Search results for "EDEMA"

Tissue hypoxia in complex regional pain syndrome.

2003

Untreated complex regional pain syndrome (CRPS) may progress from acute stages with increased hair and nail growth in the affected limb to chronic stages with atrophy of the skin, muscles and bones. The aim of this study was to investigate whether tissue hypoxia could be one mechanism responsible for this late CRPS symptoms. Nineteen patients with CRPS and two control groups (healthy control subjects, surgery patients with edema) participated in this study. Skin capillary hemoglobin oxygenation (HbO(2)) was measured non-invasively employing micro-lightguide spectrophotometry (EMPHO). The EMPHO probe was mounted force-controlled onto the skin of the affected and unaffected hand. HbO(2) was m…

Benefits and risks of danazol in hereditary angioedema: a long-term survey of 118 patients

2008

Background Hereditary angioedema (HAE) due to C1 inhibitor deficiency is clinically characterized by relapsing skin swellings, abdominal pain attacks, and life-threatening upper airway obstruction. Treatment with androgens prevents attacks for those with this condition. Objective To examine the benefits and risks of long-term treatment with danazol. Methods Data were generated retrospectively from 118 German and Danish patients who had HAE due to C1 inhibitor deficiency and were treated with danazol from 2 months to 30 years. The frequency and severity of acute attacks were registered before and during danazol treatment, and adverse effects to the treatment were noted. Data were collected b…

Effect of high-dose intravenous immunoglobulin treatment in therapy-resistant chronic spontaneous urticaria

2010

Background Chronic spontaneous urticaria (CSU) lasting more than 6 weeks is one of the most disabling types of urticaria and often results in severely impaired quality of life. Patients with CSU are often unsatisfied with the standard treatment. Another treatment option recommended for patients with so-called nonresponding CSU according to the newest guidelines is intravenous immunoglobulin (IVIG). Objective To assess the efficacy and safety of high-dose IVIG as a treatment option in patients with therapy-resistant CSU. Methods Six patients with severe CSU unresponsive to other treatment options according to the newest guidelines for several weeks were treated with high-dose IVIG (2 g/kg ev…

Does angioedema influence the quality of life in patients with chronic spontaneous urticaria?

2019

Psychometric Field Study of Hereditary Angioedema Quality of Life Questionnaire for Adults:HAE-QoL

2016

Background: Hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) may affect health-related quality of life (HRQoL). A specific HRQoL questionnaire for adult patients with C1-INH-HAE, the HAE-QoL, has recently been developed in Spain. Objective: The objective of this study was to perform a cross-cultural validation and psychometric study of the HAE-QoL in an international setting. Methods: Cross-cultural adaptation of the Spanish HAE-QoL draft version and an international rating phase with experts were performed. The resultant version of the HAE-QoL, a clinical questionnaire, and Short Form 36-item Health Survey Version 2.0 (SF-36v2) were pilot tested internationally. Item reduc…

Expanding the phenotype associated to KMT2A variants: overlapping clinical signs between Wiedemann–Steiner and Rubinstein–Taybi syndromes

2020

Lysine-specific methyltransferase 2A (KMT2A) is responsible for methylation of histone H3 (K4H3me) and contributes to chromatin remodeling, acting as “writer” of the epigenetic machinery. Mutations in KMT2A were first reported in Wiedemann–Steiner syndrome (WDSTS). More recently, KMT2A variants have been described in probands with a specific clinical diagnosis comprised in the so-called chromatinopathies. Such conditions, including WDSTS, are a group of overlapping disorders caused by mutations in genes coding for the epigenetic machinery. Among them, Rubinstein–Taybi syndrome (RSTS) is mainly caused by heterozygous pathogenic variants in CREBBP or EP300. In this work, we used next generati…

Ranibizumab for Branch Retinal Vein Occlusion Associated Macular Edema Study (RABAMES): six-month results of a prospective randomized clinical trial

2014

Purpose To compare standard-of-care grid laser photocoagulation versus intravitreal ranibizumab (IVR) versus a combination of both in the treatment of chronic (>3 months) macular oedema secondary to branch retinal vein occlusion. Methods Prospective, randomized, multicentre clinical trial. Thirty patients with a best-corrected visual acuity (BCVA) between 20/320 and 20/40 were randomized 1:1:1 to receive grid laser or three monthly injections of 0.5 mg IVR or both followed by 3 months of observation. Results Mean change from baseline BCVA at month 6 was +2 letters [laser; 0.04 logMAR, 95% confidence interval (−0.17; 0.25)], +17 letters [IVR; 0.34 (0.19; 0.5)] and +6 letters [combination; 0.…

Hereditary angioedema with normal C1-inhibitor activity in women.

2000

Summary Background Hereditary angioedema (HAE) is a well defined autosomal dominant disease (Mendelian Inheritance in Man #106100) that results from an inherited deficiency of C1 (the activated first component of complement) inhibitor function. We report an unusual variant of HAE with normal biochemical C1-inhibitor function, occurring only in women. Methods We screened 574 patients with recurrent angioedema of the skin for presence of HAE. 283 patients were selected, in whom angioedema was associated with abdominal pain attacks or recurrent life-threatening episodes of upper-airway obstruction, or both, rather than with urticaria. We measured C1-inhibitor concentration and functional activ…

Omalizumab rapidly improves angioedema-related quality of life in adult patients with chronic spontaneous urticaria: X-ACT study data

2018

Background The X-ACT study aims to examine the effect of omalizumab treatment on quality of life (QoL) in chronic spontaneous urticaria (CSU) patients with angioedema refractory to high doses of H1-antihistamines. Methods In X-ACT, a phase III, double-blind, placebo-controlled study, CSU patients (18–75 years) with ≥4 angioedema episodes during the 6 months before inclusion were randomized (1:1) to receive omalizumab 300 mg or placebo every 4 weeks for 28 weeks. Angioedema-related QoL, skin-related QoL impairment, and psychological well-being were assessed. Results Ninety-one patients were randomized and 68 (omalizumab, n=35; placebo, n=33) completed the 28-week treatment period. At baselin…

Hereditary Angioedema: New Findings Concerning Symptoms, Affected Organs, and Course

2005

Abstract Purpose Hereditary angioedema (HAE) due to C1 inhibitor deficiency is clinically characterized by relapsing skin swellings, abdominal pain attacks, and life-threatening upper airway obstruction. Our aim was to examine a temporal and spatial pattern of the edema episodes by evaluating the long-term course of hereditary angioedema in order to establish a specific swelling pattern. Subjects and methods Data were generated from 221 patients with C1 inhibitor deficiency by asking them about symptoms they experienced during their edema episodes. Documentation was accomplished through the use of standardized questionnaires. Results A total of 131110 edema episodes were observed. Clinical …