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1.  Diabetes services in the UK: fourth national survey; are we meeting NSF standards and NICE guidelines? 
Archives of Disease in Childhood  2005;90(10):1005-1009.
Background: Previous surveys of children's diabetes service provision in the UK have shown gradual improvements but continuing deficiencies.
Aim: To determine whether further improvements in services have occurred.
Methods: A questionnaire was mailed to all paediatricians in the UK identified as providing care for children and adolescents with diabetes. Responses were compared with results of three previous surveys, and with recommendations in the Diabetes NSF and the NICE type 1 diabetes guidelines.
Results: Replies were received from 187 consultant paediatricians in 169 centres looking after children; 89% expressed a special interest in diabetes, 98% saw children in a designated diabetic clinic, and 95% clinics now have more than 40 patients. In 98% of the clinics there was a specialist nurse (82% now children's trained), but 61% clinics had a nurse:patient ratio <1:100; 39% of clinics did not have a paediatric dietician and in 78% there was no access to psychology/psychiatry services in clinics. Glycated haemoglobin was measured routinely at clinics in 86%, annual screening for retinopathy performed in 80%, and microalbuminuria in 83%. All centres now have local protocols for ketoacidosis, but not for children undergoing surgery (90%) or severe hypoglycaemia (74%). Mean clinic HbA1c levels were significantly lower in the clinics run by specialists (8.9%) than generalists (9.4%). There have been incremental improvements over the last 14 years since the surveys began, but only two clinics met all the 10 previously published recommendations on standards of care.
Conclusions: The survey shows continuing improvements in organisational structure of services for children with diabetes but serious deficiencies remain. Publication and dissemination of the results of the previous surveys may have been associated with these improvements and similar recurrent service review may be applicable to services for other chronic childhood conditions.
PMCID: PMC1720116  PMID: 15941771
2.  Maternal vitamin D deficiency, refractory neonatal hypocalcaemia, and nutritional rickets 
Archives of Disease in Childhood  2005;90(4):437-438.
PMCID: PMC1720356  PMID: 15781946
4.  Doctor, who will be looking after my child's diabetes? 
PMCID: PMC1719278  PMID: 12495947
6.  Diabetes services in the UK: third national survey confirms continuing deficiencies 
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.
PMCID: PMC1719265  PMID: 12495963
7.  Diabetes unmasked by electric shock 
PMCID: PMC1719117  PMID: 11861248
8.  A multicentre trial of recombinant growth hormone and low dose oestrogen in Turner syndrome: near final height analysis 
BACKGROUND—Turner syndrome accounts for 15-20% of childhood usage of growth hormone (GH) in the UK but final height benefit remains uncertain. The most effective strategy for oestrogen replacement is also unclear.
METHODS—Fifty eight girls who, at start of treatment, were of mean age 9.1 years and projected final height 142.2 cm were randomised to receive in year 1, either low dose ethinyloestradiol 50-75 ng/kg/day, GH 28 IU/m2 surface area/week as a daily injection, or a combination of ethinyloestradiol and GH. After the first year, the ethinyloestradiol treated girls received combination treatment. After two years, girls aged over 12 years were given escalating ethinyloestradiol to promote pubertal development.
RESULTS—Near final height was available for 49 girls at age 16.5 years, 146.8 cm, representing a gain of 4.6 cm, range −7.9 to +11.7 cm. Twelve of the 49 girls gaining 7.5 cm or more were less than 13 years at the start and had received GH for at least four years. Height gain was correlated with greater initial height deficit. Fifteen girls (31%) reached 150 cm or more compared to a predicted 10%. Early supplementation with ethinyloestradiol provided no final height advantage.
CONCLUSIONS—Final height gain was modest at 4.6 cm. Younger, shorter girls gained greatest height advantage from GH. Low dosage ethinyloestradiol before planned induction of puberty was not beneficial.

PMCID: PMC1718629  PMID: 11124794
10.  Partial remission phase of diabetes in children younger than age 10 years 
Archives of Disease in Childhood  1999;80(4):367-369.
There is renewed interest in the phase of partial remission in recently diagnosed diabetes because of the potential for pharmacological and immune intervention to preserve β cell function. 95 children younger than 10 years were investigated to assess the influence of age, sex, diabetic ketoacidosis (DKA), admission at diagnosis, and ethnicity on the frequency of remission and insulin requirements during the first two years after diagnosis. Partial remission was defined as a requirement of insulin < 0.5 U/kg body weight/day. There was partial remission in 41 patients, with no differences for children aged 2-4 years and those aged 5-9 years. None of the five children aged < 2 years remitted. Forty five of 95 children were admitted to hospital at diagnosis, of whom 26 of 45 had DKA (blood pH < 7.25). In this number of children we were unable to show a statistical difference in the rate of remission with respect to DKA, admission to hospital at diagnosis, sex, or South Asian ethnic background. There were no differences in insulin requirements between the different groups by the end of two years and at that time seven of the children required insulin < 0.5 U/kg/day. The results suggest that even in preschool children there is potential for attempting to preserve β cell function.

PMCID: PMC1717883  PMID: 10086946
12.  Bartonella spp. isolated from wild and domestic ruminants in North America. 
Emerging Infectious Diseases  2000;6(3):306-311.
Bartonella species were isolated from 49% of 128 cattle from California and Oklahoma, 90% of 42 mule deer from California, and 15% of 100 elk from California and Oregon. Isolates from all 63 cattle, 14 deer, and 1 elk had the same polymerase chain reaction/restriction fragment length polymorphism profiles. Our findings indicate potential for inter- and intraspecies transmission among ruminants, as well as risk that these Bartonella spp. could act as zoonotic agents.
PMCID: PMC2640866  PMID: 10827123
13.  Metabolic cataracts in newly diagnosed diabetes 
Archives of Disease in Childhood  1997;76(2):118-120.
Accepted 29 October 1996

The morphologically distinct diabetic or `metabolic' cataract is rare in newly diagnosed insulin dependent diabetes. The cases described are of five adolescents (three girls, two boys) with newly diagnosed insulin dependent diabetes who developed metabolic cataracts close to the time of diagnosis (0-16 months). They all had a prolonged duration of symptoms before diagnosis (4-24 months) and high glycated haemoglobin levels at diagnosis (15-21%). The pathogenesis of diabetic cataract is not well understood in humans. An attempt is made to link clinical observations with evidence from experimental animal models to understand the mechanism of cataract formation, with particular reference to the aldose reductase pathway. It is recommended that the lens and retina are examined at the onset of diabetes in all children, especially those who have a prolonged duration of symptoms before diagnosis and who report persistent blurred vision.

PMCID: PMC1717061  PMID: 9068299
14.  Characterisation of carnitine palmitoyltransferases in patients with a carnitine palmitoyltransferase deficiency: implications for diagnosis and therapy. 
OBJECTIVES: Carnitine palmitoyltransferase (CPT) deficiency is one of the most common defects of mitochondrial fatty acid oxidation. Two different enzymes (CPT-I and CPT-II) are involved. Due to problems in measuring enzyme activity, relatively little is known about the substrate specificity of each of the human enzymes. This is of considerable importance in the treatment of patients. The objectives were to establish a reliable method for the measurement of CPT activity in whole cells, to use this to characterise the substrate specificity of each enzyme, and finally, to determine if medium chain triglycerides would be of benefit in the treatment of deficient patients. METHODS: A simple permeabilisation technique was used which allows the measurement of CPT activity in a small amount of cultured skin fibroblasts or peripheral blood cells. Using this technique three patients were identified with CPT deficiency. In two of these patients, one with CPT-I deficiency and one with CPT-II deficiency, a complete substrate specificity profile of the mitochondrial carnitine acyltransferases was established for all saturated even chain acyl-CoA esters. RESULTS: For both enzymes the highest CPT activity was with C12-CoA. About 70% of total cellular carnitine octanoyltransferase activity was due to mitochondrial CPT. As CPT is involved in the transport of medium chain fatty acids the metabolic response of a patient with CPT-II deficiency to dietary medium chain triglycerides was assessed. Despite the normal production of ketone bodies there was a significant medium chain dicarboxylic aciduria in the patient, indicating a limited capacity of the CPT independent mitochondrial uptake of medium chain fatty acids. CONCLUSIONS: CPT deficiency can easily be diagnosed in permeabilised cultured skin fibroblasts. Both CPT-I and CPT-II are more active with medium chain length substrates than previously assumed. Care should therefore be taken in the treatment of these patients with medium chain triglycerides.
PMCID: PMC486729  PMID: 9048718
16.  Congenital hypothyroidism--correlation between radiographic appearances of the knee epiphyses and biochemical data. 
Postgraduate Medical Journal  1991;67(788):553-556.
Plain knee radiographs of 20 neonates with congenital hypothyroidism, were reviewed. The size and appearances of the epiphyses were compared with the biochemical data at the time of referral. Fifteen infants had unequivocal evidence of delayed bone maturation based on absence of the distal femoral epiphysis or small epiphyseal size. Seven cases had fragmentation of at least one epiphysis. A positive correlation was found, at diagnosis, between the thyroxine and triiodothyronine levels and the size of the knee epiphyses. All 14 infants with thyroxine levels of less than 70 nmol/l had small epiphyses with a combined mean diameter of the proximal tibial plus distal femoral epiphyses of 7 mm or less. Conversely, of the 6 infants with thyroxine levels of 70 nmol/l or above, 5 had combined epiphyseal diameters of greater than 10 mm. We suggest that in infants with no clinical symptoms and only moderately raised raised screening thyroid stimulating hormone, a knee radiograph showing the described radiological changes should prompt institution of thyroxine treatment before awaiting biochemical confirmation of the diagnosis.
PMCID: PMC2398914  PMID: 1924025
17.  A decade of diabetes: keeping children out of hospital. 
BMJ : British Medical Journal  1993;307(6896):96-98.
OBJECTIVES--To document the number of children aged less than 15 years who developed diabetes and were managed within one large health district, and to evaluate the outcome of those children managed without hospital admission at diagnosis. DESIGN--A retrospective study over 1979-88, when a paediatrician and a physician with special interests in childhood diabetes initiated joint clinics. Data collected from the district diabetes register and files of consultants and health visitors specialising in diabetes. SETTING--Referral of children to consultants in Leicestershire (total population 863,000). MAIN OUTCOME MEASURES--The proportion of children managed without hospital admission, comparison of readmission rates and glycated haemoglobin concentrations between children admitted and those not admitted. RESULTS--Over 10 years 236 children aged 10-14 years developed diabetes (annual incidence rate 12.8/100,000 child population (95% confidence interval 11.3 to 14.7)). In total 138 were not admitted to hospital but received supervised management based at home. Admitted children were younger or acidotic or their family doctors did not contact the diabetes team. Duration of admission declined from seven days in 1979-80 to three days in 1987-8. Ninety two were not admitted to hospital during the 10 years for any reason. Significantly fewer children who received management at home were readmitted for reasons related to diabetes than the group treated in hospital (30 (22%) v 40 (41%); p = 0.004). Concentrations of glycated haemoglobin were no different between the two groups. CONCLUSIONS--Children with newly diagnosed diabetes may be safely and effectively managed out of hospital. Domiciliary or community based management depends on the commitment of consultants specialising in diabetes working in close cooperation with general practitioners, specialist nurses in diabetes, and dietitians.
PMCID: PMC1697706  PMID: 8343736
18.  Newly diagnosed diabetes: a study of parental satisfaction. 
Archives of Disease in Childhood  1992;67(8):1011-1013.
A national survey of 509 parents of children with newly diagnosed diabetes elicited a 92% response rate, showing that 96% of children were admitted to hospital, 42% staying in hospital longer than one week and 41% received an intravenous infusion. More than 90% of parents expressed satisfaction with the information given at diagnosis, the preparation they received before discharge home, and the outpatient follow up services. Home visits from a diabetes nurse specialist (DNS) were received by 73% of families and 44% reported that the DNS was the most supportive person in the first year after diagnosis. Readmission during the first 12 months after diagnosis was required by 23% of children, more often in the youngest age group. Poor liaison with schools and the lack of diabetes knowledge in teachers were the sources of greatest dissatisfaction. Children under the care of paediatricians with no specialist interest in diabetes were significantly more likely to be kept in hospital longer at diagnosis and parents were less satisfied with outpatient care and school liaison. The study supports previous recommendations that diabetes nurse specialists are a priority resource in providing support services and that every district should have a paediatrician with a special interest in diabetes and a designated children's diabetic clinic.
PMCID: PMC1793592  PMID: 1520003
20.  Reappraisal of thyroxine treatment in primary hypothyroidism. 
Archives of Disease in Childhood  1990;65(10):1129-1132.
The optimum daily dose of thyroxine was calculated for 13 children aged 3-16 years with primary hypothyroidism by titrating their doses at monthly intervals. The condition of the thyroid was assessed by sensitive assay of thyroid stimulating hormone concentrations, as well as measurement of total and free thyroid hormone concentrations and systolic time interval ratios. Serum thyroid stimulating hormone concentration was found to be the most responsive to small changes in thyroxine. The calculated optimum daily replacement dose of thyroxine (102 micrograms/m2 or 3.5 micrograms/kg) was fractionally lower than that previously recommended, and was more closely related to surface area (coefficient of variation 8.2%) than to body weight (coefficient of variation 16.2%). Our results suggest that though monthly may be the optimal time interval for increases in the dose of thyroxine, any reduction in the dose should be made more gradually.
PMCID: PMC1792337  PMID: 2248504
22.  Apnoea monitoring and cot deaths. 
PMCID: PMC1778170  PMID: 3813648
24.  High incidence of minimal change nephrotic syndrome in Asians. 
Archives of Disease in Childhood  1985;60(11):1018-1020.
Between 1973 and 1982 there was a significantly higher incidence of minimal change nephrotic syndrome among Asian compared with non-Asian children in Leicestershire. Most Asians in Leicestershire are Gujarati-speaking Hindus, but Sikhs and Muslims are also represented; no group of Asians (defined by religion, language, or birthplace) was at special risk of developing nephrotic syndrome. Nephrotic syndrome was more preponderant in Asian children living within the city of Leicester, and there was an unusually low incidence in non-Asian children within the city. Both racial and environmental factors may be important in the increased susceptibility to minimal change nephrotic syndrome in Asian children.
PMCID: PMC1777627  PMID: 4073934
25.  Parietal foramina in the Saethre-Chotzen syndrome. 
Journal of Medical Genetics  1985;22(5):413-414.
PMCID: PMC1049495  PMID: 4078875

Results 1-25 (58)