Management of Vulvovaginal Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis.

Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) is an autoimmune process resulting in painful epidermal sloughing that can involve the vulva and vagina. Current guideline recommendations are based on expert opinion and may not reflect modern management of SJS/TEN in burn centers. We performed a retrospective chart review of 34 female patients treated for SJS/TEN at our burn center from 2015 to 2023.

Event-free survival of maralixibat-treated patients with Alagille syndrome compared to a real-world cohort from GALA.

Background And Aims: Alagille syndrome (ALGS) is characterized by chronic cholestasis with associated pruritus and extrahepatic anomalies. Maralixibat, an ileal bile acid transporter inhibitor, is an approved pharmacologic therapy for cholestatic pruritus in ALGS. Since long-term placebo-controlled studies are not feasible or ethical in children with rare diseases, a novel approach was taken comparing 6-year outcomes from maralixibat trials with an aligned and harmonized natural history cohort from the G lobal AL agille A lliance (GALA) study.

Investigational non-antibiotic therapeutics for infections in hematopoietic cell transplant recipients and patients with hematologic malignancies receiving cellular therapies.

In the age of progressive antimicrobial resistance and increased difficulty combating infections in immunocompromised hosts, there has been renewed interest in the use of nontraditional therapeutics for infections. Herein, we review the use of investigational non-pharmaceutical anti-infective agents targeting fungal, bacterial, and viral infections in patients with hematologic malignancies, focusing on those receiving hematopoietic cell transplantation or cellular therapies. We discuss immune checkpoint inhibitors, granulocyte transfusions, bone marrow colony-stimulating factors, bacteriophages, fecal microbiota transplantation, and virus specific T-cell therapy.

Predictors of 6-year event-free survival in Alagille syndrome patients treated with maralixibat, an ileal bile acid transporter inhibitor.

Background And Aims: Refractory pruritus and other complications of cholestasis are indications for liver transplantation (LT) in patients with Alagille syndrome (ALGS). We evaluated predictors of event-free survival and transplant-free survival in patients with ALGS treated with maralixibat (MRX), an ileal bile acid transporter inhibitor.

Approach And Results: We assessed patients with ALGS from 3 clinical trials of MRX with up to 6 years of follow-up.

Racial/ethnic differences in the association between menthol cigarette use and mental illness among adults who smoke in the United States.

Data from the National Survey on Drug Use and Health (2012-2018) were used to characterize the association between menthol cigarette use and indicators of Any (AMI) and Serious (SMI) Mental Illness among adults who smoke in the United States. In general, people who smoke menthol cigarettes were more likely to have AMI (aOR = 1.123 [1.

Predicting future cancer incidence by age, race, ethnicity, and sex.

Introduction: Cancer remains a substantial burden on society. Our objective was to update projections on the number of new cancer diagnoses in the United States by age, race, ethnicity, and sex through 2040.

Materials And Methods: Population-based cancer incidence data were obtained using Surveillance, Epidemiology, and End Results (SEER) data.

FOLFIRINOX Pharmacodynamic Interactions in 2D and 3D Pancreatic Cancer Cell Cultures.

The multi-drug combination regime, FOLFIRINOX, is a standard of care chemotherapeutic therapy for pancreatic cancer patients. However, systematic evaluation of potential pharmacodynamic interactions among multi-drug therapy has not been reported previously. Here, pharmacodynamic interactions of the FOLFIRINOX agents (5-fluorouracil (5-FU), oxaliplatin (Oxa) and SN-38, the active metabolite of irinotecan) were assessed across a panel of primary and established pancreatic cancer cells.

Independent Predictors for Hospitalization-Associated Radiation therapy Interruptions.

Purpose: Radiation treatment interruption associated with unplanned hospitalization remains understudied. The intent of this study was to benchmark the frequency of hospitalization-associated radiation therapy interruptions (HARTI), characterize disease processes causing hospitalization during radiation, identify factors predictive for HARTI, and localize neighborhood environments associated with HARTI at our academic referral center.

Methods And Materials: This retrospective review of electronic health records provided descriptive statistics of HARTI event rates at our institutional practice.

Maralixibat Treatment Response in Alagille Syndrome is Associated with Improved Health-Related Quality of Life.

Objective: The objective of this study was to assess the impact of treatment response to the ileal bile acid transporter inhibitor maralixibat on health-related quality of life (HRQoL) in children with Alagille syndrome.

Study Design: This analysis used data from the ICONIC trial, a phase 2 study with a 4-week double-blind, placebo-controlled, randomized drug withdrawal period in children with Alagille syndrome with moderate-to-severe pruritus. Clinically meaningful treatment response to maralixibat was defined a priori as a ≥1-point reduction in the Itch-Reported Outcome (Observer) score, from baseline to week 48.

Examining the impact of trimester of diagnosis on COVID-19 disease progression in pregnancy.

Background: COVID-19 infection is associated with increased morbidity in pregnancy and adverse maternal and neonatal outcomes. Little is currently known about how the timing of infection during pregnancy affects these outcomes.

Objective: This study aimed to evaluate the effect of trimester of COVID-19 infection on disease progression and severity in pregnant patients.

Maralixibat for the treatment of PFIC: Long-term, IBAT inhibition in an open-label, Phase 2 study.

Children with progressive familial intrahepatic cholestasis, including bile salt export pump (BSEP) and familial intrahepatic cholestasis-associated protein 1 (FIC1) deficiencies, suffer debilitating cholestatic pruritus that adversely affects growth and quality of life (QoL). Reliance on surgical interventions, including liver transplantation, highlights the unmet therapeutic need. INDIGO was an open-label, Phase 2, international, long-term study to assess the efficacy and safety of maralixibat in children with FIC1 or BSEP deficiencies.

An evidence-based definition of anemia for singleton, uncomplicated pregnancies.

Background: The definition for anemia in pregnancy is outdated, derived from Scandinavian studies in the 1970's to 1980's. To identity women at risk of blood transfusion, a common cause of Severe Maternal Morbidity, a standard definition of anemia in pregnancy in a modern, healthy United States cohort is needed.

Objective: To define anemia in pregnancy in a United States population including a large county vs.

Efficacy and safety of maralixibat treatment in patients with Alagille syndrome and cholestatic pruritus (ICONIC): a randomised phase 2 study.

Background: Alagille syndrome is a rare genetic disease that often presents with severe cholestasis and pruritus. There are no approved drugs for management. Maralixibat, an apical, sodium-dependent, bile acid transport inhibitor, prevents enterohepatic bile acid recirculation.

The role of radiation therapy in the management of cutaneous malignancies. Part II: When is radiation therapy indicated?

Radiation therapy may be performed for a variety of cutaneous malignancies, depending on patient health status, tumor clinical and histologic features, patient preference, and resource availability. Dermatologists should be able to recognize the clinical scenarios in which radiation therapy is appropriate, as this may reduce morbidity, decrease risk of disease recurrence, and improve quality of life. The second article in this 2-part continuing medical education series focuses on the most common indications for radiation therapy in the treatment of basal cell carcinoma, cutaneous squamous cell carcinoma, dermatofibrosarcoma protuberans, Merkel cell carcinoma, Kaposi sarcoma, angiosarcoma, cutaneous lymphoma, melanoma, undifferentiated pleomorphic sarcoma, and sebaceous carcinoma.

The role of radiation therapy in the management of cutaneous malignancies. Part I: Diagnostic modalities and applications.

Radiation therapy offers distinct advantages over other currently available treatments for cutaneous malignancies in certain circumstances. Dermatologists and dermatologic surgeons should be familiar with the available radiation therapy techniques as well as their value and potential limitations in a variety of clinical scenarios. The first article in this 2-part continuing medical education series highlights the mechanisms, modalities, and applications of the most commonly used radiotherapy treatments as they relate to cutaneous oncology.

What influences demand for cigars among African American adult cigar smokers? Results from a hypothetical purchase task.

African Americans (AA) have historically been targeted by the tobacco industry and have the highest rates of current cigar use among racial/ethnic groups in the U.S. Yet, there is limited evidence on other factors influencing cigar use.

Hypoglycemic episodes predict length of stay in patients with acute burns.

Hypoglycemic episodes are associated with worse hospital outcomes. All adult patients admitted to our burn center from 2015 to 2019 were retrospectively reviewed. Patient demographics and burn characteristics were recorded.

The Impact of Peer Support Group Programs on Psychosocial Outcomes for Burn Survivors and Caregivers: A Review of the Literature.

Peer support group programs are often recommended for burn survivors as a way to facilitate their psychosocial recovery and reintegration into the community. Such programs provide opportunities for burn patients and their caretakers to access emotional and informational support from healthcare providers and other survivors in inpatient or outpatient settings. Despite their popularity, however, there is little information currently available on the efficacy of these groups.

Invasive Fungal Infections after Anti-CD19 Chimeric Antigen Receptor-Modified T-Cell Therapy: State of the Evidence and Future Directions.

Studies describing invasive fungal infections (IFIs) after chimeric antigen receptor-modified T-cell (CAR-T-cell) therapy are limited. Although post-CAR-T-cell IFIs appear to be uncommon, they are associated with significant morbidity and mortality. Specific risk factors for IFIs in CAR-T-cell recipients have not been fully characterized and are often extrapolated from variables contributing to IFIs in patients with other hematologic malignancies or those undergoing hematopoietic cell transplant.

The Impact of No Next of Kin Decision Makers on End-of-Life Care.

For critically ill burn patients without a next of kin, the medical team is tasked with becoming the surrogate decision maker. This poses ethical and legal challenges for burn providers. Despite this frequent problem, there has been no investigation of how the presence of a next of kin affects treatment in burn patients.

Infections after anti-CD19 chimeric antigen receptor T-cell therapy for hematologic malignancies: timeline, prevention, and uncertainties.

Purpose Of Review: Data on the infectious complications of anti-CD19 chimeric antigen receptor-modified T-cell (CAR-T-cell) therapies are scant. The approaches to preventing and managing infections among CAR-T-cell recipients are extrapolated from those of patients with other hematological malignancies. Understanding the incidence and risk factors of infections in these patients will improve clinical outcomes.

Venous Thromboembolism Chemoprophylaxis in Burn Patients: A Literature Review and Single-Institution Experience.

Hospitalized burn patients meet the criteria for Virchow's triad (endothelial damage, hypercoagulability, and stasis), predisposing them to venous thromboembolism (VTE). Although the disease burden of VTE suggests a need for prevention in this population, unreliable reported VTE rates, costly and complicated prophylaxis regimens, and chemoprophylaxis risks have prevented the establishment of a universal protocol. This paper reviews thromboprophylaxis practices both in the literature and at our own institution.

Model-Informed Drug Development of the Masked Anti-PD-L1 Antibody CX-072.

CX-072 is an anti-PD-L1 (programmed death ligand 1) Probody therapeutic (Pb-Tx) designed to be preferentially activated by proteases in the tumor microenvironment and not in healthy tissue. Here, we report the model-informed drug development of CX-072. A quantitative systems pharmacology (QSP) model that captured known mechanisms of Pb-Tx activation, biodistribution, elimination, and target engagement was used to inform clinical translation.

Small Pediatric Burns Can Be Safely Managed on an Outpatient Basis.

American Burn Association (ABA) guidelines recommend that all pediatric burns be transferred to a burn center if their presenting hospital lacks the necessary personnel or equipment for their care. Our institution often treats small burns (<10% TBSA) in pediatric patients in an ambulatory setting with a nondaily dressing. The aim of this study was to determine whether small pediatric burns could be safely managed on an outpatient basis.