Childhood Familial Mediterranean Fever in the United States: Spectrum of Clinical Phenotypes and MEFV Genotypes in a Cohort From Southeast Michigan.

Objectives: The aim of this study was to report the spectrum of Familial Mediterranean Fever (FMF) in children living in Southeast Michigan.

Methods: We reviewed prerecorded data in medical records of FMF patients. Statistical analysis of the data included Fisher exact test, Pearson χ 2 procedure, parametric independent samples t test, and parametric analysis of variance using SPSS Version 29.

Hand and Forearm Compartment Syndrome Secondary to Intravenous Infiltration.

Introduction: Acute compartment syndrome (ACS) in the hand and forearm is an uncommon yet significant orthopedic crisis. The misplacement or migration of an intravenous (IV) catheter can cause fluid extravasation into interstitial tissues, which is a rare but known cause of ACS. Diagnosis of ACS is usually clinical, but this can be challenging in anesthetized or obtunded patients who are unable to communicate.

[18F]-Fluoroestradiol (FES) brain PET in the evaluation of patients with estrogen receptor positive breast cancer and known or suspected brain metastases.

Objective: Our purpose was to describe our initial institutional experience using dedicated brain [18F]-Fluoroestradiol (FES) PET/CT or PET/MRI in the management of patients with estrogen-receptor-positive (ER+) breast cancer brain metastases (BCBM), and compare to [18F]-Fluorodeoxyglucose (FDG) PET and MRI.

Materials & Methods: Patients with biopsy-proven ER+ disease and MRI findings of suspected new, progressive, or recurrent BCBM were included in this retrospective study. Clinical and demographic data were collected.

Healthcare Utilization in Pediatric Cancer Patients Near the End-of-Life.

Describe the healthcare utilization in the last 60 days of life in pediatric patients with cancer who died at home under hospice care and those that died in the hospital. Retrospective chart review of the medical records of those children with cancer diagnosis with palliative care consult and died either under hospice care at home or in the hospital. Patients dying under hospice care spent a median of 44 days at home.

Point-of-Care-Ultrasound Quality Assurance Data From Fellow-Performed Exams in a Pediatric Emergency Department: A Descriptive Study.

Objectives: Point-of-care-ultrasound (POCUS) is increasingly used by pediatric emergency medicine (PEM) fellows, but scant data exists on the accuracy of exam interpretations. Our goal was to determine whether agreement on exam interpretation between quality assurance (QA) faculty (reference standard) and PEM fellows varied by fellowship year or exam type.

Methods: Retrospective review of fellow-performed POCUS exams between January 2019 and June 2022.

Native Aortic Valve Endocarditis Linked to Cat Exposure: A Case Report and Review.

is an uncommon human pathogen, but documented infections have been mostly associated with exposure to dogs. There are only five documented cases of endocarditis secondary to , with all cases except one documenting exposure to a canine. We present a 74-year-old male with a history of Type 2 diabetes mellitus, CKD 3, moderate aortic stenosis and remote exposure to agent orange, who was found to have native valve endocarditis without exposure to a dog.

Real-world experience of direct oral anticoagulant use in a single pediatric center.

Background: Pediatric venous thromboembolism has increased by 130%-200%, specifically in hospitalized children, and direct oral anticoagulants (DOACs) offer several therapeutic advantages.

Methods: This study aims to evaluate the real-world epidemiological and outcome data from a retrospective review of pediatric patients treated with DOACs from January 1, 2013 to December 31, 2022. In this single-center, IRB-approved study, 65 patients were identified and analyzed using SPSS statistical software, and a descriptive statistical analysis was conducted.

Predictors of Acute Kidney Injury in Patients Hospitalized With Liver Cirrhosis: A Systematic Review and Meta-Analysis.

Acute kidney injury (AKI) frequently occurs in hospitalized individuals with liver cirrhosis and represents a significant risk factor for early in-hospital mortality, holding crucial clinical and prognostic importance. The objective of this meta-analysis was to assess the risk factors associated with AKI in hospitalized individuals with cirrhosis. This systematic review and meta-analysis was conducted in concordance with guidelines provided by the Preferred Reporting Items for Systematic Reviews and Meta-Analysis statement.

Rare cardiac tumour-nodular fasciitis.

Left ventricular tumour is a rare condition in children. The causes include vegetations, thrombus, and fibroma. 2-year-old asymptomatic female presented with an innocent heart murmur at 6 months of age.

Intracranial Germinoma in Two Caucasian American Siblings With Autism Spectrum Disorder.

Intracranial germ cell tumors (IGCTs) comprise 3% to 5% of all pediatric brain tumors in the West, with a significantly higher prevalence in Asia. Although these tumors are histologically diverse, repeated somatic variants have been demonstrated. Chromosomal aneuploidies, such as Klinefelter and Down syndromes, are associated with IGCTs, but no familial germline tumor syndromes are currently known.

Postoperative complications following Schwab-grade-I versus Schwab-grade-II PCO in treating severe rigid kyphoscoliosis patients: comparative matched-group outcomes with minimum 2-year follow-up.

Background Context: Standard partial facetectomies, (Smith-Petersen Osteotomy, (SPO), (Schwab-grade-I) and complete facet resection also known as Ponte osteotomy, (PO), (Schwab-grade-II) are narrowly akin and collectively appreciated as posterior column shortening osteotomies (PCOs). The former is considered a gentler osteotomy grade than the latter. The spine literature provides very little information on their comparison regarding perioperative complications and major curve correction rate outcomes.

Association of Sex and Age with Mortality and Health-Related Quality of Life in Children with Septic Shock: A Secondary Analysis of the Life After Pediatric Sepsis Evaluation.

Sepsis is more common in males than females, but whether outcomes differ by sex in various pediatric age groups is unclear. The Life After Pediatric Sepsis Evaluation (LAPSE) was a multicenter prospective cohort study that evaluated health-related quality of life (HRQL) in children after community-acquired septic shock. In this secondary analysis, we evaluated whether male children are at increased risk of mortality or long-term decline in HRQL than female children by age group.

Small bowel obstruction secondary to metastatic urothelial bladder cancer.

Small bowel obstruction secondary to primary cancer, such as carcinoid and adenocarcinoma, is not unusual. Less frequently, hematological metastasis from breast, lung, and melanoma can occur. However, metastasis from urothelial bladder carcinoma is extremely rare.

Central Nervous System Tumor With BCL6 Corepressor Internal Tandem Duplication: Treatment Course of a Long-term Survivor.

Central nervous system (CNS) tumor with BCL6 corepressor (BCOR) internal tandem duplication (ITD) is a newly described CNS tumor, characterized by in-frame ITDs of the BCOR gene. There is no standard practice regarding the management of this tumor. We report the clinical course of a 6-year-old boy who presented to the hospital with worsening headaches.

Functional insight into a neurodevelopmental disorder caused by missense variants in an RNA-binding protein, RBM10.

The RNA-binding motif protein 10, RBM10, is an RNA splicing regulator essential for development. Loss-of-function RBM10 variants are associated with TARP syndrome, a severe X-linked recessive condition in males. We report a 3-year-old male with a mild phenotype, consisting of cleft palate, hypotonia, developmental delay, and minor dysmorphisms, associated with a missense RBM10 variant, c.

The History of Extracorporeal Membrane Oxygenation and the Development of Extracorporeal Membrane Oxygenation Anticoagulation.

Extracorporeal membrane oxygenation (ECMO) was first started for humans in early 1970s by Robert Bartlett. Since its inception, there have been numerous challenges with extracorporeal circulation, such as coagulation and platelet activation, followed by consumption of coagulation factors and platelets, and biocompatibility of tubing, pump, and oxygenator. Unfractionated heparin (heparin hereafter) has historically been the defacto anticoagulant until recently.

Informed consent in dermatology: a narrative review.

Informed consent is a legal and ethical obligation of healthcare providers, and there are several steps that must be followed for informed consent to be obtained. Numerous challenges exist to obtaining informed consent including gaps in health literacy, language barriers, impaired decision-making capacity, and inadequacy of informed consent forms. Dermatologists must understand the importance and process for obtaining informed consent to protect patients as well as avoid litigation.

Evaluation of Social Determinants of Health and Prostate Cancer Outcomes Among Black and White Patients: A Systematic Review and Meta-analysis.

Importance: As the field of medicine strives for equity in care, research showing the association of social determinants of health (SDOH) with poorer health care outcomes is needed to better inform quality improvement strategies.

Objective: To evaluate the association of SDOH with prostate cancer-specific mortality (PCSM) and overall survival (OS) among Black and White patients with prostate cancer.

Data Sources: A MEDLINE search was performed of prostate cancer comparative effectiveness research from January 1, 1960, to June 5, 2020.

Case presentation and panel discussion: Transitions in care.

There is evidence that significant quality problems arise as patients transitions in care from one setting to another. Attention to nutrition during transitions of care is important to avoid complication. During the American Society for Parenteral and Enteral Nutrition 2022 preconference course, nutrition during transition of care from pediatric to adult care, from the intensive care unit to the hospital floors and from the hospital to home was addressed.

Nutrition for the high-risk surgical patient, when they need it most: Question and answer session.

Patients requiring complex or extensive surgery are often at high risk for perioperative and postoperative nutrition risk. Despite published guidelines, providing adequate nutrition to these patients continues to remain a clinical challenge. Using the case of a patient with preoperative nutrition risk who will need to undergo timely cancer resectional surgery, speakers presenting at the American Society for Parenteral and Enteral Nutrition 2022 Preconference discussed novel strategies to assess for nutrition risk, enhanced recovery after surgery, and preoperative and postoperative nutrition management in these often complex surgical patients.

Case presentation and panel discussion: Micronutrient therapy in critical illness.

Over the past decade, the use of supraphysiologic doses of micronutrients (also called metabolic resuscitation) in critically ill patients has gained significant attention. Building upon preclinical and observational human data, numerous randomized controlled trials have tested the impact of multiple micronutrients on various outcomes in critically ill patients. At the 2022 American Society for Parenteral and Enteral Nutrition Preconference Course, three world-renowned speakers delivered talks on the (1) overall role of micronutrients and, specifically, (2) selenium and vitamin C and (3) vitamin D and zinc in critically ill patients.

Characteristics of Adult Rapid Response Events in a Freestanding Children's Hospital.

Objectives: To describe nonhospitalized adult rapid response events (adult RREs) in a freestanding children's hospital and examine the relationship between various demographic and clinical factors with the final patient disposition.

Methods: We retrospectively reviewed records for nonhospitalized patients ≥18 years of age from events that occurred in a freestanding pediatric hospital between January 2011 through December 2020. We examined the relationship between adult RREs and patient demographic information, medical history, interventions, and patient disposition following an adult RRE.