Target setting in intensive insulin management is associated with metabolic control: the Hvidoere Childhood Diabetes Study Group Centre Differences Study 2005

Swift PGF, Skinner TC, de Beaufort CE, Cameron FJ, Åman J, Aanstoot H‐J, Castaño L, Chiarelli F, Daneman D, Danne T, Dorchy H, Hoey H, Kaprio EA, Kaufman F, Kocova M, Mortensen HB, Njølstad PR, Phillip M, Robertson KJ, Schoenle EJ, Urakami T, Vanelli M, Ackermann RW, Skovlund SE for the Hvidoere Study Group on Childhood Diabetes. Target setting in intensive insulin management is associated with metabolic control: the Hvidoere Childhood Diabetes Study Group Centre Differences Study 2005. Objective: To evaluate glycaemic targets set by diabetes teams, their perception by adolescents and parents, and their influence on metabolic control. Methods: Clinical data and questionnaires were completed by adolescents, parents/carers and diabetes teams in 21 international centres. HbA1c was measured centrally. Results: A total of 2062 adolescents completed questionnaires (age 14.4 ± 2.3 yr; diabetes duration 6.1 ± 3.5 yr). Mean HbA 1c = 8.2 ± 1.4% with significant differences between centres (F = 12.3; p < 0.001) range from 7.4 to 9.1%. There was a significant correlation between parent (r = 0.20) and adolescent (r = 0.21) reports of their perceived ideal HbA1c and their actual HbA1c result (p < 0.001), and a stronger association between parents' (r = 0.39) and adolescents' (r = 0.4) reports of the HbA1c they would be happy with and their actual HbA1c result. There were significant differences between centres on parent and adolescent reports of ideal and happy with HbA1c (8.1 < F > 17.4;p < 0.001). A lower target HbA1c and greater consistency between members of teams within centres were associated with lower centre HbA1c (F = 16.0; df = 15; p < 0.001). Conclusions: Clear and consistent setting of glycaemic targets by diabetes teams is strongly associated with HbA1c outcome in adolescents. Target setting appears to play a significant role in explaining the differences in metabolic outcomes between centres.     

Ultrasound appearance of pseudomembranous colitis

A case illustrating that ultrasound can be useful in the diagnosis of pseudomembranous colitis is reported. Diagnosis of pseudomembranous colitis is usually made from microbiological or histopathological investigations. The ultrasound appearance of grossly thickened bowel wall with luminal narrowing is non-specific, but in the correct clinical context should suggest the diagnosis of pseudomembranous colitis.     

Concentration of electrolytes in the sweat of malnourished children

The sweat test was performed by the method of Gibson and Cooke on 36 children with second and third degree malnutrition, aged from 2 months to 4 years. The results were compared with those from 32 healthy, well nourished controls in the same age range. Determinations were made of sodium and chloride concentrations, chloride/sodium ratio, and the sum of the concentrations of the two electrolytes in each sample. The malnourished children were found to have higher sodium and chloride concentrations than the well nourished ones. None of the normal or malnourished children had a sweat chloride value greater than 60 mmol/l; chloride values within a suspicious range (between 40 and 60 mmol/l) were found in two malnourished children. Two well nourished controls and six malnourished children showed suspicious sweat sodium concentrations; in one child with third degree malnutrition the sodium content was greater than 60 mmol/l. In all results in the suspicious range, or greater than 60 mmol/l, the chloride/sodium ratio was less than 1, and the sum of the two electrolytes was below 140 mmol/l. Based on these results, we conclude that malnourished children have raised sweat sodium and chloride concentrations when compared with well nourished children. The electrolyte values for most of them are not in a suspicious range or consistent with a diagnosis of cystic fibrosis. Unlike that found in cystic fibrosis, the sodium/chloride ratio in our subjects was less than 1 and the sum of both electrolytes never exceeded 140 mmol/l.     

Brain swelling and ischaemia in Kenyans with cerebral malaria

Computed tomography was performed on 14 unconscious Kenyan children recovering from cerebral malaria (seven of whom had another scan 12-120 days later) to elucidate the cause of intracranial hypertension and neurological sequelae. Brain swelling, defined as a loss of cerebrospinal fluid spaces, was documented in six children, while a further two had conspicuously small ventricles only. There was severe intracranial hypertension in the two children with definite brain swelling in whom intracranial pressure was monitored. There was no evidence of acute hydrocephalus or vasogenic oedema. Four children with brain swelling also had widespread low density areas suggestive of ischaemic damage. The patterns of damage were not uniform but were consistent with a critical reduction in cerebral perfusion pressure (which was documented in the two in whom this was monitored), hypoglycaemia, or status epilepticus. All four had serious neurological sequelae. These data suggest that brain injury in cerebral malaria may be due in part to secondary systemic and intracranial factors as well as to the direct effect of intravascular sequestration.     

Impaired prenatal and postnatal growth in Dutch patients with phenylketonuria. The National PKU Steering Committee

OBJECTIVE: To assess whether physical growth is affected in early treated Dutch patients with phenylketonuria (PKU). METHODS: The birth weights of all 137 early detected patients with PKU born in the period from 1974 to 1988 in the Netherlands were compared with reference values. Height, head circumference, and weight were measured at the age at which treatment started (commonly about 2-3 weeks), at 6 months of age, and yearly from the child's first birthday up to the age of 10 years. These measurements were compared with reference values. RESULTS: The adjusted birth weight in patients with PKU was 141 g (95% confidence interval (CI) 66 to 216 g) less than Dutch reference values by Kloosterman and 103 g (95% CI 9 to 196 g) less compared with the birth weight of another reference group. At the age at which treatment started, z scores of patients for height by age were -0.23 (95% CI -0.44 to -0.02) and z scores for head circumference by the age were -0.25 (95% CI -0.44 to -0.06). From the age at which treatment started up to the age of 3 years z scores for height by age further decreased to -0.74 (95% confidence interval -0.93 to -0.56), after which no additional decrease occurred. In contrast, z scores for head circumference increased from -0.25 at the first visit to 0.08 (95% CI -0.14 to 0.30) at the age of 1 year, after which they remained close to zero. Weight by height was close to the expected centiles for all ages. CONCLUSION: Patients with PKU are growth retarded at birth and have smaller head circumferences than the normal population. In Dutch patients further growth retardation occurs in the first three years of life.     

Automated sequencing detects all mutations in Northern Irish patients with phenylketonuria and mild hyperphenylalaninaemia

In the first phase of the Northern Ireland PKU Study, we used automated sequencing to identify the spectrum of mutations in a random group of 32 unrelated phenylketonuria (PKU) families. We also investigated 7 Northern Irish patients with mild hyperphenylalaninaemia not requiring dietary intervention (MHP, previously referred to as non-PKU HPA). Disease-causing mutations were identified on all 78 investigated chromosomes. We found 23 different mutations, including 20 missense, 1 nonsense and 2 splice site mutations. All mutations were located within exons or at intronexon boundaries of the phenylalanine hydroxylase gene. Seven mutations occurred at CpG sites, confirming these sites as mutation hot-spots in PKU. Mutations R408W and I65T are the two commonest PKU mutations in the Northern Irish population. Two mutations (T380M and V245A) can be characterized as MHP mutations; they are quasi dominant markers for MHP since they cause mild hyperphenylalaninaemia even when occurring in conjunction with the most severe PKU mutations. The results have proven valuable for the development of a routine PKU mutation analysis system in Northern Ireland.     

Technology seduction: Lost opportunities in child health?

This report examines the extent to which illness-based individual care and expensive, often unevaluated, technologies in paediatrics have seduced practitioners away from more cost-effective, population-based child health activities and examples of new and unevaluated technologies in perinatology and paediatrics are given. The way in which these technologies are introduced and taken up, by 'creeping incrementalism], is described and a plea is made to implement only those aspects of paediatric care that have been demonstrated to be effective. This would result in only appropriate technologies being used, avoid harm being done to children and ensure that money is available for other effective population-based activities that improve child health.