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Therapeutic Advances in Gastroenterology Jan 2017Mitochondrial disorders (MIDs) due to respiratory-chain defects or nonrespiratory chain defects are usually multisystem conditions [mitochondrial multiorgan disorder... (Review)
Review
Mitochondrial disorders (MIDs) due to respiratory-chain defects or nonrespiratory chain defects are usually multisystem conditions [mitochondrial multiorgan disorder syndrome (MIMODS)] affecting the central nervous system (CNS), peripheral nervous system, eyes, ears, endocrine organs, heart, kidneys, bone marrow, lungs, arteries, and also the intestinal tract. Frequent gastrointestinal (GI) manifestations of MIDs include poor appetite, gastroesophageal sphincter dysfunction, constipation, dysphagia, vomiting, gastroparesis, GI pseudo-obstruction, diarrhea, or pancreatitis and hepatopathy. Rare GI manifestations of MIDs include dry mouth, paradontosis, tracheoesophageal fistula, stenosis of the duodeno-jejunal junction, atresia or imperforate anus, liver cysts, pancreas lipomatosis, pancreatic cysts, congenital stenosis or obstruction of the GI tract, recurrent bowel perforations with intra-abdominal abscesses, postprandial abdominal pain, diverticulosis, or pneumatosis coli. Diagnosing GI involvement in MIDs is not at variance from diagnosing GI disorders due to other causes. Treatment of mitochondrial GI disease includes noninvasive or invasive measures. Therapy is usually symptomatic. Only for myo-neuro-gastro-intestinal encephalopathy is a causal therapy with autologous stem-cell transplantation available. It is concluded that GI manifestations of MIDs are more widespread than so far anticipated and that they must be recognized as early as possible to initiate appropriate diagnostic work-up and avoid any mitochondrion-toxic treatment.
PubMed: 28286566
DOI: 10.1177/1756283X16666806 -
The Cochrane Database of Systematic... Apr 2016Pancreatic pseudocysts are walled-off peripancreatic fluid collections. There is considerable uncertainty about how pancreatic pseudocysts should be treated. (Review)
Review
BACKGROUND
Pancreatic pseudocysts are walled-off peripancreatic fluid collections. There is considerable uncertainty about how pancreatic pseudocysts should be treated.
OBJECTIVES
To assess the benefits and harms of different management strategies for pancreatic pseudocysts.
SEARCH METHODS
We searched the Cochrane Central Register of Controlled Trials (CENTRAL) in The Cochrane Library 2015, Issue 9, and MEDLINE, EMBASE, Science Citation Index Expanded, and trials registers until September 2015. We also searched the references of included trials and contacted trial authors.
SELECTION CRITERIA
We only considered randomised controlled trials (RCTs) of people with pancreatic pseudocysts, regardless of size, presence of symptoms, or aetiology. We placed no restrictions on blinding, language, or publication status of the trials.
DATA COLLECTION AND ANALYSIS
Two review authors independently identified trials and extracted data. We calculated the odds ratio (OR) and mean difference (MD) with 95% confidence intervals (CI) with RevMan 5, based on an available-case analysis for direct comparisons, using fixed-effect and random-effect models. We also conducted indirect comparisons (rather than network meta-analysis), since there were no outcomes for which direct and indirect evidence were available.
MAIN RESULTS
We included four RCTs, with 177 participants, in this review. After one participant was excluded, 176 participants were randomised to endoscopic ultrasound (EUS)-guided drainage (88 participants), endoscopic drainage (44 participants), EUS-guided drainage with nasocystic drainage (24 participants), and open surgical drainage (20 participants). The comparisons included endoscopic drainage versus EUS-guided drainage (two trials), EUS-guided drainage with nasocystic drainage versus EUS-guided drainage alone (one trial), and open surgical drainage versus EUS-guided drainage (one trial). The participants were mostly symptomatic, with pancreatic pseudocysts resulting from acute and chronic pancreatitis of varied aetiology. The mean size of the pseudocysts ranged between 70 mm and 155 mm across studies. Although the trials appeared to include similar types of participants for all comparisons, we were unable to assess this statistically, since there were no direct and indirect results for any of the comparisons.All the trials were at unclear or high risk of bias, and the overall quality of evidence was low or very low for all outcomes. One death occurred in the endoscopic drainage group (1/44; 2.3%), due to bleeding. There were no deaths in the other groups. The differences in the serious adverse events were imprecise. Short-term health-related quality of life (HRQoL; four weeks to three months) was worse (MD -21.00; 95% CI -33.21 to -8.79; participants = 40; studies = 1; range: 0 to 100; higher score indicates better) and the costs were higher in the open surgical drainage group than the EUS-guided drainage group (MD 8040 USD; 95% CI 3020 to 13,060; participants = 40; studies = 1). There were fewer adverse events in the EUS-guided drainage with nasocystic drainage group than in the EUS-guided drainage alone (OR 0.20; 95% CI 0.06 to 0.73; participants = 47; studies = 1), or the endoscopic drainage group (indirect comparison: OR 0.08; 95% CI 0.01 to 0.61). Participants with EUS-guided drainage with nasocystic drainage also had shorter hospital stays compared to EUS-guided drainage alone (MD -8.10 days; 95% CI -9.79 to -6.41; participants = 47; studies = 1), endoscopic drainage (indirect comparison: MD -7.10 days; 95% CI -9.38 to -4.82), or open surgical drainage group (indirect comparison: MD -12.30 days; 95% CI -14.48 to -10.12). The open surgical drainage group had longer hospital stays than the EUS-guided drainage group (MD 4.20 days; 95% CI 2.82 to 5.58; participants = 40; studies = 1); the endoscopic drainage group had longer hospital stays than the open drainage group (indirect comparison: -5.20 days; 95% CI -7.26 to -3.14). The need for additional invasive interventions was higher for the endoscopic drainage group than the EUS-guided drainage group (OR 11.13; 95% CI 2.85 to 43.44; participants = 89; studies = 2), and the open drainage group (indirect comparison: OR 23.69; 95% CI 1.40 to 400.71). The differences between groups were imprecise for the other comparisons that could be performed. None of the trials reported long-term mortality, medium-term HRQoL (three months to one year), long-term HRQoL (longer than one year), time-to-return to normal activities, or time-to-return to work.
AUTHORS' CONCLUSIONS
Very low-quality evidence suggested that the differences in mortality and serious adverse events between treatments were imprecise. Low-quality evidence suggested that short-term HRQoL (four weeks to three months) was worse, and the costs were higher in the open surgical drainage group than in the EUS-guided drainage group. Low-quality or very low-quality evidence suggested that EUS-guided drainage with nasocystic drainage led to fewer adverse events than EUS-guided or endoscopic drainage, and shorter hospital stays when compared to EUS-guided drainage, endoscopic drainage, or open surgical drainage, while EUS-guided drainage led to shorter hospital stays than open surgical drainage. Low-quality evidence suggested that there was a higher need for additional invasive procedures with endoscopic drainage than EUS-guided drainage, while it was lower in the open surgical drainage than in the endoscopic drainage group.Further RCTs are needed to compare EUS-guided drainage, with or without nasocystic drainage, in symptomatic patients with pancreatic pseudocysts that require treatment. Future trials should include patient-oriented outcomes such as mortality, serious adverse events, HRQoL, hospital stay, return-to-normal activity, number of work days lost, and the need for additional procedures, for a minimum follow-up period of two to three years.
Topics: Drainage; Humans; Pancreatic Pseudocyst; Pancreatitis; Randomized Controlled Trials as Topic; Ultrasonography, Interventional
PubMed: 27075711
DOI: 10.1002/14651858.CD011392.pub2 -
Journal of Cystic Fibrosis : Official... May 2016Hypoglycaemia in CF in the absence of diabetes or glucose lowering therapies is a phenomenon that is receiving growing attention in the literature. These episodes are... (Review)
Review
BACKGROUND
Hypoglycaemia in CF in the absence of diabetes or glucose lowering therapies is a phenomenon that is receiving growing attention in the literature. These episodes are sometimes symptomatic and likely have variable aetiologies. Our first aim was to conduct a systematic review of the literature to determine what is known about hypoglycaemia in CF. Our second aim was to assess evidence based guidelines for management strategies.
METHODS
A comprehensive search of databases and guideline compiler entities was performed. Inclusion criteria were primary research articles and evidence based guidelines that referred to hypoglycaemia in CF in the absence of insulin treatment or other glucose lowering therapies.
RESULTS
A total of 11 studies (four manuscripts and seven abstracts) and five evidence-based guidelines met the inclusion criteria. Prevalence rates of hypoglycaemia unrelated to diabetes varied between studies (7-69%). Hypoglycaemia was diagnosed during oral glucose tolerance testing or continuous glucose monitoring (CGM). Associations between hypoglycaemia and clinical parameters of BMI, lung function, liver disease and pancreatic insufficiency were measured in some studies. There was no unifying definition of hypoglycaemia in the absence of diabetes. Only two evidence based guidelines reported possible management strategies.
CONCLUSION
The systematic review found limited data on this clinical problem and supports the need for high quality methodological studies that are able to describe the experience and the aetiology(ies) of hypoglycaemia in CF.
Topics: Cystic Fibrosis; Disease Management; Glucose Tolerance Test; Humans; Hypoglycemia; Needs Assessment; Practice Guidelines as Topic; Prevalence
PubMed: 27025865
DOI: 10.1016/j.jcf.2016.02.012 -
World Journal of Gastrointestinal... Mar 2016To perform a systematic review comparing the outcomes of endoscopic, percutaneous and surgical pancreatic pseudocyst drainage.
AIM
To perform a systematic review comparing the outcomes of endoscopic, percutaneous and surgical pancreatic pseudocyst drainage.
METHODS
Comparative studies published between January 1980 and May 2014 were identified on PubMed, Embase and the Cochrane controlled trials register and assessed for suitability of inclusion. The primary outcome was the treatment success rate. Secondary outcomes included were the recurrence rates, re-interventions, length of hospital stay, adverse events and mortalities.
RESULTS
Ten comparative studies were identified and 3 were randomized controlled trials. Four studies reported on the outcomes of percutaneous and surgical drainage. Based on a large-scale national study, surgical drainage appeared to reduce mortality and adverse events rate as compared to the percutaneous approach. Three studies reported on the outcomes of endoscopic ultrasound (EUS) and surgical drainage. Clinical success and adverse events rates appeared to be comparable but the EUS approach reduced hospital stay, cost and improved quality of life. Three other studies compared EUS and esophagogastroduodenoscopy-guided drainage. Both approaches were feasible for pseudocyst drainage but the success rate of the EUS approach was better for non-bulging cyst and the approach conferred additional safety benefits.
CONCLUSION
In patients with unfavorable anatomy, surgical cystojejunostomy or percutaneous drainage could be considered. Large randomized studies with current definitions of pseudocysts and longer-term follow-up are needed to assess the efficacy of the various modalities.
PubMed: 27014427
DOI: 10.4253/wjge.v8.i6.310 -
BioMed Research International 2016Confocal laser endomicroscopy (CLE) is an endoscopic-assisted technique developed to obtain histopathological diagnoses of gastrointestinal and pancreatobiliary diseases... (Meta-Analysis)
Meta-Analysis Review
Confocal laser endomicroscopy (CLE) is an endoscopic-assisted technique developed to obtain histopathological diagnoses of gastrointestinal and pancreatobiliary diseases in real time. The objective of this systematic review is to analyze the current literature on CLE and to evaluate the applicability and diagnostic yield of CLE in patients with gastrointestinal and pancreatobiliary diseases. A literature search was performed on MEDLINE, EMBASE, Scopus, and Cochrane Oral Health Group Specialized Register, using pertinent keywords without time limitations. Both prospective and retrospective clinical studies that evaluated the sensitivity, specificity, or accuracy of CLE were eligible for inclusion. Of 662 articles identified, 102 studies were included in the systematic review. The studies were conducted between 2004 and 2015 in 16 different countries. CLE demonstrated high sensitivity and specificity in the detection of dysplasia in Barrett's esophagus, gastric neoplasms and polyps, colorectal cancers in inflammatory bowel disease, malignant pancreatobiliary strictures, and pancreatic cysts. Although CLE has several promising applications, its use has been limited by its low availability, high cost, and need of specific operator training. Further clinical trials with a particular focus on cost-effectiveness and medicoeconomic analyses, as well as standardized institutional training, are advocated to implement CLE in routine clinical practice.
Topics: Barrett Esophagus; Colorectal Neoplasms; Endoscopy; Gastrointestinal Diseases; Humans; MEDLINE; Microscopy, Confocal; Stomach Neoplasms
PubMed: 26989684
DOI: 10.1155/2016/4638683 -
Gastroenterology Research and Practice 2016Background. Researchers have evaluated various molecular tests for improving the differential diagnosis of cystic lesions of the pancreas. Methods. Six electronic...
Background. Researchers have evaluated various molecular tests for improving the differential diagnosis of cystic lesions of the pancreas. Methods. Six electronic databases were searched for articles on molecular tests for the diagnosis of pancreatic cysts. Measures of accuracy were extracted from selected articles and pooled by the random-effects model. Summary receiver operating characteristic curves were used to analyze the overall accuracy of the molecular tests. Pooled sensitivity and specificity values [95% confidence intervals] are reported. Results. The systematic review included eight studies of 428 patients in total. We determined the sensitivities and specificities of tests for KRAS mutations (0.47 [0.39-0.54], 0.98 [0.93-0.99]) and loss of heterozygosity (0.63 [0.54-0.71], 0.76 [0.63-0.87]) for distinguishing mucinous from nonmucinous cysts, as well as the sensitivities and specificities of tests for KRAS mutations (0.59 [0.46-0.71], 0.78 [0.71-0.85]) and loss of heterozygosity (0.89 [0.78-0.96], 0.69 [0.60-0.76]) for differentiating malignant from benign cysts. Conclusion. Tests of KRAS mutations could confirm but not exclude a diagnosis of a mucinous or malignant pancreatic cyst.
PubMed: 26819604
DOI: 10.1155/2016/3546085 -
Clinical and Translational... Dec 2015Pancreatic intraductal papillary mucinous neoplasias (IPMNs) represent 25% of all cystic neoplasms and are precursor lesions for pancreatic ductal adenocarcinoma. This...
OBJECTIVES
Pancreatic intraductal papillary mucinous neoplasias (IPMNs) represent 25% of all cystic neoplasms and are precursor lesions for pancreatic ductal adenocarcinoma. This study aims to identify the best imaging modality for detecting malignant transformation in IPMN, the sensitivity and specificity of risk features on imaging, and the usefulness of tumor markers in serum and cyst fluid to predict malignancy in IPMN.
METHODS
Databases were searched from November 2006 to March 2014. Pooled sensitivity and specificity of diagnostic techniques/imaging features of suspected malignancy in IPMN using a hierarchical summary receiver operator characteristic (HSROC) approach were performed.
RESULTS
A total of 467 eligible studies were identified, of which 51 studies met the inclusion criteria and 37 of these were incorporated into meta-analyses. The pooled sensitivity and specificity for risk features predictive of malignancy on computed tomography/magnetic resonance imaging were 0.809 and 0.762 respectively, and on positron emission tomography were 0.968 and 0.911. Mural nodule, cyst size, and main pancreatic duct dilation found on imaging had pooled sensitivity for prediction of malignancy of 0.690, 0.682, and 0.614, respectively, and specificity of 0.798, 0.574, and 0.687. Raised serum carbohydrate antigen 19-9 (CA19-9) levels yielded sensitivity of 0.380 and specificity of 0903. Combining parameters yielded a sensitivity of 0.743 and specificity of 0.906.
CONCLUSIONS
PET holds the most promise in identifying malignant transformation within an IPMN. Combining parameters increases sensitivity and specificity; the presence of mural nodule on imaging was the most sensitive whereas raised serum CA19-9 (>37 KU/l) was the most specific feature predictive of malignancy in IPMNs.
PubMed: 26658837
DOI: 10.1038/ctg.2015.60 -
Clinical and Translational... Jun 2015There is significant research interest in developing and validating novel pancreatic cyst-fluid biomarkers given the increasing recognition of the prevalence of...
There is significant research interest in developing and validating novel pancreatic cyst-fluid biomarkers given the increasing recognition of the prevalence of pancreatic cysts and their associated malignant potential. Although current international consensus guidelines are helpful, they fail to diagnose with certainty the cyst type and the level of epithelial dysplasia. They also fall short in predicting the future likelihood of malignant transformation. A systematic review was performed with the objective of summarizing cyst-fluid-based biomarkers that have been published in the medical literature over the past 10 years and characterizing the current quality of evidence. Our review demonstrates that there is an increasing interest in this topic with several different and innovative approaches including DNA, RNA, proteomic, and metabolomics profiling. Further techniques to improve upon cytological yield have also been studied. Besides identifying potentially useful clinical biomarkers, these empiric approaches have provided further insight into their pathogenesis. The level of evidence for the vast majority of these studies, however, is limited to retrospective early validation studies. The path forward will be to select out the most promising biomarkers and develop multicenter consortiums capable of capturing adequate sample sizes with appropriate study designs.
PubMed: 26065716
DOI: 10.1038/ctg.2015.17 -
HPB : the Official Journal of the... May 2015Endoscopic ultrasonography with fine needle aspiration (EUS-FNA) has become an integral tool in the diagnosis of pancreatic cystic lesions (PCLs) and the analysis of... (Review)
Review
BACKGROUND
Endoscopic ultrasonography with fine needle aspiration (EUS-FNA) has become an integral tool in the diagnosis of pancreatic cystic lesions (PCLs) and the analysis of molecular/DNA abnormalities might improve the accuracy of pre-operative diagnosis. A review was conducted of all studies using EUS-FNA aspirates of PCLs to assess the accuracy and added benefit that molecular analysis provides to cytological analysis.
METHODS
A systematic review of the literature was conducted using PRISMA guidelines and electronic databases: PubMed/SCOPUS/EMBASE/Cochrane/CINAHL. Surgical pathology was used as the definitive reference standard. The QUADAS-2 tool was used for quality assessment.
RESULTS
In total, 162 articles were identified; 12 articles met inclusion/exclusion criteria. Ten studies reported on cytology and 8 studies reported k-ras mutational analysis. 362 patients (of 1115 total) had surgical pathology available. The sensitivity and specificity of cytology was 0.42 and 0.99; the sensitivity and specificity of k-ras was 0.39 and 0.95; and the sensitivity and specificity of the combined test of cytology and k-ras was 0.71 and 0.88, respectively.
CONCLUSIONS
k-ras mutational analysis used as an individual screening test has a poor diagnostic accuracy, as does cytology when used alone. The benefit comes with utilization in a combined fashion. More studies are needed to evaluate the correct sequence and utility of these tests for cyst differentiation.
Topics: DNA; DNA Mutational Analysis; Endoscopic Ultrasound-Guided Fine Needle Aspiration; Genes, ras; Humans; Molecular Diagnostic Techniques; Pancreas; Pancreatic Cyst
PubMed: 25428782
DOI: 10.1111/hpb.12364 -
World Journal of Gastroenterology Jul 2014The continued need to develop less invasive alternatives to surgical and radiologic interventions has driven the development of endoscopic ultrasound (EUS)-guided... (Review)
Review
The continued need to develop less invasive alternatives to surgical and radiologic interventions has driven the development of endoscopic ultrasound (EUS)-guided treatments. These include EUS-guided drainage of pancreatic fluid collections, EUS-guided necrosectomy, EUS-guided cholangiography and biliary drainage, EUS-guided pancreatography and pancreatic duct drainage, EUS-guided gallbladder drainage, EUS-guided drainage of abdominal and pelvic fluid collections, EUS-guided celiac plexus block and celiac plexus neurolysis, EUS-guided pancreatic cyst ablation, EUS-guided vascular interventions, EUS-guided delivery of antitumoral agents and EUS-guided fiducial placement and brachytherapy. However these procedures are technically challenging and require expertise in both EUS and interventional endoscopy, such as endoscopic retrograde cholangiopancreatography and gastrointestinal stenting. We undertook a systematic review to record the entire body of literature accumulated over the past 2 decades on EUS-guided interventions with the objective of performing a critical appraisal of published articles, based on the classification of studies according to levels of evidence, in order to assess the scientific progress made in this field.
Topics: Catheter Ablation; Cholangiography; Digestive System Diseases; Digestive System Surgical Procedures; Drainage; Endoscopy, Digestive System; Endosonography; Endovascular Procedures; Ethanol; Evidence-Based Medicine; Humans; Injections; Nerve Block; Patient Selection; Predictive Value of Tests; Treatment Outcome; Ultrasonography, Interventional
PubMed: 25024600
DOI: 10.3748/wjg.v20.i26.8424