Diagnostic Utility of Impedance-pH Monitoring in Refractory Non-erosive Reflux Disease

BACKGROUND/AIMS: Approximately one-third of non-erosive reflux disease (NERD) patients are refractory to proton pump inhibitors (PPI) and face a therapeutic challenge. Therefore, it is important to differentiate between pathological and non-pathological reflux utilizing multichannel intraluminal impedance-pH (MII-pH) to analyze symptom-reflux association and diagnose true NERD versus hyper-sensitive esophagus (HE) and functional heartburn (FH). Herein, we evaluated the diagnostic yield of MII-pH in refractory NERD and sub-classified it based on quantity and quality of acid/non-acid reflux and reflux-symptom association. METHODS: Sixty symptomatic NERD patients on twice daily PPI for > 2 months were prospectively evaluated by MII-pH. Distal and proximal refluxes, bolus exposure time (BET), esophageal acid exposure time, symptom index (SI) and symptom association probability (SAP) were measured. RESULTS: Thirty-two (53%) patients had BET > 1.4% (MII-pH positive-true NERD), while 28 (47%) had BET 80% of symptoms were associated with non-acid reflux. The number of distal refluxes in true NERD versus FH or HE were significantly different, but not between FH and HE. CONCLUSIONS: Approximately 60% of refractory PPI NERD patients had positive reflux-symptom association, primarily due to non-acid reflux. Nearly half of NERD patients on PPI had normal MII-pH monitoring, sub-divided further into FH and HE equally.

Wilson disease in 71 patients followed for over two decades in a tertiary center in Saudi Arabia: a retrospective review.

Wilson disease [WD] is a rare autosomal recessive disease. Our objective was to describe the diverse patterns, therapies, and outcomes of this disease. A retrospective study over two decades on WD patients in a tertiary care center in Saudi Arabia. Clinical and laboratory findings of 71 patients with WD were retrieved from their charts, referral notes and our hospital electronic records and were analyzed. The mean age and standard deviation was 16.8 [10.7] years and 56.5% were males. The main manifestations of WD were hepatic, neurological, and mixed in 39 [54.9%], 12 [16.9%], and 20 [28.2%] patients, respectively, and 11 [15.5%] were asymptomatic cases detected by family screening. A family history of WD was positive in 41 [57.7%] patients, and consanguinity of parents was found in 26 [36.6%] patients. The mean [SD] follow-up period was 92.2 [72.9] [range, 1-320] months. Ten [14.1%] patients died during follow up, while 45 [63.4%] and 16 [22.5%] were still on or lost from follow-up, respectively. The mean [SD] age at the end of follow-up was 25.3 [12] [range, 4-62] years. Hepatoma was discovered in 5 [7.0%] patients. Penicillamine therapy was used by 58 [81.7%] patients, while zinc and trientine were given to 32 [45.1%] and 11 [15.5%] patients, respectively. Sixteen [22.5%] patients underwent liver transplantation and one died [1.4%] on the waiting list. The liver condition remained stable or improved in 35 [49.3%], and the neurological status showed improvement in 11 [34.4%] of the 32 patients who had neurological involvement. This is the biggest cohort to be reported from the Middle East. WD presentation and outcome of WD are very diverse, and its diagnosis still depends on clinical, laboratory, and radiological evidence of abnormal copper metabolism. WD should be considered in patients of any age with obscure hepatic and/or neurological abnormalities.

Predictors of sustained virological response to a 48-week course of pegylated interferon alfa-2a and ribavirin in patients infected with hepatitis C virus genotype 4

Knowledge of the predictors of sustained viral response [SVR] to pegylated interferon [PEG-INF] alfa-2a and ribavirin [RBV] therapy in patients with hepatitis C genotype-4 [HCV-4] is crucial for selecting patients who would benefit most from therapy. We assessed the predictors of SVR to this combination therapy in Saudi patients with chronic HCV-4 infection. This retrospective study included 148 patients with HCV-4 infection who underwwent clinical, biochemical and virological assessments before treatment and at 12, 24, 48 and 72 weeks posttreatment. Of the 148 patients, 90 [60.8%] were males. Mean [SD] for age was 48.5 [12.7] years and BMI was 27.9 [7.5] kg/m[2]. Seventy-nine of 148 [60.1%] patients were treatment naive and 110 [74.3%] underwent pre-treatment liver biopsy. Eighteen [12.2%] patients did not complete therapy because of side effects or they were lost to follow up. Early virological response was achieved in 84 of 91 [92.3%] patients. In the 130 [87.8%] patients who completed therapy, 34 [26.2%] were non-responders and 96 [63.8%] achieved end-of-treatment virological response [ETVR]. SVR and virological relapse [24 weeks after ETVR] occurred in 66/130 [50.7%] and 30/130 [31.2%] patients, resspectively. Compared to relapsers, sustained responders were significantly younger [P = .005], non-diabetic [P = .005], had higher serum albumin [P = .028], lower alpha-fetoprotein level [P = .026], lower aspartate aminotransferase [AST] [P = .04] levels, and were treatment-naive [P = .008]. In a multivariate regression analysis, the independent predictors of SVR were younger age [P = .016], lower serum AST [P = .012], and being treatment na‹ve [P = .021]. Approximately half of HCV-4 patients who complete the course of combination therapy achieve an SVR, especially if they are young, treatment naive and have lower AST levels

Defecation of a "colon cast" as a rare presentation of acute graft-versus-host disease

Diffuse involvement of the gastrointestinal tract by graft versus host disease [GVHD] is a common complication of allogeneic hematopoietic stem cell transplant [HSCT]. Gastrointestinal GVHD usually presents 3 or more weeks after HSCT and is characterized by profuse diarrhea, anorexia, nausea, vomiting, abdominal pain and gastrointestinal bleeding. We report a case of a 23-year-old male who had undergone allogeneic HSCT and presented with bloody diarrhea on the 90th day post-HSCT. On the fourth day of admission, the patient passed per rectum a 27-cm long pinkish colored fleshy material recognized as a "colon cast". Sigmoidoscopy showed a congested and erythematous rectum with the remaining portion of the "colon cast" attached to the proximal part of the sigmoid colon. A biopsy from the rectal wall was suggestive of grade IV GVHD. The patient was treated with methylprednisolone, cyclosporin and mycophenolate mofetil, with a partial response [diarrhea and abdominal pain improved], but then he developed multiple other medical complications and died after 3 months