Hospital for Sick Children

About: Hospital for Sick Children is a healthcare organization based out in Toronto, Ontario, Canada. It is known for research contribution in the topics: Population & Medicine. The organization has 4097 authors who have published 3746 publications receiving 129066 citations. The organization is also known as: Sick Kids Hospital & SickKids.

Factors associated with childhood cancer survivors’ knowledge about their diagnosis, treatment, and risk for late effects

Abstract: While most children with cancer survive their initial disease, cancer therapy places them at risk for late effects (LE). Knowledge of their diagnosis, treatment, and LE risk may motivate survivors to attend long-term follow-up care. The aims of this study were to examine knowledge of cancer history and future risks, and to identify factors associated with such knowledge, in a cohort of childhood cancer survivors. Survivors (i.e., patients finished cancer treatment, regardless of time since completion) aged 15 to 26 years from three Canadian cancer centers were invited to complete a questionnaire that assessed knowledge of cancer history and potential LE of treatments, including five specific LE known to have considerable long-term health impact. Clinical data were extracted from hospital records and used to validate participants’ answers. Of 250 participants, 16 (6 %) were unable to name their cancer, 79 (32 %) had partial or no knowledge of their therapy, and 83 (33 %) were unaware of at least some of their risks for LE. Decreasing age (OR for increase in age = 1.2 (1.1–1.4)), having had a renal tumor compared to leukemia (OR = 0.3 (0.1–0.9)), and lacking knowledge about treatment (OR = 0.4 (0.2–0.9)) were associated with lack of knowledge of LE. Of the five, the most and least familiar LE was LE associated with impaired pulmonary function and risk of second malignancy, respectively. This study highlights knowledge deficits in survivors, specifically regarding their risk for LE. Findings can be utilized to target survivors at risk for knowledge deficits.

A theory-driven training programme in the use of emerging commercial technology: Application to an adolescent with severe memory impairment

Abstract: We describe a theory-driven memory intervention programme for training individuals with moderate to severe memory impairment in the use of emerging commercial technology. Here we demonstrate the application of the programme to training MK, an 18-year-old woman with severe memory impairment following treatment for a suprasellar germinoma, to autonomously use a smartphone to support her day-to-day memory. A within-subject A1B1A2B2 single-case experimental design was used to evaluate the impact of smartphone use on MK's real-life functioning. Following intervention MK showed increased confidence in dealing with memory-demanding situations and generalised smartphone use across all aspects of her life as quantified by several and varied ecologically valid measures including a phone call schedule, behaviour memory observations and questionnaires. Moreover the intervention also benefited her family as indicated by a sustained reduction in caregiver strain and an increase in reported quality of life. These findin...

Type II Citrullinemia (Citrin Deficiency): A Mysterious Disease caused by a Defect of Calcium-Binding Mitochondrial Carrier Protein

Abstract: Citrullinemia (OMIM 215700) (McKusick, 1998) is an autosomal recessive disease that is caused by a deficiency of argininosuccinate synthetase (ASS; EC 6.3.4.5). The clinical, biochemical and molecular aspects of citrullinemia have been reviewed elsewhere (Walser, 1983; Saheki et al., 1987a; McKusick, 1998). So far, we have analyzed almost 200 patients with citrullinemia in our laboratory and have classified them into three types according to enzyme abnormality and into two forms according to pathogenesis (Figure 1) (Saheki et al., 1981, 1985a, 1987a, b; Kobayashi et al., 1993, 1999). The first form is the classical form (CTLN1) found in most patients with neonatal/infantile-onset citrullinemia (type I and type III), first described by (1962); the second form is the adult-onset type II citrullinemia (CTLN2) caused by a liver-specific ASS deficiency. In CTLN1, the enzyme defect is found in all tissues and cells in which ASS is expressed (Saheki et al., 1980, 1981, 1982, 1983a, 1985a, b, 1987a, b). To date, we have identified 36 mutations in the ASS gene located on chromosome 9q34 and have clarified the pathogenesis of most CTLN1 patients at the molecular level (Kobayashi et al., 1987, 1990, 1991, 1994, 1995a; Kakinoki et al., 1997).

Predicting acute ovarian failure in female survivors of childhood cancer: a cohort study in the Childhood Cancer Survivor Study (CCSS) and the St Jude Lifetime Cohort (SJLIFE).

Abstract: Summary Background Cancer treatment can cause gonadal impairment. Acute ovarian failure is defined as the permanent loss of ovarian function within 5 years of cancer diagnosis. We aimed to develop and validate risk prediction tools to provide accurate clinical guidance for paediatric patients with cancer. Methods In this cohort study, prediction models of acute ovarian failure risk were developed using eligible female US and Canadian participants in the Childhood Cancer Survivor Study (CCSS) cohort and validated in the St Jude Lifetime Cohort (SJLIFE) Study. 5-year survivors from the CCSS cohort were included if they were at least 18 years old at their most recent follow-up and had complete treatment exposure and adequate menstrual history (including age at menarche, current menstrual status, age at last menstruation, and menopausal aetiology) information available. Participants in the SJLIFE cohort were at least 10-year survivors. Participants were excluded from the prediction analysis if they had an ovarian hormone deficiency, had missing exposure information, or had indeterminate ovarian status. The outcome of acute ovarian failure was defined as permanent loss of ovarian function within 5 years of cancer diagnosis or no menarche after cancer treatment by the age of 18 years. Logistic regression, random forest, and support vector machines were used as candidate methods to develop the risk prediction models in the CCSS cohort. Prediction performance was evaluated internally (in the CCSS cohort) and externally (in the SJLIFE cohort) using the areas under the receiver operating characteristic curve (AUC) and the precision-recall curve (average precision [AP; average positive predictive value]). Findings Data from the CCSS cohort were collected for participants followed up between Nov 3, 1992, and Nov 25, 2016, and from the SJLIFE cohort for participants followed up between Oct 17, 2007, and April 16, 2012. Of 11 336 female CCSS participants, 5886 (51·9%) met all inclusion criteria for analysis. 1644 participants were identified from the SJLIFE cohort, of whom 875 (53·2%) were eligible for analysis. 353 (6·0%) of analysed CCSS participants and 50 (5·7%) of analysed SJLIFE participants had acute ovarian failure. The overall median follow-up for the CCSS cohort was 23·9 years (IQR 20·4–27·9), and for SJLIFE it was 23·9 years (19·0–30·0). The three candidate methods (logistic regression, random forest, and support vector machines) yielded similar results, and a prescribed dose model with abdominal and pelvic radiation doses and an ovarian dose model with ovarian radiation dosimetry using logistic regression were selected. Common predictors in both models were history of haematopoietic stem-cell transplantation, cumulative alkylating drug dose, and an interaction between age at cancer diagnosis and haematopoietic stem-cell transplant. External validation of the model in the SJLIFE cohort produced an estimated AUC of 0·94 (95% CI 0·90–0·98) and AP of 0·68 (95% CI 0·53–0·81) for the ovarian dose model, and AUC of 0·96 (0·94–0·97) and AP of 0·46 (0·34–0·61) for the prescribed dose model. Based on these models, an online risk calculator has been developed for clinical use. Interpretation Both acute ovarian failure risk prediction models performed well. The ovarian dose model is preferred if ovarian radiation dosimetry is available. The models, along with the online risk calculator, could help clinical discussions regarding the need for fertility preservation interventions in girls and young women newly diagnosed with cancer. Funding Canadian Institutes of Health Research, Women and Children's Health Research Institute, National Cancer Institute, and American Lebanese Syrian Associated Charities.

Excellent Outcomes With Reduced Frequency of Vincristine and Dexamethasone Pulses in Standard-Risk B-Lymphoblastic Leukemia: Results From Children's Oncology Group AALL0932.

Abstract: Purpose:AALL0932 evaluated two randomized maintenance interventions to optimize disease-free survival (DFS) while reducing the burden of therapy in children with newly diagnosed NCI standard-risk (...