Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0042109 (urticaria)
 6,569 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Variants of mastocytosis can present with puzzling cutaneous and systemic symptoms and signs that can result in an erroneous diagnosis of idiopathic urticaria or idiopathic anayphylaxis. The molecular basis of mastocytosis is now better understood, with updated classification based on distinct growth factor and oncogene abnormalities. Elicitation of a full history and careful attention to the skin examination will usually provide the clinician enough information to deduce that the condition is not simply chronic idiopathic urticaria.
 ...
PMID:Mastocytosis: the great masquerader. 1646 96

Mast cells possess an array of potent inflammatory mediators capable of inducing acute symptoms after cell activation, including urticaria, angioedema, bronchoconstriction, diarrhea, vomiting, hypotension, cardiovascular collapse, and death in few minutes. In contrast, mast cells can provide an array of beneficial mediators in the setting of acute infections, cardiovascular diseases, and cancer. The balance between the detrimental and beneficial roles of mast cells is not completely understood. Although the symptoms of acute mast cell mediator release can be reversed with epinephrine, adrenergic agonists, and mediator blockers, the continued release of histamine, proteases, prostaglandins, leukotrienes, cytokines, and chemokines leads to chronic and debilitating disease, such as mastocytosis. Identification of the molecular factors and mechanisms that control the synthesis and release of mast cell mediators should benefit all patients with mast cell activation syndromes and mastocytosis.
 ...
PMID:Mast cell mediators in allergic inflammation and mastocytosis. 1693 Dec 89

Mastocytosis is group of rare disorders characterized by abnormal mast cells growth. Pathology of the disease is unknown, mechanisms involved in mast cells delineation and growth are considered to play an important role. Unusual and broad spectrum of symptoms therefore requires cooperation of different specialists. The aim of the study was to assess diagnostic and therapeutic methods used in the Gdansk Mastocytosis Centre. 14 patients were studied (9 adults and 5 children). Bone marrow biopsy was performed in order to assess the stage of disease. Pathological, cytological, cytofotometry and genetic examination (C-KIT mutation) of the bone marrow were performed. Patients suffereing from food allergy and wasp venom anaphylaxis were diagnosed with skin prick tests and sIgE. All subjects suffered from urticaria pigmenthosa and anaphylaxis. Indolent mastocytosis was diagnosed in six subjects. Patients were treated with antihistamines, corticosteroids, cromones and immunotherapy with a marked reduction of symptoms. The experience of Gdansk Mastocytosis Centre indicates that mastocytosis is a rare and difficult to diagnose disease.
 ...
PMID:[Systemic mastocytosis]. 1698 60

The Internet sites listed in this article provide patients and physicians with extensive information on urticaria, mastocytosis and allergies in general. They are user-friendly and enable patients to easily acquire information.
 ...
PMID:[National and international information sources for medical personnel and patients]. 1734 27

Mastocytosis is a group of rare diseases characterized by abnormal growth of mast cells in skin, bone marrow, liver, spleen, lymph nodes. Signs and symptoms result mostly from mast cells mediators and mast cells organ infiltration. Pathological examination proving mast cells infiltration is crucial for the diagnosis of disease. Therapy covers patient education and symptomatic treatment (antihistamine drugs and glicocortycoids). Attempts of interferon, cladribine, imatinib treatment are made. Aggressive forms of diseases require, chemiotherapy, bone marrow transplantation. All mastocytosis subjects should be equipped in adrenaline. The paper describes case of 52 years old woman who suffered from urticaria pigmenthosa, anaphylaxis, insect venom and food allergy. Diagnosis included bone marrow examinations (pathology, cytology, genetics, cytofotometry) tryptase level, skin prick tests and sIgE. Mastocytosis was diagnosed. Therapy included symptomatic treatment and immunotherapy. The paper describes also aims of the European Competence Network on Mastocytosis.
 ...
PMID:[Mastocytosis--mastcell proliferative disease risk of anaphylactic reaction]. 1740

Mast cells are traditionally viewed as effector cells of allergic reactions and parasitic diseases, but their importance in host defense against bacteria, in tissue remodelling, their bone marrow and stem cell origin and a central role of the stem cell factor (SCF) as mast cell growth and chemotactic factor has been worked out only in recent years. Despite this, major aspects about the nature of the cells and their role in disease remain unclear. This holds in particular for the identification of mast cell precursors and the role of growth factors that stimulate specific mast cell commitment from stem cells, such as nerve growth factor, neutrotrophin-3 and certain interleukins, alone and during interaction with SCF. Early data suggesting also an involvement of specific transcription factors need to be expanded in this process. Furthermore, although mast cell proliferative disease (mastocytosis) has been shown to be often associated with SCF receptor c-kit mutations, reasons for the development of this disease remain unclear. This holds also for mast cell release mechanisms in many types of mast cell-dependent urticaria. Exciting new insights are emerging regarding the role of mast cells in bacterial infections, in defense against tumors, in wound healing and in the interplay with the nervous system, with hormones, and in the neurohormonal network. The aim of this reflection is to delineate the many known and unknown aspects of mast cells, with a special focus on their development, and to discuss in detail two mast cell-related diseases, namely mastocytosis and urticaria.
 ...
PMID:Exploring the mast cell enigma: a personal reflection of what remains to be done. 1820 12

Reported herein is an autopsy case of mast cell leukemia, a rare form of systemic mastocytosis, complicated with portal hypertension. A 52-year-old woman presented with urticaria-like skin symptoms, anemia, and thrombocytopenia. Atypical mast cells (CD2+, CD25+, CD117+) with toluidine blue metachromasia were found in the peripheral blood and on bone marrow aspiration smears. Chemotherapy with cytosine arabinoside and idarubicin was ineffective and the patient died of multi-organ failure with rapidly progressing hepatosplenomegaly and large-volume ascites 3 months after admission. At autopsy the bone marrow, spleen, liver, and lymph nodes were extensively infiltrated by atypical tumor cells with occasional bi- or multi-lobated nuclei. They were positive for mast cell tryptase and possessed an activating mutation of the c-kitgene (D816V). Ascites (2200 mL) and non-ruptured esophageal varices with submucosal hemorrhage indicated the presence of severe portal hypertension. Although there was no evidence of liver cirrhosis, the hepatic sinusoids were clogged with tumor cells, with a tendency to be more severe in the perivenular areas, and the lumens of central veins were obliterated by tumor cell infiltration. The present case demonstrates that non-cirrhotic portal hypertension due to blocking of sinusoidal and venous flow could be a serious complication in mast cell leukemia.
 ...
PMID:Mast cell leukemia with rapidly progressing portal hypertension. 1988 34

Mastocytosis is a proliferative disorder of the hematopoietic mast cell progenitor that results from expansion of a clone carrying the D816V c-kit mutation. Based on the dramatic increase in incidence of anaphylaxis in patients with mastocytosis, recent studies analyzed the presence of clonal mast cell markers, including D816V c-kit mutation, in patients with recurrent IgE- and non-IgE-mediated anaphylaxis. These studies demonstrated the presence of an aberrant mast cell clone in a significant proportion of patients with unexplained anaphylaxis, or anaphylaxis due to hymenoptera venom. Clonal mast cell disease should be suspected in particular in patients presenting with profound cardiovascular manifestations such as hypotension and syncope in the absence of urticaria.
 ...
PMID:Anaphylaxis and mast cell disease: what is the risk? 2042 12

Cutaneous mastocytosis is characterized by increased numbers of skin mast cells that release mediators causing pruritus, urticaria, and flushing. Most pediatric mastocytosis patients exhibit the pattern of urticaria pigmentosa, which typically appears during the first two years of life and resolves spontaneously in late adolescence. However, while the disease is active, patients are frequently symptomatic and uncomfortable, which justifies symptomatic treatment. We report 2 patients, a 14-month-old girl and a 26-month-old boy, with localized cutaneous erythematous lesions with a positive Darier sign. In each, a punch biopsy confirmed the diagnosis of mastocytosis. Treatment was instituted with pimecrolimus cream twice a day and oral antihistamine. An almost complete response was achieved after 4 months of therapy in both patients, with no clinical evidence of recurrence after 4 years and 2 years of follow-up, respectively. In children, the treatment of mastocytosis is directed primarily to avoiding potential mast cell degranulating agents and alleviating symptoms. Topical calcineurin inhibitors act by inhibiting T-cell activation and cytokine release; they may suppress mast cell- mediated reactions by reducing their degranulation. These two cases suggest that in localized cutaneous mastocytosis they are a safe and efficacious alternative to topical steroid therapy.
 ...
PMID:Cutaneous mastocytosis: Two pediatric cases treated with topical pimecrolimus. 2049 25

Urticaria with angioedema is a common clinical presentation that often poses a challenge for allergists. The differential diagnosis for urticaria is broad, making the evaluation and pinpointing the underlying cause difficult and frustrating for both families and physicians. Certain causes of urticaria such as infections or medications are more common and easier to identify whereas less frequently seen conditions are often overlooked because of their rarity. One such condition is mastocytosis. Mastocytosis is a rare disease that very seldom presents with urticaria but may be associated with significant morbidity and mortality if not recognized in a timely manner. We are presenting a case of a 14-year-old boy who presented with urticaria and angioedema possibly caused by a solitary mastocytoma. The learning points from this case are that mastocytosis should be considered in the differential diagnosis of urticaria and solitary mastocytomas may remain active into adolescence, raising concern for systemic progression.
 ...
PMID:A solitary mastocytoma presenting with urticaria and angioedema in a 14-year-old boy. 2170 63


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