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Aims: To obtain preliminary data on the impact of motivational interviewing, a counselling approach to behaviour change, on glycaemic control, wellbeing, and self-care of adolescents with diabetes. Methods: Twenty two […]

The appearance of this radiograph caused considerable concern in the casualty department. It shows the left wrist of an 11 year old girl and was taken following suspicion of a [...]

The world of childhood diabetes has seen more changes in clinical care being introduced in recent years than many subspecialty areas of clinical practice. This review identifies some of the key underlying evidence and considers the implications for clinical teams that personal experience of these developments has highlighted. [ABSTRACT FROM AUTHOR]

INTRODUCTION: Rapid weight gain is often observed following initiation of insulin therapy in children with type 1 diabetes mellitus (T1DM) and girls are particularly at risk of becoming overweight. The authors evaluated body composition changes in children during the first year after diagnosis and related this to markers of cardiovascular risk. METHODS: Body mass index (BMI) and body composition measured by whole body dual energy x-ray absorptiometry (DEXA) were assessed in 30 patients (18 boys) with T1DM 3-10 days after diagnosis, 6 weeks later and at 1 year, and on two occasions 1 year apart in 14 controls (8 boys). Cardiovascular risk markers were assessed in T1DM subjects at 1 year. RESULTS: T1DM subjects had lower BMI SD scores (SDS) at diagnosis than controls (mean (SD) BMI SDS -0.67 (1.34) vs 0.20 (1.14), p<0.05) and reduced percentage body fat (20.3% (4.6) vs 24.5% (7.7), p<0.05). T1DM subjects normalised their body composition at 6 weeks and this was maintained 1 year later. Girls with diabetes were thinner than boys at diagnosis (BMI SDS -1.64 (1.02) vs -0.02 (1.17), p<0.05) and at 1 year (BMI SDS -0.58(0.9) vs 0.65 (0.98), p<0.05). Girls had higher glycated haemoglobin (HbA1c) (8.8% (1.2) vs 7.8% (1.0), p<0.05), insulin dose (1.01 (0.30) vs 0.82 (0.18) U/kg/day, p=0.04), total cholesterol (4.30 (0.45) vs 3.79 (0.50) mmol/l, p<0.05) and high-density lipoprotein (2.62 (0.53) vs 2.02 (0.37) mmol/l). High sensitivity C reactive protein and fibrinogen were in the normal range and there were no differences between genders. DISCUSSION: Insulin deficiency at diagnosis of diabetes causes a catabolic state that is predominantly lipolytic. Body composition normalises within 6 weeks of treatment, though girls remain thinner than boys both at diagnosis and 1 year thereafter, in contrast to published findings. Despite girls being prescribed a larger insulin dose, their HbA1c and cholesterol levels are higher at 1 year suggesting increased insulin resistance and cardiovascular risk. [ABSTRACT FROM AUTHOR]

BACKGROUND: Body fat mass (FM) and fat free mass (FFM) in childhood are often estimated by conversion of a measured variable into compartmental body composition using constants or regression equations that have been previously derived in healthy individuals. Application of such constants or equations to children with disease states may lead to inappropriate conclusions since the 'normal' relationships may become altered. AIMS AND Methods: To test this hypothesis by taking measurements of body composition using dual energy x ray absorptiometry (DEXA) as a 'gold standard' method and calculating hydration and body potassium constants using isotopic water dilution and whole body potassium counting. Measurements of bioelectrical impedance (BIA) by two different analysers (RJL and Holtain) were also performed to allow comparison with body water measurements. RESULTS: Measurements were performed in 35 children treated for acute lymphoblastic leukaemia (ALL) and compared to those in 21 children treated for a variety of other malignancies and 32 healthy sibling controls. The mean hydration and potassium content of FFM was significantly reduced in the ALL group compared to both other malignancies and controls. Application of equations derived from controls for the measurement of FFM derived from bioelectrical impedance led to an underestimation of 1.15 kg when compared to that derived from DEXA in children treated for ALL but not in other malignancies. For all groups combined, BIA was significantly different in the two analysers. CONCLUSION: Care needs to be taken in the application of equations derived from the normal population to body composition measurement in children treated for ALL. [ABSTRACT FROM AUTHOR]

Aims: To define outcome measures for auditing the clinical care of children and adolescents with insulin dependent diabetes mellitus (IDDM) and to assess the benefit of appointing a dedicated paediatric trained diabetes specialist nurse (PDSN).Methods: Retrospective analysis of medical notes and hospital records. Glycaemic control, growth, weight gain, microvascular complications, school absence, and the proportion of children undergoing an annual clinical review and diabetes education session were assessed. The effect of the appointment of a PDSN on the frequency of hospital admission, length of inpatient stay, and outpatient attendance was evaluated.Results: Children with IDDM were of normal height and grew well for three years after diagnosis, but grew suboptimally thereafter. Weight gain was above average every year after diagnosis. Glycaemic control was poor at all ages with only 16% of children having an acceptable glycated haemoglobin. Eighty five per cent of patients underwent a formal annual clinical review, of whom 16% had background retinopathy and 20% microalbuminuria in one or more samples. After appointing the PDSN the median length of hospital stay for newly diagnosed patients decreased from five days to one day, with 10 of 24 children not admitted. None of the latter was admitted during the next year. There was no evidence of the PDSN affecting the frequency of readmission or length of stay of children with established IDDM. Non-attendance at the outpatient clinic was reduced from a median of 19 to 10%.Conclusions: Outcome measures for evaluating the care of children with IDDM can be defined and evaluated. Specialist nursing support markedly reduces the length of hospital stay of newly diagnosed patients without sacrificing the quality of care. [ABSTRACT FROM AUTHOR]

The prevalence of osteopenia in children with inflammatory bowel disease (IBD) is unknown. The effect of nutritional state, disease activity, and steroid therapy on bone mineral content (BMC) of whole body, lumbar spine, and left femoral neck measured by dual energy x ray absorptiometry in 32 children with IBD was assessed by comparison with 58 healthy local school children. Using the control data, a predicted BMC was calculated taking into account bone area, age, height, weight, and pubertal stage. The measured BMC in children with IBD was expressed as a percentage of this predicted value (% BMC). Mean (SD) % BMC was significantly reduced for the whole body and left femoral neck in the children with IBD (97.0 (4.5)% and 93.1 (12.0)% respectively, p < 0.05). Of the children with IBD, 41% had a % BMC less than 1 SD below the mean for the whole body and 47% at the femoral neck. Reduction in % BMC was associated with steroid usage but not with the magnitude of steroid dose, disease activity, or biochemical markers of bone metabolism. In conclusion, osteopenia is relatively common in childhood IBD and may be partly related to the previous use of steroids. [ABSTRACT FROM AUTHOR]

Many long term sequelae result from previous treatment for malignancy in childhood. However, little information exists on cardiopulmonary response and energy expenditure during exercise and their possible associations with excess body fat. Measurements of body composition and exercise capacity both at low intensity and maximal aerobic capacity were made on 56 long term survivors of childhood malignancy (35 survivors of acute lymphoblastic leukaemia (ALL) and 21 survivors of other malignancies) and 32 siblings acting as controls. Female survivors of ALL had significantly greater mean (SD) body fat than survivors of other malignancies and siblings (32.5 (6.4)% v 24.3 (4.4)% and 26.3 (8.5)% respectively, p < 0.005). Energy expenditure at low intensity exercise was reduced in survivors of ALL, and negatively correlated with body fat after controlling for weight (partial r range -0.21 to -0.47, p < 0.05). Stroke volume, measured indirectly, was reduced and heart rate raised in ALL survivors at sub-maximal exercise levels. Peak oxygen consumption was significantly reduced in girls and boys treated for ALL compared with siblings (30.5 v 41.3 ml/kg/min for girls, p < 0.05 and 39.9 v 47.6 ml/kg/min for boys, p < 0.05 respectively). Reduced exercise capacity may account in part for the excess adiposity observed in long term survivors of ALL. [ABSTRACT FROM AUTHOR]

Type 1 diabetes (T1D) is a chronic autoimmune disease of childhood affecting 1:500 children aged under 15 years, with around 25% presenting with life-threatening diabetic ketoacidosis (DKA). While first-degree relatives have the highest risk of T1D, more than 85% of children who develop T1D do not have a family history. Despite public health awareness campaigns, DKA rates have not fallen over the last decade. T1D has a long prodrome, and it is now possible to identify children who go on to develop T1D with a high degree of certainty. The reasons for identifying children presymptomatically include prevention of DKA and related morbidities and mortality, reducing the need for hospitalisation, time to provide emotional support and education to ensure a smooth transition to insulin treatment, and opportunities for new treatments to prevent or delay progression. Research studies of population-based screening strategies include using islet autoantibodies alone or in combination with genetic risk factors, both of which can be measured from a capillary sample. If found during screening, the presence of two or more islet autoantibodies has a high positive predictive value for future T1D in childhood (under 18 years), offering an opportunity for DKA prevention. However, a single time-point test will not identify all children who go on to develop T1D, and so combining with genetic risk factors for T1D may be an alternative approach. Here we discuss the pros and cons of T1D screening in the UK, the different strategies available, the knowledge gaps and why a T1D screening strategy is needed., Competing Interests: Competing interests: REJB reports receiving speaking honoraria from Springer Healthcare and Eli Lilly, and reports sitting on the NovoNordisk UK Foundation Research Selection Committee on a voluntary basis. TR reports receiving consultancy fees from Abbott Diabetes Care (specifically for Libre evidence reviews) and lecture/programme organiser fees from Novo Nordisk. CMD reports having been an advisor giving honorarium lectures to NovoNordisk, Sanofi-genzyme, Janssen, Servier, Lilly, AstraZeneca, Provention Bio, UCB, MSD and Vielo Bio. CMD holds a joint patent with Midatech. JWG chairs the NovoNordisk UK Foundation Research Selection Committee and is a Foundation Trustee. TB reports receiving speaking honoraria from AstraZeneca, Servier and Novo Nordisk, and has received consultancy fees from Novo Nordisk and is an NN Global Expert Panel., (© Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Long-term outcomes in young people with type 1 diabetes continue to be of interest, and may help evaluate the effects of changes to the clinical care of children that have occurred in recent decades., Aims: To identify mortality and its causes before age 30 years in patients developing type 1 diabetes before age 15 years., Methods: Since 1995, paediatricians in Wales have compiled a prospective register of incident cases of type 1 diabetes occurring before age 15 years in Wales (the Brecon Cohort). Their subsequent mortality rates were compared with mortality in the general populations of Wales and England using the patient-years exposure method. Causes of death were ascertained from death certificates and from clinicians., Results: The standardised mortality ratio for young people with type 1 diabetes in Wales was 2.91 with no clear evidence of improvement or worsening of mortality risk over time. Most deaths occurred between ages 15 and 30 years although at a slightly younger age than in the general population. There were more deaths with increasing age at diagnosis of diabetes. Ketoacidosis remains the most common cause of death before age 30 years. Hypoglycaemia was difficult to ascertain with certainty but also caused some deaths. In this age group, chronic complications of diabetes were not a cause of mortality., Conclusions: Despite the developments in clinical care in recent years, the mortality risk for people developing type 1 diabetes in childhood remains high in young adult life before the onset of chronic complications., Competing Interests: Competing interests: None declared, (© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Background/aims: Treatment with intralesional triamcinolone/betamethasone is recommended for infantile sight-threatening periocular haemangiomas. This study investigates the endocrine and weight changes in 15 infants undergoing therapy over 12 years., Methods: 15 infants, median age 19 weeks (range 10-56) receiving intra/perilesional triamcinolone/betamethasone underwent serial measurement of weight, early morning serum cortisol and adrenocorticotropic hormone (ACTH) before and after injection., Results: Cortisol fell from a median (range) of 383 (112-594) to 28 (<10-506) nmol/l (p=0.005) and ACTH from 26 (14-134) to 9 (5-20) ng/l (p=0.05) from before injection to 4 weeks after treatment. Prolonged adrenal suppression occurred in 13 out of 15 cases with time to recovery being 19.5 (4-65) weeks. Failure to gain weight appropriately was observed in 14 infants but recovered once normal adrenal function was re-established., Conclusion: Prolonged adrenal suppression following triamcinolone/betamethasone injection for periocular haemangiomas is common and associated with faltering weight gain.

Although 80% of the variance in bone mass is determined genetically, there are many other factors which influence the accumulation of bone in early life and affect future risks of osteoporosis. This review considers the genetic, fetal, and environmental influences on bone mass acquisition in healthy children, and highlights important areas where paediatricians may have a role by counselling children and their families to adopt a healthy lifestyle which promotes bone health.

Type 1 diabetes is one of the most common chronic childhood disorders, occurring with increasing frequency. Diabetes management involves the child and family learning how to inject insulin and monitor blood glucose, and adhere to a diet containing healthy food choices. Medical interventions necessary to stabilise newly diagnosed diabetes depend upon the clinical condition of the child at presentation. Hospital admission is necessary if intravenous therapy is required to correct dehydration, electrolyte imbalance, and ketoacidosis, with progression to oral fluids and subcutaneous insulin administration as the child's condition improves. If the child is mildly to moderately symptomatic and clinically well, subcutaneous insulin and oral diet and fluids may be begun from the time of diagnosis, and stabilisation at diagnosis does not necessarily require hospital admission. This article reviews the evidence concerning hospital or home based treatment at diagnosis for children with type 1 diabetes. The Cardiff approach to home management is briefly described, and the benefits and disadvantages of different approaches to initial management are discussed.

Aims: To investigate a population of individuals with 22q11 deletion syndrome for hypocalcaemia., Methods: A detailed clinical history enquiring into symptoms of hypocalcaemia and blood sampling to assess for hypocalcaemia and hypoparathyroidism, of patients outside the neonatal period known to have the 22q11 microdeletion from fluorescent in situ hybridisation studies was taken., Results: Sixty one individuals were identified, of whom 23 were untraceable and one was unable to give informed consent. Biochemical investigations were performed on 27 subjects. Ten subjects had review of notes only. Four subjects had previously identified hypoparathyroidism. A new case of hypoparathyroidism was identified. Three subjects had borderline hypocalcaemia., Discussion: In this population of patients with 22q11 deletion syndrome, 13% of the total or 30% of those biochemically assessed had evidence of reduced serum calcium concentrations. It is likely that 13-30% of patients with 22q11 deletion syndrome have possible hypoparathyroidism outside the neonatal period. Reported symptoms of hypocalcaemia did not correlate with biochemical evidence of persisting hypocalcaemia. We have shown that previously undiagnosed asymptomatic hypoparathyroidism occurs in patients with 22q11 deletion syndrome and conclude that screening of this population should be considered.

Aims: To evaluate the effects of discontinuing growth hormone (GH) treatment on energy expenditure and body composition, which might help predict those most likely to benefit from early reintroduction of GH treatment in young adult life., Methods: Body composition was calculated from skinfold thicknesses and dual energy x ray absorptometry (DXA). Resting metabolic rate (RMR) and whole body bone mineral content (BMC) were also measured. Measurements were made before stopping treatment, at discontinuation of GH treatment, and two weeks, six months, and one year later in 11 adolescents with growth hormone deficiency (GHD) and five adolescents without GHD who were treated with GH. Measurements were compared with 10 healthy controls, in whom measurements were repeated one year later., Results: During the nine months before discontinuation of GH there were no changes in body composition, RMR, or BMC of patients with GHD, nor differences when compared with controls. RMR was reduced by 11.3 kJ/kg fat free mass two weeks after stopping GH in GHD patients and remained suppressed thereafter compared with controls. Percentage body fat increased by 4.3%/year in patients with GHD after discontinuing GH, whereas no changes were noted in control or non-GHD patients at one year. The patients experiencing the greatest reductions in RMR/kg fat free mass at six months showed the largest increases in body fat at one year. No change in BMC was noted in patients one year after stopping treatment., Conclusion: Important metabolic changes occur early after discontinuing GH treatment. In patients whose growth is complete, these changes might be used to predict those most likely to benefit from continuation of GH treatment into adult life.

The androgen insensitivity syndrome is a heterogeneous disorder with a wide spectrum of phenotypic abnormalities, ranging from complete female to ambiguous forms that more closely resemble males. The primary abnormality is a defective androgen receptor protein due to a mutation of the androgen receptor gene. This prevents normal androgen action and thus leads to impaired virilisation. A point mutation of the androgen receptor gene affecting two siblings with partial androgen insensitivity syndrome is described. One had cliteromegaly and labial fusion and was raised as a girl, whereas the other sibling had micropenis and penoscrotal hypospadias and was raised as a boy. Both were shown to have the arginine 840 to cysteine mutation. The phenotypic variation in this family is thus dependent on factors other than abnormalities of the androgen receptor gene alone.

Fourteen children receiving one year of recombinant human growth hormone (rhGH) treatment underwent measurement of serial changes in body composition (measured by skinfold thickness, bioelectrical impedance, and H2(18)O dilution), resting energy expenditure (REE, estimated by ventilated hood indirect calorimetry), and total free living daily energy expenditure (TEE, measured by the doubly labelled water technique). Mean height velocity increased from 4.9 to 8.6 cm/year after six months of treatment. Fat free mass (FFM) increased more during the first six weeks (24.4 g/day) than from six to 26 weeks of treatment (6.8 g/day); fat mass decreased by 7.2 g/day and 1.1 g/day respectively. The six week increase in REE (kJ/day) was maintained after six months of treatment, though expressed per kilogram FFM (kJ/kgFFM/day), returned to pretreatment values by three months. Height velocity increases at six months correlated with six week changes in fat mass measured by skinfold thickness and REE, though use of this relationship to predict growth response in individuals is limited by the wide 95% prediction intervals. No significant changes in growth, body composition, or energy expenditure were observed between six and 12 months of treatment, in either patients who had initially responded well to treatment or those who were poor initial responders to treatment and who had their dose of rhGH doubled after six months.

In order to determine the results of steroid-sparing maintenance immunosuppression in paediatric patients who have undergone orthotopic heart transplantation (OHT), a retrospective study was undertaken in 12 children and five infants (median age 3.5 years). Preoperative diagnoses were cardiomyopathy in seven and congenital heart disease in 10 patients. Immunosuppression was induced by cyclosporin, azathioprine, methylprednisolone, and antihuman lymphocyte immune globulin. It was maintained with cyclosporin and azathioprine. After induction, five patients received no further steroids. The remainder, except one, required only pulses for rejection (13 episodes or 0.51 episodes/patient year). Long term complications included hypertension in six, and renal impairment in three children. There were no early or late deaths from infection. Actuarial survival was 94% at one year. Of the children followed up for more than one year, all demonstrated an increase in height SD scores (mean (SD) -2.15 (1.35) to -1.15 (1.16)). We conclude that a steroid-sparing maintenance immunosuppression regimen can be successfully employed in paediatric OHT, and that significant catch-up growth can be achieved postoperatively.

Changes in body composition and energy expenditure were assessed in 15 children after six weeks of human growth hormone (hGH) treatment. Body composition measurements were made by stable isotope labelled water (H2(18)O) dilution, bioelectrical impedance, and skinfold thickness techniques. Energy expenditure was assessed both by indirect ventilated hood calorimetry (resting energy expenditure) and the stable isotope doubly labelled water (2H2(18)O) technique (free living daily total energy expenditure). Mean increases in weight of 0.96 kg and fat free mass of 1.37 kg and a mean decrease in fat mass of 0.41 kg were observed. Significant increases both in resting energy expenditure and free living daily energy expenditure were detected. Absolute changes in fat mass and resting energy expenditure were correlated. The data suggest (i) that the increase in the fat free mass is the most significant early clinical measure of hGH response and (ii) that hGH increases the metabolic activity of the fat free mass. Monitoring such changes may be predictive of the efficacy of hGH in promoting growth.

Total body water was estimated as part of the assessment of body composition in children with growth disorders, using the newly commercially available method of bioelectrical impedance. This was undertaken to compare the precision and accuracy of the results with those derived from skinfold thickness against measurement of stable isotopically labelled water (H2(18)O) dilution as a standard. The comparisons were carried out to see to what extent the impedance method could be applied with confidence to assessment of children with growth disorders. Total body water was derived from impedance (I) using an association with height (Ht2/I). Impedance and skinfold thickness estimates of total body water were equally precise when compared with values obtained from H2(18)O dilution (limits of agreement -1.9 to +1.3 and -1.7 to +2.0 kg respectively). The mean intraobserver coefficient of variation for repeat measurements of impedance was 0.9% compared with 4.6% for skinfold thickness with an interobserver coefficient of variation for impedance of 2.8%. Bioelectrical impedance estimation of body composition is likely to be of value in the growth clinic when expertise in measurement of skinfold thickness is limited or repeated measurements are to be undertaken by different observers.

We describe the lysine restricted, dietary management of three out of four siblings who were identified as having hyperlysinaemia. The diets, started in the neonatal period, were maintained for varying periods with unpredictable success. The propositus, who was not treated, was diagnosed at the age of 5 years, by which time he was already severely handicapped, presumably because of his metabolic disorder. Tentative recommendations are put forward for the management of this seemingly rare disorder. Mild chronic ammonia toxicity may be a factor in the pathogenesis of this condition.