Prospective longitudinal evaluation of treatment-related toxicity and health-related quality of life during the first year of treatment for pediatric acute lymphoblastic leukemia.

BACKGROUND: Pediatric acute lymphoblastic leukemia (ALL) therapy is accompanied by treatment-related toxicities (TRTs) and impaired quality of life. In Australia and New Zealand, children with ALL are treated with either Children's Oncology Group (COG) or international Berlin-Frankfurt-Munster (iBFM) Study Group-based therapy. We conducted a prospective registry study to document symptomatic TRTs (venous thrombosis, neurotoxicity, pancreatitis and bone toxicity), compare TRT outcomes to retrospective TRT data, and measure the impact of TRTs on children's general and cancer-related health-related quality of life (HRQoL) and parents' emotional well-being. METHODS: Parents of children with newly diagnosed ALL were invited to participate in the ASSET (Acute Lymphoblastic Leukaemia Subtypes and Side Effects from Treatment) study and a prospective, longitudinal HRQoL study. TRTs were reported prospectively and families completed questionnaires for general (Healthy Utility Index Mark 3) and cancer specific (Pediatric Quality of Life Inventory (PedsQL)-Cancer Module) health related quality of life as well the Emotion Thermometer to assess emotional well-being. RESULTS: Beginning in 2016, 260 pediatric patients with ALL were enrolled on the TRT registry with a median age at diagnosis of 59 months (range 1-213 months), 144 males (55.4%), majority with Pre-B cell immunophenotype, n = 226 (86.9%), 173 patients (66.5%) treated according to COG platform with relatively equal distribution across risk classification sub-groups. From 2018, 79 families participated in the HRQoL study through the first year of treatment. There were 74 TRT recorded, reflecting a 28.5% risk of developing a TRT. Individual TRT incidence was consistent with previous studies, being 7.7% for symptomatic VTE, 11.9% neurotoxicity, 5.4% bone toxicity and 5.0% pancreatitis. Children's HRQoL was significantly lower than population norms throughout the first year of treatment. An improvement in general HRQoL, measured by the HUI3, contrasted with the lack of improvement in cancer-related HRQoL measured by the PedsQL Cancer Module over the first 12 months. There were no persisting differences in the HRQoL impact of COG compared to iBFM therapy. CONCLUSIONS: It is feasible to prospectively monitor TRT incidence and longitudinal HRQoL impacts during ALL therapy. Early phases of ALL therapy, regardless of treatment platform, result in prolonged reductions in cancer-related HRQoL.

Silver Russel syndrome in an aboriginal patient from Australia.

Silver-Russell syndrome (SRS OMIM 180860) is a rare, albeit well-recognized disorder characterized by severe intrauterine and postnatal growth retardation. It remains a clinical diagnosis with a molecular cause identifiable in approximately 60%-70% of patients. We report a 4-year-old Australian Aboriginal girl who was born at 32 weeks gestation with features strongly suggestive of SRS, after extensive investigation she was referred to our undiagnosed disease program (UDP). Genomic sequencing was performed which identified a heterozygous splice site variant in IGF2 which is predicted to be pathogenic by in-silico studies, paternal allelic origin, de novo status, and RNA studies on fibroblasts. We compare clinical findings with reported patients to add to the knowledge base on IGF2 variants and to promote the engagement of other Australian Aboriginal families in genomic medicine.

RelAte: pilot study of the effects of a mealtime intervention on social cognitive factors and energy intake among older adults living alone.

Mealtime interventions typically focus on institutionalised older adults, but we wanted to investigate whether they may also be effective among those living independently. Using a randomised controlled trial design, we assessed the effects of a novel mealtime intervention on self-efficacy, food enjoyment and energy intake. A total of 100 adults living alone aged over 60 years were randomised to the treatment or control conditions: all received a guidebook on nutrition and culinary skills. Treatment group participants received a weekly visit from a trained volunteer who prepared and shared a meal with them. Participants in the treatment group showed improvements relative to those in the control group at borderline significance (P=0·054) for self-efficacy and at significance for food enjoyment. Significant improvements were observed in female participants in the treatment but not in the control group in energy intake (although following corrections for multiple comparisons, only the effect on food enjoyment remained significant). These findings will inform the design of future complex interventions. For this type of intervention to be successful, more focus has to be placed on making interventions more personalised, potentially according to sex. Findings are important for nutritional sciences as they indicate that, in order to improve energy intake and food enjoyment among older adults, multimodal nutritional interventions including social components may be successful.

Age- and Sex-Related Differences in Nonfatal Dog Bite Injuries Among Persons Aged 0-19 Treated in Hospital Emergency Departments, United States, 2001-2017.

OBJECTIVE: No studies using national data have evaluated changes in nonfatal dog bite injuries among children, adolescents, and young adults during the past 2 decades. We examined estimates of annual nonfatal dog bite injuries among persons aged 0-19 treated in US emergency departments (EDs) from 2001 through 2017. METHODS: We examined data from the Web-based Injury Statistics Query and Reporting System to compare estimates of the annual rates of nonfatal dog bite injuries in EDs among all persons aged 0-19, by age group (0-4, 5-9, 10-14, and 15-19), and by sex. We determined significance by comparing 95% confidence intervals (CIs), and we standardized rate calculations to the year 2000 population. RESULTS: During 2001-2017, an estimated 2 406 109 (95% CI, 2 102 643-2 709 573) persons aged 0-19 were treated in EDs for nonfatal dog bite injuries (age-adjusted rate of 173.1 per 100 000 persons aged 0-19). The estimated number of nonfatal dog bite injuries declined significantly, from 181 090 (95% CI, 158 315-203 864) in 2001 to 118 800 (95% CI, 97 933-139 667) in 2017. The highest rate was among children aged 5-9. Male children, adolescents, and young adults were significantly more likely than female children, adolescents, and young adults to be treated in the ED for nonfatal dog bite injuries, driven by male-female differences in children and adolescents aged 5-14. CONCLUSIONS: Although the estimated number of nonfatal dog bite injuries among persons aged 0-19 declined significantly in the past 2 decades, additional regulatory and educational efforts specific to age and sex may lead to further reductions in nonfatal dog bite injuries.