Atezolizumab for Advanced Alveolar Soft Part Sarcoma.

Background: Alveolar soft part sarcoma (ASPS) is a rare soft-tissue sarcoma with a poor prognosis and no established therapy. Recently, encouraging responses to immune checkpoint inhibitors have been reported.

Methods: We conducted an investigator-initiated, multicenter, single-group, phase 2 study of the anti-programmed death ligand 1 (PD-L1) agent atezolizumab in adult and pediatric patients with advanced ASPS.

Vincristine Side Effects With Concomitant Fluconazole Use During Induction Chemotherapy in Pediatric Acute Lymphoblastic Leukemia.

As a mainstay of treatment for acute lymphoblastic leukemia (ALL), vincristine's side effect profile is well known. Parallel administration of the antifungal fluconazole has been shown to interfere with the metabolism of vincristine, potentially resulting in increased side effects. We conducted a retrospective chart review to determine whether concomitant administration of vincristine and fluconazole during pediatric ALL induction therapy impacted the frequency of vincristine side effects, namely, hyponatremia and peripheral neuropathy.

Pediatric Chronic Myelogenous Leukemia in T-lineage Blast Crisis: A Reminder in Relevance.

Pediatric Philadelphia chromosome positive (Ph+) acute T-cell lymphoblastic leukemia can mimic chronic myelogenous leukemia (CML) in T-lineage blast crisis (BC). Differentiating the 2 is critical in guiding therapy as most children with de novo Ph+ acute T-cell lymphoblastic leukemia are treated with chemotherapy and tyrosine kinase inhibitors, whereas T-lineage BC of CML can include hematopoietic stem cell transplantation. We present a unique case of CML in T-lineage BC.

Interdisciplinary care of children with trisomy 13 and 18.

Children with trisomy 13 and 18 (previously deemed "incompatible with life") are living longer, warranting a comprehensive overview of their unique comorbidities and complex care needs. This Review Article provides a summation of the recent literature, informed by the study team's Interdisciplinary Trisomy Translational Program consisting of representatives from: cardiology, cardiothoracic surgery, neonatology, otolaryngology, intensive care, neurology, social work, chaplaincy, nursing, and palliative care. Medical interventions are discussed in the context of decisional-paradigms and whole-family considerations.

Pediatric Acute Lymphoblastic Leukemia, Version 2.2020, NCCN Clinical Practice Guidelines in Oncology.

Acute lymphoblastic leukemia (ALL) is the most common pediatric malignancy. Advancements in technology that enhance our understanding of the biology of the disease, risk-adapted therapy, and enhanced supportive care have contributed to improved survival rates. However, additional clinical management is needed to improve outcomes for patients classified as high risk at presentation (eg, T-ALL, infant ALL) and who experience relapse.

A Review of Apps for Calming, Relaxation, and Mindfulness Interventions for Pediatric Palliative Care Patients.

Patients and families increasingly use mobile apps as a relaxation and distraction intervention for children with complex, chronic medical conditions in the waiting room setting or during inpatient hospitalizations; and yet, there is limited data on app quality assessment or review of these apps for level of engagement, functionality, aesthetics, or applicability for palliative pediatric patients. The pediatric palliative care study team searched smartphone application platforms for apps relevant to calming, relaxation, and mindfulness for pediatric and adolescent patients. Apps were reviewed using a systematic data extraction tool.

Physician approaches to drug shortages: Results of a national survey of pediatric hematologist/oncologists.

Aim: To evaluate personnel involved in scarce drug prioritization and distribution and the criteria used to inform drug distribution during times of shortage among pediatric hematologists/oncologists.

Methods: Using the American Society of Pediatric Hematology/Oncology (ASPHO) membership list, a 20 question survey of pediatric hematologists/oncologists was conducted email to evaluate personnel involved in scarce drug prioritization and distribution and criteria used to inform scarce drug distribution.

Results: Nearly 65% of the 191 study respondents had patients directly affected by drug shortages.

An ethical framework for responding to drug shortages in pediatric oncology.

The frequency of drug shortages has increased considerably over the last decade. Important ethical issues arise whenever the supply of an effective drug is insufficient to meet demand. Using the ethical principles of beneficence, non-maleficence, and justice, institutions can guide prioritization of drug distribution before a shortage occurs to avoid unfair and unethical distribution of resources.

Liver transplant for congenital factor VII deficiency.

Congenital factor VII (FVII) deficiency is a rare, autosomal recessive bleeding disorder with a spectrum of phenotypes ranging from asymptomatic to life-threatening intra-cranial hemorrhage (ICH). Orthotopic liver transplantation has been described for definitive treatment in a few patients with severe manifestations. We report a patient with congenital FVII deficiency and recurrent ICH, despite twice-weekly prophylaxis with recombinant activated FVII.

Improving pediatric hematology/oncology care in the emergency department.

Purpose: Pediatric hematology/oncology patients frequently use the emergency department (ED) for prompt care during potentially life-threatening events, such as sepsis and bleeding. One challenge of these visits is the unavailability of appropriate patient-specific medical information. Lack of information may result in ineffective ED visits for these patients with complex conditions.

Impact of cytomegalovirus (CMV) reactivation after umbilical cord blood transplantation.

This study investigated the impact of pretransplant cytomegalovirus (CMV) serostatus and posttransplant CMV reactivation and disease on umbilical cord blood transplant (UCBT) outcomes. Between 1994 and 2007, 332 patients with hematologic malignancies underwent UCBT and 54% were CMV seropositive. Pretransplant recipient CMV serostatus had no impact on acute or chronic graft-versus-host disease (aGVHD, cGVHD), relapse, disease-free survival (DFS), or overall survival (OS).

Cutaneous leishmaniasis with boggy induration and simultaneous mucosal disease.

A woman had cutaneous, mucosal, and possible visceral leishmaniasis simultaneously. Many of her cutaneous lesions consisted of boggy indurations rather than customary papules, nodules, or ulcers. This unusual case was finally cured after four courses of miltefosine, one course of antimony, and two courses of Ambisome.

Training the clinical eye and mind: using the arts to develop medical students' observational and pattern recognition skills.

Introduction: Observation, including identification of key pieces of data, pattern recognition, and interpretation of significance and meaning, is a key element in medical decision making. Clinical observation is taught primarily through preceptor modelling during the all-important clinical years. No single method exists for communicating these skills, and medical educators have periodically experimented with using arts-based training to hone observational acuity.

Pectoralis major myocutaneous flap vs revascularized free tissue transfer: complications, gastrostomy tube dependence, and hospitalization.

Objective: To evaluate the factors related to surgical complications, rate of gastrostomy tube (G-tube) dependence, and hospitalization in patients undergoing reconstruction with a pectoralis myocutaneous flap vs a soft-tissue revascularized flap.

Design: Quasi-experimental case series with a historic control group.

Population: A total of 179 patients (138 men and 41 women) with a mean (SD) age of 58 (14) years treated between January 1, 1986, and December 31, 1995, with a pectoralis flap (108 patients) or a revascularized free flap (71 patients).