AimsTherapy with an implantable cardioverter defibrillator (ICD) is established for the prevention of sudden cardiac death (SCD) in high risk patients. We aimed to determine the effectiveness of primary prevention ICD therapy by analysing registry data from 14 centres in 11 European countries compiled between 2002 and 2014, with emphasis on outcomes in women who have been underrepresented in all trials.Methods and resultsRetrospective data of 14 local registries of primary prevention ICD implantations between 2002 and 2014 were compiled in a central database. Predefined primary outcome measures were overall mortality and first appropriate and first inappropriate shocks. A multivariable model enforcing a common hazard ratio for sex category across the centres, but allowing for centre-specific baseline hazards and centre specific effects of other covariates, was adjusted for age, the presence of ischaemic cardiomyopathy or a CRT-D, and left ventricular ejection fraction ≤25%. Of the 5033 patients, 957 (19%) were women. During a median follow-up of 33 months (IQR 16–55 months) 129 women (13%) and 807 men (20%) died (HR 0.65; 95% CI: [0.53, 0.79], P-value < 0.0001). An appropriate ICD shock occurred in 66 women (8%) and 514 men (14%; HR 0.61; 95% CI: 0.47–0.79; P = 0.0002).ConclusionOur retrospective analysis of 14 local registries in 11 European countries demonstrates that fewer women than men undergo ICD implantation for primary prevention. After multivariate adjustment, women have a significantly lower mortality and receive fewer appropriate ICD shocks.

The association between neurological diseases, malignancies and cardiovascular comorbidities among patients with bullous pemphigoid: Case-control study in a specialized Polish center.

Small bowel adenocarcinoma (SBA) is a rare but increasing cause of gastrointestinal malignancy, being both a diagnostic and therapeutic challenge. The goal of treatment is margin negative resection of a lesion and local lymphadenectomy, followed by modern adjuvant chemotherapy combinations in selected cases. Improved outcomes in patients with SBA are encouraging, but elucidation of mechanisms of carcinogenesis and risk factors as well as improved treatment for this malignancy is very needed.

Summary Background Bullous pemphigoid (BP) is a distressing autoimmune bullous disease strongly associated with severe pruritus; however, data concerning pruritus in BP are still scarce. No clinical research evaluating the effect of BP on sleep quality has been conducted. Aim To evaluate the intensity of pruritus measured by nocturnal wrist movements (NWMs) and the sleep quality in patients with BP using actigraphy in comparison with nonpruritic healthy controls (HCs) with subsequent correlations with an itch visual analogue scale (VAS) as a subjective measure, disease severity [Bullous Pemphigoid Disease Area Index (BPDAI), urticaria/erythema, erosions/blisters] and serum total IgE level. Methods In total, 31 patients with newly diagnosed BP (mean ± SD age 75.4 ± 12.3 years) and 40 nonpruritic HCs (age 73.5 ± 11.7 years) were recruited. All participants wore a sleep monitor (ActiSleep+) on the dominant wrist. Results For patients with BP, median VAS score was 5.5 and median BPDAI was 43 (urticaria/erythema BPDAI was 16, erosions/blisters BPDAI was 29). Scratching, defined as bouts of NWMs, was significantly (P < 0.001) more intensive in patients with BP than in controls. Characteristic of BP was that scratching bouts corresponded with the slowest wrist movements. There were no correlations with VAS, BPDAI or total IgE level. Compared with HCs, patients with BP presented significant (P < 0.001) sleep disturbances, as determined by sleep efficiency, waking after sleep onset and average duration of awakening, and these were strongly correlated with urticaria/erythema BPDAI. Conclusion Nocturnal wrist movements measured by actigraphy are more intensive in patients with BP than in nonpruritic HCs, and characteristically slow movements. Actigraphy method showed very low sleep quality in patients with BP, thus severity of BP has a negative impact on sleep.

Gastrointestinal stromal tumours (GISTs) are the most common mesenchymal tumours of the gastrointestinal tract, but they represent less than 3% of all gastrointestinal tract malignancies. This is a detailed case study of a 52-year-old male patient treated for very uncommon histological subtype of gastric GIST with atypical clinical presentation, asymptomatic progress and late diagnosis. The resected tumour, giant in diameters, was confirmed to represent the most rare histopathologic subtype of GISTs - sarcomatoid epithelioid GIST. We report this case and review the literature with a special focus on pathomorphological evaluation, biological aggressiveness and prognostic factors. To our knowledge this is the first report of giant GIST of very uncommon sarcomatoid epithelioid subtype. It is concluded that clinicians should pay attention to the fact that initial diagnosis may be delayed due to mildly asymptomatic and non-specific clinical presentation. Asymptomatic tumours diagnosed at a late stage, which is often the case, can be large on presentation. Prognosis for patients diagnosed with GIST depend on tumour size, mitotic rate, histopathologic subtype and tumour location. That is why early diagnosis and R0 resection, which is usually feasible and safe even in giant gastric sarcomatoid epithelioid subtype of GISTs, are the key factors for further treatment and good prognosis.