Precocious Puberty in Children with Neurofibromatosis Type 1.

This multi-institutional, descriptive study of 19 children with neurofibromatosis 1 examines the link between optic pathway gliomas (OPGs) and central precocious puberty (CPP). We report that CPP can arise without OPG chiasmal involvement and that prior OPG chemotherapy does not prevent the development of CPP.

"Work through it on your own": parent-centered emotion socialization beliefs and parenting stress.

Parent-centered emotion socialization beliefs that children should manage their emotions on their own without parent support may seem convenient for parents but may actually relate to greater parenting stress if this approach is incompatible with children's developmental needs. We explored relations between mothers' and fathers' parent-centered autonomy beliefs about children's emotions and their parenting stress, controlling for child behavior problems and parent education. We also examined whether these associations varied depending on child age.

Evaluation of sputum cultures in children with spinal Muscular atrophy.

Background: Spinal Muscular Atrophy (SMA) is a severe neuromuscular disorder. Despite increased survival due to novel therapies, morbidity from respiratory complications still persists. We aim to describe these patients' sputum cultures as an expression of chronic infectious airway disease.

Pulmonary function tests for evaluating the severity of Duchenne muscular dystrophy disease.

Aim: In Duchenne muscular dystrophy (DMD), lung disease contributes significantly to morbidity and mortality. This study aimed to assess the usefulness of various pulmonary function tests in evaluating DMD severity.

Methods: This retrospective study analysed lung function tests of patients with DMD-treated in the multidisciplinary respiratory neuromuscular clinic at Schneiders' Children Medical Center of Israel.

Impact on neonatal morbidities after a change in policy to administer antenatal corticosteroids to mothers at risk for late preterm delivery.

Objectives: Antenatal corticosteroids (ACS) administered to mothers at risk for preterm delivery before 34 weeks has been standard care to improve neonatal outcomes. After introducing a new obstetric policy based on updated recommendations advising the administration of ACS to pregnant women at risk for late preterm (LPT) delivery (34-36 6/7 weeks), we set out to determine the short-term clinical impact on those LPT neonates.

Methods: Retrospective chart review of LPT neonates delivered at NYU Langone Medical Center both one year before and after the policy went into place.

Effectiveness of a "Suppression Bundle" to improve HIV virologic suppression in an outpatient infectious disease clinic: a pilot implementation study.

Despite effectiveness and accessibility of combined anti-retroviral therapy (cART), only 85% of people living with HIV (PLHIV) in the United States are virologically suppressed. Improving suppression is complex. Our objective was to consider unique factors in PLHIV with non-suppressed viral loads in clinic and improve the percentage of suppressed patients by implementing a "Suppression Bundle" consisting of three to five bundled interventions with the goal of improved suppression.

COVID-19 in advanced Duchenne/Becker muscular dystrophy patients.

Duchenne muscular dystrophy (DMD) is the most common childhood muscular dystrophy. As a result of progressive muscle weakness, pulmonary function decreases during the second decade of life and lung disease contributes significantly to morbidity and mortality in these patients. Corticosteroids are the current standard of care for patients with DMD, despite known adverse effects such as obesity and immunosuppression.

Genetics, not the uterine environment, drive the formation of trophoblast inclusions: Insights from a twin study.

Introduction: Trophoblast inclusions (TIs) are associated with aneuploidy and pregnancy loss and have thus been considered to be a marker of genetic abnormality. However, to date, no study has specifically explored whether TIs are a manifestation of fetal genetics or, rather, the result of the intrauterine environment. The goal of this study was to compare the frequency of TIs in the placentas of monozygotic (MZ) and dizygotic (DZ) twin pairs in order to determine whether the formation of TIs is genetically driven or not.

Prenatal and perinatal metabolic risk factors for autism: a review and integration of findings from population-based studies.

Purpose Of Review: Given the ongoing rise in prevalence of autism spectrum disorder (ASD) and the challenges in developing and administering interventions to significantly alleviate ASD symptoms, there is an urgent need to identify modifiable risk factors for ASD. The goal of this review is to systematically evaluate the current evidence for an association between conditions related to maternal metabolic syndrome and risk for ASD in offspring focusing on methodically rigorous studies.

Recent Findings: In recent years, multiple studies explored the association between various conditions related to maternal metabolic syndrome (obesity, hypertension, or diabetes prior to, or with onset during pregnancy) and ASD risk in the offspring.

A 15-Year-Old With Tetralogy of Fallot With Pulmonic Atresia, Multiple Aorticopulmonary Collateral Arteries and Bilateral Peripheral Pulmonary Stenosis.

Tetralogy of Fallot (TOF) is a congenital disorder comprised of four heart defects. It is considered a critical condition that requires surgical repair. TOF may be complicated by pulmonary valve atresia (PA) and the development of major aorticopulmonary collateral arteries (MAPCA).

Doctors are seen as Godlike: Moral typecasting in medicine.

Objective: Doctors are generally thought of as very intelligent and capable. This perception has upsides-doctors are afforded respect and esteem-but it may also have downsides, such as neglecting the mental and physical health of physicians. Two studies examine how Americans "typecast" doctors as Godlike "thinkers" who help others, rather than as vulnerable "feelers" who might themselves need help.

Haptoglobin therapy has differential effects depending on severity of canine septic shock and cell-free hemoglobin level.

Background: During sepsis, higher plasma cell-free hemoglobin (CFH) levels portend worse outcomes. In sepsis models, plasma proteins that bind CFH improve survival. In our canine antibiotic-treated Staphylococcus aureus pneumonia model, with and without red blood cell (RBC) exchange transfusion, commercial human haptoglobin (Hp) concentrates bound and compartmentalized CFH intravascularly, increased CFH clearance, and lowered iron levels, improving shock, lung injury, and survival.

Development of home cage social behaviors in BALB/cJ vs. C57BL/6J mice.

BALB/cJ and C57BL/6J inbred mouse strains have been proposed as useful models of low and high levels of sociability (tendency to seek social interaction), respectively, based primarily on behaviors of ∼30-day-old mice in the Social Approach Test (SAT). In the SAT, approach and sniffing behaviors of a test mouse toward an unfamiliar stimulus mouse are measured in a novel environment. However, it is unclear whether such results generalize to a familiar environment with a familiar social partner, such as with a littermate in a home cage environment.