaluation of lavage fluid. First, the identification of cellular constituents helps to separate inflammatory processes in which lymphocytes predominate (for example, sarcoidosis, hypersensitivity pneumonitis, and berylliosis) from those in which neutrophils or macrophages predominate (for example, idiopathic pulmonary fibrosis and histiocytosis X). Second, the cells removed during lavage can be studied for their immune properties and function; tested with specific antigens, in diseases such as berylliosis and hypersensitivity pneumonitis; and examined for the presence of unique surface antigens with monoclonal antibodies (for example, histiocytosis X). Third, in conjunction with scanning electron microscopy and electron probe analysis, lavage makes possible the identification of inorganic particles in alveolar macrophages of patients with pneumoconiotic lung disease. Finally, although lavage is still an investigative procedure for most pulmonary disorders, it has an established role in the diagnosis of opportunistic infections in the immunocompromised patient.
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PMID:Bronchoalveolar lavage: role in the pathogenesis, diagnosis, and management of interstitial lung disease. 388 Oct 71

Chemotaxis is a property common to all free cells or unicellular microorganisms. It is not a simple spontaneous cellular migration but one which is directed towards the source or nucleus, producer of the chemotactic substance. One of the first phenomenon which is established as a defense mechanism of the organism is the attraction of polymorphonuclears. In 1955 Rebuck and Crowley described a method, "skin window" for the study of in vivo leukocyte chemotaxis. The aim of this work was to go deeper into the study of this test and to establish its clinical use. Two hundred and seventy patients from both sexes were studied and divided into five groups: Group I - 60 healthy subjects as control. Group II - 60 patients with pathologic leukocyte response: 10 cirrhotics, 15 Hodgkin's disease, 15 chronic renal insufficiency, 2 drepanocytosis and 3 sarcoidosis. Group III - 60 patients with no theoretical alterations in the leukocyte chemotaxis: 22 bronchial asthma, 23 nonlymphoid neoplasm, 13 iritis and 2 histiocytosis X. Group IV - 40 active tuberculosis patients. Group V - 30 patients with bacterial pneumonia non-tuberculosis. The Rebuck test was carried out on all patients. As lymphocyte markers, E rosettes, superficial immunoglobulins and the lymphoblast transformation test against PHA were performed on all the groups of patients. As to the results obtained, the positive responses for Groups I, II, III, IV and V were 87%, 28%, 83.3%, 45% and 63.3%, respectively. These results were evaluated in relation to the Mantoux reaction. The modified Rebuck test is useful for leukocyte chemotactic study. This was found to be altered in 13% of the healthy population.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Behavior of leukocyte chemotaxis in various clinico-immunological situations]. 389 89

A case of Langerhans' cell granulomatosis associated with gastric adenocarcinoma is reported. A review of the literature demonstrate an association of this entity with Hodgkin or non-Hodgkin lymphomas alone. The discussion is centred on differential diagnosis from the pseudo-sarcoid granulomatous reaction. Further reports may assist in classifying the granulomatous reaction to tumours into two types, epithelioid and Langerhans.
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PMID:Solitary Langerhans' cell granulomatosis of the stomach associated with gastric carcinoma. 393 69

The histopathologic and ultrastructural features of intraluminal organizing and fibrotic changes were studied in open lung biopsies and autopsy specimens from 373 patients with interstitial lung disorders, including hypersensitivity pneumonitis (n = 44), idiopathic pulmonary fibrosis (n = 92), collagen-vascular diseases (n = 20), chronic eosinophilic pneumonia (n = 10), pulmonary histiocytosis X (n-90), pulmonary sarcoidosis (n = 62), pneumoconioses (n = 25), Legionnaire's disease (n = 5), drug- and toxin-induced pneumonitis (n = 4), radiation-induced pneumonitis (n = 2), lymphangioleiomyomatosis (n = 11), and chronic organizing pneumonia of unknown cause (n = 8). Three patterns of intraluminal organization and fibrosis were recognized: 1) intraluminal buds, which partially filled the alveoli, alveolar ducts and/or distal bronchioles; 2) obliterative changes, in which loose connective tissue masses obliterated the lumens of alveoli, alveolar ducts or distal bronchioles, and 3) mural incorporation of previously intraluminal connective tissue masses, which fused with alveolar, alveolar ductal, or bronchiolar structures and frequently became reepithelialized. All three patterns had common morphologic features, suggesting that, regardless of their severity, they resulted from a common pathogenetic mechanism, ie, the migration of activated connective tissue cells, through defects in the epithelial lining and its basement membrane, from the interstitial into the intraluminal compartment. Intraluminal buds were observed most frequently in hypersensitivity pneumonitis, chronic eosinophilic pneumonia, and organizing pneumonia of unknown cause. Mural incorporation and, to a lesser extent, obliterative changes were observed in most interstitial disorders and were very prominent in idiopathic pulmonary fibrosis. Mural incorporation and obliterative changes play an important role in pulmonary remodeling, especially when several adjacent alveoli and/or other air spaces are involved. Under these circumstances, intraluminal organization can mediate the fusion of adjacent alveolar structures by intraluminal connective tissue.
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PMID:Intraluminal fibrosis in interstitial lung disorders. 395 68

Bronchoalveolar lavage is a simple technique, complementary to fiberoptic bronchoscopy. The material yielded comprises cells and supernatant, and both components may provide information about the distal lung structures. A few counter-indications should be respected. Normally, the cell differential comprises: 93 +/- 5% alveolar macrophages, 7 +/- 1% lymphocytes and about 1% neutrophils, eosinophils and basophils. Total cellularity and cell differentials are altered in many conditions, according to different patterns, which permits a classification. The increase in numbers may relate to macrophages (smokers, pneumoconiotic disorders...), to lymphocytes (sarcoidosis, hypersensitivity pneumonitis...) and to neutrophils and/or eosinophils (fibrotic disorders, chronic eosinophilic pneumonia, histiocytosis X...). In the last disease, the diagnosis may be established by detecting Langerhans cells. In other conditions, bronchoalveolar lavage mostly provides a trend to diagnosis. Usually well tolerated, bronchoalveolar lavage may usefully be repeated for monitoring patients with chronic interstitial lung disorders. It also gives an insight into the pathogenesis of many pulmonary pathological processes.
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PMID:[Exploratory bronchoalveolar lavage]. 623 22

The activity of the endothelial and macrophage derived angiotensin-converting enzyme in blood was within normal range in 12 patients with histiocytosis X, independent of extent and activity of the disease. In contrast to sarcoidosis and Gaucher's disease, histiocytosis X is not associated with elevated levels of angiotensin-converting enzyme.
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PMID:Histiocytosis X. VI. Angiotensin-converting enzyme activity. 626 40

Respiratory tract fluid, produced from an enormous area spanning the mucosa of the nose to the alveolar surface, is a complex mixture of serum transudate and locally secreted proteins and glycomucoproteins and of inflammatory and immune effector cells intermingled. Its analysis is important in understanding the pathogenesis of respiratory diseases and remains essential for the clinical diagnosis of most lung disorders. Many basic facts about the formation and composition of this fluid remain unknown, and little information exists about absorptive mechanisms along the airways. Respiratory fluid is not homogeneous but has unique regional characteristics that are becoming better appreciated as more selective sampling methods are devised. Above all, it is a dynamic substance in healthy airways and diseased ones, and any specimen is just a point-in-time sample that can change in composition, often making serial analysis and comparisons necessary. Nasal fluids currently have limited diagnostic application except in allergic rhinitis. Expectorant (sputum) telescopes fluid and cells from all areas and is not from a specific locale, so the trend is to retrieve more selective and regional specimens of airways fluids. Technology largely does not exist to collect area specimens, except for bronchoalveolar lavage, which generally samples the alveolar surface. Clearly, bronchoalveolar lavage fluid analysis has been the favored way to characterize the peripheral air-spaces for the past 10 years or so, and most of this monograph has been devoted to normal data derived from lavage specimens and to a few examples of lung disease that reflect this burgeoning application. In many respects, results obtained from lavage fluid are virtually in catalogue form at present, and it remains to the future to know how some of the observations will help make diagnosis better or elucidate pathogenic mechanisms. Generally, bronchoalveolar lavage fluid analysis has led to better concepts of immunopathology of many diseases and provided new ways to monitor the evolution of certain diseases, especially the diffuse interstitial lung disorders, but development of specific criteria for diagnosis has been less rewarding. However, certain patterns of lymphocyte-predominant alveolitis, suggesting sarcoidosis or hypersensitivity diseases, recognition of specific T lymphocyte defects and opportunistic microorganisms as in AIDS, and the use of microprobe electron analysis to identify cellular particulates all point to more precision of diagnosis. Alveolar proteinosis and histiocytosis X may be detected from lavage fluid components. The prospects are truly exciting.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Respiratory tract fluids: analysis of content and contemporary use in understanding lung diseases. 636 22

Based on the finding that Langerhans cells and histiocytosis X cells react with the monoclonal antibody OKT6, raised against a subset of thymocytes, we used this antibody to study the cells collected by bronchoalveolar lavage (BAL) from 131 patients, including 18 with pulmonary histiocytosis X, 43 with pulmonary sarcoidosis, 67 with miscellaneous pulmonary disorders, and 3 controls. Immunofluorescence studies demonstrated the presence of OKT6-reactive cells in all patients with pulmonary histiocytosis X (mean +/- SEM, 5.29% +/- 1.14% of all cells in BAL fluid). Immunoelectron microscopic studies revealed that the cells labeled in these patients (n = 13) contained Langerhans granules. The number of fluorescent cells in the other 113 patients was significantly smaller (mean +/- SEM, 0.20% +/- 0.04% of all cells; P less than 0.001). In the 3 control patients, in the 43 patients with sarcoidosis, and in 61 of the 67 patients with miscellaneous disorders unrelated to histiocytosis X, no cells or less than 1% of the total were labeled; however, in the 6 remaining patients in this miscellaneous group, 1.3 to 2.8% of all cells in BAL were labeled. In 3 of these 6 patients, immunoelectronmicroscopic examination showed that the cells labeled by OKT6 had the general characteristics of Langerhans cells but lacked Langerhans granules. OKT3, OKT4, and OKT8 monoclonal antibodies did not stain histiocytosis X cells in BAL fluid.
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PMID:Diagnosis of pulmonary histiocytosis X by immunodetection of Langerhans cells in bronchoalveolar lavage fluid. 637 96

Seven cases are presented in which enlargement of the pituitary stalk was demonstrated by computed tomography (CT). Histiocytosis X, sarcoidosis, and metastatic cancer were the proven or presumed causes. The discovery of pituitary stalk enlargement prompted radiation treatment in three patients and led to the diagnosis of previously unsuspected diabetes insipidus in one.
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PMID:CT of the abnormal pituitary stalk. 642 Nov 24


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