Resolution of refractory hepatic hydrothorax in patients with hepatitis C virus cirrhosis after treatment with direct-acting antiviral agents.

Hepatic hydrothorax (HH) is a transudative pleural effusion that complicates advanced liver cirrhosis. Cases refractory to medical treatment in the form of salt restriction and diuretics are labeled refractory hepatic hydrothorax (RHH) and may require transjugular intrahepatic portosystemic shunts (TIPSS) or even liver transplantation. Renal impairment is common in advanced liver disease, worsens its prognosis, and makes the management of HH more challenging. Successful antiviral therapy reduces some of the complications of cirrhosis secondary to hepatitis C virus (HCV) infection. We herein report two cirrhotic patients with chronic kidney disease who developed RHH which resolved after the successful treatment of their HCV infection with direct-acting antivirals (DAAs). In cases of RHH associated with HCV cirrhosis, a trial of DAAs is warranted before resorting to TIPSs or liver transplantation.

Mitochondrial Neurogastrointestinal Encephalomyopathy Syndrome Treated with Stem Cell Transplant: A Case Series and Literature Review.

Mitochondrial neurogastrointestinal encephalomyopathy syndrome is a rare autosomal recessive multisystem disorder caused by nuclear TYMP gene mutations, which leads to deficiency in thymidine phosphorylase enzyme. This deficiency then leads to mitochondrial dysfunction, which causes the features characteristic of this syndrome, including severe muscle wasting, gastrointestinal dysmotility, leukoencephalopathy, peripheral neuropathy, and ophthalmoplegia. Here, we present a case series of 3 patients with mitochondrial neurogastrointestinal encephalomyopathy from Saudi Arabia who underwent allogeneic stem cell transplant at King Faisal Specialist Hospital (Riyadh, Saudi Arabia). Two patients died within the first year of transplant, and the third is still alive but without improvement in clinical features. Allogeneic hematopoietic stem cell transplant-related mortality appears to be high; this may at least be partially related to established end-organ effects with decreased performance status. Although allogeneic hematopoietic stem cell transplant clearly affects correction of genetic and biochemical defects in mitochondrial neurogastrointestinal encephalomyopathy, its ability to reverse or improve established clinical manifestations has not been proven.

Mitochondrial Neurogastrointestinal Encephalomyopathy Treated with Stem Cell Transplantation: A Case Report and Review of Literature.

Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is a rare autosomal recessive disorder. The mutation in the ECGF1 gene causes severe deficiency of thymidine phosphorylase (TP), which in turn increases thymidine and deoxyuridine in the blood, serum, and tissue. The toxic levels of these products cause malfunction of the mitochondrial respiratory chain and mitochondrial DNA. Commonly, patients become symptomatic between 15 and 20 years of age (range 5 months to 35 years). The most commonly affected systems are gastrointestinal, followed by ocular, and nervous system. The disease is often fatal; high mortality rate is reported between 20 and 40 years of age. Treatment modalities that can increase thymidine phosphorylase activity and decrease thymidine and deoxy-uridine have shown symptomatic improvements in patients with MNGIE. Platelet transfusion, hemodialysis, peritoneal dialysis or allogeneic hematopoietic stem cell transplantation (HSCT) have been tried. The survival and long-term benefits of these measures are still not clear. Engrafted patients after stem cell transplantation have showed improvements in serum thymidine and deoxyuridine. We are reporting a case of MNGIE from Saudi Arabia, who underwent allogeneic hematopoietic stem cell transplantation. No MNGIE case has been previously reported from Saudi Arabia or the Gulf Arab countries. From the available literature, so far only 11 patients with MNGIE have undergone stem cell transplantation.

Levofloxacin-based first-line therapy versus standard first-line therapy for Helicobacter pylori eradication: meta-analysis of randomized controlled trials.

BACKGROUND: First-line levofloxacin-based treatments eradicate Helicobacter pylori with varying success. We examined the efficacy and safety of first-line levofloxacin-based treatment in comparison to standard first-line therapy for H pylori eradication. MATERIALS AND METHODS: We searched literature databases from Medline, EMBASE, and the Cochrane Register of Randomized Controlled Trials through March 2013 for randomized controlled trials comparing first-line levofloxacin and standard therapy. We included randomized controlled trials conducted only on naïve H pylori infected patients in adults. A systematic review was conducted. Meta-analysis was performed with Review Manager 5.2. Treatment effect was determined by relative risk with a random or fixed model by the Mantel-Haenszel method. RESULTS: Seven trials were identified with 888 patients receiving 7 days of first-line levofloxacin and 894 treated with standard therapy (Amoxicillin, Clarithromycin and proton pump inhibitor) for 7 days. The overall crude eradication rate in the Levofloxacin group was 79.05% versus 81.4% in the standard group (risk ratio 0.97; 95% CI; 0.93, 1.02). The overall dropout was 46 (5.2%) in the levofloxacin group and 52 (5.8%) for standard therapy. The dizziness was more common among group who took Levofloxacin based treatment and taste disturbance was more common among group who took standard therapy. Meta-analysis of overall adverse events were similar between the two groups with a relative risk of 1.06 (95% CI 0.72, 1.57). CONCLUSION: Helicobacter pylori eradication with 7 days of Levofloxacin-based first line therapy was safe and equal compared to 7 days of standard first-line therapy.

Entecavir for the treatment of real-life chronic hepatitis B patients: a study from Saudi Arabia.

BACKGROUND AND OBJECTIVES: Entecavir is a nucleoside analog used in the treatment of chronic hepatitis B. The efficacy of ETV has not been studied in the Saudi population. The objective of the study was to find undetectable HBV DNA after 48 weeks completion of ETV treatment in real-life versus clinical trial patients. DESIGN AND SETTING: A retrospective study in a tertiary care center in Saudi Arabia of patients treated from 2006 January to 2010 June. PATIENTS AND METHODS: Of 43 eligible patients, 24 patients were treatment-naïve and 19 were treatment refractory. RESULTS: Mean HBV DNA viral load was 51 million IU/mL prior to treatment and decreased to 0.16 million IU/mL at 48 weeks. Mean HBV DNA log10 IU/mL was 6.3 before treatment and decreased to 2.3 log10 IU/mL(P=.001) at 48 weeks. After 48 weeks treatment, ALT significantly decreased from a mean ALT of 88.7 U/L before treatment to 37.5U/L (P=.04). After 48 weeks, the HBV DNA was undetectable in 14 (58.4%) in treatment-naïve patients and in 6 (31.6%) treatment-refractory patients. At 48 weeks 17 (60.7%) of HBeAg-negative patients and 3 (20%) HBeAg-positive patients achieved undetectable HBV DNA (P=.003). When the treatment was extended for a median of 24 months (range 12 months to 60 months), 29 (67.4%) achieved undetectable HBV DNA. Among 29 patients who achieved undetectable HBV DNA, the treatment refractory patients reached undetectability within a mean of 32.4 (18.6) months and treatment-naïve patients in a mean of 18.8 (10.5) months(P=.01). Two (13.3%) of HBeAg-reactive patients converted to HBeAg-negative status and one patient (2.3%)lost HBsAg. CONCLUSION: After treatment with entecavir, HBV DNA undetectable at 48 weeks in 58.4% of naïve patients.The response rate was better in HBeAg-negative and treatment-naïve patients compared to HBeAg-positive and treatment-refractory patients.

Liver transplantation in Wilson's disease: Single center experience from Saudi Arabia.

AIM: To determine liver transplantation outcomes in Wilson's disease (WD) patients, focusing on neurological manifestations. METHODS: This retrospective study assessed data from 16 WD patients (nine males, 56%) who had liver transplants between 1991 and 2007. Survival, graft function, and neurological complications were assessed during a follow-up period of up to 15 years. In addition, each patient's medical record was reviewed in detail to find the type of Wilson's disease (hepatic or hepatic plus neurological WD), indication for liver transplantation, use of chelating agents prior to transplantation, immediate and long term complications following transplantation, the donor details, and the pathology of explanted liver. RESULTS: End-stage liver disease was the indication for transplantation in all 16 WD patients. Four patients displayed WD-related neurological symptoms in addition to liver disease. Living-related liver transplantation was done in three cases. One patient died on postoperative day 6 due to primary graft non-function. One-year post liver transplant survival was 94%. Neurological manifestations of all four patients disappeared during their follow-up. Four patients developed acute cellular rejection, but all responded to treatment. One patient developed chronic ductopenic rejection after 15 years post-transplantation and their graft failed; this patient is currently waiting for re-transplantation. Fourteen patients (88%) are still living. The long-term average survival is currently 10.5 years, with a current median survival of 8 years. Long-term graft survival is currently 81%. CONCLUSION: Short- and long-term survival in WD patient liver transplantation was excellent, and neurological and psychological WD manifestations disappeared during long-term follow-up.

Inflammatory bowel disease in Saudi Arabia: a hospital-based clinical study of 312 patients.

BACKGROUND AND OBJECTIVES: The epidemiology, clinical characteristics, and natural course of inflammatory bowel disease (IBD) in Saudi Arabia are still largely unknown. Hence, we decided to conduct a large retrospective, cohort study to determine these features of the disease. DESIGN AND SETTING: Retrospective study conducted in a tertiary care hospital in Riyadh from January 1970 to December 2008. PATIENTS AND METHODS: We reviewed all the cases of IBD diagnosed and collected all data pertaining to patients with IBD. RESULTS: A total of 312 patients with IBD were included for this analysis, including 197 (63%) patients with Crohn disease (CD) and 115 (37%) patients with ulcerative colitis (UC). The mean age (standard deviation) of patients with IBD was 25.5 (10.6) years; 152 (48.7%) were males and 160 females. The referral rate in the past 10 years was 72.1% compared with preceding 20 years, and 56% (n=178) of patients with IBD were from the central region of Saudi Arabia. The patients were followed up for a mean duration of 9.5 years; during their follow-up, 206 patients (66%) required hospital admission and 9 patients (2.9%) with UC developed colon cancer. A total of 6 patients died during the follow-up. Fifty-three percent (n=104) of the patients with CD underwent surgeries as part of their treatment, whereas only 20% (n=23) of the patients with UC underwent colectomy. CONCLUSIONS: The incidence of IBD has been gradually increasing in Saudi Arabia over the years. Clinical features and morbidity in patients are not different from patients with IBD seen in the West.

Colorectal cancer distribution in 220 Indian patients undergoing colonoscopy.

AIM: Colorectal cancer is one of the major cancers in the developed world. The incidence of colorectal cancer is low in India. The aim of the present study was to describe the anatomical distribution and age at diagnosis of colorectal cancer in India. METHODS: Retrospective descriptive analysis of anatomical distribution, age at diagnosis and demography of 220 cases (149 [67.7%] men) of adenocarcinoma of the colon or rectum diagnosed at colonoscopy over a period of five years. RESULTS: The mean age at diagnosis was 58.4 years (SD 13.3; range 23-85 years). Twenty-eight (12.7%) cases were below the age of 40 years. The majority (31.8%) cases were aged between 61-70 years. Most of the tumors (n=163, 74%) were located distal to the splenic flexure. Multivariate logistic regression analysis showed that bleeding per rectum (OR 2.8; 95% CI 1.2-6.2) was associated with distal cancer, and palpable mass (OR 3.9; 95% CI 1.7-8.6) was associated with proximal cancer. CONCLUSIONS: Almost one-third of the colorectal cancers in this series occurred in the seventh decade and were located distal to the splenic flexure.