Publications by authors named "Squires R"

Article Synopsis
  • Progressive familial intrahepatic cholestasis (PFIC) is an autosomal recessive disorder mainly caused by BSEP deficiency, leading to issues like bile formation disruption and severe itching (pruritus).!* -
  • The MARCH-PFIC study was a phase 3, double-blind, placebo-controlled trial involving participants aged 1-17 from 29 centers across 16 countries, focusing on the effects of the drug maralixibat on different types of PFIC.!* -
  • The study aimed to measure changes in pruritus severity and serum bile acid levels over 26 weeks, specifically looking at outcomes in cohorts with varying forms of PFIC, comparing those treated with maralixibat
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Substantial evidence supports that delay of surgery after breast cancer diagnosis is associated with increased mortality risk, leading to the introduction of a new Commission on Cancer quality measure for receipt of surgery within 60 days of diagnosis for non-neoadjuvant patients. Breast cancer subtype is a critical prognostic factor and determines treatment options; however, it remains unknown whether surgical delay-associated breast cancer-specific mortality (BCSM) risk differs by subtype. This retrospective cohort study aimed to assess whether the impact of delayed surgery on survival varies by subtype (hormone [HR]+/HER2-, HR-/HER2-, and HER2+) in patients with loco-regional breast cancer who received surgery as their first treatment between 2010-2017 using the SEER-Medicare.

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Article Synopsis
  • Researchers aimed to create and validate a prediction tool for pediatric acute liver failure (PALF) mortality, focusing on its rapidly changing clinical course.* -
  • They analyzed data from 1,144 PALF patients over 15 years and developed a dynamic prediction model using joint modeling of multiple clinical and laboratory variables.* -
  • The model showed improved accuracy in predicting 7-day and 21-day mortality when using updated information from the first few days of treatment, demonstrating the importance of continuous disease monitoring.*
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Introduction: This study aimed to investigate the association between cardiorespiratory fitness (CRF) and perioperative morbidity and long-term mortality in operable patients with early-stage non-small cell lung cancer (NSCLC).

Patients And Methods: This prospective study included consecutive patients with early-stage NSCLC who underwent presurgical cardiopulmonary exercise testing between November 2014 and December 2019 (registration number: ChiCTR2100048120). Logistic and Cox proportional hazards regression were applied to evaluate the correlation between CRF and perioperative complications and long-term mortality, respectively.

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Background: Our objective was to better understand the natural history and disease modifiers of Alpha-1-antitrypsin deficiency (AATD), a common genetic liver disease causing hepatitis and cirrhosis in adults and children. The clinical course is highly variable. Some infants present with neonatal cholestasis, which can resolve spontaneously or progress to cirrhosis; others are well in infancy, only to develop portal hypertension later in childhood.

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Childhood obesity is a serious concern in the United States, with over one third of the pediatric population classified as obese. Abdominal pain is one of the most common chief complaints among pediatric emergency department (ED) visits. We hypothesized that overweight and obese children being evaluated in the ED for abdominal pain would have higher resource utilization than their normal and underweight peers.

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Article Synopsis
  • The study aimed to identify causes of death and the prevalence of non-cardiovascular (non-CV) deaths in a group of patients referred for exercise testing, and to see if exercise test results could predict these deaths.
  • Over a follow-up period, 849 out of 13,382 patients died, with 80.9% of those deaths categorized as non-CV, meaning exercise test results did have predictive value for both CV and non-CV mortality.
  • Key findings indicated that low functional aerobic capacity, poor heart rate recovery, and a low chronotropic index significantly increased the risk of non-CV deaths, while overall, exercise test abnormalities were more strongly linked to CV deaths.
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Background & Aims: Biliary atresia (BA) is poorly understood and leads to liver transplantation (LT), with the requirement for and associated risks of lifelong immunosuppression, in most children. We performed a genome-wide association study (GWAS) to determine the genetic basis of BA.

Methods: We performed a GWAS in 811 European BA cases treated with LT in US, Canadian and UK centers, and 4,654 genetically matched controls.

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BACKGROUND The presence of anatomical variations of the hepatic artery poses a challenge for normothermic machine perfusion (NMP). Here, we describe our experience of creating a single arterial cannulation for NMP in 3 donor livers with replaced right hepatic artery. CASE REPORT Three donor livers with replaced right hepatic artery were perfused using NMP (OrganOx® metra®) for liver transplantation.

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Background: Dyspnea and fatigue are characteristics of long SARS-CoV-2 (COVID)-19. Cardiopulmonary exercise testing (CPET) can be used to better evaluate such patients.

Research Question: How significantly and by what mechanisms is exercise capacity impaired in patients with long COVID who are coming to a specialized clinic for evaluation?

Study Design And Methods: We performed a cohort study using the Mayo Clinic exercise testing database.

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Background: The role of minute ventilation/carbon dioxide production ( / CO 2 ) slope, a ventilation efficiency marker, in predicting short-term and long-term health outcomes for patients with nonsmall-cell lung cancer (NSCLC) undergoing lung resection has not been well investigated.

Material And Methods: This prospective cohort study consecutively enrolled NSCLC patients who underwent a presurgical cardiopulmonary exercise test from November 2014 to December 2019. The association of / CO 2 slope with relapse-free survival (RFS), overall survival (OS), and perioperative mortality was evaluated using the Cox proportional hazards and logistic models.

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Background: Alterations in both mitochondrial DNA (mtDNA) and nuclear DNA genes affect mitochondria function, causing a range of liver-based conditions termed mitochondrial hepatopathies (MH), which are subcategorized as mtDNA depletion, RNA translation, mtDNA deletion, and enzymatic disorders. We aim to enhance the understanding of pathogenesis and natural history of MH.

Methods: We analyzed data from patients with MH phenotypes to identify genetic causes, characterize the spectrum of clinical presentation, and determine outcomes.

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Article Synopsis
  • The review highlights the benefits and challenges of cardiac rehabilitation (CR) in managing cardiovascular disease (CVD), emphasizing its positive impact on patient outcomes.
  • New delivery methods using remote technologies are being explored alongside traditional center-based approaches, though more research is needed to determine their long-term effectiveness.
  • Efforts are being made to increase participation in CR, improve therapy effectiveness, and broaden access to new patient groups, with a focus on advancing policies and practices in the field.
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Cardiac transplantation is the final therapeutic option for patients with end-stage heart failure. Most patients experience a favorable functional ability post-transplant. However, episodes of acute rejection, and multiple comorbidities such as hypertension, diabetes mellitus, chronic kidney disease and cardiac allograft vasculopathy are common.

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Background: Approximately 3.5 million children and adolescents worldwide are chronically infected with HCV. This study uses pharmacokinetic modeling to identify pediatric doses of elbasvir/grazoprevir (EBR/GZR) that achieve plasma concentrations similar to those seen in adults receiving the approved fixed-dose combination regimen of EBR/GZR.

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Background: Since 2007, national public health laboratories in the WHO Eastern Mediterranean Region (EMR) have participated in a regional external quality assessment scheme in bacteriology to improve testing proficiency.

Aims: To assess laboratory performance in bacteriology in the EMR between 2011 and 2019 using the regional external quality assessment scheme.

Methods: We analysed the accuracy of participant-reported data in bacterial identification, Gram stain microscopy, and antimicrobial susceptibility testing.

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Article Synopsis
  • In medical studies, therapeutic decisions can lead to dependent censoring, impacting the analysis of survival outcomes, especially evident in cases like pediatric acute liver failure where death can be influenced by liver transplantation.
  • Traditional methods for evaluating biomarkers typically assume independent censoring, which makes them ineffective in these scenarios.
  • The authors propose new estimators that account for dependent censoring using multiple longitudinal risk factors and demonstrate their effectiveness through simulations and real-world applications for predicting mortality.
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Introduction: It is important for individuals and families to prepare for potential disasters to enable communities to generate a consolidated response. It is estimated that 30 percent of residents of the fourth largest city in Idaho, Idaho Falls, are not prepared to deal with disasters. A 1-day training workshop for healthcare professionals and students at Idaho State University in Pocatello was organized to build their capacity for acute disaster response and preparedness.

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Purpose: Nonexercise predictions of peak oxygen uptake (V˙ o2peak ) are used clinically, yet current equations were developed from cohorts of apparently healthy individuals and may not be applicable to individuals with cardiovascular disease (CVD). Our purpose was to develop a CVD-specific nonexercise prediction equation for V˙ o2peak .

Methods: Participants were from the Fitness Registry and Importance of Exercise International Database (FRIEND) with a diagnosis of coronary artery bypass surgery (CABG), myocardial infarction (MI), percutaneous coronary intervention (PCI), or heart failure (HF) who met maximal effort criteria during a cardiopulmonary exercise test (n = 15 997; 83% male; age 63.

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Objective: To determine whether the number of cardiac rehabilitation (CR) sessions attended and selected clinical characteristics were predictive of patients who exhibited improvement in peak oxygen uptake (VOpeak) after CR.

Patients And Methods: Using the Rochester Epidemiology Project records-linkage system, we identified all consecutive patients aged 18 years or older from Olmsted County, Minnesota, who underwent cardiopulmonary exercise testing before and after CR from 1999 to 2017. Regression models were created to assess the clinical predictors of VOpeak improvement (>0% baseline) after CR.

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Background And Aims: Detailed investigation of the biological pathways leading to hepatic fibrosis and identification of liver fibrosis biomarkers may facilitate early interventions for pediatric cholestasis.

Approach And Results: A targeted enzyme-linked immunosorbent assay-based panel of nine biomarkers (lysyl oxidase, tissue inhibitor matrix metalloproteinase (MMP) 1, connective tissue growth factor [CTGF], IL-8, endoglin, periostin, Mac-2-binding protein, MMP-3, and MMP-7) was examined in children with biliary atresia (BA; n = 187), alpha-1 antitrypsin deficiency (A1AT; n = 78), and Alagille syndrome (ALGS; n = 65) and correlated with liver stiffness (LSM) and biochemical measures of liver disease. Median age and LSM were 9 years and 9.

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Introduction: Peak rating of perceived exertion (RPE) is measured during clinical cardiopulmonary exercise testing (CPX) and is commonly used as a subjective indicator of maximal effort. However, no study to date has reported reference standards or the distribution of peak RPE across a large cohort of apparently healthy individuals.

Purpose: This study aimed to determine reference standards for peak RPE when using the 6-20 Borg scale for both treadmill and cycle tests.

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Purpose: Survivors of coronary artery disease (CAD) events are at risk for repeat events. Although evidence supports cardiac rehabilitation (CR) after an initial CAD event, it is unclear whether a repeat course of CR (CR × 2) is beneficial after a recurrent CAD event. The purpose of this study was to determine the association of CR × 2 with clinical outcomes in persons undergoing repeat percutaneous coronary intervention (PCI).

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