Health-Related Quality of Life in Patients Living with Wilson Disease in Spain: A Cross-Sectional Observational Study.

Wilson disease (WD) is a rare copper metabolism disorder caused by mutations in the ATP7B gene. It usually affects young individuals and can produce hepatic and/or neurological involvement, potentially affecting health-related quality of life (HRQoL). We assessed HRQoL in a cohort of Spanish patients with WD and evaluated disease impact on several domains of patients' lives, treatment adherence, drug preference and satisfaction, and healthcare resource utilisation in a cross-sectional, retrospective, multicentric, observational study. A total of 102 patients were included: 81.4% presented isolated liver involvement (group H) and 18.6% presented neurological or mixed involvement (group EH). Up to 30% of patients reported a deteriorated emotional status with anxiety and depression, which was greater in the EH subgroup; the use of neuropsychiatric drugs was high. Over 70% of the patients were satisfied with their current treatment but complained about taking too many pills, stating they would consider switching to another more patient-friendly treatment if available. The Simplified Medication Adherence Questionnaire revealed only 22.5% of patients were fully adherent to therapy, suggesting that alternative therapies are needed. This real-world study, even though is highly enriched with hepatic patients and mild disease, shows that WD impacts patients' HRQoL, especially in the emotional domain.

'Why should I feel guilty? I always give my best'. Guilt in Spanish women caring for dependent relatives from a gender perspective.

OBJECTIVES: The sense of guilt in informal caregivers has been associated with lower levels of overall life-satisfaction, an increase in feelings of overwork, stress, and health problems, including anxiety and depression. Even though women who care for dependent relatives have higher levels of feelings of guilt than male caregivers, little research has been done on the dynamics of guilt from a gender perspective. This study examines the dynamics of this moral emotion in women caregivers' psychosocial health. METHODS: This study is based on semi-structured interviews with nineteen Spanish women who are informal caregivers looking after dependent family members. The interviews were subsequently transcribed and subjected to thematic analysis. RESULTS: Three themes emerged from qualitative analysis: first, doing more for the people being cared for than for oneself; secondly, being demanding on oneself; and thirdly, controlling one's own desires. CONCLUSION: The results show dynamics of self-control that seem to function as mechanisms for the women caregivers to avoid feelings of guilt, but at the price of taking on greater caring responsibilities, denying their own desires, and endangering their psychosocial health. We consider that intervention to help women reduce anticipatory guilt is essential, as these feelings put women caregivers' psychosocial health at risk.

Hepatocellular carcinoma risk in hepatitis C stage-3 fibrosis after sustained virological response with direct-acting antivirals.

BACKGROUND & AIMS: Patients with chronic hepatitis C and stage 3 fibrosis are thought to remain at risk of hepatocellular carcinoma after sustained virological response. We investigated this risk in a large cohort of patients with well-defined stage 3 fibrosis. METHODS: We performed a multicentre, ambispective, observational study of chronic hepatitis C patients with sustained virological response after treatment with direct-acting antivirals started between January and December 2015. Baseline stage 3 was defined in a two-step procedure: we selected patients with transient elastography values of 9.5-14.5 kPa and subsequently excluded those with nodular liver surface, splenomegaly, ascites or collaterals on imaging, thrombopenia or esophago-gastric varices. Patients were screened twice-yearly using ultrasound. RESULTS: The final sample comprised 506 patients (median age, 57.4 years; males, 59.9%; diabetes, 17.2%; overweight, 44.1%; genotype 3, 8.9%; HIV coinfection, 18.4%; altered liver values, 15.2%). Median follow-up was 33.7 (22.1-39.1) months. Five hepatocellular carcinomas and 1 cholangiocarcinoma were detected after a median of 29.4 months (95% CI: 26.8-39.3), with an incidence of 0.47/100 patients/year (95% CI: 0.17-1.01). In the multivariate analysis, only males older than 55 years had a significant higher risk (hazard ratio 7.2 [95% CI: 1.2-41.7; P = .029]) with an incidence of 1.1/100 patients/year (95% CI: 0.3-2.8). CONCLUSIONS: In a large, well-defined cohort of patients with baseline hepatitis C stage-3 fibrosis, the incidence of primary liver tumours was low after sustained virological response and far from the threshold for cost-effectiveness of screening, except in males older than 55 years.

Correction: Randomized prospective study evaluating tenofovir disoproxil fumarate prophylaxis against hepatitis B virus reactivation in anti-HBc-positive patients with rituximab-based regimens to treat hematologic malignancies: The Preblin study.

[This corrects the article DOI: 10.1371/journal.pone.0184550.].

Randomized prospective study evaluating tenofovir disoproxil fumarate prophylaxis against hepatitis B virus reactivation in anti-HBc-positive patients with rituximab-based regimens to treat hematologic malignancies: The Preblin study.

BACKGROUND: Hepatitis B virus (HBV) reactivation in patients with resolved HBV infection (HBsAg negative, antiHBc positive) is uncommon, but potentially fatal. The role of HBV prophylaxis in this setting is uncertain. The aim of this study was to compare the efficacy of tenofovir disoproxil fumarate (TDF) prophylaxis versus close monitoring in antiHBc-positive, HBsAg-negative patients under treatment with rituximab (RTX)-based regimens for hematologic malignancy. METHODS: PREBLIN is a phase IV, randomized, prospective, open-label, multicenter, parallel-group trial conducted in 17 hospitals throughout Spain. Anti-HBc-positive, HBsAg-negative patients with undetectable HBV DNA were randomized to receive TDF 300 mg once daily (Group I) or observation (Group II). The primary endpoint was the percentage of patients showing HBV reactivation during 18 months following initiation of RTX treatment. Patients with detectable HBV DNA (Group III) received the same dose of TDF and were analyzed together with Group I to investigate TDF safety. RESULTS: Sixty-one patients were enrolled in the study, 33 in the TDF treatment group and 28 in the observation group. By ITT analysis, HBV reactivation was 0% (0/33) in the study group and 10.7% (3/28) in the observation group (p = 0.091). None of the patients in either group showed significant differences in liver function parameters between baseline and the last follow-up sample. TDF was generally well tolerated and there were no severe treatment-related adverse events. CONCLUSION: In patients with hematological malignancy and resolved hepatitis B infection receiving RTX-based regimens, HBV reactivation did not occur in patients given TDF prophylaxis.

An Exercise in Exploring Big Data for Producing Reliable Statistical Information.

The availability of copious data about many human, social, and economic phenomena is considered an opportunity for the production of official statistics. National statistical organizations and other institutions are more and more involved in new projects for developing what is sometimes seen as a possible change of paradigm in the way statistical figures are produced. Nevertheless, there are hardly any systems in production using Big Data sources. Arguments of confidentiality, data ownership, representativeness, and others make it a difficult task to get results in the short term. Using Call Detail Records from Ivory Coast as an illustration, this article shows some of the issues that must be dealt with when producing statistical indicators from Big Data sources. A proposal of a graphical method to evaluate one specific aspect of the quality of the computed figures is also presented, demonstrating that the visual insight provided improves the results obtained using other traditional procedures.

Manometric findings in adult eosinophilic oesophagitis: a study of 12 cases.

OBJECTIVE: To describe the manometric findings detected in adult patients with dysphagia that were diagnosed of eosinophilic oesophagitis, and to compare with the cases of eosinophilic infiltration of the oesophagus reported in the literature. PATIENTS AND METHODS: We present 12 adult patients diagnosed as suffering from this disorder in our department in a 1.5-year period, according to histological criteria and discarding any other cause of eosinophilic infiltration of the oesophagus. Stationary oesophageal manometry using a hydropneumocapillary perfusion system was performed in every case. The recommendations of the Spanish Group of Digestive Motility were followed for the interpretation of the results. In seven patients who presented motor disorder in manometric evaluation, treatment with steroid oesophageal lavage using fluticasone propionate was carried out and these patients were subsequently re-evaluated. RESULTS: All patients were young predominantly men, and the first endoscopic examination showed regular concentric stenosis or a 'ring oesophagus'. Six patients had a severe nonspecific oesophageal motor disorder characterized by up to 80% of nontransmitted or very low-amplitude waves in the lower two-thirds of the organ. Three patients presented a manometric disturbance characterized by hyperkinetic peristaltic waves in distal oesophageal third. One patient had an alteration of the oesophageal motor dynamics characterized by 80% of deglutory complexes formed by a primary simultaneous wave in the two lower oesophageal thirds followed by a secondary peristaltic wave in 50% of cases that had a normal duration and amplitude. The remaining two patients had normal oesophageal motility. The upper oesophageal sphincter showed no alterations, and the manometric evaluation of the lower oesophageal sphincter tone proved normal in 10 patients, with slight hypotension in two cases. In seven of the nine patients who presented an oesophageal motor disorder, treatment with steroid oesophageal lavage using fluticasone propionate was administered and a new oesophageal manometry was performed afterwards, in which the motor disorder was clearly improved as soon as dysphagia, endoscopic lesions and histopathologic alteration disappeared. DISCUSSION: In the literature, 61 cases of eosinophilic infiltration of the oesophageal mucosa subjected to oesophageal manometric study had been described, and 60.6% of them showed evidence of different types of manometric alterations, mainly with spastic or hypercontractility characteristics. Although six of our cases showed very deficient peristalsis with very low-amplitude or nontransmitted waves, and in another three high-amplitude peristaltic waves were recorded. Motor disorders improved parallel to the disappearance of the eosinophilic infiltration of the mucosa. These data suggest that motor disorders in eosinophilic oesophagitis are a consequence of eosinophil infiltration of the oesophagus and should be considered in the differential diagnosis of dysphagia. These manometric alterations could be considered as primary nonspecific disorders and included in the 'ineffective oesophageal motility' group.