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https://www.readbyqxmd.com/read/28229176/denosumab-for-the-treatment-of-mastocytosis-related-osteoporosis-a-case-series
#1
Giovanni Orsolini, Irene Gavioli, Gaia Tripi, Ombretta Viapiana, Davide Gatti, Luca Idolazzi, Roberta Zanotti, Maurizio Rossini
The purpose of this study was to investigate the therapeutic effect of denosumab, an anti-RANKL monoclonal antibody for the treatment of bone loss in indolent systemic mastocytosis (ISM) patients intolerant to bisphosphonates. Four patients underwent upon informed consent a treatment with denosumab 60 mg administered subcutaneously every 6 months with the same regimen used for postmenopausal osteoporosis. Bone mineral density (BMD) was measured at lumbar and femoral sites at baseline and after 1 year. C-terminal telopeptide of collagen type I (CTX), bone alkaline phosphatase (bALP) and tryptase serum level were determined at baseline and after 12 months with fasting blood samples withdrawals...
February 22, 2017: Calcified Tissue International
https://www.readbyqxmd.com/read/28229121/intertriginous-maculopapular-mastocytosis-in-a-patient-with-acute-myeloid-leukemia
#2
María Herrero-Moyano, Tania Marusia Capusan, Alejandra Pérez-Plaza, Almudena Godoy, Javier Sánchez-Perez
No abstract text is available yet for this article.
January 2017: JAAD Case Reports
https://www.readbyqxmd.com/read/28185248/mastering-gut-permeability-new-roles-for-old-friends
#3
Michael Bramhall, Colby Zaph
Mast cells are innate immune cells that respond rapidly to infection in barrier tissues such as the skin and intestinal mucosa. Expulsion of parasitic worms in the gut involves a robust type 2 host response, and an acute mastocytosis is often generated at the site of infection. However, the role of mast cells in resistance to worm infections appears to be parasite specific. Mast cells are also involved in tissue repair, but the long-term contribution of mast cell activation after worm expulsion has not been definitively studied...
February 2017: European Journal of Immunology
https://www.readbyqxmd.com/read/28181948/kounis-syndrome-during-anesthesia-presentation-of-indolent-systemic-mastocytosis-a-case-report
#4
Elena de la Fuente Tornero, Arantza Vega Castro, Pedro Álvarez de Sierra Hernández, Javier Balaguer Recena, Sofía Carmen Zaragoza Casares, Francisco Miguel Serrano Baylin, Paloma Gallardo Culebradas, Beatriz Amorós Alfonso, Jose Ramón Rodríguez Fraile
Mastocytosis comprises a heterogeneous group of disorders characterized by mast cell accumulation and proliferation in distinct organs. Kounis syndrome is defined as the concurrence of acute coronary syndromes with mast cell activation in a setting of allergic or hypersensitivity reactions. This is the first reported case of an intraoperative Kounis syndrome as the onset of an indolent systemic mastocytosis probably triggered by succinylated gelatin infusion during general anesthesia. The presentation of this case is intended to contribute to the knowledge of mastocytosis and Kounis syndrome at the time of diagnostic workup during intraoperative anaphylaxis or myocardial ischemia...
February 8, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28181942/successful-management-of-a-patient-with-possible-mast-cell-activation-syndrome-undergoing-pulmonary-embolectomy-a-case-report
#5
Ellen W Richter, Kai-Ling Hsu, Vanessa Moll
We report the successful perioperative management of a patient with presumed mastocytosis undergoing pulmonary embolectomy. Postoperatively the patient went into vasodilatory shock, which was partly attributed to mast cell mediator release. H1- and H2-antagonists, steroids, and a single dose of methylene blue were given with improvement of hemodynamics. The patient was weaned off vasoactive substances and extubated by postoperative day 2. We discuss the perioperative management of patients with mastocytosis, briefly review the literature concerning anesthetic management for cardiac surgery in patients with this disorder, and discuss our patient's alternative but related diagnosis of idiopathic mast cell activation syndrome...
February 8, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28141734/anaesthesia-and-orphan-disease-rapid-sequence-induction-in-systemic-mastocytosis
#6
Christoph Unterbuchner, Marina Hierl, Timo Seyfried, Thomas Metterlein
No abstract text is available yet for this article.
March 2017: European Journal of Anaesthesiology
https://www.readbyqxmd.com/read/28135563/pathogenesis-and-pathology-of-mastocytosis
#7
Dean D Metcalfe, Yoseph A Mekori
Systemic mastocytosis is a clonal disorder of mast cells that may variably present with characteristic skin lesions, episodes of mast cell mediator release, and disturbances of hematopoiesis. No curative therapy presently exists. Conventional management has relied on agents that antagonize mediators released by mast cells, inhibit mediator secretion, or modulate mast cell proliferation. Recent advances in the molecular understanding of the pathophysiology of systemic mastocytosis have provided new therapeutic considerations, including new and novel tyrosine kinase inhibitors...
January 24, 2017: Annual Review of Pathology
https://www.readbyqxmd.com/read/28133781/longitudinal-study-of-pediatric-urticaria-pigmentosa
#8
Adam Heinze, Travis J Kuemmet, Yvonne E Chiu, Sheila S Galbraith
BACKGROUND/OBJECTIVES: Urticaria pigmentosa (UP) is the most common form of mastocytosis in children and is associated with systemic signs, symptoms, and triggers. To our knowledge, the effect of UP on children's quality of life has not been studied. The objective of the current study was to characterize the natural history, triggers, and complications of pediatric UP, identify prognostic indicators, and determine its effect on quality of life. METHODS: Between 2002 and 2007, children with three or more mastocytomas diagnosed by a pediatric dermatologist were recruited during visits at the Children's Hospital of Wisconsin Dermatology Clinic (Milwaukee, WI)...
January 30, 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28112808/nerve-growth-factor-a-neuroimmune-crosstalk-mediator-for-all-seasons
#9
REVIEW
Stephen D Skaper
Neurotrophic factors comprise a broad family of biomolecules - most of which are peptides or small proteins - that support the growth, survival, and differentiation of both developing and mature neurons. The prototypical example and best-characterized neurotrophic factor is nerve growth factor (NGF) which is widely recognized as a target-derived factor responsible for the survival and maintenance of the phenotype of specific subsets of peripheral neurons and basal forebrain cholinergic nuclei during development and maturation...
January 23, 2017: Immunology
https://www.readbyqxmd.com/read/28095350/progressive-multifocal-leukoencephalopathy-in-a-patient-with-systemic-mastocytosis-treated-with-cladribine
#10
Karl B Alstadhaug, Randi Fykse Halstensen, Francis Odeh
BACKGROUND: Progressive multifocal leukoencephalopathy (PML) is a rare opportunistic brain infection caused by the human polyomavirus JC (JCPyV). A particular problem with the drug cladribine seems to be prolonged suppression of the CD4+ T-cells, a well-known risk factor for PML. CASE DESCRIPTION: A 67-year-old male presented with a 3-weeks history of unsteady gait, dysarthria and a dysfunctional right arm. Seven years earlier, he had been diagnosed with urticaria pigmentosa, and 2 years later aggressive systemic mastocytosis...
March 2017: Journal of Clinical Virology: the Official Publication of the Pan American Society for Clinical Virology
https://www.readbyqxmd.com/read/28074523/familial-gastrointestinal-stromal-tumors-lentigines-and-caf%C3%A3-au-lait-macules-associated-with-germline-c-kit-mutation-treated-with-imatinib
#11
Divya Gupta, Laxmisha Chandrashekar, Lidia Larizza, Elisa A Colombo, Laura Fontana, Cristina Gervasini, Devinder M Thappa, Medha Rajappa, Kalai Selvi Rajendiran, Gubbi Shamanna Sreenath, Vikram Kate
BACKGROUND: Familial lentiginosis syndromes are characterized by a wide array of manifestations resulting from activation of molecular pathways which control growth, proliferation, and differentiation of a broad range of tissues. Familial gastrointestinal stromal tumors (GISTs) are often accompanied by additional features like hyperpigmentation, mastocytosis, and dysphagia. They have been described with mutations in c-kit (most commonly), platelet-derived growth factor receptor A, neurofibromatosis-1, and succinate dehydrogenase genes...
February 2017: International Journal of Dermatology
https://www.readbyqxmd.com/read/28074480/clonal-reticulohistiocytosis-of-the-skin-and-bone-marrow-associated-with-systemic-mastocytosis-and-acute-myeloid-leukaemia
#12
Nicola Fusco, Arturo Bonometti, Claudia Augello, Sonia Fabris, Leonardo Boiocchi, Stefano Fiori, Denise Morotti, Nicola Fracchiolla, Emilio Berti, Umberto Gianelli
AIMS: The aims of this study were to define whether diffuse cutaneous reticulohistiocytosis could be underpinned by somatic genetic alterations and represent precursor to more aggressive forms of disease. METHODS AND RESULTS: A 59-year-old man with diffuse cutaneous reticulohistiocytosis, experienced bone marrow localisation of the disease, with associated systemic mastocytosis and acute myeloid leukaemia. Cytogenetic analyses of the bone marrow aspirate revealed the presence of a derivative chromosome giving rise to a partial trisomy of chromosome 1q and a partial monosomy of chromosome 9q...
January 11, 2017: Histopathology
https://www.readbyqxmd.com/read/28069280/a-new-therapeutic-advance-for-symptomatic-systemic-mastocytosis
#13
Michel Arock
No abstract text is available yet for this article.
January 6, 2017: Lancet
https://www.readbyqxmd.com/read/28069279/masitinib-for-treatment-of-severely-symptomatic-indolent-systemic-mastocytosis-a-randomised-placebo-controlled-phase-3-study
#14
Olivier Lortholary, Marie Olivia Chandesris, Cristina Bulai Livideanu, Carle Paul, Gérard Guillet, Ewa Jassem, Marek Niedoszytko, Stéphane Barete, Srdan Verstovsek, Clive Grattan, Gandhi Damaj, Danielle Canioni, Sylvie Fraitag, Ludovic Lhermitte, Sophie Georgin Lavialle, Laurent Frenzel, Lawrence B Afrin, Katia Hanssens, Julie Agopian, Raphael Gaillard, Jean-Pierre Kinet, Christian Auclair, Colin Mansfield, Alain Moussy, Patrice Dubreuil, Olivier Hermine
BACKGROUND: Indolent systemic mastocytosis, including the subvariant of smouldering systemic mastocytosis, is a lifelong condition associated with reduced quality of life. Masitinib inhibits KIT and LYN kinases that are involved in indolent systemic mastocytosis pathogenesis. We aimed to assess safety and efficacy of masitinib versus placebo in severely symptomatic patients who were unresponsive to optimal symptomatic treatments. METHODS: In this randomised, double-blind, placebo-controlled, phase 3 study, we enrolled adults (aged 18-75 years) with indolent or smouldering systemic mastocytosis, according to WHO classification or documented mastocytosis based on histological criteria, at 50 centres in 15 countries...
February 11, 2017: Lancet
https://www.readbyqxmd.com/read/28041932/quantification-of-human-diamine-oxidase
#15
Thomas Boehm, Sophie Pils, Elisabeth Gludovacz, Helen Szoelloesi, Karin Petroczi, Otto Majdic, Andrea Quaroni, Nicole Borth, Peter Valent, Bernd Jilma
OBJECTIVES: Diamine oxidase (DAO) is essential for extracellular degradation of histamine. For decades activity assays with inherent limitations were used to quantify the relative amounts of DAO. No reference DAO standard is available. Absolute DAO amounts cannot be determined. Controversy exists, whether DAO is circulating or not in non-pregnant individuals. The role of DAO as biomarker in various diseases is ambiguous. It is not clear, whether precise quantification of human DAO antigen using commercially available enzyme-linked immunosorbent assays (ELISAs) is possible...
December 29, 2016: Clinical Biochemistry
https://www.readbyqxmd.com/read/28038453/histone-deacetylase-inhibitor-saha-mediates-mast-cell-death-and-epigenetic-silencing-of-constitutively-active-d816v-kit-in-systemic-mastocytosis
#16
Katarina Lyberg, Hani Abdulkadir Ali, Jennine Grootens, Matilda Kjellander, Malin Tirfing, Michel Arock, Hans Hägglund, Gunnar Nilsson, Johanna Ungerstedt
Systemic mastocytosis (SM) is a clonal bone marrow disorder, where therapeutical options are limited. Over 90% of the patients carry the D816V point mutation in the KIT receptor that renders this receptor constitutively active. We assessed the sensitivity of primary mast cells (MC) and mast cell lines HMC1.2 (D816V mutated), ROSA (KIT WT) and ROSA (KIT D816V) cells to histone deacetylase inhibitor (HDACi) treatment. We found that of four HDACi, suberoyl anilide hydroxamic acid (SAHA) was the most effective in killing mutated MC...
February 7, 2017: Oncotarget
https://www.readbyqxmd.com/read/28031180/mastocytosis-2016-updated-who-classification-and-novel-emerging-treatment-concepts
#17
Peter Valent, Cem Akin, Dean D Metcalfe
Over the past few years substantial advances have been made in understanding the pathogenesis, evolution, and complexity of mast cell neoplasms. New diagnostic and prognostic parameters and novel therapeutic targets with demonstrable clinical impact have been identified. A number of these new markers, molecular targets, and therapeutic approaches have been validated and translated into clinical practice. At the same time, the classification of mastocytosis and related diagnostic criteria have been refined and updated by the consensus group and the World Health Organization (WHO)...
December 28, 2016: Blood
https://www.readbyqxmd.com/read/28003089/an-unusual-case-of-tubulointerstitial-renal%C3%A2-mastocytosis
#18
Maria E Sanhueza, Paula Segura, Guillermo Murray, Ruben Torres, Luis Toro
No abstract text is available yet for this article.
January 2017: Kidney International
https://www.readbyqxmd.com/read/27995272/-mastocytosis-clinical-aspects-diagnostics-therapy
#19
U Lippert
Mastocytosis is a rare, almost exclusively sporadically occurring disease involving an increase in clonal tissue mast cells. The disease spectrum is heterogenous, ranging from isolated skin lesions with a normal life expectancy to rare, aggressive forms with very poor prognosis. Children are often affected. But whereas these almost invariantly display solely a cutaneous mastocytosis with polymorphous skin lesions, in adults the lesions are small and maculopapular and in over 80% of cases accompanied by involvement of bone marrow and the D816V activating mutation of the gene for the c‑Kit receptor...
December 19, 2016: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
https://www.readbyqxmd.com/read/27981619/familial-progressive-hyperpigmentation-cutaneous-mastocytosis-and-gastrointestinal-stromal-tumor-as-clinical-manifestations-of-mutations-in-the-c-kit-receptor-gene
#20
Tatiana Piqueres-Zubiaurre, Zuriñe Martínez de Lagrán, Ricardo González-Pérez, Amaia Urtaran-Ibarzabal, Guiomar Perez de Nanclares
BACKGROUND: Familial progressive hyperpigmentation (FPH) is an autosomal dominant disorder characterized by the appearance of hyperpigmented patches on the skin from early infancy that increase in size and number with age. METHODS: We report the clinical and molecular studies of an 11-year-old boy who had areas of hyperpigmentation since birth that had spread across his body as irregular hyperpigmented macules and papules, and include relevant history in family members...
December 16, 2016: Pediatric Dermatology
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