Search results for " Connective tissue disease"
showing 10 items of 848 documents
Changes in plasma and urine globotriaosylceramide levels do not predict Fabry disease progression over 1 year of agalsidase alfa.
2013
Globotriaosylceramide concentrations were assessed as potential predictors of change from baseline after 12 months by estimated glomerular filtration rate and left-ventricular mass index using pooled data from three randomized, placebo-controlled agalsidase alfa trials and open-label extensions of patients with Fabry disease.Males (aged 18 years or older) with Fabry disease received agalsidase alfa (0.2 mg/kg every other week for 12 months). A backward-elimination approach evaluated potential predictors (baseline estimated glomerular filtration rate and left-ventricular mass index; age at first dose; baseline and change from baseline at 12 months of globotriaosylceramide (urine, plasma); ur…
Levocetirizine for the treatment of itch in psoriasis patients: An open-label pilot study in a real-world setting.
2019
Itch is the most bothersome symptom in psoriasis, often leading to impaired quality of life. Treatment of psoriasis-induced itch is frequently unsatisfactory as the various therapies employed have a delayed onset of effect. Histamine-1 receptor (H1) antihistamines are not recommended in treatment guidelines as histamine is not considered a key mediator in psoriasis. However, patients using H1 antihistamines frequently report benefits in questionnaire-based studies. To address these contradictions, we examined the short-term effects of levocetirizine, a nonsedating H1 antihistamine, on psoriasis-related itch and itch-related quality of life. In this pilot study, patients with psoriasis-relat…
Clinical performance of a new medullary humeral nail: antegrade versus retrograde insertion.
2001
Objective A new intramedullary nail system for humeral shaft fractures is evaluated to determine whether retrograde nailing is as reliable as antegrade nailing. Study design Prospective multicenter nonrandomized clinical study. Patients Eighty-four patients with acute humeral shaft fractures were nailed with the new unreamed humeral nail (UHN) system. Fifty-seven nails were introduced retrogradely, and twenty-seven antegradely. Bone healing and functional outcome were the follow-up parameters. Results The ratio of perioperative complications was equivalent for both groups, but one shaft fracture and three fractures or fissures at the entry point occurred in the group with retrograde nail in…
Cutaneous, genital and oral lichen planus: a descriptive study of 274 patients
2018
Background Lichen planus (LP) is a chronic autoimmune disease that affects the oral mucosa as well as the skin, genital mucosa and other sites. Objective: to evaluate the correlation between oral, genital and cutaneous lichen planus, in a sample of LP patients. Material and Methods This descriptive study reviewed 274 clinical histories of patients, who all presented histological confirmation of lichen planus verified by a pathologist, attending research centers in Barcelona. Results A total of 40 LP patients (14.59%) presented genital lesions. Of 131 patients with cutaneous LP (47.8%), the most commonly affected zones were the body’s flexor surfaces, representing 60.1% of cases. 24% of pati…
Hereditary Angioedema: Long-Term Treatment with One or More Injections of C1 Inhibitor Concentrate per Week
2009
<i>Background:</i> Hereditary angioedema due to C1 inhibitor deficiency (HAE-C1-INH) is characterized by recurrent edema attacks in various organs. The objective of the present study was to assess the efficacy and safety of weekly long-term replacement treatment with one or more injections of plasma-derived C1-INH concentrate per week (WLTC) in patients with HAE-C1-INH. <i>Methods:</i> Nineteen patients with HAE-C1-INH underwent WLTC for 9 years on average. The benefits and risks were determined based on regular recording by the patients of the severity and number of attacks at the beginning and the end of the study. <i>Results:</i> All patients reported …
A comparison between nailfold capillaroscopy patterns in adulthood in juvenile and adult-onset systemic sclerosis: A EUSTAR exploratory study.
2015
Objective: Qualitative capillaroscopy patterns in juvenile- and adult-onset systemic sclerosis (SSc) were studied in adulthood using data from the EULAR Scleroderma Trials and Research (EUSTAR) database. Methods: Data collected between June 2004 and April 2013 were examined with focus on capillaroscopy. In this retrospective exploratory study, series of patients with juvenile-onset SSc were matched with series of adult-onset SSc having the same gender and autoantibody profile. Results: 30 of 123 patients with juvenile-onset and 2108 of 7133 with adult-onset SSc had data on capillaroscopy. Juvenile-onset SSc showed scleroderma pattern more frequently than adult-onset SSc (93.3% and 88%). The…
Controversies and challenges in the management of chronic urticaria
2016
This supplement reports proceedings of the second international Global Urticaria Forum, which was held in Berlin, Germany in November 2015. Despite the clear international guideline, there remain a number of controversies and challenges in the management of patients with chronic urticaria (CU). As a result of major advancements in urticaria over the past 4 years, the current EAACI/GA(2) LEN/EDF/WAO urticaria guideline treatment algorithm requires updating. Case studies from patients with chronic spontaneous urticaria (CSU) [also called chronic idiopathic urticaria (CIU)], chronic inducible urticaria (CIndU) or diseases and syndromes related to CU are useful in describing and exploring chall…
Development and validation of the Urticaria Control Test: a patient-reported outcome instrument for assessing urticaria control.
2013
Background Chronic urticaria is a frequent and debilitating skin disease. Its symptoms commonly fluctuate considerably from day to day. As of yet, the only reliable tool to assess disease activity is the Urticaria Activity Score, which prospectively documents the signs and symptoms of urticaria for several days. Objective We sought to develop and validate a novel patient-reported outcome instrument to retrospectively assess urticaria control, the Urticaria Control Test (UCT). Methods Potential UCT items were developed by using established methods (literature research and expert and patient involvement). Subsequently, item reduction was performed by using a combined approach, applying impact…
Detection of dissection of the aortic intima and media after angioplasty of coarctation of the aorta. An angiographic, computer tomographic, and echo…
1990
Balloon coarctation angioplasty (BCA) was performed in eight patients (five male and three female) who were 14-49 years old (mean, 27.3 years) with isolated discrete unoperated coarctation of the aorta (n = 7) and postoperative recoarctation (n = 1). BCA was successful in seven of eight patients, resulting in a decrease in the gradient (64 +/- 19 to 16 +/- 13 mm Hg, p less than 0.01), an increase in the diameter at the coarctation site (0.9 +/- 0.4 to 1.6 +/- 0.4 mm, p less than 0.01). Follow-up (6 months) has demonstrated continued gradient relief (6 +/- 9 mm Hg) and diameter increase (1.6 +/- 0.2 cm). Monitoring was performed by transesophageal echocardiography (TEE) during BCA, and befor…
One year longitudinal study of the straight gyrus morphometry in first-episode schizophrenia-spectrum patients
2012
Brief report.-- El pdf es la versión post-print.-- et al.