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Classic Timothy Syndrome Associated With Bilateral Border Digit Syndactyly: A Case Series

J Hand Surg Am. 2022 Nov 3:S0363-5023(22)00538-X. doi: 10.1016/j.jhsa.2022.09.003. Online ahead of print.

ABSTRACT

PURPOSE: Timothy syndrome (TS) is characterized by congenital long-QT arrhythmia and limb syndactyly. Patients who undergo syndactyly repair with undiagnosed TS may have their abnormal cardiac electrical activity unmasked during surgery. The purpose of this study was to detail the extremity phenotype seen in patients with TS, which may help hand surgeons in their preoperative assessment.

METHODS: This was a retrospective review of all patients with TS seen at our institution from 1998 to 2022. Descriptive statistics regarding their demographics, medical and surgical histories, and syndactyly phenotypes were obtained.

RESULTS: Seven patients (5 males and 2 females) with TS were seen at our institution for multidisciplinary evaluation (median age at presentation was 23 months). Six patients had finger syndactyly and 5 had toe syndactyly. One patient did not have any extremity syndactyly and was noted to have a specific TS mutation known to lack musculoskeletal abnormalities. All patients with finger syndactyly had border digit involvement, with 5 out of 6 patients displaying syndactyly of the middle-ring and ring-little finger web spaces. Toe syndactyly was more heterogeneous, with 1 patient lacking any lower extremity syndactyly and others having variable involvement of the second-third, third-fourth, and fourth-little toe web spaces. Complexity ranged from simple to complete. Four patients had intraoperative cardiac events leading to TS diagnoses after surgery.

CONCLUSIONS: Bilateral border digit hand syndactyly, with or without bilateral toe syndactyly, should raise concerns for TS and prompt further investigation into potential cardiac disease to avoid perioperative cardiac morbidity and mortality.

TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic V.

PMID:36336572 | DOI:10.1016/j.jhsa.2022.09.003

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Electrocardiographic Measures of Repolarization Heterogeneity are not Predictive For Torsades De Pointes Among Undifferentiated Patients with Prolonged QTc: A Case Control Study

J Cardiovasc Electrophysiol. 2022 Nov 6. doi: 10.1111/jce.15735. Online ahead of print.

ABSTRACT

INTRODUCTION: Torsades de Pointes (TdP) is a potentially lethal polymorphic ventricular tachydysrhythmia associated with and caused by prolonged myocardial repolarization. However, prediction of TdP is challenging. We sought to determine if electrocardiographic myocardial repolarization heterogeneity is necessary and predictive of TdP.

METHODS: We performed a case control study of TdP at a large urban hospital. We identified cases based on a hospital center ECG database search for tracings from 1/2005 to 6/2019 with QTc>500, QRS<120, and HR<60, and a subsequent natural language search of electronic health records for the terms: TdP, polymorphic ventricular tachycardia, sudden cardiac death, and relevant variants. Controls were drawn in a 2:1 ratio to cases from a similar pool of ECGs, and matching for QTc, heart rate, sex, and age. We abstracted historical, laboratory, and ECG data using detailed written instructions and an electronic database. We included a second blinded data abstractor to test data abstraction and manual ECG measurement reliability. We used GE QT Guard software for automated repolarization measurements. We compared groups using unpaired statistics.

RESULTS: We included 75 cases and 150 controls. The number of current QTc prolonging medications and serum electrolytes were substantially the same between the two groups. We found no significant difference in measures of QT or T wave repolarization heterogeneity.

CONCLUSION: Electrocardiographic repolarization heterogeneity is not greater in otherwise unselected patients with QTc prolongation who suffer TdP and does not appear predictive of TdP. However, previous observations suggest specific repolarization characteristics may be useful for defined patient subgroups at risk for TdP. This article is protected by copyright. All rights reserved.

PMID:36335640 | DOI:10.1111/jce.15735

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Validation of the Spanish Acute Cystitis Symptoms Score (ACSS) in native Spanish-speaking women of Europe and Latin America

Neurourol Urodyn. 2022 Nov 6. doi: 10.1002/nau.25079. Online ahead of print.

ABSTRACT

INTRODUCTION AND OBJECTIVES: The Acute Cystitis Symptom Score (ACSS) is a patient self-reporting questionnaire for clinical diagnostics and patient-reported outcome (PRO), which may assess the symptoms and the effect on the quality of life in women with acute cystitis (AC). The current study aimed to create a validated Spanish version of the ACSS questionnaire.

MATERIAL AND METHODS: The process of linguistic validation of the Spanish version of the ACSS consisted of the independent forward and backward translations, revision and reconciliation, and cognitive assessment. Clinical evaluation of the study version of the ACSS was carried out in clinics in Spain and Latin America. Statistical tests included the calculation of Cronbach’s α, split-half reliability, specificity, sensitivity, diagnostic odds ratio, positive and negative likelihood ratio, and area under the receiver-operating characteristic curve (AUC).

RESULTS: The study was performed on 132 patients [age (mean;SD) 45.0;17.8 years] with AC and 55 controls (44.5;12.2 years). Cronbach’s α of the ACSS was 0.86, and the split-half reliability was 0.82. The summary scores of the ACSS domains were significantly higher in patients than in controls, 16.0 and 2.0 (p < 0.001), respectively. The predefined cut-off point of ≥6 for a summary score of the “Typical” domain resulted in a specificity of 83.6% and a sensitivity of 99.2% for the Spanish version of the ACSS. AUC was 0.91 [0.85; 0.97].

CONCLUSIONS: The validated Spanish ACSS questionnaire evaluates the symptoms and clinical outcomes of patients with AC. It can be used as a patient’s self-diagnosis of AC, as a PRO measure tool, and help to rule out other pathologies in patients with voiding syndrome.

PMID:36335613 | DOI:10.1002/nau.25079

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Mitral valve surgery via repeat median sternotomy versus right mini-thoracotomy: A systematic review and meta-analysis of clinical outcomes

J Card Surg. 2022 Nov 6. doi: 10.1111/jocs.17101. Online ahead of print.

ABSTRACT

BACKGROUND: Redo mitral valve surgeries have high mortality and morbidity and can be physically demanding for patients. Median sternotomy remains the gold standard for most cardiac surgeries. To tackle certain risks with a re-sternotomy, alternative procedures such as the right anterolateral minithoracotomy have been explored. This review aims to compare the clinical outcomes of re-sternotomy (MS) versus right mini thoracotomy (MT) in mitral valve surgery.

METHODS: A systematic, electronic search was performed according to Preferred Reporting items for Systematic Reviews and Meta-analysis guidelines to identify relevant articles that compared outcomes of the MS versus MT procedures in patients who have had cardiac surgery via a MS approach.

RESULTS: Twelve studies were identified, enrolling 4514 patients. Length of hospital stay(MD = -3.71, 95% confidence interval [CI] [-4.92, -2.49]), 30-day mortality(odds ratio [OR] = 0.59, 95% CI [0.39, 0.90]), and new-onset renal failure(OR = 0.38, 95% CI [0.22, 0.65]) were statistically significant in favor of the MT approach. Infection rates(OR = 0.56, 95% CI[0.25, 1.21]) and length of intensive care unit (ICU) stay (MD = -0.55, 95% CI[-1.16, 0.06]) was lower in the MT group; however, the difference was not significant. No significant differences were observed in the CPB time(MD = -2.33, 95% CI [-8.15, 3.50]), aortic cross-clamp time MD = -1.67, 95% CI[-17.07, 13.76]), and rates of stroke(OR = 1.03, 95% CI[0.55, 1.92]).

CONCLUSION: Right MT is a safe alternative to the traditional re-sternotomy for patients who have had previous cardiac surgery. The approach offers a reduced length of hospital stay, ICU stay, and a lower risk of new-onset renal failure requiring dialysis. This review calls for robust trials in the field to further strengthen the evidence.

PMID:36335611 | DOI:10.1111/jocs.17101

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The efficacy of nitroglycerin to prevent radial artery spasm and occlusion during and after transradial catheterization: A systematic review and meta-analysis of randomized controlled trials

Clin Cardiol. 2022 Nov 6. doi: 10.1002/clc.23906. Online ahead of print.

ABSTRACT

Radial artery spasm (RAS) is the most common cause of transradial access site crossover and is a common intra-procedural complication. RAS incidence can lead to radial artery occlusion (RAO) postprocedure, preventing the radial artery as a future access site. We evaluated the efficacy of nitroglycerin preventing RAS and RAO during transradial catheterization discussing the different routes of administration, including topical, subcutaneous, and intra-arterial. A systematic review and meta-analysis included all relevant articles until April 23, 2022. We searched six databases Google Scholar, Web of Science, SCOPUS, EMBASE, PubMed (MEDLINE), and CENTRAL. We registered our review protocol in PROSPERO with ID: CRD42022330356. We included 11 trials with 5814 patients. Compared to placebo, the pooled analysis favored subcutaneous nitroglycerin in preventing RAS (risk ratio [RR]: 0.57 with 95% confidence interval [CI] [0.43-0.77], p = .0003) and RAO (RR: 0.39 with 95% CI [0.16-0.98], p = .05). In contrast to the intra-arterial nitroglycerin that showed nonstatistically significant results in preventing RAS and RAO (RR: 0.8 with 95% CI [0.63-1.02], p = .07)- (RR: 0.78 with 95% CI [0.6-1.01], p = .06)), respectively. Also, topical nitroglycerin did not prevent RAS (RR: 0.73 with 95% CI [0.42-1.24], p = .24). Compared with placebo, subcutaneous nitroglycerin during transradial catheterization reduced the incidence of RAS and RAO. Meanwhile, Intra-arterial and topical nitroglycerin did not show statistically significant outcomes. Subcutaneous nitroglycerin may be a practical and cost-effective technique to facilitate transradial catheterization; however, more RCTs are needed to evaluate the subcutaneous versus intra-arterial nitroglycerin administration.

PMID:36335609 | DOI:10.1002/clc.23906

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Peripheral venous catheter-related phlebitis: A meta-analysis of topical treatment

Nurs Open. 2022 Nov 6. doi: 10.1002/nop2.1449. Online ahead of print.

ABSTRACT

AIM: To systematically evaluate the efficacy of different topical treatments for PVC-related phlebitis in hospital in-patients.

DESIGN: A systematic review and meta-analysis.

METHODS: A selection was made of experimental and quasi-experimental studies published in English or Spanish. These should provide data on the degree of phlebitis, pain and infiltration (means and standard deviations, mainly) of hospitalized patients with phlebitis secondary to peripheral venous catheter. All those studies that reflected systemic or exclusive prevention treatments were excluded. Searches were from inception to April 2020. The date of data collection was from December 2020 to May 2021. The selection criteria were based on the PICOS model. Risk of bias was assessed using the Cochrane Collaboration tool.

RESULTS: Twelve studies (726 patients) met the inclusion criteria. With respect to the decrease in the degree of phlebitis, was found ichthammol glycerine, followed by heparinoids. As for degree of pain, sesame oil obtained the most marked reduction. In terms of degree of infiltration, heparinoids and ichthammol glycerine were the only products to achieve a statistically significant reduction. The most important limitations are the low quantity and quality of the trials included. Insufficient data are available to draw valid conclusions about the efficacy of any treatment.

PMID:36335576 | DOI:10.1002/nop2.1449

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How well do covariates perform when adjusting for sampling bias in online COVID-19 research? Insights from multiverse analyses

Eur J Epidemiol. 2022 Nov 6. doi: 10.1007/s10654-022-00932-y. Online ahead of print.

ABSTRACT

COVID-19 research has relied heavily on convenience-based samples, which-though often necessary-are susceptible to important sampling biases. We begin with a theoretical overview and introduction to the dynamics that underlie sampling bias. We then empirically examine sampling bias in online COVID-19 surveys and evaluate the degree to which common statistical adjustments for demographic covariates successfully attenuate such bias. This registered study analysed responses to identical questions from three convenience and three largely representative samples (total N = 13,731) collected online in Canada within the International COVID-19 Awareness and Responses Evaluation Study ( www.icarestudy.com ). We compared samples on 11 behavioural and psychological outcomes (e.g., adherence to COVID-19 prevention measures, vaccine intentions) across three time points and employed multiverse-style analyses to examine how 512 combinations of demographic covariates (e.g., sex, age, education, income, ethnicity) impacted sampling discrepancies on these outcomes. Significant discrepancies emerged between samples on 73% of outcomes. Participants in the convenience samples held more positive thoughts towards and engaged in more COVID-19 prevention behaviours. Covariates attenuated sampling differences in only 55% of cases and increased differences in 45%. No covariate performed reliably well. Our results suggest that online convenience samples may display more positive dispositions towards COVID-19 prevention behaviours being studied than would samples drawn using more representative means. Adjusting results for demographic covariates frequently increased rather than decreased bias, suggesting that researchers should be cautious when interpreting adjusted findings. Using multiverse-style analyses as extended sensitivity analyses is recommended.

PMID:36335560 | DOI:10.1007/s10654-022-00932-y

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Intake of prebiotic fibers and the risk of laryngeal cancer: the PrebiotiCa study

Eur J Nutr. 2022 Nov 6. doi: 10.1007/s00394-022-03030-7. Online ahead of print.

ABSTRACT

PURPOSE: To evaluate whether the intake of specific fibers with prebiotic activity, e.g., inulin-type fructans (ITFs), fructo-oligosaccharides (FOSs), and galacto-oligosaccharides (GOSs), is associated with laryngeal cancer risk.

METHODS: Within the PrebiotiCa study, we used data from a case-control study (Italy, 1992-2009) with 689 incident, histologically confirmed laryngeal cancer cases and 1605 controls. Six prebiotic molecules (ITFs, nystose [FOS], kestose [FOS], 1F-β-fructofuranosylnystose [FOS], raffinose [GOS] and stachyose [GOS]) were quantified in various foods via ad hoc conducted laboratory analyses. Subjects’ prebiotic fiber intake was calculated by multiplying food frequency questionnaire intake by the prebiotic content of each food item. The odds ratios (OR) of laryngeal cancer for prebiotic fiber intake were calculated using logistic regression models, including, among others, terms for tobacco, alcohol, and total energy intake.

RESULTS: The intakes of kestose, raffinose and stachyose were inversely associated with laryngeal cancer, with ORs for the highest versus the lowest quartile of 0.70 (95% confidence interval, CI 0.50-0.99) for kestose, 0.65 (95% CI 0.45-0.93) for raffinose and 0.61 (95% CI 0.45-0.83) for stachyose. ITFs, nystose and 1F-β-fructofuranosylnystose were not associated with laryngeal cancer risk. Current smokers and heavy drinkers with medium-low intakes of such prebiotic fibers had, respectively, an over 15-fold increased risk versus never smokers with medium-high intakes and a five to sevenfold increased risk versus never/moderate drinkers with medium-high intakes.

CONCLUSION: Although disentangling the effects of the various components of fiber-rich foods is complex, our results support a favorable role of selected prebiotic fibers on laryngeal cancers risk.

PMID:36335543 | DOI:10.1007/s00394-022-03030-7

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Gigantism: microsurgical treatment by transsphenoidal approach and prognostic factors

Pituitary. 2022 Nov 6. doi: 10.1007/s11102-022-01286-0. Online ahead of print.

ABSTRACT

PURPOSE: We present the results of transsphenoidal microsurgical treatment in 14 patients with gigantism. The influence on the prognosis of factors such as the tumor size and preoperative levels of GH and IGF-1 is also quantified.

MATERIALS AND METHODS: The patients, operated between 1982 and 2004, were reviewed retrospectively in June 2022. All patients had complete endocrinological studies in the preoperative period and a postoperative control between 6 days and 3 weeks. Follow-up has been supported with annual check-ups between 3 and 31 years. We have compared the preoperative levels of GH and IGF-1 of these patients with the levels of a series of acromegalic patients operated on in the same Center.

RESULTS: In this series there were 4 women and 10 men. The age ranged between 14 and 21 years. In 6 patients, postoperative hormone levels achieved the disease control criteria (42.8%). The CT/MRI studies revealed the existence of invasive tumors in 10 of the patients (71.4%). Postoperative CT/MRI showed no tumor tissue in 3 patients but in 7 patients there were tumor remains. The remaining 4 patients had abnormal images although not considered as tumor. A statistical comparison of preoperative serum GH and IGF-1 levels in patients with gigantism and patients with acromegaly showed a significant elevation in the former.

CONCLUSION: Pituitary adenomas that cause gigantism are generally large and invasive, which makes them difficult to cure. High preoperative levels of GH and IGF-1 are also factors that decrease remission.

PMID:36335516 | DOI:10.1007/s11102-022-01286-0

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Long-Term Vault Changes in Different Levels and Factors Affecting Vault Change After Implantation of Implantable Collamer Lens with a Central Hole

Ophthalmol Ther. 2022 Nov 6. doi: 10.1007/s40123-022-00606-8. Online ahead of print.

ABSTRACT

INTRODUCTION: This study evaluated vault changes in eyes implanted with an implantable Collamer lens (ICL) with a central hole (ICL V4c) for myopia and astigmatism correction as well as factors related to vault changes over time.

METHODS: This retrospective study enrolled 169 myopic eyes from 169 patients (137 women and 32 men) who underwent ICL V4c implantation to correct myopia and astigmatism. Vault values were measured quantitatively using a rotating Scheimpflug camera. Each patient underwent at least four postoperative follow-up visits at 1 month, 6 months, 1 year, and more than 2 years. We compared postoperative values between groups and identified factors affecting vault changes over time.

RESULTS: The mean vaults at 1 month (baseline), 6 months, 1 year, and the last follow-up time following ICL implantation were 540.83 ± 186.13, 520.00 ± 196.08, 503.79 ± 198.30, and 471.42 ± 211.35 μm, respectively. Eyes with baseline vaults of 250-750 and ≥ 750 μm exhibited a trend of vault decrease over time, and the differences were statistically significant (P < 0.05) at 6 months and 1 year postoperatively, respectively. The variables relevant to the value of vault decrease between baseline and last follow-up time were anterior chamber depth (ACD) and ICL power (adjusted R2 = 0.121, P < 0.001).

CONCLUSIONS: We found a trend of decreased mean objective vault values over time, which was more obvious in eyes with higher baseline vault values. The vault value of the ICL decreased more in eyes with shallower ACDs, higher ICL power, or both. Moreover, the decrease in vault values became statistically significant earlier in eyes with higher baseline vaults or shallower ACDs.

PMID:36335511 | DOI:10.1007/s40123-022-00606-8