Autosomal dominant polycystic kidney disease and coronary artery dissection or aneurysm: A systematic review

Importance Autosomal dominant polycystic kidney disease (ADPKD) has been associated with cardiovascular abnormalities such as intracranial and aortic aneurysms. Objective To systematically review the case reports and case series of ADPKD patients with coronary artery dissection or aneurysm. Evidence review Systematic review registration number: CRD42015015723. Data sources: MEDLINE, Web of Science and OpenGrey, reference lists of studies. Study selection: Published case reports and case series. Data extraction: Two parties analyzed the studies. Disagreements were solved by consensus or by a third party. Funding: none. Findings The reports of 23 patients (22 from 17 studies-six with coronary artery dissection and 16 with coronary artery aneurysm-and one with coronary dissection) were analyzed and reported here. Most patients were symptomatic. Coronary dissection showed female and left descending anterior artery predominance, features similar to non-ADPKD patients, but a median diagnostic age below expected (41 vs. 50 years old). Coronary aneurysms had male and right coronary artery predominance but lower median diagnostic age (44 years old) and higher rate of multiple vessel affection than reported for non-ADPKD patients. Conclusion and relevance Clinical disparities may suggest a different mechanism of aneurysm formation compared to the population without ADPKD. Nevertheless, lack of access to data of one patient and text of one article limited our conclusions. Coronary aneurysms and dissections represent a source of coronary syndromes and death in ADPKD. Mutation of ADPKD-related genes may predispose to coronary abnormalities, especially aneurysms. Further analysis regarding this association is necessary

When the Diagnosis is a Victim of the Circumstances

Introduction: Haemoptysis is a common symptom which can sometimes mimic gastrointestinal bleeding. Case description: We describe the case of a 31-year-old man who presented to the emergency department after an episode of sudden nausea and presumed massive haematemesis. The situation was interpreted as gastrointestinal bleeding but clinical evolution and greater attention to the anamnesis resulted in a diagnosis of pulmonary tuberculosis and the provision of appropriate care. Discussion: This report emphasizes the difficulty of differentiating between haemoptysis and haematemesis and the importance of a careful anamnesis and attention to all clinical circumstances for an accurate diagnosis. Learning points: Haematemesis and haemoptysis are not always distinguished from each other when a patient's history is being collected.Despite being a rare manifestation of tuberculosis, in the correct epidemiological context, haemoptysis should raise the suspicion of pulmonary tuberculosis.The clinical setting and the need for immediate care should not limit clinical investigation or the differential diagnosis.info:eu-repo/semantics/publishedVersio

Primary biliary cirrhosis in a rheumatoid arthritis patient treated with rituximab, a case-based review

Primary biliary cirrhosis (PBC) is an autoimmune disease in which intrahepatic bile ducts are targeted by an immune-mediated injury. This disease tends to progress to fibrosis and cirrhosis with hepatic failure. The authors report a case of a 50-year-old rheumatoid arthritis (RA) patient, with erosions and seropositive for rheumatoid factor and anti-citrullinated peptide antibodies, with 18 years disease duration refractory to prednisolone and several disease-modifying antirheumatic drugs, either conventional or biological (adalimumab and etanercept). In April 2007, she started therapy with rituximab (RTX) with good European League Against Rheumatism response achieved 9 months later. In June 2008, she was admitted with intrahepatic cholestasis, steatorrhea, and spontaneous fractures of various ribs. After excluding cholelitiasis, as well as infectious and neoplastic diseases a liver biopsy was performed that was compatible with the diagnosis of PBC. The antinuclear antibodies (1/160) were positive as well as the antimitochondrial antibodies (1/640). Other antibodies were negative such as anti-SSA and anti-SSB. Afterwards, the patient started ursodesoxycholic acid 15 mg kg(-1) day(-1) with progressive improvement of cholestatic markers. A labial salivary gland biopsy was performed and showed findings compatible with the concomitant diagnosis of Sjögren's syndrome. Based on this clinical report, a detailed review of the clinical aspects of PBC is presented as well as its association with other immune-mediated inflammatory diseases, particularly, with RA

Patients- and physicians- priorities for improvement: The case of rheumatic diseases

OBJECTIVES: To compare the health priorities elected by patients with rheumatic diseases and by their attending rheumatologists. PATIENTS AND METHODS: We undertook a cross-sectional study among patients and rheumatologists in Portuguese rheumatology outpatient clinics. 75% of all Portuguese Rheumatology Departments agreed to participate. Rheumatologists from non-participating hospital departments were asked to collaborate through their private practices. All patients were eligible for inclusion except if they were under 18 years of age or had a mental disorder that would affect their participation. Data were collected through dedicated questionnaires. Patients were asked to indicate 3 priorities for improvement out of 12 health domains (Arthritis Impact Measurement Scale 2) regarding their rheumatic disease. Rheumatologists were asked similar questions focused around rheumatoid arthritis (RA) and osteoarthritis (OA). RESULTS: 1,868 patients and 56 rheumatologists entered the study. The most commonly selected priorities by patients with rheumatic diseases were: "Rheumatic pain" (70%), "Walking and bending" (45%), and "Hand and Finger Function" (40%). The main priority for improvement among patients with RA was "Rheumatic Pain" (69%), while rheumatologists more commonly elected "Work" (55%) as their main priority for these patients. Among patients with OA, "Rheumatic Pain" was the first priority for both patients and doctors (elected by 75%, and 55% of respondents, respectively). CONCLUSIONS: Our study showed discordance between the priorities for improvement elected by patients and by their respective physicians. This was more pronounced in RA than in OA. Studying and addressing such differences may support physicians and institutions to better achieve the prime goal of incorporating and responding to patients' needs and preferences

An investigation of product design and production in an automotive assembler and engine suppliers that adopt modularity

Modularity can be understood as a set of concepts that leads to new product architecture and production organization. This enables the transference of value-added activities throughout the automotive supply chain. In this context, the aim of this paper is to investigate modularity adoption in product design and in production by considering a specific situation of the relationship between a commercial vehicle assembler and engine suppliers. In order to fulfil those objectives, a case-based research was employed as the methodological approach. The results presented some influences that occur between product design and production and vice-versa - part of them were expected. This reinforces the confirmatory nature of the empirical data. The modularity concept may be applied to a productive process not necessarily designed in modules, i.e. it is possible to assemble components as from an existing product, resulting in sub-sets in a modular logic. Data also confirmed that the product architecture of a commercial vehicle favours product division in modules. Moreover, the supply chain management is simplified compared to the traditional production system, as part of the responsibilities is transferred to the suppliers

Understanding patient-reported knowledge of hernia surgery: a quantitative study

Abdominal wall; Knowledge; PatientPared abdominal; Conocimiento; PacienteParet abdominal; Coneixement; PacientPurpose The objective of this study was to gather information on patient-reported knowledge (PRK) in the field of hernia surgery. Methods A prospective quantitative study was designed to explore different aspects of PRK and opinions regarding hernia surgery. Patients referred for the first time to a surgical service with a presumed diagnosis of hernia and eventual hernia repair were eligible, and those who gave consent completed a simple self-assessment questionnaire before the clinical visit. Results The study population included 449 patients (72.8% men, mean age 61.5). Twenty (4.5%) patients did not have hernia on physical examination. The patient’s perceived health status was “neither bad nor good” or “good” in 56.6% of cases. Also, more patients considered that hernia repair would be an easy procedure (35.1%) rather than a difficult one (9.8%). Although patients were referred by their family physicians, 32 (7.1%) answered negatively to the question of coming to the visit to assess the presence of a hernia. The most important reason of the medical visit was to receive medical advice (77.7%), to be operated on as soon as possible (40.1%) or to be included in the surgical waiting list (35.9%). Also, 46.1% of the patients considered that they should undergo a hernia repair and 56.8% that surgery will be a definitive solution. Conclusion PRK of patients referred for the first time to an abdominal wall surgery unit with a presumed diagnosis of hernia was quite limited and there is still a long way towards improving knowledge of hernia surgery.Open Access Funding provided by Universitat Autonoma de Barcelona

Prevalence of Vitamin D Deficiency and its Predictors in the Portuguese Population: a Nationwide Population-Based Study

Vitamin D deficiency is prevalent worldwide, but its prevalence is unknown in adult Portuguese population. In Portugal, 66% of adults present Vitamin D insufficiency/deficiency. Winter, living in Azores, older age, and obesity were the most important risk factors. It highlights the need of strategies to prevent vitamin D deficiency in Portugal. Objective: To estimate the prevalence and risk factors of vitamin D deficiency in the adult Portuguese population. Methods: Adults (≥ 18 years old) from the EpiReumaPt Study (2011-2013) were included. Standardized questionnaires on socio-demographic and lifestyle features were obtained. Serum 25-hydroxyvitamin D [25(OH)D] concentrations were evaluated using ADVIA Centaur VitD competitive immunoassay (Siemens Healthineers) in 2015-2017 as 25 (OH)D Level 0: ≤ 10 ng/mL; Level 1: 11-19 ng/mL; Level 2: 20-29 ng/mL, and Level 3: ≥ 30 ng/mL. Weighted multinomial regression analysis was conducted to evaluate the association between socio-demographic and lifestyle variables and vitamin D status. Results: Based on weighted analysis, the estimated prevalence of levels of 25(OH)D ≤ 10, < 20, and < 30 ng/mL was 21.2, 66.6, and 96.4%, respectively. The strongest independent predictors of serum 25 (OH)D ≤ 10 ng/mL were living in the Azores archipelagos (OR 9.39; 95%CI 1.27-69.6) and having the blood sample collection in winter (OR 18.53; 95%CI 7.83-43.87) or spring (11.55; 95%CI 5.18-25.74). Other significant predictors included older age (OR 5.65, 95%CI 2.08-15.35), obesity (OR 2.61; 95%CI 1.35-5.08), current smoking (OR 2.33; 95%CI 1.23-4.43), and female gender (OR 1.9, 95%CI 1.1-3.28). Conversely, physical exercise (OR 0.48, 95%CI 0.28-0.81) and occasional alcohol intake (OR 0.48, 95%CI 0.29-0.81) were associated with a lower risk of 25(OH)D ≤ 10 ng/mL. Conclusion: Vitamin D deficiency/insufficiency [25(OH)D 60% of all Portuguese adults, with strong geographical and seasonal variation. This study highlights the need to critically assess the relevance of vitamin D deficiency as a public health problem and the urgent need for a wide and scientifically robust debate about the most appropriate interventions at the individual and societal levels.info:eu-repo/semantics/publishedVersio