Comorbidity and Operative Time are Stronger Predictors than Age for Palatoplasty Adverse Airway Events, A NSQIP-P Study of 6668 Cases.

Background: Adverse airway events (AAEs) are rare but devastating complications following palatoplasty. The purpose of this study is to evaluate patient risk factors for their effect on these complications. We hypothesize that prolonged operative time and the presence of multiple medical comorbidities are risk factors for AAEs.

Refractory Keratolimbal Allograft Rejection in Autoimmune Polyglandular Syndrome-Associated Keratopathy Treated With Intravenous Immunoglobulin.

Purpose: The aim of this study was to describe the use of intravenous immunoglobulin (IVIG) in the management of a 20-year-old woman with autoimmune polyglandular syndrome-associated keratopathy who developed acute transplant rejection after keratolimbal allograft (KLAL) surgery.

Case: Nine weeks after KLAL surgery, a 20-year-old woman with autoimmune polyglandular syndrome-related limbal stem cell deficiency presented with graft injection, hemorrhage, and an epithelial rejection line. This was concerning for acute rejection in the setting of triple-agent systemic immunosuppression (albeit nonadherence at times).

Penile diameter during puberty in boys: a retrospective analysis of longitudinally obtained data.

Objectives: Tanner staging is the standard for rating sexual maturation (SMR) in boys (pubic hair (PH) and genital (G) development). G staging is tripartite in nature and is prone to ambiguity because it is based upon somewhat vague visual cues that may lead to erroneous assessments and medical errors. Measurement of penile growth (penile girth or diameter) may provide an additional tool (in addition to the orchidometer) to make G staging more valid.

Empirically Derived Age-Based Vital Signs for Children in the Out-of-Hospital Setting.

Study Objective: To compare Pediatric Advanced Life Support (PALS) vital signs criteria to empirically derived vital signs cut-points for predicting out-of-hospital interventions in children.

Methods: We performed a cross-sectional study of pediatric encounters (<18 years) using the 2019 to 2020 datasets of the National Emergency Medical Services Information System, which we randomly divided into equal size derivation and validation samples. We developed age-based centile curves for initial heart rate, respiratory rate, and systolic blood pressure using generalized additive models for location, scale, and shape, which we evaluated in the validation sample.

A Heterozygous Gain-of-Function Variant in IKBKB Associated with Autoimmunity and Autoinflammation.

Purpose: Biallelic loss-of-function variants in IKBKB cause severe combined immunodeficiency. We describe a case of autoimmunity and autoinflammation in a male infant with a heterozygous gain-of-function (GOF) IKBKB variant.

Methods: Case report and review of the literature.

Parent's Hesitation with COVID-19 Vaccinations in Infants and Children Aged 6 Months to 5 Years.

We implemented an in-person survey of parents/guardians concerning COVID-19 vaccine for a predominantly African-American Medicaid pediatric patient population between the ages of 6-59 months at a Children's Hospital General Pediatric Clinic in Norfolk, VA. Vaccine hesitancy was predominantly based on concerns surrounding safety and overall need for the vaccine.

Changes in Distribution of Severe Neurologic Involvement in US Pediatric Inpatients With COVID-19 or Multisystem Inflammatory Syndrome in Children in 2021 vs 2020.

Importance: In 2020 during the COVID-19 pandemic, neurologic involvement was common in children and adolescents hospitalized in the United States for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)-related complications.

Objective: To provide an update on the spectrum of SARS-CoV-2-related neurologic involvement among children and adolescents in 2021.

Design, Setting, And Participants: Case series investigation of patients reported to public health surveillance hospitalized with SARS-CoV-2-related illness between December 15, 2020, and December 31, 2021, in 55 US hospitals in 31 states with follow-up at hospital discharge.

Team debriefing in the COVID-19 pandemic: a qualitative study of a hospital-wide clinical event debriefing program and a novel qualitative model to analyze debriefing content.

Background: Healthcare workers faced unique challenges during the early months of the COVID-19 pandemic which necessitated rapid adaptation. Clinical event debriefings (CEDs) are one tool that teams can use to reflect after events and identify opportunities for improving their performance and their processes. There are few reports of how teams have used CEDs in the COVID-19 pandemic.

A Hypothetical Case Example Illustrating the Importance of a Multidisciplinary Approach to Treating Chronic/Functional Abdominal Pain in Pediatric Patients.

Irritable bowel syndrome (IBS) can have a substantial impact on the physical, academic, and psychosocial functioning of pediatric patients. As a functional gastrointestinal disorder, pediatric patients with IBS are thought to benefit from a multidisciplinary approach to target the biopsychosocial factors of this condition. In this co-authored article by a Pediatric Gastroenterologist, Pediatric Pain and Palliative Care specialist and Pediatric GI Psychologist, we present a hypothetical case of a pediatric patient who will undergo evaluation and treatment by each of these specialists demonstrating how a collaborative effort amongst multidisciplinary specialists is the ideal approach to care.

Association of Apnea vs Hypopnea Predominance With Pediatric Sleep Apnea Outcomes: A Secondary Analysis of the Childhood Adenotonsillectomy Trial.

Importance: Children with apnea-predominant obstructive sleep apnea (OSA) are hypothesized to have a more severe form of the disease. However, research is lacking as to whether there is a significant difference in outcomes between children with apnea-predominant vs hypopnea-predominant OSA.

Objective: To assess the association between baseline apnea-predominant or hypopnea-predominant OSA on polysomnography and quality of life (QOL) outcomes in children with obstructive sleep apnea managed by watchful waiting with supportive care (WWSC) or adenotonsillectomy (AT).

Epilepsy in Coffin-Siris syndrome: A report from the international CSS registry and review of the literature.

Coffin-Siris syndrome (CSS, MIM135900) is a rare multiple congenital anomaly syndrome caused by pathogenic variants in the BAF complex; up to 28% of patients have previously been reported to have seizures, however, a comprehensive review of epilepsy has not been undertaken in this population. The International CSS Patient Report Database was queried for patients with self-reported seizures, epilepsy, and EEG results. Data gathered included demographic data, pathogenic gene variants, seizure characteristics and treatments, and EEG findings.

Changing temporal trends in patient volumes in a pediatric emergency department during a COVID-19 pandemic lockdown: A retrospective cohort study.

Objective: Emergency department (ED) teams have had to adjust limited staffing resources to meet the fluctuating levels of patient volume and acuity during the COVID-19 pandemic. Historically, Mondays have had the highest reported ED volumes. We are unaware of any studies reporting on the change of this Monday effect during the COVID-19 pandemic.

Adenotonsillectomy outcomes in obese adolescents with obstructive sleep apnea.

Study Objectives: (1) To assess adenotonsillectomy (AT) outcomes in adolescents with obesity and with obstructive sleep apnea (OSA); (2) To identify clinical factors predicting OSA in adolescents following AT.

Methods: Adolescents 12 to 18 years old with obesity who underwent AT for OSA were included. Subjects had pre-AT and post-AT polysomnogram.

Intravenous Fluid Bolus Rates Associated with Outcomes in Pediatric Sepsis: A Multi-Center Analysis.

Purpose: Pediatric sepsis guidelines recommend rapid intravenous fluid (IVF) bolus administration rates (BAR). Recent sepsis studies suggest that rapid BAR may be associated with increased morbidity. We aimed to describe the association between emergency department (ED) IVF BAR and clinical outcomes in pediatric sepsis.

Bell's palsy with facial bone involvement: A rare presentation of chronic nonbacterial osteomyelitis with literature review.

Chronic nonbacterial osteomyelitis (CNO) is a chronic, sterile, inflammatory disease. It primarily presents with nonspecific bone pain and swelling but ultimately can cause bone destruction and deformities, if left untreated. The involvement of the cranial bones (apart from the mandible) is rare in CNO.

Evidence for an association between Coffin-Siris syndrome and congenital diaphragmatic hernia.

Coffin-Siris syndrome (CSS) is an autosomal dominant neurodevelopmental syndrome that can present with a variety of structural birth defects. Pathogenic variants in 12 genes have been shown to cause CSS. Most of these genes encode proteins that are a part of the mammalian switch/sucrose non-fermentable (mSWI/SNF; BAF) complex.

Inborn Errors of Metabolism: Becoming Ready for Rare.

Inborn errors of metabolism (IEMs) are a large group of disorders that can present in any age group and must be considered in the differential diagnosis for a variety of signs and symptoms appearing in infants and children. The rarity and complexity of these conditions often make them difficult to recognize, as they may mimic more common conditions. This review article discusses some of the more commonly presenting IEMs that are important for the general pediatrician to understand when evaluating a sick patient.