Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Circulating immune complexes composed of HBcAg and anti-HBc have been demonstrated recently in patients with hepatitis B virus replication. After dissociation of immune complexes by chaotropic ions, HBcAg was quantified radioimmunologically. In the present study, we describe 10 patients with hepatitis B virus replication, absent or delayed anti-HBc formation and exposed HBcAg in serum. Four of the 10 patients had acute hepatitis, and six patients had chronic persistent hepatitis. In seven of 10 patients, a secondary immune defect was apparent due to acquired immunodeficiency syndrome, leukemia, histiocytosis X, sarcoidosis or end-stage renal disease. Electron microscopy demonstrated that Dane particles from anti-HBc-negative patients were agglutinated after addition of monoclonal anti-HBc antibodies, whereas Dane particles from anti-HBc-positive sera did not show agglutination. Monoclonal HBsAg-specific antibodies aggregated Dane particles independent of the presence of anti-HBc. Circulating HBcAg was always associated with the Dane particle fraction after density gradient separation. Hepatitis B virus core proteins from patients with and without anti-HBc studied by immunoblotting after sodium dodecyl sulfate-gel electrophoresis showed identical patterns. Hepatocytes from anti-HBc-negative patients were positive for HBcAg but negative for immunoglobulin G by immunofluorescence technique. The data indicate that HBcAg may also be expressed on the surface of Dane particles, where it is commonly masked by anti-HBc.
 ...
PMID:HBcAg expressed on the surface of circulating Dane particles in patients with hepatitis B virus infection without evidence of anti-HBc formation. 274 30

The diagnostic value of immunocytochemical tests was analysed for 19 cases of pulmonary histiocytosis X (PHX) and eight of other types of fibrosing pulmonary disease (sarcoidosis, 3; exogenous allergic alveolitis, 3; chronic pneumonia, 1; fibrosing alveolitis, 1). The cellular, proliferative-fibroblastic and fibrocystic stages in the course of pulmonary changes were differentiated. PHX cells reacted with anti-S 100 protein in all stages. In three cases for which unfixed tissue was available, all PHX cells reacted with antibody Leu-6, and 35% of these cells also reacted with the proliferation marker Ki-67. The few S-100 positive cells from the eight controls were limited to peribronchial tissue. Thus the antibodies Leu-6 and S-100 are useful aids in the diagnosis of PHX.
 ...
PMID:[Immunocytochemical studies in the diagnosis of pulmonary histiocytosis X]. 278 67

In our recent experience, the posttraumatic diabetes insipidus (PT.DI) and idiopathic (I.DI) are the most common forms of central diabetes insipidus (C.DI) in adult patients. The hypothalamo-pituitary function in these patients may be quite heterogeneous. We evaluated this aspect in 32 patients with different forms of C.DI (19 males and 13 females; aged 16-55 yrs): 12 with previous severe cranial or general trauma; 8 with CNS lesion due to Tuberculosis, Sarcoidosis, Histiocytosis X or to other pathogenic noxa (Secondary DI); 12 with idiopathic form. In all we measured ACTH, TSH, FSH, LH, PRL and target hormones (pl. cortisol, T3 T4, Testosterone) in baseline conditions with and without substitutive DDAVP therapy. In all cases the hormonal pattern was within the normal range. In several patients stimulation test with specific releasing factors (TRH, LHRH, oCRH) were carried out. Although basal anterior pituitary function is usually normal in patients with central DI (post-traumatic, idiopathic or secondary), an isolated subclinical secondary or tertiary hypothyroidism can be observed in some cases. Thus, a more accurate, periodical, and complete hormonal evaluation is indicated in some patients. The maintained response of ACTH to CRH (even increased after acute withdrawal therapy) indicated that AVP is not necessary to ensure normal function to the CRH-ACTH axis.
 ...
PMID:[Anterio-hypophyseal function in central diabetes insipidus]. 281 47

A series of human multinucleate giant cells (MGCs) of the endocytotic type were studied using enzyme histochemical methods for dehydrogenases, glycosidases, phosphatases, and peptidases. Several enzyme patterns were found. The subgroup of MGCs associated with inflammatory granulomatous processes (sarcoidosis, granulomatous myositis, familial granulomatosis, lymphogranuloma, granulomatous cholangitis) was characterized by high activities of nonspecific esterase (NE) and tartrate-sensitive acid phosphatase (AcPase-Ts). There was no detectable activity of peptidases or tartrate-resistant isoenzyme of acid phosphatase (AcPase-Tr). This enzyme equipment was indistinguishable from that in mononuclear precursors in the granulomas. The other MGCs of the series displayed enzyme patterns substantially different from their monocytic precursors (blood monocytes and Langerhans cells). The subgroup of foreign body associated MGCs (resorption of fat, keratin, and suture material) was characterized by high activities of NE, AcPase-Tr, and greatly variable activities of both peptidases studied. The latter lacked predilection for certain subcellular regions. The subgroup of osteoclasts and so-called giant cell tumours (osteoclastoma, giant cell tumour of soft parts, giant cell epulis of peripheral, and central types) displayed very low activity of NE, high activity of AcPase-Tr, and strong activities of peptidases. The latter were localized near the surface membrane of the polykarya. MGCs in histiocytosis X (HX) differed from the previous group by higher values of NE in average. All MGC types had common denominator in the absence of alkaline phosphatase activity, on average intense dehydrogenase activities, mostly low beta-glucuronidase and highly variable alpha-mannosidase activities. The enzyme pattern heterogeneity is discussed with regard to the phenomenon of enzyme induction and depression occurring in course of polykaryon production. The variability of phenomenon may reflect reactive adaptation to varying functional demands imposed on MGCs under different conditions.
 ...
PMID:Enzyme patterns in human endocytotic multinucleate giant cells--a histochemical study. 287 82

Epithelial membrane antigen (EMA) appears to be a marker of activation, proliferation, and/or neoplasia in some epithelial and nonepithelial cells, including histiocytes. We performed immunoperoxidase stains for EMA on a variety of histiocytic lesion specimens, including specimens from two cases of interdigitating reticulum cell lymphoma, 12 cases of histiocytosis X, seven cases of sarcoidosis, five cases of granuloma annulare, 13 juvenile xanthogranulomas, two reticulohistiocytomas, five xanthelasmas, three dermatopathic lymph nodes, and three foreign body reactions. Only the two cases of interdigitating reticulum cell lymphoma and two of the 12 cases of histiocytosis X exhibited significant EMA positivity. These findings may prove useful in the differential diagnosis of histiocytic lesions.
 ...
PMID:Epithelial membrane antigen staining patterns of histiocytic lesions. 310 82

Skeleton and soft tissue findings in systemic dermatoses sometimes allow early differentiation between diseases affecting the skin only and those involving other organs too. Rarely, ossious alterations can be detected even before any cutaneous manifestations have shown up. Apart from a tabular survey, we refer in detail to the frequency as well as the diagnostic value of ossious and soft tissue findings in dermatomyositis, lupus erythematosus, systemic sclerosis, psoriasis, Reiter's disease, sarcoidosis, neurofibromatosis (Recklinghausen's disease), histiocytosis X, and pretibial myxedema. Particular attention is paid to the problems of differential diagnosis with special regard to rheumatoid arthritis.
 ...
PMID:[Roentgenmorphologic findings in systemic dermatoses]. 329 34

The clinical and histopathological findings are presented in five patients with granuloma faciale. The lesions most often occur on the face and are characterized by single or multiple soft, elevated, well-circumscribed nodules or plaques ranging in color from reddish purple to brown. The sites most commonly affected are the nose, temple, checks and forehead. The etiology of granuloma faciale is unknown. The condition is extremely persistent and may last for many years. Histologically, a narrow zone of uninvolved dermis is usually observed between the epidermis and the dermal, dense, polymorphous infiltrate at all levels of the corium. The infiltrate is usually diffuse or shows a nodular perivascular pattern, consisting of lymphocytes, plasma cells, neutrophils and histiocytes together with a varying number of eosinophils. In older lesions the formation of the fibrous tissue may be seen, accompanied by capillary proliferation. Differential diagnosis includes sarcoidosis, discoid lupus erythematosus, erythema elevatum et diutinum, angiolymphoid hyperplasia with eosinophilia and histiocytosis X. The diagnosis of granuloma faciale requires a synopsis of clinical and histological findings.
 ...
PMID:[Facial granuloma. On the clinic-histologic extent of variations of finding in 5 patients]. 338 62

Cigarette smoking produces marked alterations in the lung parenchyma and in the population of immune and inflammatory cells present in the lower respiratory tract. These cigarette-induced changes appear to influence the incidence of two different interstitial lung diseases, histiocytosis X and sarcoidosis. Smoking is a strong risk factor for the development of pulmonary histiocytosis X, since the incidence of smoking is very high among patients with histiocytosis X: 90% of the patients with histiocytosis X were smokers; 46% of the controls were smokers (p less than .001). In contrast, smoking appears to reduce the incidence of sarcoidosis: 31% of the patients with sarcoidosis were smokers (p less than .05 compared to controls). In an effort to understand how cigarette smoking influences the incidence of these two disorders, we compared the numbers and types of immune and inflammatory cells recovered by bronchoalveolar lavage from nonsmoking and smoking controls and patients with histiocytosis X and sarcoidosis. Although nonsmoking patients with histiocytosis X did not have a significant increase in the number of alveolar macrophages recovered by lavage (p greater than .2 compared to normals), smoking patients had an increase in the number of alveolar macrophages similar to that observed in the control population. In contrast, the number of macrophages recovered from patients with sarcoidosis who smoked was considerably less than that observed in normal smokers (p less than .05 comparing patients with sarcoidosis and controls who smoked 1-20 cigarettes/day). This difference in the intensity of the cigarette-induced macrophage alveolitis observed in the two patient groups may be important in explaining the opposite effects of cigarette smoking on the incidence of histiocytosis X and sarcoidosis.
 ...
PMID:Smoking and interstitial lung disease. The effect of cigarette smoking on the incidence of pulmonary histiocytosis X and sarcoidosis. 348 4

Granulomatous lesions of the cranio-facial area are frequent and various in their nature: lymphohistiocytic with or without eosinophils, tuberculoid-like with epithelioid and giant cells, or sometimes made essentially of giant cells. Their etiology can be known or easy to find: foreign body granuloma, sarcoidosis, leprosy, rhinoscleroma, fungal diseases especially zygomycosis and rhinosporidiosis, parasitic diseases. The lethal midline granuloma is a clinical entity characterized by its necrotic and relentlessly progressive destructive presentation. After elimination of a malignant process, especially lymphoid, and of a Wegener's granulomatosis the diagnosis will be "idiopathic midline non-healing granuloma". Some of them will stay located at the facial area; others will disseminate as a malignant disease. Central giant cell granuloma and histiocytosis X, especially eosinophilic granuloma, are two other varieties of granuloma, different of the former granulomatous infiltrates by their clinical presentation and their evolution.
 ...
PMID:[Craniofacial granulomatous lesions]. 352 24

Morphologic and immunohistochemical studies were made of open lung biopsies from 9 patients with pulmonary histiocytosis X (HX) and 12 patients with other conditions, and of skin biopsies from patients with cutaneous sarcoidosis, Chester-Erdheim disease, and eruptive histiocytoma. The monoclonal antibody OKT6 was detected with the use of goat anti-mouse IgG labeled with fluorescein (FITC) for light microscopy, and sheep antimouse Fab'2 fragment of IgG labeled with horseradish peroxidase (HRP) for immunoelectron microscopy. The presence of S-100 protein was revealed by an antibody prepared against bovine S-100 protein, using sheep anti-rabbit IgG labeled with FITC for light microscopy and with HRP for immunoelectron microscopy. OKT6 antibody and S-100 protein were detected simultaneously by double labeling with FITC and rhodamine. In all patients with pulmonary HX, the major cellular components (HX cells) of the granulomas showed labeling of the plasma membranes by OKT6 and of the cytoplasm by the anti S-100 protein antibody. The double-labeling technique demonstrated that the same cells carried both reactivities. Immunoelectron microscopy showed that the reactive cells had all the structural characteristics of Langerhans cells, including Langerhans cell granules. Cells reacting with OKT6 showed discrete internal labeling in some of the Langerhans granules, especially those in continuity with the plasma membranes. However, internal labeling of Langerhans granules was not demonstrated in preparations for the localization of S-100 protein. Control samples of sarcoid lesions and other pulmonary lesions unrelated to HX did not show any reactivity except in Langerhans cells; a skin lesion from a patient with eruptive histiocytoma contained OKT6-positive cells which did not have Langerhans granules.
 ...
PMID:Immunocytochemical characterization of pulmonary histiocytosis X cells in lung biopsies. 387 76


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>