Παρασκευή 9 Νοεμβρίου 2018

Autoimmune Enteropathy: An Updated Review with Special Focus on Stem Cell Transplant Therapy

Abstract

Autoimmune enteropathy (AIE) is a complex disease affecting both children and adults. Although associated with significant morbidity and mortality, the pathophysiology of the disease and its treatment have not been well characterized. This study aims to review the medical literature available on this rare but clinically significant ailment, to help establish a better understanding of its pathophysiology and enumerate the available diagnostic and treatment modalities. A literature search was conducted on PubMed using key terms related to autoimmune enteropathy and intractable diarrhea, with no restrictions on the date of publication or language. We found a total of 98 reports of AIE published in the form of case reports and case series. The evidence reviewed suggests that AIE is a multifaceted disorder that requires a high index of suspicion in the appropriate clinical setting to be able to make an early diagnosis. Current evidence supports the use of supportive care to correct nutritional and metabolic deficiencies, and immunosuppressives and immunomodulators as directed therapies. Hematopoietic stem cell transplant is an aggressive, but successful curative modality for patients with AIE as part of immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome. Cumulative clinical experience with management of AIE has allowed improved outcomes in transplanted and non-transplanted AIE patients even though morbidity and mortality with are still high in patients with this condition. More research is needed to further define the role of new therapies for AIE, and a central registry with participation of multiple institutions might help share and standardize care of patients with this rare but serious condition.



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Short-Term Symptomatic Relief in Gastroesophageal Reflux Disease: A Comparative Study of Esomeprazole and Vonoprazan

Abstract

Background and Aim

Proton pump inhibitors and vonoprazan (a potassium-competitive acid blocker) are recommended as first-line treatments for gastroesophageal reflux disease (GERD). However, few reports have investigated the onset of action of these agents for GERD symptom relief. The present study compared the symptom relief of esomeprazole with that of vonoprazan via monitoring self-reported GERD symptoms after treatment initiation.

Methods

This was a prospective, multicenter, randomized, open-label, parallel group, comparative clinical study between esomeprazole (20 mg/day) and vonoprazan (20 mg/day) administered for 4 weeks to patients with GERD symptoms. Patients who had scores ≥ 8 on the Gastroesophageal Reflux Disease Questionnaire (GerdQ) were defined as having GERD and enrolled in this study. Sixty patients were randomly assigned to either the esomeprazole group (n = 30) or the vonoprazan group (n = 30). Treatment response rates in each drug group were evaluated according to the GerdQ. The Frequency Scale for the Symptoms of GERD (FSSG) scores from the 1st day after treatment initiation and the Global Overall Symptom (GOS) scale scores during treatment were also evaluated.

Results

At 4 weeks, the treatment response rates for symptom relief according to the GerdQ were 88.0% in the esomeprazole group and 81.8% in the vonoprazan group. The GOS scales, which reflect the impact of GERD symptoms, were similar for both groups. The FSSG scores decreased from the 1st to the 14th day in both groups.

Conclusions

There were no substantial differences in the symptom relief between the two groups at any time point in this short-term study.



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Malignant Transformation of Ectopic Pancreas

Abstract

Malignant transformation of ectopic pancreas tissue is a diagnostic challenge as clinical symptoms and radiographic features of these tumors are non-specific. Given the rarity of these lesions, it is usually neither suspected nor included in the diagnostic workup of different tumors. We conducted a comprehensive literature review regarding malignancy arising from ectopic pancreas for a better understanding of its frequency, clinicopathological features, and prognosis. A literature search was performed in three major databases: PubMed, Cochrane, and Web of Science. Fifty-four well-documented cases of malignant ectopic pancreas were identified in the published literature. Our analysis provided the following observations: (1) there was a slight predominance of males over females; (2) most patients with malignant transformation of ectopic pancreas were middle-aged; (3) most commonly, the tumor was located in the stomach; (4) most tumors were adenocarcinomas; (5) most frequently, the malignancy arose within a type I heterotopia according to Heinrich classification; (6) macroscopically, a subepithelial-like appearance was most frequently observed; and (7) improved prognosis for ectopic pancreatic malignancies in comparison with reported survival data for orthotopic pancreatic cancer. Even if the majority of cases of ectopic pancreas are incidental findings and malignant transformation is a rare event, pancreatic heterotopy should be considered as a source of potentially malignant lesions.



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The early-citation trend: an analysis of seven rehabilitation journals concerning the 2015–2017 window

No abstract available

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Effectiveness of robot-assisted gait training on motor impairments in people with Parkinson’s disease: a systematic review and meta-analysis

imageThe aim of this systematic review and meta-analysis was to evaluate the effectiveness of robot-assisted gait training (RAGT) on motor impairments in people with Parkinson's disease (PD). A computer-based systematic literature search was performed in six databases according to PRISMA guidelines. Randomized controlled trials (RCTs) that assessed the effects of RAGT on motor impairments in people with PD were included. GRADE approach and PEDro scale were used to determine the studies' quality of evidence. Meta-analyses were performed by calculating the weighted mean difference (WMD) at 95% confidence interval. Seven RCTs (PEDro: 5–8) met the inclusion criteria for systematic review and meta-analyses. The meta-analysis showed significant improvement on Unified Parkinson Disease Rating Scale Part III after intervention [WMD=3.292; 95% confidence interval (CI)=1.378–5.207; P=0.000], and after 1-month follow-up (WMD=5.512; 95% CI=2.396–8.629; P=0.001). Stride length (WMD=9.283; 95% CI=7.153–11.414; P=0.00) and gait speed (WMD=0.166; 95% CI=−0.090 to 0.243; P=0.000) showed significant improvements after RAGT. Balance as measured by Berg Balance Scale was improved significantly after intervention (WMD=3.87; 95% CI=0.374–6.735; P=0.029) and at 1-month follow-up (WMD=3.87; 95% CI=1.324–6.413; P=0.002). The pooled analysis did not detect any significant changes regarding stride time, cadence and functional balance scales. GRADE level of evidence ranged between high and low. The RAGT showed better outcomes than conventional interventions on some motor aspects in PD. However, RAGT did not seem superior to control interventions. Further RCTs that examine the effect of RAGT on more specific outcomes and at different medication statuses are required.

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Feasibility of a targeted strengthening program to improve gait in people with multiple sclerosis: a brief report

imageThis study aims to determine feasibility of strengthening muscles that are important contributors to gait for people with multiple sclerosis, yet are not routinely targeted in the literature. An 8-week strengthening intervention targeted ankle plantarflexion, hip abduction, and trunk muscles using a repeated-measures design. Outcomes included satisfaction, adherence, muscle strength, gait speed (timed 25-foot walk), gait endurance (6-min walk test), and self-reported gait-related participation (Multiple Sclerosis Walking Scale-12). Ten participants (Expanded Disability Status Scale: 3.5–5.5) completed the intervention. All participants were at least 'satisfied'; adherence was 87% (supervised sessions) and 75% (home sessions). All quantitative measures improved: muscle strength (23.1–47.6%, P

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Reliability and validity of the shortened Singapore versions of the Chedoke Arm and Hand Activity Inventory

imageUpper limb deficits are common sequelae after a stroke and negatively affect daily living and quality of life. The use of outcome measures to evaluate upper limb function is essential to assess sensorimotor recovery and to determine the effectiveness of rehabilitation. The aim of this study was to estimate the construct validity and inter-rater reliability of three shortened versions of the Singapore version of the Chedoke Arm and Hand Activity Inventory (CAHAI-SG) comprising seven, eight, and nine test items. The sample consisted of 55 inpatients with acute/subacute stroke to whom the CAHAI-SG, Fugl-Meyer Assessment of Upper Extremity (FMA-UE) and the Action Research Arm Test (ARAT) were administered. To estimate convergent and discriminative construct validity, Spearman's rank correlation coefficient and 95% confidence intervals were computed for CAHAI-SG scores with FMA-UE and ARAT scores. Reliability was estimated using intraclass correlation coefficient (relative reliability) and the standard error of measurement (absolute reliability). Convergent validity with the FMA-UE was 0.79, 0.80, and 0.81 for seven-item, eight-item, and nine-item versions of the CAHAI-SG, respectively, and 0.81 with the ARAT for all shortened versions. Discriminative validity with the FMA-UE pain subscale was between 0.37 and 0.38. The absolute reliability was 3.09, 3.65, and 3.98, and relative reliability was 0.96, 0.95, and 0.96 for the seven-item, eight-item, and nine-item versions, respectively. All shortened versions of the CAHAI-SG demonstrated similar psychometric properties to the full (13 item) version, meaning clinicians may use these shorter versions that require less time to administer and score.

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