WRS is the most common cause of permanent neonatal diabetes mellitus in consanguineous pedigrees. In addition to testing patients with a definite clinical diagnosis, EIF2AK3 should be tested in patients with isolated neonatal diabetes diagnosed after 3 wk of age from known consanguineous families, isolated populations, or countries in which inbreeding is frequent.
This audit analysed the Tanner and Whitehouse II twenty bone (TW2) method of bone age assessment which was used in our department, and compared it with the Greulich and Pyle (GP) method. 50 previous bone ages were independently re-calculated by each of three registrars using both techniques, with the time taken to perform each assessment being recorded. For each method the interobserver variation was analysed in terms of the spread of results. The intraobserver variation in TW2 was determined by comparing the bone age originally reported with that subsequently calculated on the same film by the same registrar. The average spread of results was 0.74 years for TW2 method, and 0.96 years for the GP method and this difference is not statistically significant at the 5% level. The average intraobserver variation to TW2 was 0.33 years, but with 95% confidence limits of -0.87 to +1.53 years. The average time taken was 7.9 min for TW2 and 1.4 min for GP assessments. It was concluded that the GP method gave similar reproducibility and was faster than the TW2 method. Following clinical discussion the routine departmental bone age assessment method was changed from the TW2 to the GP method.
SUMMARY Insulin dependent diabetes mellitus presenting in children under five years old exhibits several clinical and management features that differ from diabetes presenting in older children. In this review of the current population of the Oxford children's diabetes clinic, children with diabetes diagnosed aged 0-<5 years are compared with those diagnosed aged 5-<10 years to illustrate these differences.The mean annual age specific incidence of diabetes for children aged 0-<5 is 9-9/100 000 compared with 13*8/100 000 for the children diagnosed aged 5-<10. Although children with diabetes currently aged less than 5 comprise only 8% of the clinic population, such children ultimately make up 41% of the total number of children with diabetes aged under 15 attending the clinic. Diabetes diagnosed in children under the age of 5 seems to have increased in incidence over the past 10 years, exhibits a male preponderence (1-5:1), and shows an unusual seasonal variation in incidence with an autumn/early winter trough, late winter/early spring peak, and the absence of the mid-summer trough seen in other age groups. First degree family history was positive in 16% of children diagnosed under the age of 5 compared with 10% of the group diagnosed aged 5-<10. In none of these children was the mother the affected relative.
Aims: To determine the current level of diabetes services and to compare the results with previous national surveys. Methods: A questionnaire was mailed to all paediatricians in the UK identified as providing care for children with diabetes aged under 16 years. Information was sought on staffing, personnel, clinic size, facilities, and patterns of care. Responses were compared with results of two previous national surveys. Results: Replies were received from 244 consultant paediatricians caring for an estimated 17 192 children. A further 2234 children were identified as being cared for by other consultants who did not contribute to the survey. Of 244 consultants, 78% expressed a special interest in diabetes and 91% saw children in a designated diabetic clinic. In 93% of the clinics there was a specialist nurse (44% were not trained to care for children; 47% had nurse:patient ratio >1:100), 65% a paediatric dietitian, and in 25% some form of specialist psychology or counselling available. Glycated haemoglobin was measured routinely at clinics in 88%, retinopathy screening was performed in 87%, and microalbuminuria measured in 66%. Only 34% consultants used a computer database. There were significant differences between the services provided by paediatricians expressing a special interest in diabetes compared with "non-specialists", the latter describing less frequent clinic attendance of dietitians or psychologists, less usage of glycated haemoglobin measurements, and less screening for vascular complications. Non-specialist clinics met significantly fewer of the recommendations of good practice described by Diabetes UK. Conclusions: The survey shows improvements in services provided for children with diabetes, but serious deficiencies remain. There is a shortage of diabetes specialist nurses trained to care for children and paediatric dietitians, and a major shortfall in the provision of psychology/counselling services. The services described confirm the need for more consultant paediatricians to receive specialist training and to develop expertise and experience in childhood diabetes.
A multiple insulin injection regimen using a self-contained injection device Novopen (Novo Industries, Bagsvaerd, Copenhagen, Denmark) was assessed in 11 adolescents (age range 12.1-16.9 years) with diabetes. Ten patients completed the 3-month study period and eight expressed a wish to continue with the regimen, finding that the advantages it offered out-weighed the inconvenience of multiple injections. Mean glycosylated haemoglobin fell over the 3 months of the study period from 13.7 +/- 2.7% to 11.7 +/- 3.4% (NS) and there was a trend towards lower mean blood values which reached significance at the pre-lunch measurement (p less than 0.02).
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