Hereditary Transthyretin Amyloidosis: How to Differentiate Carriers and Patients Using Speckle-Tracking Echocardiography

Background: Hereditary transthyretin amyloidosis is a rare disease caused by transthyretin (TTR) gene mutations. The aim of our study was to identify early signs of cardiac involvement in patients with a TTR gene mutation in order to differentiate carriers from patients with neurological or cardiac disease. Methods: A case-control study was carried out on 31 subjects with the TTR mutation. Patients were divided into three groups: 23% with cardiac amyloidosis and polyneuropathy (group A), 42% with only polyneuropathy (group B) and 35% carriers (group C). Speckle-tracking echocardiography (left-ventricular global longitudinal strain-GLS, atrial stiffness) was performed in all patients. The apical/basal longitudinal strain ratio (SAB) and relative apical sparing (RAS) were assessed in all subjects. Results: Analyzing groups C and B, we only found a significant difference in the SAB (p-value 0.001) and RAS (p-value 0.039). These parameters were significantly more impaired in group A compared to group B (SAB p-value 0.008; RAS p-value 0.002). Also, atrial stiffness was significantly impaired in groups A and B compared to group C. Conclusions: Our study suggests the diagnostic role of the SAB and RAS in cardiac amyloidosis. The SAB and RAS showed a gradual increase from carriers to patients with neurological and cardiac diseases. Thus, these parameters, in addition to atrial stiffness, could be used to monitor carriers. More extensive data are needed

Efficacy of 5-ASA in the treatment of colonic diverticular disease

OBJECTIVE: To assess the therapeutic efficacy of 5-aminosalicylic acid (5-ASA) in patients with colonic diverticular disease performing a systematic review of the literature. DATA SOURCES: Cochrane Trial Register (until Issue 4, 2008), Medical Literature Analysis and Retrieval System Online (1966 to October 1, 2008), Excerpta Medica Database (1980 to October 1, 2008), and abstracts from the major US, European, and Asian gastroenterology conferences. Expert opinions sought and reference lists of identified studies and any relevant published reviews checked. STUDY SELECTION: Randomized controlled trial (RCT) or a controlled clinical trial with a parallel group design using 5-ASA as 1 treatment arm. RESULTS: Six RCTs enrolling 818 patients were found: 3 were performed in patients with uncomplicated diverticulitis, and the remaining in patients with symptomatic uncomplicated diverticular disease. The results of these studies showed that patients treated with 5-ASA had significantly better outcomes and that also mesalazine scheduled daily was superior to cyclic administration to prevent relapse of diverticular disease. However, several of these studies have not included an endoscopy at the start of the study and when patients had recurrences. CONCLUSIONS: From this systematic review, it seems that 5-ASAs may have a role in patients with colonic diverticular disease. However, high-quality well-designed RCTs are necessary to confirm these initial observations. Copyright © 2010 by Lippincott Williams & Wilkins

European private law

Lawyers around the world roughly agree on the meaning of private law. Whatever their national origins, they will point to contract and tort and identify their roots in the national private law order. Understanding European private law requires clarification of each of the three composite elements which includes Europe is not a state but a quasi-state with a multilevel governance structure, the law is not only private but also has a strong regulatory (public) dimension and law cannot be equated with codified law but has a multifaceted character-ranging from private regulation within a public regulatory frame to contract governance and self-regulation. Constitutionalization of private law through the increasing use of fundamental rights and human rights ranks high on the political, judicial, and academic agendas. The Draft Common Frame of Reference (DCFR) is designed to operate in a framework based on conventional actors: individual private parties, judges, and the legislator