The Social Networks and Social Support of Siblings of Children with Cancer.

Siblings of children with cancer need support to ameliorate the challenges they encounter; however, little is known about what types and sources of support exist for siblings. This study addresses this gap in our understanding of the social networks and sources of support for adolescents with a brother or sister who has cancer. Additionally, we describe how the support siblings receive addresses what they feel are the hardest aspects of being a sibling of a child with cancer.

46 XY undervirulized male DSD: Reporting a patient with prenatally diagnosed disorder/difference of sex development (DSD) with heterozygous mutations.

Leydig cell hypoplasia is a rare autosomal recessive condition caused by mutations in luteinizing hormone/chorionic gonadotropin receptor (LHCGR) genes in which 46, XY patients demonstrate a wide spectrum of disorders/differences of sex development (DSD) phenotypes ranging from normal female external genitalia in severe subtypes to micropenis or hypospadias in patients with less severe presentations. Although most patients with LHCGR defects are diagnosed at puberty, here we describe the prenatal diagnosis of 46, XY DSD due to two likely pathogenic variants in , one of which has never been reported.

Workforce demographics and unit structure in paediatric cardiac critical care in the United States.

Objective: To assess current demographics and duties of physicians as well as the structure of paediatric cardiac critical care in the United States.

Design: REDCap surveys were sent by email from May till August 2019 to medical directors ("directors") of critical care units at the 120 United States centres submitting data to the Society of Thoracic Surgeons Congenital Heart Surgery Database and to associated faculty from centres that provided email lists. Faculty and directors were asked about personal attributes and clinical duties.

Newborn Screening for Biliary Atresia: a Review of Current Methods.

Purpose Of Review: Biliary atresia is a serious neonatal liver disease due to obstructed bile ducts that has better outcomes when detected and treated in the first 30-45 days of life. This review examines different methods to screen newborns for biliary atresia as well as discusses observations from ongoing screening programs implemented in parts of the United States.

Recent Findings: Screening strategies for biliary atresia include detecting persistent jaundice, examining stool color, testing fractionated bilirubin levels, or measuring bile acid levels from dried blood spot cards.

The indispensable role of pharmacy services and medication therapy management in cystic fibrosis.

Care for people with cystic fibrosis (PWCF) is highly complex and requires a multidisciplinary approach where the pharmacist plays a vital role. The purpose of this manuscript is to serve as a guideline for pharmacists and pharmacy technicians who provide care for PWCF by providing background and current recommendations for the use of cystic fibrosis (CF)-specific medications in both the acute and ambulatory care settings. The article explores current literature surrounding the role of pharmacists and pharmacy technicians, proven pharmacy models to emulate, and pharmacokinetic idiosyncrasies unique to the CF population while also identifying areas of future research.

Optimization of antibiotics for cystic fibrosis pulmonary exacerbations due to highly resistant nonlactose fermenting Gram negative bacilli: Meropenem-vaborbactam and cefiderocol.

We are writing this letter to provide an update of published information on antibiotics for cystic fibrosis (CF) pulmonary exacerbations to the State of the Art articles by Zobell et al. Information on meropenem-vaborbactam and cefiderocol were not available when the original articles were published. These new antibiotics, approved in 2017 and 2019, possess antipseudomonal properties like the other carbapenems and cephalosporins in the original articles however, existing literature refers to their use for other less common bacteria.

Phase IIa Global Study Evaluating Rituximab for the Treatment of Pediatric Patients With Granulomatosis With Polyangiitis or Microscopic Polyangiitis.

Objective: To assess the safety, tolerability, pharmacokinetics, and efficacy of rituximab (RTX) in pediatric patients with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA).

Methods: The Pediatric Polyangiitis Rituximab Study was a phase IIa, international, open-label, single-arm study. During the initial 6-month remission-induction phase, patients received intravenous infusions of RTX (375 mg/m body surface area) and glucocorticoids once per week for 4 weeks.

Recurrence of Primary Sclerosing Cholangitis After Liver Transplant in Children: An International Observational Study.

Background And Aims: Recurrent primary sclerosing cholangitis (rPSC) following liver transplant (LT) has a negative impact on graft and patient survival; little is known about risk factors for rPSC or disease course in children.

Approach And Results: We retrospectively evaluated risk factors for rPSC in 140 children from the Pediatric PSC Consortium, a multicenter international registry. Recipients underwent LT for PSC and had >90 days of follow-up.

State of the art in cystic fibrosis pharmacology optimization of antimicrobials in the treatment of cystic fibrosis pulmonary exacerbations: III. Executive summary.

Acute pulmonary exacerbations are complications of cystic fibrosis (CF) and are associated with increased morbidity and mortality. Methicillin-resistant Staphylococcus aureus (MRSA) and Aspergillus fumigatus are organisms that have been detected in the lungs of CF patients. The focus of this review is to provide an overview of the classes of antimicrobials used for MRSA and allergic bronchopulmonary aspergillosis (ABPA), a hypersensitivity reaction caused by A.

Outpatient Opioid Prescriptions are Associated With Future Substance Use Disorders and Overdose Following Adolescent Trauma.

Objective: This study aims to determine if outpatient opioid prescriptions are associated with future SUD diagnoses and overdose in injured adolescents 5 years following hospital discharge.

Summary Of Background Data: Approximately, 1 in 8 adolescents are diagnosed with an SUD and 1 in 10 experience an overdose in the 5 years following injury. State laws have become more restrictive on opioid prescribing by acute care providers for treating pain, however, prescriptions from other outpatient providers are still often obtained.

Understanding the expanding role of pharmacy services in outpatient cystic fibrosis care.

Cystic fibrosis (CF) patients utilize an average of 10 (±5) medications per day. Given the complexity of the medication regimen, the CF Foundation (CFF) recommends pharmacists as members of the CF care team. The areas of pharmacy services have been identified in the literature.

Screening Adolescent Trauma Patients for Substance Use at 10 Pediatric Trauma Centers.

Background: The American College of Surgeons Committee on Trauma recommends universal alcohol screening be part of the evaluation of admitted trauma patients. Yet, suboptimal screening rates have been reported for admitted adult and adolescent trauma patients. This lack of screening, in turn, has limited the ability of trauma services to provide patients with brief interventions during their hospital admission and subsequent referrals to treatment after discharge.

Management of Velopharyngeal Insufficiency in Cleft Patients With and Without Multidisciplinary Team Care.

Objective: To evaluate the effect of an American Cleft Palate-Craniofacial Association (ACPA)-approved multidisciplinary team on velopharyngeal insufficiency (VPI) diagnosis and treatment.

Design: Retrospective cohort setting; tertiary children's hospital patients; children with cleft palate repair identified through procedure codes.

Main Outcome Measures: Velopharyngeal insufficiency diagnosis was assigned based on surgeon or team assessment.

Optimization of antimicrobials in the treatment of cystic fibrosis pulmonary exacerbations: II. Therapies for allergic bronchopulmonary aspergillosis.

This review is the second article in the State-of-the-Art series and aims to evaluate medications used in the treatment of allergic bronchopulmonary aspergillosis (ABPA) in pediatric and adult patients with cystic fibrosis (CF). ABPA is one of several organisms that are found in the airways of CF patients. This review provides an evidence-based summary of pharmacokinetic (PK)/pharmacodynamic (PD), tolerability, and efficacy studies of medications including corticosteroids, amphotericin B, azole antifungals (isavuconazole, itraconazole, posaconazole, and voriconazole), and a monoclonal antibody omalizumab in the treatment of ABPA and identifies areas where further study is warranted.

Vedolizumab Therapy in Children With Primary Sclerosing Cholangitis: Data From the Pediatric Primary Sclerosing Cholangitis Consortium.

Objectives: Most patients with primary sclerosing cholangitis (PSC) also have inflammatory bowel disease (IBD). The liver and colon express MAdCAM-1, a target of lymphocyte homing integrins. Vedolizumab (VDZ) is an α4β7 integrin antibody used to treat IBD.

Continence outcomes following a modification of the Mitchell bladder neck reconstruction in myelomeningocele: A single institution experience.

Background: Many surgical procedures have been developed to improve continence in myelomeningocele patients. Our modification of the Mitchell bladder neck reconstruction involves removal of a diamond-shaped wedge of the anterior bladder neck, tubularization of the bladder neck and urethra to increase outlet resistance, and addition of a bladder neck autologous fascial sling.

Objective: We aimed to evaluate rates of continence and re-operation in children with myelomeningocele undergoing this Modified Mitchell bladder neck reconstruction.

I-PASS Mentored Implementation Handoff Curriculum: Frontline Provider Training Materials.

Introduction: The I-PASS Handoff Program is a comprehensive handoff curriculum that has been shown to decrease rates of medical errors and adverse events during patient handoffs. Frontline providers are the key individuals participating in handoffs of patient care. It is important they receive robust handoff training.