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

We identify and describe clinical findings in hypocomplementemic urticarial vasculitis syndrome (HUVS), an uncommon to rare illness related to systemic lupus erythematosus (SLE). A patient with recurrent, idiopathic urticaria-like lesions was diagnosed as having HUVS if a lesional biopsy showed leukocytoclastic vasculitis, the serum C1q was markedly decreased, and antibody to C1q was detected in the patient's serum. The clinical characteristics, serologic findings, and outcome of patients who met these criteria were determined from prospective and retrospective data, including hospital and office records, patient interviews, previously banked serum samples, and freshly drawn sera. Eighteen patients with HUVS were identified, and high incidences of angioedema, ocular inflammation, glomerulonephritis, and obstructive pulmonary disease were found. Renal and lung biopsies showed mesangial or membranoproliferative glomerulonephritis and severe pulmonary emphysema without vasculitis. Pulmonary function was measured in 17 patients, 11 of whom had dyspnea. All dyspneic patients had moderate to severe airflow obstruction, which progressed in all 11 and subsequently improved in only 1. Six of these 11 patients died of respiratory failure, 1 underwent lung transplantation, and 3 of the remaining 4 have moderately severe to life-threatening respiratory insufficiency. Treatment did not appear to alter the progression of obstructive lung disease. In contrast, renal insufficiency improved with treatment in 2 of 2 patients. Angioedema, ocular inflammation, obstructive lung disease, and glomerulonephritis appear to be common in HUVS, and lung disease causes substantial morbidity and mortality. The pathogenesis of HUVS may involve humoral autoimmunity, although it is not clear how autoimmunity would participate in development of obstructive lung disease. Cigarette smoking appears to be a risk factor for fatal lung disease in HUVS. All patients with HUVS should be made aware of this possibility and should be advised, encouraged, and helped to avoid tobacco smoke.
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PMID:Hypocomplementemic urticarial vasculitis syndrome. Clinical and serologic findings in 18 patients. 783 68

Anaphylaxis, a multisystem allergic reaction, represents a true medical emergency. Anaphylaxis is characterized by a combination of the following symptoms: urticaria, angioedema, distributive shock, and respiratory failure. Most often, the patient is rapidly treated with prompt resolution of the anaphylaxis in either the out-of-hospital or emergency department (ED) setting. Infrequently, recurrent or multiphasic anaphylaxis is encountered, involving a reappearance of allergic phenomena after complete resolution of the original reaction. Recurrence may involve nuisance-level issues such as urticaria; alternatively, multiphasic reactions may be characterized by cardiovascular collapse or respiratory compromise. Initially aggressive pharmacological therapy followed by prolonged observation in either the ED or the in-hospital setting is strongly recommended to monitor for potential recurrence.
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PMID:Multiphasic anaphylaxis: report of a case with prehospital and emergency department considerations. 927 99

Anaphylaxis, a multisystem allergic reaction, represents a true medical emergency. Anaphylaxis is characterized by a combination of the following: urticaria, angioedema, distributive shock, and respiratory failure. Most often, the patient is rapidly treated with prompt resolution of the anaphylaxis in either the out-of-hospital or emergency department (ED) setting. Infrequently, recurrent, or multiphasic, anaphylaxis is encountered, involving a reappearance of allergic phenomena after complete resolution of the original reaction. Recurrence may involve nuisance-level issues such as urticaria; alternatively, multiphasic reactions may be characterized by cardiovascular collapse and/or respiratory compromise. Initially aggressive pharmacological therapy followed by prolonged observation in either the ED or the in-hospital setting is strongly recommended to monitor for potential recurrence.
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PMID:Occurrence of multiphasic anaphylaxis during a transcontinental air flight. 1059 93

Recombinant human alpha-l-iduronidase (Aldurazyme), laronidase) is approved as an enzyme replacement therapy to treat the lysosomal storage disorder, mucopolysaccharidosis type I (MPS I) at a dose of 0.58 mg/kg by once-weekly intravenous infusion. To assess whether alternate dosing regimens might provide a better reduction in lysosomal storage, a 26-week, randomized, open-label, multinational dose-optimization trial was conducted. The pharmacodynamic effect and safety of the approved laronidase dose was compared to three alternative regimens (1.2mg/kg every 2 weeks; 1.2mg/kg every week; 1.8 mg/kg every 2 weeks) among 33 MPS I patients. The four treatment regimens showed no significant differences in the reduction of urinary glycosaminoglycan excretion or liver volume. Laronidase had an acceptable safety profile in all dose regimen groups. Infusion-associated reactions were the most common drug-related adverse events across dose regimens (by patient incidence), and included pyrexia (21%), vomiting (15%), rash (15%), and urticaria (12%). Patients in the approved dose group had the lowest incidence of drug-related adverse events (38% vs. 63-75%) and infusion-associated reactions (25% vs. 25-63%). There was one death: a patient with acute bronchitis died of respiratory failure 6h after completing the first laronidase infusion. The approved 0.58 mg/kg/week laronidase dose regimen provided near-maximal reductions in glycosaminoglycan storage and the best benefit-to-risk ratio. The 1.2mg/kg every 2 weeks regimen may be an acceptable alternative for patients with difficulty receiving weekly infusions, but the long-term effects of this regimen are unknown.
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PMID:A dose-optimization trial of laronidase (Aldurazyme) in patients with mucopolysaccharidosis I. 1903 63

Intravenous contrast medium (ICM) rarely induces anaphylactic reactions, including urticaria, hypotension and respiratory failure. Even the most modern ICM may cause such adverse events. Thrombocytopenia has been reported as an extreme rare consequence of ICM. Here we report on a case of a 72-year-old male patient with a self-limiting severe acute thrombocytopenia following administration of intravenous non-ionic low-osmolarity contrast medium. No such low platelet count has ever been reported. We also present a review of the literature.
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PMID:Acute severe thrombocytopenia following non-ionic low-osmolarity intravenous contrast medium injection. 2277 75