Lessons learnt in the first year of an Australian pediatric cardio oncology clinic.

Background: Modern oncological therapies together with chemotherapy and radiotherapy have broadened the agents that can cause cardiac sequelae, which can manifest for pediatric oncology patients while on active treatment. Recommendations for high-risk patients who should be monitored in a pediatric cardio-oncology clinic have previously been developed by expert Delphi consensus by our group. In 2022 we opened our first multidisciplinary pediatric cardio-oncology clinic adhering to these recommendations in surveillance and management.

Cardio-Oncology Recommendations for Pediatric Oncology Patients: An Australian and New Zealand Delphi Consensus.

Cardio-oncology is a new multidisciplinary area of expertise that seeks to pre-emptively and proactively address cardiac complications that emerge during and following cancer therapy. Modern therapies including molecular targeted therapy and immunotherapy have broadened the agents that can cause cardiac sequelae, often with complications arising within days to weeks of therapy. Several international guidelines have been developed for the acute monitoring of cardio-oncology side effects.

Standardization of Fertility Preservation Discussion Among Pediatric Oncology and Bone Marrow Transplant Patients: A Single Institution Experience.

Infertility is an impactful late effect of cancer therapy. Options for fertility preservation exist, however, barriers remain. Within our division, we lacked a standard approach to discussing fertility preservation.

ETV6 germline mutations cause HDAC3/NCOR2 mislocalization and upregulation of interferon response genes.

ETV6 is an ETS family transcription factor that plays a key role in hematopoiesis and megakaryocyte development. Our group and others have identified germline mutations in ETV6 resulting in autosomal dominant thrombocytopenia and predisposition to malignancy; however, molecular mechanisms defining the role of ETV6 in megakaryocyte development have not been well established. Using a combination of molecular, biochemical, and sequencing approaches in patient-derived PBMCs, we demonstrate abnormal cytoplasmic localization of ETV6 and the HDAC3/NCOR2 repressor complex that led to overexpression of HDAC3-regulated interferon response genes.

Cost-Effectiveness of the International Late Effects of Childhood Cancer Guideline Harmonization Group Screening Guidelines to Prevent Heart Failure in Survivors of Childhood Cancer.

Purpose: Survivors of childhood cancer treated with anthracyclines and/or chest-directed radiation are at increased risk for heart failure (HF). The International Late Effects of Childhood Cancer Guideline Harmonization Group (IGHG) recommends risk-based screening echocardiograms, but evidence supporting its frequency and cost-effectiveness is limited.

Patients And Methods: Using the Childhood Cancer Survivor Study and St Jude Lifetime Cohort, we developed a microsimulation model of the clinical course of HF.

Malignant Granular Cell Tumor of the Lower Extremity in an Adolescent Male.

Granular cell tumors (GCTs) are extremely rare soft tissue tumors, with only 2% of tumors being malignant. Malignant GCTs are more often seen in women between the ages 40 and 60. There has been no case reported of a malignant GCT in a pediatric patient.

Implementing a stepwise educational approach for bridging the gap between specialty and primary care for childhood cancer survivors.

Purpose: To create a community of learning involving primary care providers and subspecialist to enhance providers' knowledge regarding care of adult childhood cancer survivors (CCS).

Methods: A stepwise approach was used to develop educational opportunities for providers. This process started with a local/regional in-person conference, which informed a webinar series, and resulted in the development of enduring material using a dynamic learning management system.

Desensitization to pegaspargase in children with acute lymphoblastic leukemia and lymphoblastic lymphoma.

Hypersensitivity to pegaspargase is associated with inferior survival in pediatric patients with acute lymphoblastic leukemia and lymphoblastic lymphoma. In the past year, drug-supply shortages have led to the lack of an available alternative to pegaspargase. Rather than omit asparaginase from the treatment of acute lymphoblastic leukemia or lymphoblastic lymphoma patients with hypersensitivity to pegaspargase, we continued pegaspargase treatments for nine pediatric patients, utilizing a rapid desensitization protocol.

Utility of pediatric female fertility preservation discussions following pelvic radiation.

Introduction: While many childhood cancers are curable with therapy, adverse consequences in fertility exist. We sought to assess the number of female patients with pelvic tumors receiving radiation therapy, and the proportion that undergo measures for fertility preservation (FP).

Methods: A total of 53 female patients treated with pelvic tumors from 2000 to 2016 were retrospectively identified.

Comparative oncology approach to drug repurposing in osteosarcoma.

Background: Osteosarcoma is an orphan disease for which little improvement in survival has been made since the late 1980s. New drug discovery for orphan diseases is limited by the cost and time it takes to develop new drugs. Repurposing already approved FDA-drugs can help overcome this limitation.

Establishment of Personalized Pain Goals in Oncology Patients to Improve Care and Decrease Costs.

Purpose: Cancer-related pain is common, negatively affects quality of life and survival, and often requires treatment with opioid analgesics. Patient-reported data that describe the incidence and severity of pain, medication use, and patient satisfaction with care are lacking.

Methods: We analyzed 18 months of outpatient oncology clinic encounters from the electronic medical record to obtain data on pain levels and opioid and nonopioid treatments.

Medium-term assessment of cardiac function in pediatric cancer survivors. Comparison of different echocardiographic methods, cardiac MRI and cardiac biomarker testing in adolescent cancer survivors.

Purpose: To evaluate the feasibility and correlation of 3D echocardiography (echo) and cardiac biomarkers with cardiac MRI, in surveillance of cardiac function for cancer survivors.

Methods: Subjects ≥10 years of age who have survived >2 years after completion of cancer treatment from a single center were enrolled. Cardiac MRI and 3D echo images were obtained on the same day when routine echo was obtained.

Germline hemizygous deletion of locus in a patient presenting with Li-Fraumeni syndrome.

Li-Fraumeni syndrome (LFS) is a rare cancer predisposition syndrome usually associated with germline alterations. Its genetic basis in wild-type pedigrees is less understood. Using whole-genome sequencing, we identified a germline hemizygous deletion ablating in a wild-type patient presenting with high-grade sarcoma, laryngeal squamous cell carcinoma and a family history suggestive of LFS.

Cardiomyopathy in Childhood Cancer Survivors: Lessons from the Past and Challenges for the Future.

Childhood cancer survivors are at substantial risk for cancer treatment-related cardiomyopathy. Identification of those at highest risk has presented a longstanding challenge for survivorship researchers. To date, risk stratification approaches to screening and subsequent intervention have largely been driven by demographic and treatment-related exposures, possibly missing an opportunity for a more personalized approach.

Phototherapy induced cutaneous eruption in a neonate with transient porphyrinemia.

Unlabelled: Phototherapy for hyperbilirubinemia has rare complications. We report a case of phototherapy induced eruption in a neonate with transient porphyrinemia. Our patient received phototherapy due to hyperbilirubinemia secondary to erythroblastosis fetalis (hemolytic disease of the newborn).

Assessment of S-NPP VIIRS On-orbit Radiometric Calibration and Performance.

The VIIRS instrument on board the S-NPP spacecraft has successfully operated for more than four years since its launch in October, 2011. Many VIIRS environmental data records (EDR) have been continuously generated from its sensor data records (SDR) with improved quality, enabling a wide range of applications in support of users in both the operational and research communities. This paper provides a brief review of sensor on-orbit calibration methodologies for both the reflective solar bands (RSB) and the thermal emissive bands (TEB) and an overall assessment of their on-orbit radiometric performance using measurements from instrument on-board calibrators (OBC) as well as regularly scheduled lunar observations.

Analysis of redox and apoptotic effects of anthracyclines to delineate a cardioprotective strategy.

Purpose: Cardiotoxic side effects of anthracyclines limit their use as effective chemotherapeutics. One mechanistic model of anthracycline-induced cardiotoxicity is attributed to the generation of intracellular reactive oxygen species (ROS). However, this theory has been questioned because several cardioprotective strategies have included the use of antioxidants without significant clinical benefit.

A Collaborative Step-Wise Process to Implementing an Innovative Clinic for Adult Survivors of Childhood Cancer.

With a 5 year survival rate of approximately 80%, there is an increasing number of childhood cancer survivors in the United States. Childhood cancer survivors are at an increased risk for physical and psychosocial health problems many years after treatment. Long-term follow-up care should include education, development of individualized follow up plans and screening for health problems in accordance with the Children's Oncology Group survivor guidelines.

A Case of Immune Thrombocytopenic Purpura After Rabies Vaccination.

We describe a case of immune thrombocytopenic purpura (ITP) occurring 15 days after the first dose of a 4-dose rabies vaccination series. ITP is thought to be an immune-mediated process triggered by an infection or toxin. There is little evidence in the literature beyond case reports of an association of ITP with vaccines other than with the measles, mumps, and rubella vaccine.

Humanism and professionalism education for pediatric hematology-oncology fellows: A model for pediatric subspecialty training.

Background: Humanism and professionalism are virtues intrinsic to the practice of medicine, for which we lack a standard, evidence-based approach for teaching and evaluation. Pediatric hematology-oncology (PHO) fellowship training brings new and significant stressors, making it an attractive setting for innovation in humanism and professionalism training.

Procedure: We electronically surveyed a national sample of PHO fellows to identify fellows' educational needs in humanism and professionalism.

Childhood cancer for the primary care physician.

Childhood cancer is rare among childhood diseases and requires a high index of suspicion by the primary care physician to entertain the possibility of cancer when managing common childhood diseases. This article presents an overview of common pediatric cancers, their presentations, and how the primary care physician can work up patients whom they suspect have a malignancy. The goal is to help primary care doctors in early recognition and appropriate referral of patients, in order for patients to receive required specialized care in a timely manner.