The outcome in Australian children with hyperinsulinism of infancy: early extensive surgery in severe cases lowers risk of diabetes

AIMS Hyperinsulinism of infancy (HI) is characterized by unregulated insulin secretion in the presence of hypoglycaemia, often resulting in brain damage. Pancreatic resection for control of hypoglycaemia is frequently resisted because of the risk of diabetes mellitus (DM). We investigated retrospectively 62 children with HI from nine Australian treatment centres born between 1972 and 1998, comparing endocrine and neurological outcome in 28 patients receiving medical therapy alone with 34 who required pancreatic resection to control their hypoglycaemia. METHODS History, treatment and clinical course were ascertained from file audit and interview. Risk of DM (hazard ratio) attributable to age at surgery (< vs. greater than or equal to 100 days at last pancreatectomy) and extent of resection (< vs. greater than or equal to 95%) were calculated using Cox proportional hazards regression and categorical variables compared by the chi(2) -test. Neurological outcome (normal, mild deficit or severe deficit) was derived from the most authoritative source. RESULTS Surgically treated patients had a greater birthweight, earlier presentation and higher plasma insulin levels. Of 18 infants < 100 days and 16 greater than or equal to 100 days of age at surgery, four (all greater than or equal to 100 days) became diabetic as an immediate consequence of surgery and five (two < 100 days and three greater than or equal to 100 days) became diabetic 7-18 years later. Surgery greater than or equal to 100 days and pancreatectomy greater than or equal to 95% were associated with development of diabetes (HR = 12.61, CI 1.53-104.07 and HR = 7.03, CI 1.43-34.58, respectively). Neurodevelopmental outcome was no different between the surgical and medical groups with 44% overall with neurological deficits. Patients euglycaemic within 35 days of the first symptom of hypoglycaemia (Group A) had a better neurodevelopmental outcome than those still hypoglycaemic > 35 days from first presentation (Group B) (P = 0.007). Prolonged hypoglycaemia in Group B was due either to delayed diagnosis or to need for repeat surgery because of continued hypoglycaemia. Within Group A, medically treated patients (who presented later with apparently milder disease) had a higher incidence of neurodevelopmental deficit (n = 15, four mild, three severe deficit) compared with surgically treated patients (n = 18, two mild, none severe deficit) (P < 0.025). CONCLUSIONS Poor neurodevelopmental outcome remains a major problem in hyperinsulinism of infancy. Risk of diabetes mellitus with pancreatectomy varies according to age at surgery and extent of resection. Patients presenting early with severe disease have a better neurodevelopmental outcome and lower risk of diabetes if they are treated with early extensive surgery.

Parenting and adolescent self-regulation

This study examined the relationship between adolescents' academic and non-academic self-regulation (SR), authoritative parenting (as demonstrated by high levels of Involvement, Strictness, and Autonomy Granting), and parent self-efficacy in four areas. Participants were 214 Australian high school students and their parents. There was a moderate correlation (r = 0.63) between academic and non-academic SR. Adolescents and their parents differed significantly in their perceptions of parenting behaviours, with parents rating themselves higher than their children on Involvement, Autonomy Granting, and Strictness behaviours. A model of the relationships between the constructs was developed showing a strong path from parent self-efficacy to both academic and non-academic SR via high parental Involvement (as perceived by adolescents). Strict parenting and the granting by parents of psychological autonomy to their adolescent children did not appear to be important in the development of young people's self-regulatory behaviours. (C) 2004 The Association for Professionals in Services for Adolescents. Published by Elsevier Ltd. All rights reserved.

The handbook of physical education

What is the current condition of the field of physical education? How has it adapted to the rise of kinesiology, sport and exercise science and human movement studies over the last thirty years? This Handbook provides an authoritative critical overview of the field and identifies future challenges and directions. The Handbook is divided in to six parts: - Perspectives and Paradigms in Physical Education Pedagogy Research; - Cross-disciplinary Contributions to Research on Physical Education; - Learners and Learning in Physical Education; - Teachers, Teaching and Teacher Education in Physical Education; - Physical Education Curriculum; - Difference and Diversity in Physical Education. This benchmark work is essential reading for educators and students in the field of physical education.