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.

Dissonance between conceptions of learning and ways of learning for indigenous Australian university students

This article reports on a phenomenographic investigation into conceptions of learning for 15 Indigenous Australian university students over the three years of their degree courses. The ways in which they went about learning were also investigated along with the relationship between individual students' 'core' conceptions of learning and the ways in which they learned. Results indicated that their conceptions and ways of learning were similar in some respects to those found for other university students. However, some students went about learning in ways that were incongruent with the core conception of learning they held. This can be regarded as dissonance between strategies and conceptions of learning. The implications of this for teaching and learning for such students are discussed.

Seclusion use with children and adolescents: an Australian experience

Objective: To summarize the current state of knowledge on the use of seclusion and restraint with children and adolescents and to report the findings of an exploratory study to identify factors that place a child or adolescent at increased risk of seclusion during their admission. Method: Literature searches were undertaken on MEDLINE, CINAHL and PsycINFO databases. Articles were identified that focused specifically on seclusion and restraint use with children and adolescents or contained material significant to this population. The study reports findings from a retrospective review of patient charts, seclusion registers and staffing from an Australian acute inpatient facility. Results: The data available in regard to seclusion use in this population is limited and flawed. Further research is needed on the use and outcomes of seclusion and restraint and on alternative measures in the containment of dangerousness. Both the literature and this study find that patients with certain factors are at increased risk of being secluded during an inpatient stay. These factors include being male, diagnoses of disruptive behaviour disorder and a previous history of physical abuse. Staffing factors did not show a relationship to the use of seclusion. Conclusions: There are patient factors that predict increased risk of seclusion; these factors and their interrelationships require further elucidation. Further research is also needed on the outcomes, both positive and negative, of seclusion use and of alternatives to seclusion.

Kidney volume, blood pressure, and albuminuria: Findings in an Australian Aboriginal community

Background. Australian Aborigines are experiencing epidemic proportions of renal disease, marked by albuminuria and, variably, hematuria. They also have high rates of low birth weight, which have been associated with lower kidney volumes and higher blood pressures. The authors evaluated relationships between kidney volume, blood pressure, albuminuria, and hematuria in 1 homogeneous group. Methods Forty-three percent (672 of 1,560) of the population in a remote coastal Australian Aboriginal community aged 4.4 to 72.1 years participated in the study. Results: Kidney size correlated closely with body size. Systolic blood pressure (SBP) was correlated inversely with kidney length and kidney volume, after adjusting for age, sex, and body surface area (BSA); a 1-cm increase in mean kidney length was associated with a 2.2-mm Hg decrease in SBP, and a 10-mL increase in mean kidney volume was associated with a 0.6-mm Hg decrease in SBP (P = 0.001). Mean kidney volume explained 10% of the variance in SBP in a multivariate model containing age, sex, and BSA. In addition to higher SBP, adults who had the lowest quartiles of kidney volume also had the highest levels of overt albuminuria (P = 0.044). Conclusion: Smaller kidneys predispose to higher blood pressures and albuminuria in this population. The lower volumes possibly represent kidneys with reduced nephron numbers, which might be related to an adverse intrauterine environment. Susceptibility to renal disease could be a direct consequence of reduced nephron numbers; the higher blood pressures with which they are associated could also contribute to, as well as derive from, this association.

Oral glucose tolerance test in children and adolescents: Positives and pitfalls

Objectives: To describe the glycaemic status (assessed by an oral glucose tolerance test (OGTT)) and associated comorbidities in a cohort of Australian children and adolescents at risk of insulin resistance and impaired glucose homeostasis (IGH). Methods: Twenty-one children and adolescents (three male, 18 female) (18 Caucasian, one Indigenous, two Asian) (20 obese, one lipodystrophy) referred to the Paediatric Endocrinology and Diabetes Clinic underwent a 2-h OGTT with plasma glucose and insulin measured at baseline, + 60 and + 120 min. If abnormal, the OGTT was repeated. Results: The mean (SD) age was 14.2 (1.6) years, BMI 38.8 (7.0) kg/m(2) and BMI-SDS 3.6 (0.6). Fourteen patients had fasting insulin levels >21 mU/L. Type 2 diabetes mellitus was diagnosed in one patient, impaired glucose tolerance (IGT) in four patients and impaired fasting glycaemia (IFG) in one patient. Despite no weight loss, only one patient had a persistently abnormal OGTT on repeat testing. Three patients with IGH were medicated with risperidone at the time of the initial OGTT. One patient who had persistent IGT had continued risperidone. The other two patients had initial OGTT results of IGT and diabetes mellitus type 2. They both ceased risperidone between tests and repeat OGTT showed normal glycaemic status. Conclusions: Use of fasting glucose alone may miss cases of IGH. Diagnosis of IGT should not be made on one test alone. Interpretation of glucose and insulin responses in young people is limited by lack of normative data. Larger studies are needed to generate Australian screening recommendations. Further assessment of the potential adverse effects of atypical antipsychotic medication on glucose homeostasis in this at-risk group is important.

A longitudinal study of learning for a group of indigenous Australian university students: Dissonant conceptions and strategies

Conceptions of learning and strategies used by 15 indigenous students in three Australian universities were studied longitudinally over three years. Their academic achievements were good, but at a high cost in terms of time and effort. In spite of the fact that almost half of the students expressed higher-order (qualitative) conceptions of learning in the first year and more in the second and third years, all of the students reported using highly repetitive strategies to learn. That is, they did not vary their way of learning, reading or writing in the beginning of their studies and less than half of them did so at the end of the three years. It is argued that encountering variation in ways of learning is a prerequisite for the development of powerful ways of learning and studying.

Hot beverage scalds in Australian children

Our objective was to compile data on the mechanism and severity of injuries associated with hot beverage burns in children. We identified 152 children over a 3-year period who attended a tertiary level burns center, representing 18% of all children treated. Their median age was 17.5 months and median body surface area burned was 4% (range, 0.25% to 32%). Significantly, 52% of children required admission, 18% received a split skin graft, and 26% required long-term scar management. In 70% of all cases, the mechanism of injury was the child pulling the hot beverage over himself or herself. In 80% of incidents, a primary care giver witnessed the injury. These findings indicate that scalding from hot beverages carries significant morbidity and is an important pediatric public health issue. It is clear that further research towards effective education programs for primary caregivers is warranted.