Serum vitamin D levels are lower in Australian children and adolescents with type 1 diabetes than in children without diabetes.

Vitamin D is synthesised in the skin through the action of UVB radiation (sunlight), and 25-hydroxy vitamin D (25OHD) measured in serum as a marker of vitamin D status. Several studies, mostly conducted in high latitudes, have shown an association between type 1 diabetes mellitus (T1DM) and low serum 25OHD. We conducted a case-control study to determine whether, in a sub-tropical environment with abundant sunlight (latitude 27.5°S), children with T1DM have lower serum vitamin D than children without diabetes. Fifty-six children with T1DM (14 newly diagnosed) and 46 unrelated control children participated in the study. Serum 25OHD, 1,25-dihydroxy vitamin D (1,25(OH)(2) D) and selected biochemical indices were measured. Vitamin D receptor (VDR) polymorphisms Taq1, Fok1, and Apa1 were genotyped. Fitzpatrick skin classification, self-reported daily hours of outdoor exposure, and mean UV index over the 35 d prior to blood collection were recorded. Serum 25OHD was lower in children with T1DM (n = 56) than in controls (n = 46) [mean (95%CI) = 78.7 (71.8-85.6) nmol/L vs. 91.4 (83.5-98.7) nmol/L, p = 0.02]. T1DM children had lower self-reported outdoor exposure and mean UV exposure, but no significant difference in distribution of VDR polymorphisms. 25OHD remained lower in children with T1DM after covariate adjustment. Children newly diagnosed with T1DM had lower 1,25(OH)(2) D [median (IQR) = 89 (68-122) pmol/L] than controls [121 (108-159) pmol/L, p = 0.03], or children with established diabetes [137 (113-153) pmol/L, p = 0.01]. Children with T1DM have lower 25OHD than controls, even in an environment of abundant sunlight. Whether low vitamin D is a risk factor or consequence of T1DM is unknown.

Randomised controlled trial of the Healthy Living Triple P-Positive Parenting Program for families of children with type 1 diabetes.

This randomised controlled trial examined the efficacy of a brief, group-based parenting program in improving child and family outcomes for families of children with type 1 diabetes. Families (N = 50) of children (2-10 years) with type 1 diabetes were randomly allocated to intervention (n = 22) or care-as-usual (n = 28). Assessments (pre-intervention, post-intervention and 6-month follow-up) evaluated parent- and child-reported parenting behaviour, child behaviour/adjustment and child quality of life (primary outcomes); and metabolic control (routinely-collected blood glucose data), parents' self-efficacy with diabetes management, diabetes-specific child behaviour difficulties, family quality of life, parents' diabetes-related and general parenting stress and observed parent and child behaviour (secondary outcomes). Intent-to-treat analyses indicated greater rate of improvement over time for families allocated to intervention compared to care-as-usual for use of corporal punishment (primary caregivers only), and confidence with managing children's emotions/behaviours, parent-rated child quality of life and adjustment to the child's illness (secondary caregivers only). There were no other intervention effects. Although families found the intervention useful, low levels of psychosocial problems at baseline limited the scope for group-level improvement and there was limited evidence for intervention efficacy. Individually-tailored measures of goal-specific behaviour change may be considered in future research.

Adolescents seeking weight management: who is putting their hand up and what are they looking for?

We describe the characteristics of adolescents seeking treatment for obesity via the 'Eat Smart' feasibility study; an intensive 12 week dietitian-led weight management programme with an emphasis on lifestyle change. It was designed to test the feasibility of a structured low-fat diet compared with a semi-structured reduced carbohydrate plan compared with a model of 'standard care'- an unstructured low fat approach. When compared with non-participants, participants were predominantly female and lived in lower socioeconomic areas. When given the choice of dietary approach, 50% elected reduced dietary carbohydrate, 43% structured low fat and 7% chose 'standard care'. Modest weight loss was achieved over 12 weeks, with both the structured low fat and reduced carbohydrate formats. Families showed a strong preference for structured eating plans, in particular seeking assistance with correct portion size. The current standard model of unstructured advice was both unpopular and relatively unsuccessful.

The Eat Smart Study: a randomised controlled trial of a reduced carbohydrate versus a low fat diet for weight loss in obese adolescents.

BACKGROUND: Despite the recognition of obesity in young people as a key health issue, there is limited evidence to inform health professionals regarding the most appropriate treatment options. The Eat Smart study aims to contribute to the knowledge base of effective dietary strategies for the clinical management of the obese adolescent and examine the cardiometablic effects of a reduced carbohydrate diet versus a low fat diet. METHODS AND DESIGN: Eat Smart is a randomised controlled trial and aims to recruit 100 adolescents over a 2 1/2 year period. Families will be invited to participate following referral by their health professional who has recommended weight management. Participants will be overweight as defined by a body mass index (BMI) greater than the 90th percentile, using CDC 2000 growth charts. An accredited 6-week psychological life skills program 'FRIENDS for Life', which is designed to provide behaviour change and coping skills will be undertaken prior to volunteers being randomised to group. The intervention arms include a structured reduced carbohydrate or a structured low fat dietary program based on an individualised energy prescription. The intervention will involve a series of dietetic appointments over 24 weeks. The control group will commence the dietary program of their choice after a 12 week period. Outcome measures will be assessed at baseline, week 12 and week 24. The primary outcome measure will be change in BMI z-score. A range of secondary outcome measures including body composition, lipid fractions, inflammatory markers, social and psychological measures will be measured. DISCUSSION: The chronic and difficult nature of treating the obese adolescent is increasingly recognised by clinicians and has highlighted the need for research aimed at providing effective intervention strategies, particularly for use in the tertiary setting. A structured reduced carbohydrate approach may provide a dietary pattern that some families will find more sustainable and effective than the conventional low fat dietary approach currently advocated. This study aims to investigate the acceptability and effectiveness of a structured reduced dietary carbohydrate intervention and will compare the outcomes of this approach with a structured low fat eating plan. TRIAL REGISTRATION: The protocol for this study is registered with the International Clinical Trials Registry (ISRCTN49438757).

Klebsiella pneumoniae bacteraemia complicating rotavirus gastroenteritis in two infants with glucocorticoid deficiency.

Rotavirus gastroenteritis was complicated by Klebsiella Pneumoniae bacteraemia in two infants with glucocorticoid deficient conditions who were treated with 'stress dose' hydrocortisone during their illness. Delayed healing in the context of glucocorticoid administration combined with damage from rotavirus infection may result in increased risk of mucosal invasion by gastrointestinal bacteria and subsequent enteric gram-negative bacteraemia.

The feasibility of a home-based moderate-intensity physical activity intervention in obese children and adolescents.

OBJECTIVES: To explore the feasibility of conducting a 10-week home-based physical activity (PA) programme and evaluate the changes in insulin sensitivity (S(I)) commensurate with the programme in obese young people. DESIGN: Open-labelled intervention. SETTING: Home-based intervention with clinical assessments at a tertiary paediatric hospital. SUBJECTS: 18 obese (body mass index (BMI)>International Obesity Task Force age and sex-specific cut-offs) children and adolescents (8-18 years, 11 girls/7 boys) were recruited. 15 participants (nine girls/six boys, mean+/-SE age 11.8+/-0.6 years, BMI-SD scores (BMI-SDS) 3.5+/-0.1, six prepubertal/nine pubertal) completed the intervention. INTERVENTION: The programme comprised biweekly home visits over 10 weeks with personalised plans implemented aiming to increase moderate-intensity PA. Pedometers and PA diaries were used as self-monitoring tools. The goals were to (1) teach participants behavioural skills related to adopting and maintaining an active lifestyle and (2) increase daily participation in PA. OUTCOME MEASURES: Mean steps/day were assessed. S(I) assessed by the frequently sampled intravenous glucose tolerance test and other components of the insulin resistance syndrome were measured. RESULTS: Mean steps/day increased significantly from 10 363+/-927 (baseline) to 13 013+/-1131 (week 10) (p<0.05). S(I) was also significantly increased, despite no change in BMI-SDS, and remained so after an additional 10-week follow-up. CONCLUSIONS: The results suggest that such a home-based PA programme is feasible. S(I) improved without changes in BMI-SDS. More rigorous evaluations of such programmes are warranted.

Thyrotoxic Periodic Paralysis: An Incidental Diagnosis!

Thyrotoxic periodic paralysis is a rare presentation of thyrotoxicosis where the patient develops a transient motor deficit secondary to acute hypokalemia. The thyroid hormone augments gene transcription and post-transcriptional modification of Na-K ATPase, a cell membrane protein that regulates the electrical potential of the cell. Na-K ATPase increases active transport of potassium (K+) ions into the intracellular compartment causing hypokalemia without total body potassium deficit. Severe hypokalemia affects depolarization of the muscle cell membrane, clinically evidenced as paralysis. Other factors that may trigger hypokalemia and paralysis in the setting of hyperthyroidism include diet intake high in carbohydrates and salt, alcohol ingestion, trauma, infections, certain medication, and strenuous exercise. This rare but possible clinical presentation of thyrotoxicosis is significantly more predominant in males of Asian descent. We are reporting a case of a 44-year-old Asian-American male who presented to the emergency department with complaints of acute onset of bilateral lower extremity weakness. He had severe hypokalemia and was diagnosed with primary hyperthyroidism due to Graves' disease.

Advantages and Disadvantages of the Ketogenic Diet: A Review Article.

The ketogenic diet (KD) has gained immense popularity during the last decade, primarily because of its successful short-term effect on weight loss. In the United States, KD is utilized in a variety of patient populations for weight management, despite limited evidence regarding its efficacy and risks. This literature review provides an evaluation of data on the benefits and risks associated with the chronic use of KD, including its metabolic, endocrinological, and cardiovascular effects.

A Systematic Review of Pediatric Telediabetes Service Models.

Telediabetes may improve patient access to clinicians who specialize in the management of pediatric diabetes. Due to the diversity of telehealth modes, many different service models for pediatric telediabetes have been developed. This review describes pediatric telediabetes service models identified in the literature, investigates the reported changes in HbA1c of these interventions, and describes enablers and barriers to implementing a telediabetes service. Evaluation of current literature may inform the development and sustainability of telehealth services for pediatric diabetes management. Twenty-nine studies met inclusion criteria and were reviewed. This review has demonstrated that pediatric telediabetes can be delivered by remote monitoring and real-time videoconference modes. Overall, pediatric telediabetes increased interactions between patients and clinicians, improved access to specialized care, and facilitated increased diabetes monitoring. In some contexts, telediabetes also improved short-term glycemic control. Key enablers reported for telediabetes services were integration with existing workflows, dedicated staff, clinician and patient training, appropriate data security, technology with good usability, and the availability of technical support. Barriers included increases in patient responsibilities and clinician workload, and technical issues with equipment and software.

Utility of AVP gene testing in familial neurohypophyseal diabetes insipidus.

CONTEXT: Familial neurohypophyseal diabetes insipidus (FNDI) is a rare disorder resulting from arginine vasopressin (AVP) gene mutations. A partial defect in AVP secretion occurs early in the course of FNDI and may not be detected by a water deprivation test (WDT). Testing for AVP gene mutations may confirm a diagnosis of FNDI when a WDT is inconclusive and may also predict individuals who will later develop FNDI. OBJECTIVE: To test the utility of AVP gene analysis in confirming the diagnosis of FNDI. PATIENTS: Five families (20 subjects, 14 symptomatic and six asymptomatic) with FNDI and nine children with idiopathic neurohypophyseal diabetes insipidus (INDI). MEASUREMENTS: Genomic DNA was analysed for AVP gene mutations using polymerase chain reaction (PCR) amplification and sequencing. RESULTS: Heterozygous AVP gene mutations were found in all subjects with FNDI but none of the ICDI patients. Each family had their own distinct mutation. We identified two novel mutations (C44W and C105S). One asymptomatic subject developed diabetes insipidus (DI) 4 months after detection of an AVP gene mutation. The WDT suggested partial DI in 4/6 but was normal in 2/6 children with FNDI. CONCLUSION: AVP gene testing allowed diagnostic confirmation of FNDI when the WDT was inconclusive in symptomatic children, therefore obviating the need for a repeat WDT and enabling earlier initiation of appropriate treatment. AVP gene testing also has the potential to identify which asymptomatic children will later develop FNDI.

A Randomized Controlled Trial of Two Different Macronutrient Profiles on Weight, Body Composition and Metabolic Parameters in Obese Adolescents Seeking Weight Loss.

OBJECTIVE: Adolescent obesity is difficult to treat and the optimal dietary pattern, particularly in relation to macronutrient composition, remains controversial. This study tested the effect of two structured diets with differing macronutrient composition versus control, on weight, body composition and metabolic parameters in obese adolescents. DESIGN: A randomized controlled trial conducted in a children's hospital. METHODS: Eighty seven obese youth (means: age 13.6 years, BMI z-score 2.2, waist: height ratio 0.65, 69% female) completed a psychological preparedness program and were then randomized to a short term 'structured modified carbohydrate' (SMC, 35% carbohydrate; 30% protein; 35% fat, n = 37) or a 'structured low fat' (SLF, 55% carbohydrate; 20% protein; 25% fat, n = 36) or a wait listed control group (n = 14). Anthropometric, body composition and biochemical parameters were measured at randomization and after 12 weeks, and analyzed under the intention to treat principle using analysis of variance models. RESULTS: After 12 weeks, data was collected from 79 (91%) participants. BMI z-scores were significantly lower in both intervention groups compared to control after adjusting for baseline values, SLF vs. control, mean difference = -0.13 (95%CI = -0.18, -0.07), P<0.001; SMC vs. control, -0.14 (-0.19, -0.09), P<0.001, but there was no difference between the two intervention diet groups: SLF vs. SMC, 0.00 (-0.05, 0.04), P = 0.83. CONCLUSIONS: Both dietary patterns resulted in similar changes in weight, body composition and metabolic improvements compared to control. The use of a structured eating system which allows flexibility but limited choices can assist in weight change and the rigid application of a low fat eating pattern is not exclusive in its efficacy. TRIAL REGISTRATION: International Clinical Trials Registry ISRCTN49438757.

Indexes of insulin resistance and secretion in obese children and adolescents: a validation study.

OBJECTIVE: To assess the concurrent validity of fasting indexes of insulin sensitivity and secretion in obese prepubertal (Tanner stage 1) children and pubertal (Tanner stages 2-5) adolescents using estimates from the modified minimal model frequently sampled intravenous glucose tolerance test (FSIVGTT) as a criterion measure. RESEARCH DESIGN AND METHODS: Eighteen obese children and adolescents (11 girls and 7 boys, mean age 12.2 +/- 2.4 years, mean BMI 35.4 +/- 6.2 kg/m(2), mean BMI-SDS 3.5 +/- 0.5, 7 prepubertal and 11 pubertal) participated in the study. All participants underwent an insulin-modified FSIVGTT on two occasions, and 15 repeated this test a third time (mean 12.9 and 12.0 weeks apart). S(i) measured by the FSIVGTT was compared with homeostasis model assessment (HOMA) of insulin resistance (HOMA-IR), quantitative insulin-sensitivity check index (QUICKI), fasting glucose-to-insulin ratio (FGIR), and fasting insulin (estimates of insulin sensitivity derived from fasting samples). The acute insulin response (AIR) measured by the FSIVGTT was compared with HOMA of percent beta-cell function (HOMA-beta%), FGIR, and fasting insulin (estimates of insulin secretion derived from fasting samples). RESULTS: There was a significant negative correlation between HOMA-IR and S(i) (r = -0.89, r = -0.90, and r = -0.81, P < 0.01) and a significant positive correlation between QUICKI and S(i) (r = 0.89, r = 0.90, and r = 0.81, P < 0.01) at each time point. There was a significant positive correlation between FGIR and S(i) (r = 0.91, r = 0.91, and r = 0.82, P < 0.01) and a significant negative correlation between fasting insulin and S(i) (r = -90, r = -0.90, and r = -0.88, P < 0.01). HOMA-beta% was not as strongly correlated with AIR (r = 0.60, r = 0.54, and r = 0.61, P < 0.05). CONCLUSIONS: HOMA-IR, QUICKI, FGIR, and fasting insulin correlate strongly with S(i) assessed by the FSIVGTT in obese children and adolescents. Correlations between HOMA-beta%, FGIR and fasting insulin, and AIR were not as strong. Indexes derived from fasting samples are a valid tool for assessing insulin sensitivity in prepubertal and pubertal obese children.

Turner syndrome in childhood and adolescence.

Turner syndrome (TS) is the most common chromosomal disorder causing short stature in females. The short stature is caused at least in part by haploinsufficiency of the short stature homeobox (SHOX) gene. Complete spontaneous puberty may occur in approximately 16% of patients, with spontaneous pregnancy in up to 4%. The final height of untreated TS girls is 86-88% of the mean adult female height. Growth hormone (GH) given alone or with oxandrolone improves final height. The major factors determining the outcome of GH therapy are the dose of GH used and the number of years of GH therapy prior to oestrogenization. Pubertal induction in TS should be individualized bearing in mind growth optimization and psychological issues. Adolescents and adults with TS may face a range of medical, fertility and psychosocial issues. Psychological support for TS individuals and families is important throughout life and should ideally be provided by both health professionals and TS support groups.

Vitamin A levels in patients with CF are influenced by the inflammatory response.

BACKGROUND: Serum vitamin A, normally depressed in inflammatory conditions, is frequently low in people with CF. Vitamin A is important in respiratory epithelial regeneration and repair. We hypothesised that serum vitamin A would be associated with inflammation and disease severity. METHODS: Serum vitamin A (as retinol), C-reactive protein (CRP), vitamin E, 25-hydroxy vitamin D (25OHD), 1,25-dihydroxy vitamin D (1,25(OH)(2)D), weight, and lumbar spine bone mineral density (LSBMD) were measured in 138 subjects with CF (5-56 years) and 138 control subjects (5-48 years). FEV(1), presence of CF liver disease (CFLD) and hospital admissions were recorded in those with CF. RESULTS: Serum vitamin A level was lower in CF subjects than in controls (mean, 95% CI: 1.29, 1.0-1.37 vs. 1.80, 1.7-1.87 micromol/l, p < 0.0001), and inversely correlated with CRP (r(s) = -0.37, p < 0.0001). CF subjects with low vitamin A (45%) level had poorer FEV(1), weight z-score, LSBMD z-score, and higher CRP compared with those with normal levels. In the CF group CRP, vitamin E, 1,25(OH)(2)D, presence of CFLD, admissions, and age were associated with vitamin A level. CONCLUSIONS: Serum vitamin A is negatively associated with CRP in subjects with CF, consistent with normal population studies. It is important to distinguish between low serum vitamin A associated with the inflammatory response and that due to poor nutritional stores. The role of vitamin A in CF warrants further study, in the contexts both of chronic recurrent inflammatory disease and acute pulmonary exacerbation.

The use of online health techniques to assist with the delivery of specialist paediatric diabetes services in Queensland.

We have tested an alternative method of delivering health services to regional areas of Queensland. By integrating telepaediatrics into an existing outreach programme for children with diabetes and endocrine conditions, we were able to reduce travel for specialist hospital staff while maintaining (and sometimes increasing) the contact patients had with the specialist team. In the first 28 months, we facilitated 160 patient consultations and 10 education sessions via videoconference through the telepaediatric service. By the end of the study, site visits were taking place annually and routine videoconference clinics were scheduled quarterly for the review of new patients and follow-up. Telepaediatric services in endocrinology and diabetes were established at three levels: the coordination of routine specialist clinics via videoconference; ad hoc patient consultations for collaborative management during acute presentations and at times of urgent clinical need; and the delivery of education to staff and patients throughout the state. The net result was improved access to specialist services from rural and remote areas of Queensland.

Predicting success: factors associated with weight change in obese youth undertaking a weight management program.

OBJECTIVE: To explore which baseline physiological and psychosocial variables predict change in body mass index (BMI) z-score in obese youth after 12 weeks of a dietary weight management study. METHODS: Participants were obese young people participating in a dietary intervention trial in Brisbane Australia. The outcome variable was change in BMI z-score. Potential predictors considered included demographic, physiological and psychosocial parameters of the young person, and demographic characteristics of their parents. A multivariable regression model was constructed to examine the effect of potential predictive variables. RESULTS: Participants (n = 88) were predominantly female (69.3%), and had a mean(standard deviation) age of 13.1(1.9) years and BMI z-score of 2.2(0.4) on presentation. Lower BMI z-score (p < 0.001) and insulin resistance (p = 0.04) at baseline, referral from a paediatrician (p = 0.02) and being more socially advantaged (p = 0.046) were significantly associated with weight loss. Macronutrient distribution of diet and physical activity level did not contribute. CONCLUSIONS: Early intervention in obesity treatment in young people improves likelihood of success. Other factors such as degree of insulin resistance, social advantage and referral source also appear to play a role. Assessing presenting characteristics and factors associated with treatment outcome may allow practicing clinicians to individualise a weight management program or determine the 'best-fit' treatment for an obese adolescent.

The use of web-based interventions to prevent excessive weight gain.

We reviewed web-based interventions for overweight and obesity prevention. A literature search was conducted using seven electronic databases. Manually searched articles were also included. Thirty studies fulfilled the inclusion criteria. Of these, 13 studied physical activity, eight studied dietary practices and nine studied a combination of physical activity and dietary practice. Twenty-eight of the studies (93%) reported positive changes in moderate to vigorous physical activity level, fruit and vegetable intake and psychological factors. A meta-analysis showed there were improvements, though not significant, in fruit and vegetable consumption (standardised mean difference, SMD = 0.61; 95% CI =-0.13 to 1.35) and physical activity (SMD = 0.15; 95% CI =-0.06 to 0.35). The review suggests that web-based interventions are a useful educational tool for increasing awareness and making healthy behaviour changes in relation to an excessive weight gain problem.

Neonatal complete generalized glucocorticoid resistance and growth hormone deficiency caused by a novel homozygous mutation in Helix 12 of the ligand binding domain of the glucocorticoid receptor gene (NR3C1).

CONTEXT: Glucocorticoid resistance is a rare genetic condition characterized by reduced sensitivity to cortisol signaling and subsequent hyperactivation of the hypothalamic-pituitary-adrenal axis. OBJECTIVE: The objective was to confirm the diagnosis of glucocorticoid resistance in the patient, to determine the degree of suppression of cortisol and ACTH levels in response to dexamethasone, and to determine the underlying genetic abnormality and functional consequences of the mutation. PATIENT AND METHODS: The patient presented on the first day of life with profound hypoglycemia. Initial cortisol levels were appropriately elevated; however, the patient was found to have persistently elevated levels of both cortisol and ACTH. The baby developed a tanned appearance and severe hypertension and fatigued easily with feeding. Serial oral dexamethasone suppression tests were performed with doses escalating from 0.125 mg to 12 mg dexamethasone given at 2300 h. Sequencing of the glucocorticoid receptor gene was performed along with functional studies of the glucocorticoid receptor. GH secretion was assessed with an arginine glucagon stimulation test. RESULTS: Cortisol and ACTH levels did not suppress with doses of up to 12 mg dexamethasone. A 2-bp deletion was found at amino acid position 773 of the glucocorticoid receptor ligand binding domain. A complete lack of dexamethasone binding and in vitro biological effect was demonstrated. GH stimulation testing was consistent with GH deficiency. CONCLUSION: The homozygous mutation in the ligand-binding domain of the glucocorticoid receptor gene resulted in a functionally inactive glucocorticoid receptor and apparent complete glucocorticoid resistance with biochemical GH deficiency.