Childhood-onset Erdheim-Chester disease in the molecular era: clinical phenotypes and long-term outcomes of 21 patients.

Erdheim-Chester disease (ECD) is a rare histiocytic disorder that can present as a localized infiltration of foamy histiocytes or a multisystem disease that may be life-threatening. It is extremely rare in children. Pegoraro and colleagues present the clinical and molecular features of 21 patients with pediatric ECD through a large international collaboration, documenting that it resembles its adult counterpart, with similar molecular features and responses to agents targeting BRAF and MEK.

Active Surveillance and Contact Precautions for Preventing Methicillin-Resistant Staphylococcus aureus Healthcare-Associated Infections During the COVID-19 Pandemic.

Background: Statistically significant decreases in methicillin-resistant Staphylococcus aureus (MRSA) healthcare-associated infections (HAIs) occurred in Veterans Affairs (VA) hospitals from 2007 to 2019 using a national policy of active surveillance (AS) for facility admissions and contact precautions for MRSA colonized (CPC) or infected (CPI) patients, but the impact of suspending these measures to free up laboratory resources for testing and conserve personal protective equipment for coronavirus disease 2019 (COVID-19) on MRSA HAI rates is not known.

Methods: From July 2020 to June 2022 all 123 acute care VA hospitals nationwide were given the rolling option to suspend (or re-initiate) any combination of AS, CPC, or CPI each month, and MRSA HAIs in intensive care units (ICUs) and non-ICUs were tracked.

Results: There were 917 591 admissions, 5 225 174 patient-days, and 568 MRSA HAIs.

Fertility assessment and treatment in adolescent and young adult cancer survivors.

In the survivorship setting, adolescent and young adult (AYA) cancer survivors frequently demonstrate little knowledge of infertility risk, are unclear regarding their fertility status, and may under- or overestimate their treatment-related risk for infertility. In female AYA survivors, ovarian function usually parallels fertility, and can be assessed with serum hormone levels and ultrasonography. Posttreatment fertility preservation may be appropriate for survivors at risk for primary ovarian insufficiency.

Schizophrenia-associated NRXN1 deletions induce developmental-timing- and cell-type-specific vulnerabilities in human brain organoids.

De novo mutations and copy number deletions in NRXN1 (2p16.3) pose a significant risk for schizophrenia (SCZ). It is unclear how NRXN1 deletions impact cortical development in a cell type-specific manner and disease background modulates these phenotypes.

Fertility preservation in pediatric leukemia and lymphoma: A report from the Children's Oncology Group.

Certain chemotherapy agents, radiation, and surgery can all negatively impact future fertility. Consults regarding treatment-related risk for infertility and gonadal late effects of these agents should occur at the time of diagnosis as well as during survivorship. Counseling on fertility risk has traditionally varied significantly across providers and institutions.

Short-Term Patient Outcomes After Placement of an Internal Joint Stabilizer for Terrible Triad Injuries: A Multicenter Study.

Purpose: The purpose of this study was to assess the functional and patient-reported outcomes after the use of the internal joint stabilizer (IJS) for unstable terrible triad injuries. Specifically, we sought to determine our complication rate and the impact of complications on patient outcomes.

Methods: We identified all patients who had an IJS placed as a supplemental fixation for a terrible triad injury at two urban, level 1 academic medical centers.

Clin-STAR corner: 2021 update in musculoskeletal pain in older adults with a focus on osteoarthritis-related pain.

Chronic musculoskeletal (MSK) pain remains a leading cause of disability and functional impairment among older adults and is associated with substantial societal and personal costs. Chronic pain is particularly challenging to manage in older adults because of multimorbidity, concerns about treatment-related harm, as well as older adults' beliefs about pain and its management. This narrative review presents data on nine high-quality, peer-reviewed clinical trials published primarily over the past two years that focus on MSK pain management in older adults, of which four were comprehensively reviewed.

Outcomes of patients undergoing single-level arthroplasty versus anterior lumbar interbody fusion.

Background: Compared to vertebral body fusion, artificial discs are thought to lessen the risks of adjacent segment disease and the need for additional surgery by maintaining spinal mobility as they mimic the intervertebral disc structure. No studies have compared the rates of postoperative complications and the requirement for secondary surgery at adjacent segments among patients who have undergone anterior lumbar interbody fusions (ALIF) versus those undergoing lumbar arthroplasty.

Methods: An all-payer claims database identified 11,367 individuals who underwent single-level ALIF and lumbar arthroplasty for degenerative disc disease (DDD) between January 2010 and October 2020.

LPCAT1 levels in the placenta, the maternal plasma and the fetal plasma do not predict fetal lung responses to glucocorticoids in a sheep model of pregnancy.

Introduction: Lysophosphatidylcholine acyltransferase 1 (LPCAT1) is important for saturated phosphatidylcholine (Sat-PC) production in the lung. Sat-PC is a critical component of pulmonary surfactant, which maintains low alveolar surface tension, facilitating respiration. Previous studies have reported an association between maternal and fetal LPCAT1 levels and neonatal lung function.

Identifying and Validating Pediatric Hospitalizations for MIS-C Through Administrative Data.

Background: Individual children's hospitals care for a small number of patients with multisystem inflammatory syndrome in children (MIS-C). Administrative databases offer an opportunity to conduct generalizable research; however, identifying patients with MIS-C is challenging.

Methods: We developed and validated algorithms to identify MIS-C hospitalizations in administrative databases.

Chronic Mucosal Inflammation in Pediatric Intestinal Failure Patients-A Unique Phenomenon.

Introduction/objectives: As intestinal failure (IF) management improves and long-term survival rate increases, its physiological complications have become more apparent. The development of chronic intestinal inflammation resembling inflammatory bowel disease (IBD) in this population has been reported, but the literature describing it in detail is sparse. The present study was designed to characterize children with IF who developed chronic intestinal inflammation and identify the potential predisposing clinical factors.

Structural effects on custom, flexible iris prostheses passing through injector systems.

Purpose: To determine the suitability of various commercially available intraocular lens injection systems for the implantation of custom, flexible artificial irides of various sizes, both with and without fiber meshwork.

Setting: Cincinnati Eye Institute, Blue Ash, Ohio.

Design: Laboratory study.

Early triage echocardiography to predict outcomes in patients admitted with COVID-19: a multicenter study.

Introduction: Cardiac involvement seems to impact prognosis of COVID-19, especially in critically ill patients. We aimed to assess the prognostic value of right ventricular (RV) and left ventricular (LV) dysfunction, evaluated by bedside triage echocardiography (echo), in patients admitted to emergency departments (ED) in the US with COVID-19. We also assessed the feasibility of using cloud imaging for sharing and interpreting echocardiograms.

Predictors for operative treatment in pediatric proximal third both-bone diaphyseal forearm fractures in children include age and translation, but not initial angulation.

Introduction: Proximal third diaphyseal fractures of the radius and ulna represent an onerous fracture pattern due to difficulty maintaining acceptable alignment with nonoperative management. Our aim was to identify the factors that increase the odds for a surgical treatment of these fractures. Recognizing these factors can raise awareness to patients who are more likely to require additional care and assist clinicians in counseling families, targeting treatment plans, and constructing follow-up protocols.

H, N, and C chemical shift backbone resonance NMR assignment of the accumulation-associated protein (Aap) lectin domain from Staphylococcus epidermidis.

Staphylococcus epidermidis is the leading causative agent for hospital-acquired infections, especially device-related infections, due to its ability to form biofilms. The accumulation-associated protein (Aap) of S. epidermidis is primarily responsible for biofilm formation and consists of two domains, A and B.

Unlocking the potential of induced pluripotent stem cells for neonatal disease modeling and drug development.

Neonatal lung and heart diseases, albeit rare, can result in poor quality of life, often require long-term management and/or organ transplantation. For example, Congenital Heart Disease (CHD) is one of the most common type of congenital disabilities, affecting nearly 1% of the newborns, and has complex and multifactorial causes, including genetic predisposition and environmental influences. To develop new strategies for heart and lung regeneration in CHD and neonatal lung disease, human induced pluripotent stem cells (hiPSCs) provide a unique and personalized platform for future cell replacement therapy and high-throughput drug screening.

Paediatric rheumatologists do not score the physician's global assessment of juvenile idiopathic arthritis disease activity in the same way.

Objectives: To assess the heterogeneity in factors affecting physician's global assessment of disease activity (PhGA) and in PhGA scoring of multiple JIA patient's case scenarios.

Methods: An electronic web-based questionnaire of factors potentially considered in PhGA was sent worldwide to members of PRINTO and the Pediatric Rheumatology Care and Outcomes Improvement Network (PR-COIN). The respondents were asked to rate from 0 to 100 the relevance of 17 factors possibly affecting PhGA scoring and to derive a PhGA score of 17 detailed JIA patient cases.

Spatial Tuning in Nociceptive Processing Is Driven by Attention.

When the source of nociception expands across a body area, the experience of pain increases due to the spatial integration of nociceptive information. This well-established effect is called spatial summation of pain (SSp) and has been the subject of multiple investigations. Here, we used cold-induced SSp to investigate the effect of attention on the spatial tuning of nociceptive processing.