Enfermedad de Wilson: espectro clínico de la enfermedad hepática

Wilson's disease is a hereditary autosomal recessive disorder of copper metabolism,characterized by copper accumulation in the liver and brain. This rare entity, which has a broad clinical spectrum, is often difficult to diagnose and should therefore always be suspected in patients with liver disease of unclear cause. We describe two types of manifestation of liver disease in two patients; the first developed fulminant hepatic failure requiring urgent liver transplantation and the second showed advanced chronic liver disease and received standard medical treatment. The objective of this clinical observation is to analyze the diagnosis of Wilson's disease in two patients with distinct onset, illustrating the broad clinical spectrum of the disease, and its treatment.     

Treating psoriatic arthritis to target: discordance between physicians and patients’ assessment,non-adherence,and restricted access to drugs precluded therapy escalation in a real-world cohort

Clinical Rheumatology - The treat-to-target strategy (T2T) was associated with better outcomes in psoriatic arthritis (PsA) compared to standard care in clinical trials. This study aimed to analyze...     

Cachexia in patients with rheumatoid arthritis: a cohort study

Clinical Rheumatology - Rheumatoid arthritis (RA) is an inflammatory disease that leads to altered body composition. The loss of lean mass with a preservation or increase in fat mass has been...     

Histological analysis of liver parenchyma and choledochal wall, and external diameter and intraluminal pressure of the common bile duct in controls and patients with common bile duct stones with and without acute suppurative cholangitis

The purpose of this prospective controlled study was to determine the changes in intraluminal pressure and diameter of the common bile duct in a total of 121 bile patients with choledocholithiasis, and the consequences of these alterations for choledochal mucosa and liver histology. In fact, the reflux of bacteria from the obstructed biliary tract into the bloodstream is responsible for producing the clinical syndrome of acute suppurative cholangitis. Group I (26 patients) served as controls, Group II (50) had choledocholithiasis with clear green bile, and Group III (45) were patients with acute suppurative cholangitis with pus in the biliary tract. Ultrasonography revealed gallstones in all the patients. The external diameter of the common bile duct in patients with choledocholithiasis and acute suppurative cholangitis was significantly greater than in those of the other groups. Patients with acute suppurative cholangitis also had a higher intraluminal pressure than those of Groups I or II.     

Completely laparoscopic versus open gastrectomy for early and advanced gastric cancer: a matched cohort study

Introduction

The application of laparoscopic gastric surgery has rapidly increased for the treatment of early gastric cancer. However, laparoscopic gastrectomy for advanced tumor remains controversial, particularly in terms of oncologic outcomes. This study was designed to compare 3-year survival of laparoscopic versus open curative gastrectomy in early and advanced gastric cancer.

Methods

This was a retrospective matched cohort study. We included patients between 2003 and 2010 with an R0 resection. A totally laparoscopic technique was used and D2 lymph node dissection was practiced routinely. We performed an intracorporeal hand-sewn esophagojejunostomy in all laparoscopic total gastrectomy cases. We matched all laparoscopic cases 1:1 with open cases according to TNM AJCC seventh edition. We used Mann–Whitney or t test and Chi-square test to compare both groups. Kaplan–Meier analysis with log-rank test was performed to compare survival.

Results

We included 31 open and 31 laparoscopic cases (mean age 63?±?14?years; 66% males). Both groups were identical in type of gastrectomy (71% total and 29% subtotal). There were no statistical difference between laparoscopic and open groups in age, sex, N category, tumor location and size, histological differentiation, and T category (48% T1, 13% T2, 16% T3, and 23% T4 in both groups), with 48% early and 52% advanced tumors. The median number of resected lymph nodes was similar: 35 (23–53) for laparoscopic and 39 (23–45) for open cases (P?=?0.81). The median follow-up was 50?months. The overall 3-year survival was 82% for laparoscopic surgery and 87% for the open surgery group (P?=?0.56). There were no difference in 3-year survival for the laparoscopic versus the open surgery groups for advanced tumors (74 vs. 75%, P?=?0.88), N+?tumors (73 vs. 73%, P?=?0.99) and for the different AJCC stages (stage 1: 94 vs. 100%, stage 2: 89 vs. 82%, and stage 3: 50 vs. 50%, P?=?0.32, 0.83, and 0.98 respectively).

Conclusions

In this preliminary report, with 52% of advanced tumor, the 3-year overall and stage-by-stage survival was comparable for laparoscopic and open curative gastrectomy.