Treatment Refractory Soft Tissue Myoepithelial Carcinoma With an ARID1A Mutation.

Soft tissue myoepithelial carcinoma is a rare tumor first reported in the salivary gland. There is considerable tumor heterogeneity between pathology findings, tumor aggressiveness, and response to treatment. Recent molecular testing has identified recurrent genetic changes with PLAG mutations in salivary gland primary tumors and loss of SMARCB1 and EWSR1/FUS gene changes in myoepithelial carcinoma. SMARCB1 is a component of the switch/sucrose nonfermentable (SWI/SNF) complex, an essential cellular regulator. ARID1A is another SWI/SNF complex subunit and is a potent oncogenic driver in other tumor types. In this case, we describe the case of an adolescent/young adult patient with treatment refractory soft tissue myoepithelial carcinoma and a previously unreported ARID1A mutation.

Affirming Hormone Treatment for a Transgender Adolescent After a Venous Thromboembolic Event.

BACKGROUND: Medical affirmation, including gender-affirming hormones, is an essential component in the treatment of many transgender and gender-diverse youth. The risk of venous thromboembolism (VTE) during testosterone therapy for gender-affirming care is not fully elucidated. OBSERVATION: The case describes a 17-year-old transgender male treated with testosterone therapy who presented with an occlusive deep vein thrombosis of right axillary and subclavian veins. Testosterone level was 920 ng/dL at the time of the deep vein thrombosis, and he had no risk factors for VTE. A complete hypercoagulable workup was negative. CONCLUSIONS: The possibility of testosterone therapy as a risk factor for VTE may suggest the need to include this information during informed consent discussions. Long-term anticoagulation may be considered for those restarting testosterone therapy.

A Novel Use of Romiplostim for SARS-CoV-2-induced Thrombocytopenia.

The literature regarding coronavirus disease of 2019 (COVID-19) infection in pediatrics indicates that children have less severe clinical presentations and lower mortality rates. There remains limited data regarding hematologic sequelae in pediatric patients with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Romiplostim has shown a platelet response in pediatric patients with chronic immune thrombocytopenic purpura, and eltrombopag is proven to increase platelet counts in patients with inherited thrombocytopenia. We review SARS-CoV-2-associated thrombocytopenia and present a pediatric patient with acute on chronic thrombocytopenia in the setting of COVID-19 with subsequent platelet recovery using romiplostim.

Treatment response with sirolimus for a pediatric patient with an EBV-associated smooth-muscle tumor after bone marrow transplantation.

Epstein-Barr virus-associated smooth-muscle tumors (EBV-SMTs) are unique and rare neoplasms described in immunocompromised patients. The case describes a nine-year-old female with a history of acute lymphoblastic leukemia with relapse and subsequent allogeneic bone marrow transplantation who presented with multiple EBV-SMTs of the liver. EBV utilizes the mammalian target of rapamycin (mTOR) pathway for tumor growth, and sirolimus, a mTOR inhibitor, has shown to result in a short-term response. We now report an extended treatment response with sirolimus in a pediatric patient with an EBV-SMT.

A Quality Improvement Initiative to Reduce Unnecessary Rapid Responses Using Early Warning Scores.

OBJECTIVES: The Pediatric Early Warning Score (PEWS) is an evidence-based tool that allows early collaborative assessment and intervention for a rapid response team (RRT) activation. The goal of our quality improvement initiative was to reduce the percentage of unnecessary RRT activations by 50% over 2 years without increasing PICU transfers or compromising patient safety and timely evaluation. METHODS: A PEWS system replaced preexisting vital signs-based pediatric RRT criteria and was modified through plan-do-study-act cycles. Unnecessary RRT activations, total RRT activation rate, transfers to the PICU, total clinical interventions performed per RRT, and missed RRT activation rate were compared between intervention periods. Likert scale surveys were administered to measure satisfaction with each modification. RESULTS: There was a significant decrease in the percentage of unnecessary RRT activations from 33% to 3.5% after the implementation of the PEWS and modified-PEWS systems (P < .05). The RRT activation rate decreased from 22.6 to 13.3 RRT activations per 1000 patient care days after implementation of the PEWS and modified-PEWS systems (P < .05), without changes in PICU transfer rates. Physicians reported that the PEWS system improved nursing communication and accuracy of RRT criteria (P < .05). Nursing reported that the PEWS system improved patient management and clinical autonomy (P < .05). CONCLUSIONS: The PEWS systems have been an effective means of identifying deteriorating pediatric patients and reducing unnecessary RRT activations. The new system fosters collaboration and communication at the bedside to prevent acute deterioration, perform timely interventions, and ultimately improve patient safety and outcomes.

HIV drug resistance early warning indicators in namibia with updated World Health Organization guidance.

BACKGROUND: In response to concerns about the emergence of HIV drug resistance (HIVDR), the World Health Organization (WHO) has developed a comprehensive set of early warning indicators (EWIs) to monitor HIV drug resistance and good programme practice at antiretroviral therapy (ART) sites. METHODS: In 2012, Namibia utilized the updated WHO EWI guidance and abstracted data from adult and pediatric patients from 50 ART sites for the following EWIs: 1. On-time Pill Pick-up, 2. Retention in Care, 3. Pharmacy Stock-outs, 4. Dispensing Practices, and 5. Virological Suppression. RESULTS: Data for EWIs one through four were abstracted and validated. EWI 5--Virological Suppression was not included due to poor data entry at many sites. On-time Pill Pick-up national estimate was 87.9% (87.2-88.7) of patients picking up pills on time for adults and 90.0% (88.9-90.9) picking up pills on time for pediatrics. Retention in Care national estimate was 82% of patients retained on ART after 12 months for adults and 83% for pediatrics. Pharmacy Stock-outs national estimate was 99% of months without a stock-out for adults and 97% for pediatrics. Dispensing Practices national estimate was 0.01% (0.003-0.064) of patients dispensed mono- or dual-therapy for adults and 0.25% (0.092-0.653) for pediatrics. CONCLUSIONS: The successful 2012 EWI exercise provides Namibia a solid evidence base, which can be used to make national statements about programmatic functioning and possible HIVDR. This evidence base will serve to contextualize results from Namibia's surveys of HIVDR, which involves genotype testing. EWI abstraction has prompted the national program and its counterparts to engage sites in dialogue regarding the need to strengthen adherence and retention of patients on ART. The EWI collection process and EWI results will serve to optimize patient care and support Namibia in making evidence-based recommendations and take action to minimize the emergence of preventable HIVDR.