Tangeskesen9076

Background Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare inherited disorder causing life-threatening arrhythmias. Long-term outcome studies of the channelopathy are limited. Objective The aim of the present study was to summarize our knowledge on CPVT patients, including the clinical profile treatment approach and long-term outcome. Methods In this single center study, we retrospectively and prospectively collected data from nine CPVT patients and analyzed them. Results We reviewed nine patients with CPVT in seven families (22% male), with a median follow-up time of 8.6 years. Mean age at diagnosis was 26.4 12 years. Symptoms at admission were syncope (four patients) and aborted cardiac arrest (four patients). Family history of sudden cardiac death was screened in five patients. In genetic analyses, we found five patients with ryanodine type 2 receptor (RYR2) mutations. Seven patients were treated with beta-blockers, and if symptoms persisted flecainide was added (four patients). Despite beta-blocker treatment, three patients suffered from seven adverse cardiac events. An implantable cardioverter defibrillator was implanted in seven patients (one primary, six secondary prevention). Over the follow-up period, three patients suffered from ventricular tachycardia (ten times) and five patients from ventricular fibrillation (nine times). No one died during follow-up. Conclusion Our CPVT cohort showed a high risk of cardiac events. Family screening, optimal medical therapy and individualized treatment are necessary in affected patients in referral centers.Atopic dermatitis is the most common chronic inflammatory skin disease. It is often the first indicator of allergic diseases, and a certain percentage of patients are affected by allergic rhinitis and/or asthma as a consequence. The study aimed to investigate the link between atopic dermatitis and comorbidity in family medicine. In the specialist family medicine practice Osijek, a retrospective study was conducted in the period from January 1, 2016 to July 1, 2017 on the percentage of patients with atopic dermatitis in the total number of patients, and their comorbid diseases. The data source was the E-chart. The results showed that 195 (10.53%) out of 2056 patients had atopic dermatitis, 80 (41%) patients had atopic dermatitis and allergic rhinitis, 34 (17.4%) asthma, 132 (67.7%) infections, 59 (30.3%) gastrointestinal disturbances, and 68 (34.3%) had mental disorders. Patients up to 18 years old were more likely to have infections, and adult patients were exposed to chronic stress. The most commonly used drug was loratadine (60.5%), while mometasone was the most commonly administered topical drug (40%). The result of this research showed the steps of the ˝atopic march˝. Atopic dermatitis is followed by changes in the skin and its progression to other organ systems in most of the patients.A recent phase 3 trial showed that the outcome of patients with relapsed/refractory (R/R) FLT3-mutated acute myeloid leukemia (AML) improved with gilteritinib, a single-agent second-generation FLT3 tyrosine kinase inhibitor (TKI), compared with standard of care. In this trial, the response rate with standard therapy was particularly low. We retrospectively assessed the characteristics and outcome of patients with R/R FLT3-mutated AML included in the Toulouse-Bordeaux DATAML registry. Among 347 patients who received FLT3 TKI-free intensive chemotherapy as first-line treatment, 174 patients were refractory (n = 48, 27.6%) or relapsed (n = 126, 72.4%). Salvage treatments consisted of intensive chemotherapy (n = 99, 56.9%), azacitidine or low-dose cytarabine (n = 9, 5.1%), other low-intensity treatments (n = 17, 9.8%), immediate allogeneic stem cell transplantation (n = 4, 2.3%) or best supportive care only (n = 45, 25.9%). Among the 114 patients who previously received FLT3 TKI-free intensive chemotherapy as first-line treatment (refractory, n = 32, 28.1%; relapsed, n = 82, 71.9%), the rate of CR (complete remission) or CRi (complete remission with incomplete hematologic recovery) after high- or low-intensity salvage treatment was 50.0%, with a bridge to transplant in 34.2% (n = 39) of cases. The median overall survival (OS) was 8.2 months (interquartile range, 3.0-32); 1-, 3- and 5-year OS rates were 36.0% (95%CI 27-45), 24.7% (95%CI 1-33) and 19.7% (95%CI 1-28), respectively. In this real-word study, although response rate appeared higher than the controlled arm of the ADMIRAL trial, the outcome of patients with R/R FLT3-mutated AML remains very poor with standard salvage therapy.BACKGROUND No dural substitute has proven to be complication-free in a large clinical trial, even suggesting some benefit during watertight closure. However, primary dural closure is not always possible due to dural shrinkage from electrocautery for dural bleeding. OBJECTIVE This study is performed to analyze the clinical outcomes related to cerebrospinal fluid (CSF) leakage after microvascular decompression (MVD) using a simple surgical technique. METHODS Three hundred and sixty consecutive cases were treated with MVD and followed up for more than one month after surgery. Bleeding from the cut veins during dural incision was controlled by pulling stay sutures instead of electrocautery to avoid dural shrinkage. Additionally, a wet cottonoid was placed on the cerebellar side dural flap to prevent dural dehydration. During dural closure, primary dural closure was always attempted. this website If not possible, a "plugging muscle" method was used for watertight dural closure. RESULTS The mean age was 54.1 ± 10.8 years (range, 24-85 years), and 238 (66.1%) were female. Primary MVD was performed in 345 (95.8%) patients. The mean operation time (from skin incision to skin closure) was 96.7 ± 33.0 min (range, 38-301 min). Primary dural closure was possible in 344 (95.6%) patients. The "plugging muscle method" was performed more frequently in patients older than 60 years (8 of 99 cases, 8.08%) than in younger cases (8 of 261 cases, 3.07%) (p = 0.039; chi-squared test). After surgery, 5 (1.4%) patients were treated for middle ear effusion, and another 5 (1.4%) patients experienced transient CSF rhinorrhea, which was spontaneously resolved within 1 to 7 days. No patients required additional treatments for CSF leakage. CONCLUSION A simple technique using pulling stay sutures to stop bleeding from the dural edges and placing a wet cottonoid on the exposed dura can make primary dural closure easier.