Clinical evaluation of code-based algorithms to identify patients with pulmonary arterial hypertension in healthcare databases.

Pulmonary arterial hypertension (PAH) is a rare subgroup of pulmonary hypertension (PH). Claims and administrative databases can be particularly important for research in rare diseases; however, there is a lack of validated algorithms to identify PAH patients using administrative codes. We aimed to measure the accuracy of code-based PAH algorithms against the true clinical diagnosis by right heart catheterization (RHC).

Pulmonary Vein Sign on Computed Tomography Pulmonary Angiography in Proximal and Distal Chronic Thromboembolic Pulmonary Hypertension With Hemodynamic Correlation.

Background: Pulmonary vein sign (PVS) indicates abnormal pulmonary venous flow on computed tomography pulmonary angiography (CTPA) is a frequent finding in proximal chronic thromboembolic pulmonary hypertension (CTEPH). PVS's occurrence in distal CTEPH and correlation to disease severity is unknown. Using right heart catheterization data, we evaluated the relationship between PVS and CTEPH disease distribution and severity.

Endogenous Retroviral Elements Generate Pathologic Neutrophils in Pulmonary Arterial Hypertension.

The role of neutrophils and their extracellular vesicles (EVs) in the pathogenesis of pulmonary arterial hypertension is unclear. To relate functional abnormalities in pulmonary arterial hypertension neutrophils and their EVs to mechanisms uncovered by proteomic and transcriptomic profiling. Production of elastase, release of extracellular traps, adhesion, and migration were assessed in neutrophils from patients with pulmonary arterial hypertension and control subjects.

Severe Pulmonary Arterial Hypertension Is Characterized by Increased Neutrophil Elastase and Relative Elafin Deficiency.

Background: Preclinical evidence implicates neutrophil elastase (NE) in pulmonary arterial hypertension (PAH) pathogenesis, and the NE inhibitor elafin is under early therapeutic investigation.

Research Question: Are circulating NE and elafin levels abnormal in PAH and are they associated with clinical severity?

Study Design And Methods: In an observational Stanford University PAH cohort (n = 249), plasma NE and elafin levels were measured in comparison with those of healthy control participants (n = 106). NE and elafin measurements were then related to PAH clinical features and relevant ancillary biomarkers.

Cognitive Outcomes of Young Children After Prenatal Exposure to Medications for Opioid Use Disorder: A Systematic Review and Meta-analysis.

Importance: The number of children with prenatal opioid exposure to medication for addiction treatment (MAT) with methadone and buprenorphine for maternal opioid use disorder is increasing, but the associations of this exposure with cognitive outcomes are not well understood.

Objective: To examine the strength and consistency of findings in the medical literature regarding the association of prenatal exposure to MAT with early childhood cognitive development, particularly when accounting for variables outside MAT exposure.

Data Sources: A search strategy obtained publications from PubMed, CINAHL, PsycINFO, Web of Science, and Embase from January 1972 to June 2019.

Discovery of Distinct Immune Phenotypes Using Machine Learning in Pulmonary Arterial Hypertension.

Rationale: Accumulating evidence implicates inflammation in pulmonary arterial hypertension (PAH) and therapies targeting immunity are under investigation, although it remains unknown if distinct immune phenotypes exist.

Objective: Identify PAH immune phenotypes based on unsupervised analysis of blood proteomic profiles.

Methods And Results: In a prospective observational study of group 1 PAH patients evaluated at Stanford University (discovery cohort; n=281) and University of Sheffield (validation cohort; n=104) between 2008 and 2014, we measured a circulating proteomic panel of 48 cytokines, chemokines, and factors using multiplex immunoassay.

Circulating plasmablasts are elevated and produce pathogenic anti-endothelial cell autoantibodies in idiopathic pulmonary arterial hypertension.

Idiopathic pulmonary arterial hypertension (IPAH) is a devastating pulmonary vascular disease in which autoimmune and inflammatory phenomena are implicated. B cells and autoantibodies have been associated with IPAH and identified as potential therapeutic targets. However, the specific populations of B cells involved and their roles in disease pathogenesis are not clearly defined.

Features and Outcomes of Methamphetamine-associated Pulmonary Arterial Hypertension.

Rationale: Although amphetamines are recognized as "likely" agents to cause drug- and toxin-associated pulmonary arterial hypertension (PAH), (meth)amphetamine-associated PAH (Meth-APAH) has not been well described.

Objectives: To prospectively characterize the clinical presentation, histopathology, and outcomes of Meth-APAH compared with those of idiopathic PAH (iPAH).

Methods: We performed a prospective cohort study of patients with Meth-APAH and iPAH presenting to the Stanford University Pulmonary Hypertension Program between 2003 and 2015.

Prognostic utility of right atrial emptying fractions in pulmonary arterial hypertension.

Although left atrial function has been extensively studied in patients with heart failure, the determinants and clinical correlates of impaired right atrial (RA) function have been poorly studied. We investigated measures of RA function in pulmonary arterial hypertension (PAH). We identified all treatment-naive patients with World Health Organization category 1 PAH seen at our center during 2000-2011 who had right heart catheterization and 6-minute walk test (6MWT) within 1 month of initial echocardiographic examination.

Neonatal opioid withdrawal syndrome.

Neonatal opioid withdrawal syndrome is common due to the current opioid addiction epidemic. Infants born to women covertly abusing prescription opioids may not be identified as at risk until withdrawal signs present. Buprenorphine is a newer treatment for maternal opioid addiction and appears to result in a milder withdrawal syndrome than methadone.

Impact of insulin resistance on ventricular function in pulmonary arterial hypertension.

Background: Insulin resistance (IR) is an independent prognostic marker in pulmonary arterial hypertension (PAH), although the mechanism by which it engenders risk is unknown. We prospectively investigated the clinical, laboratory, hemodynamic, and echocardiographic characteristics of insulin-sensitive (IS) and IR patients with PAH.

Methods: This was a prospective cohort study including well-phenotyped patients with PAH proven at cardiac catheterization.

Safety and efficacy of transition from systemic prostanoids to inhaled treprostinil in pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) is a disease characterized by increased pulmonary pressures and chronic right heart failure. Therapies for moderate and severe PAH include subcutaneous (SQ) and intravenous (IV) prostanoids that improve symptoms and quality of life. However, treatment compliance can be limited by severe side effects and complications related to methods of drug administration.

Association between intrapartum fetal heart rate patterns and neonatal abstinence syndrome in methadone exposed neonates.

Objective: Determine whether infants exposed to chronic maternal methadone with abnormal intrapartum fetal heart rate (FHR) patterns are more likely to require treatment for neonatal abstinence syndrome (NAS).

Study Design: Intrapartum FHR tracings analyzed in 104 pregnancies at ≥ 34 weeks gestation for FHR variability, accelerations, and decelerations. FHR patterns compared between neonates based on treatment with methadone for NAS.

Acanthosis nigricans and diabetes risk factors: prevalence in young persons seen in southwestern US primary care practices.

Purpose: Evidence shows acanthosis nigricans is often associated with hyperinsulinemia and may indicate increased risk of type 2 diabetes mellitus. The purpose of this study was to determine the association of acanthosis nigricans with type 2 diabetes risk factors and disease in young persons.

Methods: We conducted a cross-sectional study in the Research in Outpatient Settings Network, a practice-based research network in southwestern US communities.

Comparison of skin sites for estimating serum total bilirubin in in-patients and out-patients: chest is superior to brow.

Objective: [corrected] To compare transcutaneous bilirubin readings from the chest and forehead of inpatient and outpatient infants to investigate whether one site is more accurate for estimating serum bilirubin concentration.

Methods: In all, 31 infants were followed with serum and transcutaneous bilirubins using BiliChek trade mark at two skin sites.

Results: For inpatients average chest bilirubin was 0.