Attack Frequency In Patients Research Articles

Exploring factors for predicting colchicine responsiveness in children with PFAPA

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome (PFAPA) are the most common autoinflammatory syndromes in children. This study aimed to evaluate the clinical and laboratory parameters that may predict colchicine responsiveness.This retrospective, multicenter, cross-sectional study involved nine pediatric rheumatology centers from our country., The patients diagnosed with PFAPA were compared on the basis of their responses to colchicine. In the 806 (42.3% female 57.7% male) patients, the most common clinical findings were fever (100%), exudative tonsillitis (86.5%), pharyngitis (80.9%), and aphthous stomatitis (50.5%). The mean attack frequency was 13.5 ± 6.8 attacks per year lasting for a mean of 3.9 ± 1.1 days. Colchicine treatment was attempted in 519 (64.4%) patients, with 419 (80.7%) showing a favorable response. In patients who underwent MEFV gene analysis (70.8%), the most common variant was M694V heterozygous (16.8%). The presence of pharyngitis (p = 0.03, 95% CI 0.885 to 0.994), the presence of arthralgia (p = 0.04, 95% CI 0.169 to 0.958), and having more frequent attacks (p = 0.001, 95% CI 0.028 to 0.748) were found to be associated with colchicine unresponsiveness, whereas the carriage of the M694V variant (p = 0.001, 95% CI 0.065 to 0.242) was associated with colchicine responsiveness.Conclusion: This study identified the presence of pharyngitis, arthralgia, and increased attack frequency in patients with PFAPA as factors predicting colchicine unresponsiveness, whereas the carriage of the M694V variant emerged as a predictor of colchicine responsiveness. Predicting colchicine response at disease onset may facilitate a more effective management of PFAPA.What is Known:• Colchicine treatment can be used in the prophylaxis of PFAPA disease.• Having the MEFV variant is the most commonly known factor in predicting response to colchicine.What is New:• The presence of pharyngitis or arthralgia, and more frequent attacks in PFAPA disease were found to be independently associated with colchicine unresponsiveness. • Carrying the M694V variant was identified as the sole factor predicting colchicine responsiveness.

Despite Prophylactic Treatments, Break-through Attacks Continue among Patients with Hereditary Angioedema

There are several FDA-approved medications for prophylactic treatment of Hereditary Angioedema (HAE). Despite increased use of these therapies, patients continue to experience HAE attacks. We sought to evaluate attack frequency in patients receiving prophylactic treatment.

The efficacy of acupuncture for stable angina pectoris: A systematic review and meta-analysis.

The aim of this study was to assess the efficacy and safety of acupuncture in the treatment of patients with stable angina pectoris. A literature search was performed in nine databases, including PubMed and the Cochrane Library, from their inception to 30 August 2018. Randomized controlled trials that compared acupuncture therapy with sham acupuncture or no treatment were included. Two reviewers under the guidance of the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines assessed the eligibility of each record and extracted essential information independently. The data were merged using a fixed-effect model. Pooled analysis of 17 eligible trials with 1516 participants showed that acupuncture was associated with reduced angina attack frequency (-4.91; 95% confidence interval, -6.01- -3.82; p < 0.00001) and improved depression (-1.23; 95% confidence interval, -1.47- -1.00; p < 0.00001) and anxiety level (-0.96; 95% confidence interval, -1.16- -0.75; p < 0.00001) relative to sham treatment or standard care alone. No increased risk of adverse events was observed during treatment (relative risk, 0.70; 95% confidence interval, 0.33-1.48; p = 0.35). No significant improvement was shown in nitroglycerin use or angina intensity. The included studies were associated with unclear to high risk of selection or performance bias, and the quality of evidence was low to moderate. Acupuncture may safely and effectively improve physical restrictions, emotional distress, and attack frequency in patients with stable angina pectoris. However, angina intensity and medication use were not reduced. Studies with adequate blinding and a valid sham control group are still warranted due to the current low quality of evidence.

Frequency of attacks in patients with psychogenic non-epileptic seizures.

The aim of this study was to investigate the frequency of attacks (psychogenic seizures) in patients with psychogenic non-epileptic seizures (PNES) and to characterize factors potentially associated with attack frequency. In this retrospective study, all patients with PNES, who were studied at Shiraz Comprehensive Epilepsy Center at Shiraz University of Medical Sciences, Iran, from 2008 until 2018, were reviewed. We categorized the attack frequency in the patients as (1) daily; (2) weekly; and (3) frequency of less than one per week. Three hundred and ten patients were studied. Attack frequency in patients was 34 ± 67 per month. One hundred and eleven patients (36%) had daily attacks, 93 (30%) had weekly attacks, and 106 (34%) had less than weekly attacks. Sixty-five patients (21%) reported having more than one attack per day. Demographic variables, attack-related variables, PNES associated factors, and use of AEDs were not significantly associated with attack frequency in the patients. We observed that two thirds of the patients with PNES had frequent daily or weekly attacks. The findings of our study could be helpful in designing future clinical trials. First, attack frequency is an unbiased outcome measure in the design of such studies. Second, it is easily measurable using attack calendars; we suggest that attack frequency be assessed daily using daily attack calendars. Finally, it is very easy to recruit patients with PNES for clinical trials (with regards to their attack frequency) since many of them have frequent attacks.

High attack frequency in patients with angioedema due to C1-inhibitor deficiency is a major determinant in switching to home therapy: a real-life observational study.

BackgroundHereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) is characterized by recurrent attacks of swelling that affect various body sites. Such attacks are a frequent cause of visits to the emergency department and are often treated in the hospital. In recent years, self-administration of C1-inhibitor (C1-INH) concentrates at home has become an increasingly used option, with a positive impact on patient outcomes and quality of life.MethodsThis was an observational study of 6 months’ duration in 56 patients with C1-INH-HAE referred to a HAE center in southern Italy. The patients received three types of treatment for their swelling attacks: C1-INH concentrates administered at home (n = 25); icatibant administered at home (n = 12); and C1-INH concentrates administered in the hospital (n = 19). The objectives of this observational study were to compare therapy compliance (defined as the proportion of treated attacks) and quality of life in home- and hospital-treated patients, and to identify factors associated with the decision to use home therapy.ResultsOverall, 918 attacks were reported over 6 months, of which 544 (59.2 %) were treated. Total number of reported attacks and the mean (±SD) number of attacks per patient, respectively, in the three groups were: 611 and 24.4 (±26.1) for home-based C1-INH; 191 and 15.9 (±12.0) for home-based icatibant; 166 and 6.1 (±6.5) for hospital-based C1-INH. Differences in attack frequency between home- and hospital-based treatments were statistically significant (p = 0.002), while patient demographic characteristics and the disease severity score did not correlate with the use of home therapy. Compliance with therapy was significantly better with home-based therapy (71.2 % of treated attacks with C1-INH and 44.0 % with icatibant) than with hospital-based therapy (21.6 %, p = 0.003). Quality of life showed an opposite trend, with patients on hospital-based treatment reporting the highest quality of life.ConclusionsHome-based therapy was associated with better compliance compared with hospital-based therapy. The choice to adopt home-based therapy appeared to correlate with a high attack frequency. Home-based therapy is a valid treatment option for patients with C1-INH-HAE and should be offered to all such patients, and especially to those with high attack frequency.

Modified-release sildenafil reduces Raynaud's phenomenon attack frequency in limited cutaneous systemic sclerosis

To examine the effect of sildenafil in patients with Raynaud's phenomenon (RP) secondary to limited cutaneous systemic sclerosis (lcSSc). In this double-blind, placebo-controlled study, 57 patients with RP secondary to lcSSc were randomized to receive modified-release sildenafil 100 mg once daily for 3 days followed by modified-release sildenafil 200 mg once daily for 25 days or placebo. The primary assessment was the percentage change in the number of RP attacks per week in the per-protocol population. Secondary end points included Raynaud's Condition Score, duration of attacks, RP pain score, endothelial dysfunction assessed by a peripheral arterial tonometric (PAT) device, and serum biomarker levels. The mean percentage reduction from baseline to day 28 in attacks per week was greater for modified-release sildenafil than for placebo (-44.0% versus -18.1%, P = 0.034); the mean number of attacks per week improved from 25.0 at baseline to 19.3 after placebo treatment and from 30.5 to 18.7 after modified-release sildenafil treatment (P = 0.244). Decreases from baseline in Raynaud's Condition Score, duration of attacks, and RP pain score were not significantly different between groups. Mean values and changes from baseline in PAT responses and serum biomarker levels were similar between groups. The most frequent adverse events were headache and dyspepsia; the majority of adverse events were mild or moderate. Our findings indicate that modified-release sildenafil reduced attack frequency in patients with RP secondary to lcSSc and was well tolerated. Modified-release sildenafil may be a treatment option in this patient population.

Cluster Headache and Alpha 1-antitrypsin Deficiency

Little is known about the pathophysiology of cluster headache (CH), one of the most debilitating primary headaches. Interestingly, associations of lung affecting diseases or lifestyle habits such as smoking and sleep apnoea syndrome and CH have been described. Certain genotypes for alpha 1-antitrypsin (alpha(1)-AT) are considered risk factors for emphysema. Our aim was to investigate possible associations between common genotypes of the SERPINA1 gene and CH. Our study included 55 CH patients and 55 controls. alpha(1)-AT levels in serum and the genotype were analysed. Patients CH characteristics were documented. We could not detect any association between CH and a genotype that does not match the homozygous wild type for alpha(1)-AT. Interestingly, there is a significant difference of CH attack frequency in patients who are heterozygous or homozygous M allele carriers. We conclude that the presence of an S or Z allele is associated with higher attack frequency in CH.