Acid sphingomyelinase deficiency and Gaucher disease: Underdiagnosed and often treatable causes of hepatomegaly, splenomegaly, and low HDL cholesterol in lean individuals.

Background: Acid sphingomyelinase deficiency (ASMD) and Gaucher disease type 1 (GD1) are rare inherited sphingolipid disorders with multisystemic manifestations, including liver disease and dyslipidemia. Despite effective treatments, insufficient disease awareness frequently results in diagnostic delays during which irreversible complications occur. We delineated the shared and distinctive features of hepatic, splenic, and lipoprotein phenotypes in ASMD and GD1.

The impact of sphingomyelin on the pathophysiology and treatment response to olipudase alfa in acid sphingomyelinase deficiency.

Acid sphingomyelinase deficiency (ASMD) is a rare progressive genetic disorder caused by pathogenic variants in the gene causing low or absent activity of the enzyme acid sphingomyelinase, resulting in subsequent accumulation of its substrate, sphingomyelin. Signs and symptoms of excessive lysosomal sphingomyelin storage, such as hepatosplenomegaly and pulmonary impairment, and in a subset of patients, progressive neurological manifestations, have long been recognized as hallmarks of the disease. Uncontrolled accumulation of sphingomyelin has important and complex downstream metabolic and immunologic consequences that contribute to the disease burden.

Medication adherence among people living with hypertension and diabetes in Puno, Peru: A secondary analysis of formative data of the ANDES trial.

Background: Hypertension is the leading modifiable risk factor for premature death globally despite the existence of evidence-based and cost-effective treatments. Medication nonadherence is cited as the main cause of treatment failure for hypertension. In Peru, adherence to anti-hypertensive medications of individuals with both hypertension and type 2 diabetes (T2D) is not well studied.

Health system barriers to hypertension care in Peru: Rapid assessment to inform organizational-level change.

Traditional patient- and provider-level hypertension interventions have proven insufficient to halt hypertension as the leading cause of morbidity and mortality globally. Systems-level interventions are required to address factors challenging hypertension control across a social ecological framework, an under-studied topic particularly salient in low- and middle-income countries (LMICs) such as Peru. To inform such interventions, we sought to identify key health systems barriers to hypertension care in Puno, Peru.

The application of antimicrobial stewardship knowledge to nursing practice: A national survey of United Kingdom pre-registration nursing students.

Aim: To assess student nurses understanding and skills in the application of antimicrobial stewardship knowledge to practice.

Design: Quantitative.

Methods: Cross-sectional survey.

Transcriptomic analysis delineates preterm prelabor rupture of membranes from preterm labor in preterm fetal membranes.

Background: Globally, preterm birth remains the leading cause of death in children younger than 5 years old. Spontaneous preterm birth is comprised of two events that may or may not occur simultaneously: preterm labor and preterm prelabor rupture of membranes (PPROM). To further explore the concept that spontaneous preterm birth can result from the initializing of two separate but overlapping pathological events, we compared fetal membrane tissue from preterm labor deliveries to fetal tissue from preterm labor with PPROM deliveries.

Fidelity and adherence to a liquefied petroleum gas stove and fuel intervention: The multi-country Household Air Pollution Intervention Network (HAPIN) trial.

Background: Reducing household air pollution (HAP) to levels associated with health benefits requires nearly exclusive use of clean cooking fuels and abandonment of traditional biomass fuels.

Methods: The Household Air Pollution Intervention Network (HAPIN) trial randomized 3,195 pregnant women in Guatemala, India, Peru, and Rwanda to receive a liquefied petroleum gas (LPG) stove intervention (n = 1,590), with controls expected to continue cooking with biomass fuels (n = 1,605). We assessed fidelity to intervention implementation and participant adherence to the intervention starting in pregnancy through the infant's first birthday using fuel delivery and repair records, surveys, observations, and temperature-logging stove use monitors (SUMs).

Successes and challenges to implementing the Fondo de Inclusión Social Energético LPG access Program in Peru: novel insights from front-end implementers.

Background: Household air pollution due to the burning of solid fuels is one of the leading risk factors for disease and mortality worldwide, resulting in an estimated three million deaths annually. Peru's national LPG access program, FISE, aims to reduce the use of biomass fuels and increase access to cleaner fuels for cooking in low-income Peruvian households through public-private partnerships. Perspectives from front-end program implementers are needed to better understand barriers and facilitators to program implementation and to identify strategies to strengthen program reach, uptake, and health impact.

Post-birth exposure contrasts for children during the Household Air Pollution Intervention Network randomized controlled trial.

Exposure to household air pollution is a leading cause of ill-health globally. The Household Air Pollution Intervention Network (HAPIN) randomized controlled trial evaluated the impact of a free liquefied petroleum gas stove and fuel intervention on birth outcomes and maternal and child health. As part of HAPIN, an extensive exposure assessment was conducted.

Fidelity and adherence to a liquefied petroleum gas stove and fuel intervention: the multi-country Household Air Pollution Intervention Network (HAPIN) trial.

Background: Reducing household air pollution (HAP) to levels associated with health benefits requires nearly exclusive use of clean cooking fuels and abandonment of traditional biomass fuels.

Methods: The Household Air Pollution Intervention Network (HAPIN) trial randomized 3,195 pregnant women in Guatemala, India, Peru, and Rwanda to receive a liquefied petroleum gas (LPG) stove intervention (n=1,590), with controls expected to continue cooking with biomass fuels (n=1,605). We assessed fidelity to intervention implementation and participant adherence to the intervention starting in pregnancy through the infant's first birthday using fuel delivery and repair records, surveys, observations, and temperature-logging stove use monitors (SUMs).

Effects of a LPG stove and fuel intervention on adverse maternal outcomes: A multi-country randomized controlled trial conducted by the Household Air Pollution Intervention Network (HAPIN).

Household air pollution from solid cooking fuel use during gestation has been associated with adverse pregnancy and birth outcomes. The Household Air Pollution Intervention Network (HAPIN) trial was a randomized controlled trial of free liquefied petroleum gas (LPG) stoves and fuel in Guatemala, Peru, India, and Rwanda. A primary outcome of the main trial was to report the effects of the intervention on infant birth weight.

Exposure-response relationships for personal exposure to fine particulate matter (PM), carbon monoxide, and black carbon and birthweight: an observational analysis of the multicountry Household Air Pollution Intervention Network (HAPIN) trial.

Background: Household air pollution (HAP) from solid fuel use is associated with adverse birth outcomes, but data for exposure-response relationships are scarce. We examined associations between HAP exposures and birthweight in rural Guatemala, India, Peru, and Rwanda during the Household Air Pollution Intervention Network (HAPIN) trial.

Methods: The HAPIN trial recruited pregnant women (9-<20 weeks of gestation) in rural Guatemala, India, Peru, and Rwanda and randomly allocated them to receive a liquefied petroleum gas stove or not (ie, and continue to use biomass fuel).

Availability, affordability and access to essential medications for asthma and chronic obstructive pulmonary disease in three low- and middle-income country settings.

Introduction: Despite the rising burden of chronic respiratory disease globally, and although many respiratory medications are included in the World Health Organization Essential Medications List (WHO-EML), there is limited information concerning the availability and affordability of treatment drugs for respiratory conditions in low- and middle-income countries (LMICs).

Methods: All public and private pharmacies in catchment areas of the Global Excellence in COPD outcomes (GECo) study sites in Bhaktapur, Nepal, Lima, Peru, and Nakaseke, Uganda, were approached in 2017-2019 to assess pricing and availability of medications for the management of asthma and COPD.

Results: We surveyed all 63 pharmacies in respective study areas in Nepal (95.

Baseline associations between household air pollution exposure and blood pressure among pregnant women in the Household Air Pollution Intervention Network (HAPIN) multi-country randomized controlled trial.

Cooking and heating using solid fuels can result in dangerous levels of exposure to household air pollution (HAP). HAPIN is an ongoing randomized controlled trial assessing the impact of a liquified petroleum gas stove and fuel intervention on HAP exposure and health in Guatemala, India, Peru, and Rwanda among households that rely primarily on solid cooking fuels. Given the potential impacts of HAP exposure on cardiovascular outcomes during pregnancy, we seek to characterize the relationship between personal exposures to HAP and blood pressure among pregnant women at baseline (prior to intervention) in the study.

Visualizing Field Data Collection Procedures of Exposure and Biomarker Assessments for the Household Air Pollution Intervention Network Trial in India.

Here, we present a visual representation of standard procedures to collect population-level data on personal exposures to household air pollution (HAP) from two different study sites in a resource-constrained setting of Tamil Nadu, India. Particulate matter PM2.5 (particles smaller than 2.

Incentivizing Pregnant Women to Quit Smoking in the Real World-A Community-Based Pilot Intervention.

Smoking during pregnancy is a leading preventable cause of poor pregnancy outcomes. Financial incentives interventions yield quit rates of approximately 30% during pregnancy, versus approximately 4% in traditional smoking cessation programs. This pilot study assessed the feasibility of translating an efficacious University of Vermont research-based intervention into a rural community setting delivered by the Vermont Department of Health.

Liquefied Petroleum Gas or Biomass for Cooking and Effects on Birth Weight.

Background: Exposure during pregnancy to household air pollution caused by the burning of solid biomass fuel is associated with adverse health outcomes, including low birth weight. Whether the replacement of a biomass cookstove with a liquefied petroleum gas (LPG) cookstove would result in an increase in birth weight is unclear.

Methods: We performed a randomized, controlled trial involving pregnant women (18 to <35 years of age and at 9 to <20 weeks' gestation as confirmed on ultrasonography) in Guatemala, India, Peru, and Rwanda.

Exposure Contrasts of Pregnant Women during the Household Air Pollution Intervention Network Randomized Controlled Trial.

Background: Exposure to arising from solid fuel combustion is estimated to result in million premature deaths and 91 million lost disability-adjusted life years annually. Interventions attempting to mitigate this burden have had limited success in reducing exposures to levels thought to provide substantive health benefits.

Objectives: This paper reports exposure reductions achieved by a liquified petroleum gas (LPG) stove and fuel intervention for pregnant mothers in the Household Air Pollution Intervention Network (HAPIN) randomized controlled trial.

Cross-sectional analysis of the association between personal exposure to household air pollution and blood pressure in adult women: Evidence from the multi-country Household Air Pollution Intervention Network (HAPIN) trial.

Elevated blood pressure (BP) is a leading risk factor for the global burden of disease. Household air pollution (HAP), resulting from the burning of biomass fuels, may be an important cause of elevated BP in resource-poor communities. We examined the exposure-response relationship of personal exposures to HAP -fine particulate matter (PM), carbon monoxide (CO), and black carbon (BC) - with BP measures in women aged 40-79 years across four resource-poor settings in Guatemala, Peru, India and Rwanda.

Large-Scale Climatic Patterns Have Stronger Carry-Over Effects than Local Temperatures on Spring Phenology of Long-Distance Passerine Migrants between Europe and Africa.

Earlier springs in temperate regions since the 1980s, attributed to climate change, are thought to influence the earlier arrival of long-distance migrant passerines. However, this migration was initiated weeks earlier in Africa, where the Southern Oscillation, Indian Ocean Dipole, North Atlantic Oscillation drive climatic variability, and may additionally influence the migrants. Multiple regressions investigated whether 15 indices of climate in Africa and Europe explained the variability in timing of arrival for seven trans-Saharan migrants.