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Hellenic Journal of Cardiology : HJC =... 2023Duchenne muscular dystrophy is a fatal X-linked recessive disease affecting approximately 1 in 3500 births. It is characterized by a genetic lack of dystrophin, which is... (Review)
Review
Duchenne muscular dystrophy is a fatal X-linked recessive disease affecting approximately 1 in 3500 births. It is characterized by a genetic lack of dystrophin, which is an essential protein for maintaining muscle integrity. The lack of dystrophin plays a pathophysiological role in the development of dilated cardiomyopathy in Duchenne muscular dystrophy. Currently, no consensus exists on specific pharmacological therapy guidelines for these patients; however, it centers around the guidelines for heart failure management. This systematic review investigated 12 randomized control trials dating back to 2005 in the pharmacotherapy of patients with dilated cardiomyopathy Duchenne muscular dystrophy. This review specifically included angiotensin-converting enzyme inhibitors, aldosterone receptor blockers, angiotensin receptor/neprilysin inhibitors, beta-blockers, and mineralocorticoid receptor antagonists. Despite their limitations, these studies have shown promising effects in improving the overall heart function and prognosis in patients with this condition. However, to attain higher statistical significance, future studies should investigate larger populations and for longer periods.
Topics: Humans; Cardiomyopathy, Dilated; Muscular Dystrophy, Duchenne; Dystrophin; Angiotensin-Converting Enzyme Inhibitors; Adrenergic beta-Antagonists
PubMed: 37406964
DOI: 10.1016/j.hjc.2023.06.007 -
Sexual Medicine Reviews Oct 2022Vaginal stenosis is a distressing side effect of radiation therapy that can impair quality of life. Dilator therapy is an option for patients undergoing pelvic... (Review)
Review
INTRODUCTION
Vaginal stenosis is a distressing side effect of radiation therapy that can impair quality of life. Dilator therapy is an option for patients undergoing pelvic radiotherapy to mitigate vaginal stenosis. Currently, the dilators given to patients by most hospitals are made of plastic, compared to silicone dilators which are available on the market for purchase.
OBJECTIVES
We conducted a systematic literature review to find information to guide clinical recommendations to pelvic radiotherapy patients on potential differences regarding the use of plastic vs silicone dilators with regard to efficacy, cost, and patient preferences.
METHODS
A systematic literature review was conducted in Embase, MEDLINE, and PubMed using Emtree terms. To be included in the review, papers needed to: focus on female patients undergoing radiation therapy, assess a vaginal dilator, measure any dilator intervention outcome, and specifically compare plastic vs silicone dilators for any measured outcome (either qualitative or quantitative).
RESULTS
The initial search yielded 195 articles. Two area experts, with a third expert for arbitration, read each article and found that none met all review inclusion criteria. No studies were found that compared silicone to plastic dilators with regard to efficacy in treating vaginal stenosis due to radiation therapy, no studies were found that compared cost or cost-effectiveness of the 2 dilator types, and no studies were found comparing patient preferences or experiences (eg, comfort, adherence, ease of use) between the 2 dilator types.
CONCLUSION
The materials used to create dilators have never been rigorously compared in the context of radiotherapy-related vaginal stenosis. Institutions and patients have no data to guide their choice. Significantly more research at the patient and institutional level is needed to explore the potential long-term quality of life and cost benefits of improved adherence with silicone dilator use, and to guide shared decision-making regarding dilator choice. Morgan O, Lopez MD, Martinez AJC, et al. Systematic Review of Comparisons Between Plastic and Silicone Dilators: Revealing a Knowledge Gap. Sex Med Rev 2022;10:513-519.
Topics: Constriction, Pathologic; Female; Humans; Plastics; Quality of Life; Silicones; Vagina
PubMed: 36030181
DOI: 10.1016/j.sxmr.2022.06.008 -
Obstetrics and Gynecology Jun 2021To assess the comparative effectiveness and potential harms of cervical ripening in the outpatient compared with the inpatient setting, or different methods of ripening... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
To assess the comparative effectiveness and potential harms of cervical ripening in the outpatient compared with the inpatient setting, or different methods of ripening in the outpatient setting alone.
DATA SOURCES
Searches for articles in English included MEDLINE, EMBASE, CINAHL, Cochrane Library, ClinicalTrials.gov, and reference lists (up to August 2020).
METHODS OF STUDY SELECTION
Using predefined criteria and DistillerSR software, 10,853 citations were dual-reviewed for randomized controlled trials (RCTs) and cohort studies of outpatient cervical ripening using prostaglandins and mechanical methods in pregnant women at or beyond 37 weeks of gestation.
TABULATION, INTEGRATION, AND RESULTS
Using prespecified criteria, study data abstraction and risk of bias assessment were conducted by two reviewers, random-effects meta-analyses were conducted and strength of evidence was assessed. We included 30 RCTs and 10 cohort studies (N=9,618) most generalizable to women aged 25-30 years with low-risk pregnancies. All findings were low or insufficient strength of evidence and not statistically significant. Incidence of cesarean delivery was not different for any comparison of inpatient and outpatient settings, or comparisons of different methods in the outpatient setting (most evidence available for single-balloon catheters and dinoprostone). Harms were inconsistently reported or inadequately defined. Differences were not found for neonatal infection (eg, sepsis) with outpatient compared with inpatient dinoprostone, birth trauma (eg, cephalohematoma) with outpatient compared with inpatient single-balloon catheter, shoulder dystocia with outpatient dinoprostone compared with placebo, maternal infection (eg, chorioamnionitis) with outpatient compared with inpatient single-balloon catheters or outpatient prostaglandins compared with placebo, and postpartum hemorrhage with outpatient catheter compared with inpatient dinoprostone. Evidence on misoprostol, hygroscopic dilators, and other outcomes (eg, perinatal mortality and time to vaginal birth) was insufficient.
CONCLUSION
In women with low-risk pregnancies, outpatient cervical ripening with dinoprostone or single-balloon catheters did not increase cesarean deliveries. Although there were no clear differences in harms when comparing outpatient with inpatient cervical ripening, the certainty of evidence is low or insufficient to draw definitive conclusions.
SYSTEMATIC REVIEW REGISTRATION
PROSPERO, CRD42020167406.
Topics: Ambulatory Care; Catheters; Cervical Ripening; Cesarean Section; Dilatation; Dinoprostone; Female; Hospitalization; Humans; Labor, Induced; Obstetric Labor Complications; Oxytocics; Pregnancy
PubMed: 33752219
DOI: 10.1097/AOG.0000000000004382 -
Pediatric Rheumatology Online Journal Mar 2023The relationship between anti-SSA/RO antibodies and pregnancy has been reported previously, and we aim to visualize the rates of maternal and infant outcomes with... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
The relationship between anti-SSA/RO antibodies and pregnancy has been reported previously, and we aim to visualize the rates of maternal and infant outcomes with anti-SSA/RO.
METHODS
We systematically searched records from Pubmed, Cochrane, Embase, and Web of Science databases, pooled incidence rates of adverse outcomes of pregnancy, and 95% confidence intervals (CIs) were performed with RStudio.
RESULTS
A total of 890 records comprising 1675 patients and 1920 pregnancies were searched from the electronic databases. For maternal outcomes, the pooled estimate rates were 4% for termination of pregnancy, 5% for spontaneous abortion, 26% for preterm labor, and 50% for cesarean operation. While for fetal outcomes, the pooled estimate rates were 4% for perinatal death, 3% for intrauterine growth retardation, 6% for endocardial fibroelastosis, 6% for dilated cardiomyopathy, 7% for congenital heart block, 12% for congenital heart block recurrence, 19% for cutaneous neonatal lupus erythematosus, 12% for hepatobiliary disease and 16% for hematological manifestations. A subgroup analysis of congenital heart block prevalence was performed, diagnostic method and study region were found to affect heterogeneity to some extent.
CONCLUSION
Cumulative analysis of data from real-world studies confirmed adverse pregnancy outcomes of women with anti-SSA/RO, serves as a reference and a guide for the diagnosis and subsequent treatment of these women, thereby enhancing maternal and infant health. Additional studies with real-world cohorts are required to validate these results.
Topics: Pregnancy; Infant, Newborn; Humans; Female; Infant; Abortion, Spontaneous; Antibodies, Antinuclear; Cardiomyopathy, Dilated; Lupus Erythematosus, Cutaneous
PubMed: 36870970
DOI: 10.1186/s12969-023-00803-0 -
Journal of Cardiovascular Development... Apr 2022Traditional definitions of Ebstein's anomaly (EA) and left ventricular noncompaction (LVNC), two rare congenital heart defects (CHDs), confine disease to either the... (Review)
Review
Traditional definitions of Ebstein's anomaly (EA) and left ventricular noncompaction (LVNC), two rare congenital heart defects (CHDs), confine disease to either the right or left heart, respectively. Around 15-29% of patients with EA, which has a prevalence of 1 in 20,000 live births, commonly manifest with LVNC. While individual EA or LVNC literature is extensive, relatively little discussion is devoted to the joint appearance of EA and LVNC (EA/LVNC), which poses a higher risk of poor clinical outcomes. We queried PubMed, Medline, and Web of Science for all peer-reviewed publications from inception to February 2022 that discuss EA/LVNC and found 58 unique articles written in English. Here, we summarize and extrapolate commonalities in clinical and genetic understanding of EA/LVNC to date. We additionally postulate involvement of shared developmental pathways that may lead to this combined disease. Anatomical variation in EA/LVNC encompasses characteristics of both CHDs, including tricuspid valve displacement, right heart dilatation, and left ventricular trabeculation, and dictates clinical presentation in both age and severity. Disease treatment is non-specific, ranging from symptomatic management to invasive surgery. Apart from a few variant associations, mainly in sarcomeric genes and , the genetic etiology and pathogenesis of EA/LVNC remain largely unknown.
PubMed: 35448091
DOI: 10.3390/jcdd9040115 -
Surgical Endoscopy May 2021Achalasia is a rare, chronic, and morbid condition with evolving treatment. Peroral endoscopic myotomy (POEM) has gained considerable popularity, but its comparative... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Achalasia is a rare, chronic, and morbid condition with evolving treatment. Peroral endoscopic myotomy (POEM) has gained considerable popularity, but its comparative effectiveness is uncertain. We aim to evaluate the literature comparing POEM to Heller myotomy (HM) and pneumatic dilation (PD) for the treatment of achalasia.
METHODS
We conducted a systematic review of comparative studies between POEM and HM or PD. A priori outcomes pertained to efficacy, perioperative metrics, and safety. Internal validity of observational studies and randomized trials (RCTs) was judged using the Newcastle Ottawa Scale and the Cochrane Risk of Bias 2.0 tool, respectively.
RESULTS
From 1379 unique literature citations, we included 28 studies comparing POEM and HM (n = 21) or PD (n = 8), with only 1 RCT addressing each. Aside from two 4-year observational studies, POEM follow-up averaged ≤ 2 years. While POEM had similar efficacy to HM, POEM treated dysphagia better than PD both in an RCT (treatment "success" RR 1.71, 95% CI 1.34-2.17; 126 patients) and in observational studies (Eckardt score MD - 0.43, 95% CI - 0.71 to - 0.16; 5 studies; I 21%; 405 patients). POEM needed reintervention less than PD in an RCT (RR 0.19, 95% CI 0.08-0.47; 126 patients) and HM in an observational study (RR 0.33, 95% CI 0.16, 0.68; 98 patients). Though 6-12 months patient-reported reflux was worse than PD in 3 observational studies (RR 2.67, 95% CI 1.02-7.00; I 0%; 164 patients), post-intervention reflux was inconsistently measured and not statistically different in measures ≥ 1 year. POEM had similar safety outcomes to both HM and PD, including treatment-related serious adverse events.
CONCLUSIONS
POEM has similar outcomes to HM and greater efficacy than PD. Reflux remains a critical outcome with unknown long-term clinical significance due to insufficient data and inconsistent reporting.
Topics: Deglutition Disorders; Dilatation; Esophageal Achalasia; Esophageal Sphincter, Lower; Esophagitis, Peptic; Gastroesophageal Reflux; Heller Myotomy; Humans; Laparoscopy; Natural Orifice Endoscopic Surgery; Observational Studies as Topic; Postoperative Complications; Treatment Outcome
PubMed: 33655443
DOI: 10.1007/s00464-021-08353-w -
European Journal of Clinical... Nov 2022Accumulating evidence suggests that endothelial dysfunction is implicated in the pathogenesis and severity of coronavirus disease 2019 (COVID-19). In this context,... (Review)
Review
BACKGROUND
Accumulating evidence suggests that endothelial dysfunction is implicated in the pathogenesis and severity of coronavirus disease 2019 (COVID-19). In this context, vascular impairment in COVID-19 might be associated with clinical manifestations and could refine risk stratification in these patients.
METHODS
This systematic review aims to synthesize current evidence on the frequency and the prognostic value of vascular dysfunction during acute and post-recovery COVID-19. After systematically searching the MEDLINE, clinicaltrials.gov and the Cochrane Library from 1 December 2019 until 05 March 2022, we identified 24 eligible studies with laboratory confirmed COVID-19 and a thorough examination of vascular function. Flow-mediated dilation (FMD) was assessed in 5 and 12 studies in acute and post-recovery phase respectively; pulse wave velocity (PWV) was the marker of interest in three studies in the acute and four studies in the post-recovery phase.
RESULTS
All studies except for one in the acute and in the post-recovery phase showed positive association between vascular dysfunction and COVID-19 infection. Endothelial dysfunction in two studies and increased arterial stiffness in three studies were related to inferior survival in COVID-19.
DISCUSSION
Overall, a detrimental effect of COVID-19 on markers of endothelial function and arterial stiffness that could persist even for months after the resolution of the infection and provide prognostic value was congruent across published studies. Further research is warranted to elucidate clinical implications of this association.
Topics: Brachial Artery; COVID-19; Endothelium; Endothelium, Vascular; Humans; Pulse Wave Analysis; Vascular Stiffness
PubMed: 35986716
DOI: 10.1111/eci.13859 -
Neuroscience and Biobehavioral Reviews Nov 2021The use of pupillometry to track emotional learning processes in humans is generating an increasing interest. Here, we provide a first systematic review and... (Meta-Analysis)
Meta-Analysis
The use of pupillometry to track emotional learning processes in humans is generating an increasing interest. Here, we provide a first systematic review and meta-analysis on the value of pupil dilation as a marker of Pavlovian conditioning, focusing on the roles of UCS valence (aversive vs. appetitive), the time course across trials and response intervals within trials. Based on data from 39 independent samples (total n = 1303), our results revealed strong evidence for the overall validity of conditioned pupil responses, with a trend for larger effects in aversive (average g = 0.73) vs. appetitive conditioning (g = 0.39). Response differentiation increased over the course of acquisition. Substantial differentiation effects were found in both early and late response windows. Moderator analyses revealed a consistent influence of UCS modality on differential conditioning, while evidence for moderation by contingency instructions and length of acquisition phase was mixed. The results highlight pupil dilation as a sensitive and reliable index of Pavlovian conditioning across valence categories and stimulus modalities. Important implications regarding methodological considerations and research goals are discussed.
Topics: Conditioning, Classical; Humans; Pupil
PubMed: 34499928
DOI: 10.1016/j.neubiorev.2021.09.005 -
World Journal of Gastrointestinal... Jul 2023Candy cane syndrome (CCS) is a condition that occurs following gastrectomy or gastric bypass. CCS remains underrecognized, yet its prevalence is likely to rise due to...
BACKGROUND
Candy cane syndrome (CCS) is a condition that occurs following gastrectomy or gastric bypass. CCS remains underrecognized, yet its prevalence is likely to rise due to the obesity epidemic and increased use of bariatric surgery. No previous literature review on this subject has been published.
AIM
To collate the current knowledge on CCS.
METHODS
A literature search was conducted with PubMed and Google Scholar for studies from May 2007, until March 2023. The bibliographies of the retrieved articles were manually searched for additional relevant articles.
RESULTS
Twenty-one articles were identified (135 patients). Abdominal pain, nausea/vomiting, and reflux were the most reported symptoms. Upper gastrointestinal (GI) series and endoscopy were performed for diagnosis. Surgical resection of the blind limb was performed in 13 studies with resolution of symptoms in 73%-100%. In surgical series, 9 complications were reported with no mortality. One study reported the surgical construction of a jejunal pouch with clinical success. Six studies described endoscopic approaches with 100% clinical success and no complications. In one case report, endoscopic dilation did not improve the patient's symptoms.
CONCLUSION
CCS remains underrecognized due to lack of knowledge about this condition. The growth of the obesity epidemic worldwide and the increase in bariatric surgery are likely to increase its prevalence. CCS can be prevented if an elongated blind loop is avoided or if a jejunal pouch is constructed after total gastrectomy. Diagnosis should be based on symptoms, endoscopy, and upper GI series. Blind loop resection is curative but complex and associated with significant complications. Endoscopic management using different approaches to divert flow is effective and should be further explored.
PubMed: 37547243
DOI: 10.4253/wjge.v15.i7.510 -
Scandinavian Journal of Gastroenterology Dec 2021Empiric esophageal dilation is frequently performed for non-obstructive dysphagia. Studies evaluating its efficacy have reported conflicting results. In this... (Meta-Analysis)
Meta-Analysis
BACKGROUND AND AIMS
Empiric esophageal dilation is frequently performed for non-obstructive dysphagia. Studies evaluating its efficacy have reported conflicting results. In this meta-analysis, we have evaluated the efficacy of esophageal dilation in the management of non-obstructive dysphagia.
METHODS
We reviewed several databases from inception to 26 May 2021 to identify randomized controlled trials (RCTs) and observational studies that evaluated the role of empiric esophageal dilation for non-obstructive dysphagia. Our outcomes of interest were clinical success (improvement in dysphagia after dilation) and difference in post-operative dysphagia score between groups. For categorical variables, we calculated pooled odds ratios (OR) with 95% confidence intervals (CI); for continuous variables, we calculated standardized mean difference (SMD) with 95% CI. Data were analyzed using a random effects model. We used GRADE framework to ascertain the quality of evidence.
RESULTS
We included 4 studies (3 RCTs and one observational) with 243 patients; there were 133 treated with empiric dilation and 110 controls. We found no significant difference in clinical success (OR (95% CI) 1.91 (0.89, 4.08)) or post-procedure dysphagia score between groups (SMD (95% CI) 0.38 (-0.37, 1.14)). Our findings remained consistent on subgroup analysis including RCTs only. Quality of evidence ranged from low to very low based on GRADE framework.
CONCLUSIONS
Our meta-analysis does not support the use of empiric esophageal dilation in patients with non-obstructive dysphagia. More studies are required to confirm these findings.
Topics: Deglutition Disorders; Dilatation; Endoscopy; Exercise Therapy; Humans
PubMed: 34477033
DOI: 10.1080/00365521.2021.1971288