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

Seven tetramethylrhodamine B isothiocyanate- (TRITC) labeled lectins: lens culinaris (LCH), ulex europeus-1 (UEA-1), lycopersicon esculentum (LEA), wheat germ agglutinin (WGA), dolichos biflorus (DBA), soybean agglutinin (SBA) and erythrina cristagalli (ECA) were applied on cultured human megakaryocytes (Megs) detected by immunofluorescence. All stages of Megs (from lymphocyte-like Megs to mature Megs) and platelets were labeled by LCH, LEA, UEA-1 and WGA. ECA binds to platelets but only to some Megs. DBA did not bind to platelets but did bind to some Megs, irrespective of stage. SBA binds to all stages of Megs, but did not bind to platelets. These results indicate the presence of mannose, glucose (LCH), sialic acid (WGA), and glucosamine (UEA-1, LEA, WGA) on the surface of all cells of the Meg lineage, a variable presence of galactosamine (DBA, SBA, ECA), and a discrepancy in the presence of some galactosamine compounds between platelets and Megs (DBA, SBA).
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PMID:Carbohydrate composition of human megakaryocyte membranes in culture: identification using binding of seven lectins. 206 23

The carbohydrate compounds of the mucus of flask cells in the kidney of claw-frogs (Xenopus laevis) were analysed through lectin binding studies. After removing epoxy resin semithin sections were incubated with 7 lectins (WGA, RCA I, PNA, LCH, UEA, LPA) marked by horseradish peroxidase and 2 unmarked lectins (VAA, Con A). The glycosaminoglycans in the canalicular lumen of flask cells showed a strong reaction with WGA and RCA, whereas the binding of PHA, Con A, and LCH was weaker. No reaction was observed with PNA, UEA, LPA, and VAA. The mucus of the flask cells seems to be rich in N-acetyl-glycosamine and -galactosamine. It contains also mannose, glucose, and galactose, but seems to have no fucose or N-acetyl-sialic acid residues.
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PMID:Lectin binding on carbohydrate compounds of the flask cells in the claw-frog kidney. 314 43

Binding patterns of fluorescein isothiocyanate (FITC)- and gold-conjugated lectins to extracellularly melanized sheathed and exsheathed microfilariae of subperiodic Brugia malayi, isolated from and in situ in the abdominal hemocoel of Anopheles quadrimaculatus 72-hr postinfection, were examined. Five FITC-conjugated lectins [Helix pomatia agglutinin (HPA), Arachis hypogaea (peanut agglutinin-PNA), Triticum vulgaris (wheat germ agglutinin-WGA), Lens culinaris (lentil-LCH), and Concanavalin A (Con A)] with specificities for different carbohydrate moieties were tested for binding to isolated melanized microfilariae and observed with transmitted light and fluorescence microscopy. All five FITC-lectins bound strongly to the acellular material accompanying the melanin deposits on the surface of isolated melanized microfilariae. Significant inhibition of FITC-lectin binding occurred when lectins were preincubated with their complementary carbohydrates before testing. H. pomatia agglutinin binding was totally inhibited by N-acetyl-D-glucosamine and N-acetyl-D-galactosamine. Other lectins were partially inhibited, such as PNA by galactose and lactose; WGA by N-acetylneuraminic acid; LCH by N-acetyl-D-glucosamine, mannose, glucose, and methyl alpha-D-mannopyranoside; and Con A by mannose and methyl alpha-D-mannopyranoside. Three gold-conjugated lectins (HPA, PNA, and Con A), examined by using transmission electron microscopy, bound to the outer surface of the acellular material associated with the melanin deposits on isolated melanized microfilarial sheaths and melanized microfilariae and to the remnants of lysed hemocytes found in the proximity of the melanized deposits. Con A in the presence of gold-labeled horseradish peroxidase, examined by using transmission electron microscopy, showed random binding within the melanized capsule formed around the microfilarial sheath in situ. These results indicate that the acellular material accompanying melanin deposits on melanized microfilarial sheaths and sheathed and exsheathed microfilariae contain several glycoconjugates with exposed carbohydrate moieties and are possibly glycoproteins. These glycoproteins could be the by-products of the activation of the prophenoloxidase by the microfilariae.
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PMID:Lectin binding to extracellularly melanized microfilariae of Brugia malayi from the hemocoel of Anopheles quadrimaculatus. 856 82

An 18 year old girl presented with acute visual loss. T2 weighted brain MRI showed areas of hyperintensities in the thalamic nuclei, internal capsule, lentiform nuclei, the subarachnoidal spaces, and a retrobulbar infiltration. Analysis of CSF showed numerous foamy histiocytes without malignant cells, raised protein, and depressed glucose concentration. Biopsy of the right thalamus demonstrated aggregates of histiocytes with immunohistological and ultrastructural characteristics of non-Langerhans cell histiocytosis. The patient improved with chemotherapy and corticosteroids. After 3 months of treatment, CSF analysis showed no more histiocytes. Cytological examination of CSF can be helpful for the management of patients with extensive histiocytic infiltration.
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PMID:Atypical form of non-Langerhans histiocytosis with disseminated brain and leptomeningeal lesions. 1103 28

We used positron emission tomography (PET) to characterize cerebral metabolism in 7 patients (serial examinations in 2 patients) with central nervous system disease in Langerhans cell histiocytosis (CNS-LCH) who had neuroendocrine abnormalities (n = 7), neuropsychiatric disabilities (n = 3), and CNS degenerative disease (n = 1). The PET scan alterations occurred at localizations with known CNS-LCH disease. The PET scans revealed areas where the metabolism and function were altered in 6 of the 7 patients studied, with either an increased or a decreased metabolism (uptake of glucose). Serial examinations may indicate alterations in the degree of ongoing disease activity, but further studies on functional imaging are desired. The additional information of PET compared with MRI is the ability to detect alterations in CNS metabolic activity in certain patients with CNS-LCH. PET may also provide a tool for longitudinal follow-up of therapeutic measures in selected patients.
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PMID:Brain 18-FDG PET scan in central nervous system langerhans cell histiocytosis. 1221 70

Central diabetes insipidus with an onset in adulthood is very rare. Unlike in children, central diabetes insipidus in adults is more frequently caused by inflammatory processes and neoplastic infiltrations that do not originate from the neuronal tissue than primary neuronal tissue tumours. Rare histiocytic neoplasias (Langerhans cell histiocytosis, xanthogranulomatosis and Erdheim-Chester disease) have a specific affinity to hypothalamus and the pituitary stalk not only in paediatric patients but also when occurring in adults. We describe 3 cases of central diabetes insipidus with an onset in adulthood. Diabetes insipidus was the first sign of Langerhans cell histiocytosis in 2 patients, and it was the first sign of Erdheim-Chester disease in one patient. MR imaging showed pathological infiltration and dilated pituitary stalks in all 3 patients. PET-CT proved useful in differential diagnosis, showing further extracranial pathological changes either on the basis of significant glucose accumulation or on the basis of CT imaging. The Langerhans cell histiocytosis in the first patient has also manifested itself as an infiltration of the perianal area with intensive accumulation of fluorodeoxyglucose (FDG) - SUV 8.6 and gingival inflammation indistinguishable from parodontosis. Histology of the perianal infiltrate confirmed Langerhans cell histiocytosis. Infiltration of the pituitary stalk disappeared from the MR image after 4 cycles of 2-chlordeoxyadenosin (5 mg/m2 5 consecutive days). The PET-CT of the 2nd patient showed only borderline accumulation of FDG in the ENT area, while simultaneously performed CT imaging showed cystic restructuring of the pulmonary parenchyma and nodulations consistent with pulmonary Langerhans cell histiocytosis. Bronchoalveolar lavage identified higher number of CD1 and S100 positive elements, consistent, once again, with pulmonary LCH also affecting pituitary stalk and ear canal. The PET-CT of the third patient showed increased activity in the long bones and ilium near the sacroiliac joint. Biopsy of the focus in the ilium confirmed foam histiocyte infiltration immunochemically corresponding to Erdheim-Chester disease. Additional imaging assessments revealed the presence of further signs of the disease. Pituitary infiltrate biopsy in this patient did not elucidate the diagnosis but resulted in complete panhypopituarism. Central diabetes insipidus in adulthood might be the first sign of so far undiagnosed extracranial disease, in our case of histiocytic neoplasias, and PET-CT has an excellent potential to detect extracranial symptoms of these conditions. Therefore, the high-risk pituitary stalk infiltrate biopsy should always be preceded by comprehensive examination aimed at identification of extracranial manifestations of the pituitary gland diseases.
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PMID:[Central diabetes insipidus in adult patients--the first sign of Langerhans cell histiocytosis and Erdheim-Chester disease. Three case studies and literature review]. 2032 85

Growth without growth hormone (GH) has occasionally been described in patients who have had tumors removed in the hypothalamic-pituitary area. Most of these patients have metabolic abnormalities such as obesity, dyslipidemia and fatty liver. This report describes the metabolic beneficial effects of GH replacement in pediatric patients with growth without GH. Two children in whom the growth without GH phenomenon occurred after therapy for brain tumors participated in this study. Case 1 is a 15-yr-old Japanese girl, diagnosed as having Langerhans cell histiocytosis with multiple intracranial lesions at the age of two. She showed a slight body fat increase, dyslipidemia and fatty liver. Case 2 is a 10-yr-old Indonesian boy, diagnosed with craniopharyngioma at the age of three. He was obese and had low bone mineral density (BMD). In both cases, GH replacement therapy was started at 0.042 mg/kg/week for 12 months. Body composition, BMD, and visceral abdominal area were measured every 3 months. Serum fasting blood glucose, insulin, ALT, lipid profile, leptin, and adiponectin levels were also measured every 3 months. Case 1 showed improvement of transaminase (ALT from 64 to 16 IU/L) and triglyceride (from 239 to 129 mg/dL) over 12 months, but did not show a decrease in visceral fat area or of body fat percentage. Case 2 showed a decrease in body fat percentage and visceral fat area, accompanied by elevated serum adiponectin and decreased leptin levels. In conclusion, twelve months GH replacement therapy improves metabolic abnormalities in pediatric patients with growth without GH.
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PMID:Metabolic effects of growth hormone replacement in two pediatric patients with growth without growth hormone. 2066 Sep 85

Langerhans cell histiocytosis (LCH) is a rare idiopathic benign disease characterized by proliferation of Langerhans cells, most commonly in the skull. In extremely rare cases, the tumor can occur in the sternum. A 4-year-old girl presented to our institution with a firm, nontender mass in her anterior chest that had indolently enlarged to approximately 2 cm in diameter over the previous several months. Computed tomography, magnetic resonance imaging, and F18-flurodeoxy-glucose positron emission tomography computed tomography were performed for preoperative diagnosis, but the findings were inconclusive. Therefore, we performed surgical excision under general anesthesia, followed by microscopic and immunohistochemical analysis of the excised specimen. The mass was eventually diagnosed as a LCH arising from the sternum. No postoperative oncological treatment was given, and follow-up has continued for 1 year until the time of writing without any tumor recurrence. To our knowledge, only 10 cases of LCH arising from the sternum have been reported in the medical literature. Among them, surgical approaches including curettage and partial sternotomy were performed in only 6 cases. Without exception, all patients experienced excellent clinical outcomes. Therefore, additional clinical experiences are required. No standard treatment of choice for this disease currently exists. In our experience, curettage of the involved soft tissue mass and bone followed by appropriate reconstruction of the defect is considered a good option for the treatment of solitary LCH of the sternum. In addition, LCH should be considered in the differential diagnoses when a sharp delineated osteolytic mass is detected in the sternum.
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PMID:Solitary Langerhans cell histiocytosis arising from sternum: a case report. 2224 35

Tolerance of microorganisms to diverse stresses (i.e., multistress tolerance) is a very useful property with industrial applications. We have developed a simple method for isolating multistress-tolerant semidominant mutants of the budding yeast Saccharomyces cerevisiae by one-step selection under lethal hydrogen peroxide (H(2)O(2)) stress condition, which we named the lethal concentration of H(2)O(2) (LCH) method. This method involves simply isolating colonies after plating of mutagenized S. cerevisiae cells, which are cultivated overnight in liquid media, on agar plates containing a lethal concentration of H(2)O(2) for the wild-type strain. Phenotypic and genetic analyses of the ten strains isolated by this method revealed that two strains exhibiting stress tolerance to H(2)O(2), ethanol, heat shock, salt, organic solvent, freeze-thaw, chronological aging, and high concentrations of glucose possess semidominant and distinct single-gene mutations designated as MLT1-1 (multistress tolerance) and MLT2-1, which are responsible for multistress tolerance. From these results, we expect this method to confer multistress tolerance on industrial yeasts.
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PMID:A new simple method for isolating multistress-tolerant semidominant mutants of Saccharomyces cerevisiae by one-step selection under lethal hydrogen peroxide stress condition. 2339 1

Langerhans cell histiocytosis (LCH) is a rare disorder of mononuclear phagocytic system whose clinical presentation varies from the localised involvement of a single bone to a widely disseminated disease. Langerhans cell histiocytosis rarely involves the thyroid gland and isolated involvement of thyroid is even rarer. We report a case of an eight-year-old male child diagnosed with Langerhans cell histiocytosis limited to thyroid gland with review of literature. Should thyroidectomy be done or proceed with chemotherapy? Our case report raises this question with a note on the role of 18 fluoro deoxy glucose positron emission tomography-computed tomography in the management of the same.
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PMID:Isolated Langerhans Histiocytosis in Thyroid: Thyroidectomy or Chemotherapy? 2650 Sep 97


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