Advice to Clinicians on Communication from Adolescents and Young Adults with Cancer and Parents of Children with Cancer.

Effective communication is integral to patient and family-centered care in pediatric and adolescent and young adult (AYA) oncology and improving healthcare delivery and outcomes. There is limited knowledge about whether AYAs and parents have similar communication preferences and needs. By eliciting and comparing communication advice from AYAs and parents, we can identify salient guidance for how clinicians can better communicate.

Attenuation of PKCδ enhances metabolic activity and promotes expansion of blood progenitors.

A finely tuned balance of self-renewal, differentiation, proliferation, and survival governs the pool size and regenerative capacity of blood-forming hematopoietic stem and progenitor cells (HSPCs). Here, we report that protein kinase C delta (PKCδ) is a critical regulator of adult HSPC number and function that couples the proliferative and metabolic activities of HSPCs. PKCδ-deficient mice showed a pronounced increase in HSPC numbers, increased competence in reconstituting lethally irradiated recipients, enhanced long-term competitive advantage in serial transplantation studies, and an augmented HSPC recovery during stress.

The phenotypic spectrum of germline variants: from isolated sideroblastic anemia to mitochondrial myopathy, lactic acidosis and sideroblastic anemia 2.

variants have previously been described in patients with myopathy, lactic acidosis and sideroblastic anemia 2 (MLASA2). encodes the mitochondrial tyrosyl-tRNA synthetase, which is responsible for conjugating tyrosine to its cognate mt-tRNA for mitochondrial protein synthesis. Here we describe 14 individuals from 11 families presenting with sideroblastic anemia and variants that we identified using a sideroblastic anemia gene panel or exome sequencing.

Refractory autoimmune disease: an overview of when first-line therapy is not enough.

A recent Intercontinental Cooperative ITP Study Group (ICIS) meeting in September 2015 focused on immunomodulation across the spectrum of autoimmune conditions. It became clear to the attendees that in this wide range of conditions, there is a subset of patients that remain highly refractory to first line therapy. Therapeutic approaches to these patients vary greatly and while many different immunomodulatory agents have been investigated, few have seen universal success.

Ethics and professionalism education during neonatal-perinatal fellowship training in the United States.

Objective: The objectives of this study were to determine the perceived adequacy of ethics and professionalism education for neonatal-perinatal fellows in the United States, and to measure confidence of fellows and recent graduates when navigating ethical issues.

Study Design: Neonatal-Perinatal Fellowship Directors, fellows and recent graduates were surveyed regarding the quality and type of such education during training, and perceived confidence of fellows/graduates in confronting ethical dilemmas.

Result: Forty-six of 97 Directors (47%) and 82 of 444 fellows/graduates (18%) completed the surveys.

Safety update on the use of recombinant activated factor VII in approved indications.

This updated safety review summarises the large body of safety data available on the use of recombinant activated factor VII (rFVIIa) in approved indications: haemophilia with inhibitors, congenital factor VII (FVII) deficiency, acquired haemophilia and Glanzmann's thrombasthenia. Accumulated data up to 31 December 2013 from clinical trials as well as post-marketing data (registries, literature reports and spontaneous reports) were included. Overall, rFVIIa has shown a consistently favourable safety profile, with no unexpected safety concerns, in all approved indications.

The role of Lin28b in myeloid and mast cell differentiation and mast cell malignancy.

Mast cells (MCs) are critical components of the innate immune system and important for host defense, allergy, autoimmunity, tissue regeneration and tumor progression. Dysregulated MC development leads to systemic mastocytosis (SM), a clinically variable but often devastating family of hematologic disorders. Here we report that induced expression of Lin28, a heterochronic gene and pluripotency factor implicated in driving a fetal hematopoietic program, caused MC accumulation in adult mice in target organs such as the skin and peritoneal cavity.

Increase in the incidence of differentiated thyroid carcinoma in children, adolescents, and young adults: a population-based study.

Objective: To investigate trends in incidence of differentiated thyroid carcinomas among children and adolescents and young adults.

Study Design: In this ecological time-trends study, we selected cases of differentiated thyroid carcinomas (1984-2010) in patients <30 years from Surveillance, Epidemiology, and End Results 9 cancer registries by using International Classification of Diseases for Oncology, 3rd edition, codes for papillary and follicular cancers. Patients with multiple other primary diseases before differentiated thyroid carcinomas were excluded.