Prevailing therapeutic regimes and predictive factors for prandial insulin substitution in 26 687 children and adolescents with Type 1 diabetes in Germany and Austria.

AIMS: To analyse current therapeutic strategies for prandial insulin substitution in a large number of children and adolescents with Type 1 diabetes in Germany and Austria, along with changes in therapeutic habits and outcome. METHODS: We classified the data of 26 687 patients, treated from 1995 to 2005 in 152 paediatric clinics, using a database established for quality control and scientific surveys in paediatric diabetology (DPV). RESULTS: Seventy-three per cent of all patients (mean age 13.6 years., mean duration of diabetes 5.4 years.) were treated with > or = 4 daily injections (intensified conventional treatment; ICT), 14% with continuous subcutaneous insulin infusion (CSII), 13% with 1-3 injections per day (conventional treatment). Frequency of daily injections increased with age, duration of diabetes and insulin dose. The insulin dose at breakfast was higher than for the evening meal or lunch, from diagnosis onwards. Individuals using insulin analogues received up to 11% higher insulin doses per day compared with patients treated with human insulin. The time of day, age, duration of diabetes, female gender, insulin analogues and ICT all had a significant influence on prandial insulin doses. Although the number of patients treated with ICT or CSII increased over the period of observation, mean glycated haemoglobin (HbA(1c)) was approximately 8.0% each year, and decreased by only 0.01%. CONCLUSIONS: Eighty-seven per cent of patients were treated with ICT or CSII. However, while this percentage increased over the observation period, mean HbA(1c) was almost constant. Longer duration of diabetes, increasing age, female gender, insulin analogues and ICT were associated with higher prandial insulin doses.

Seizure control and acceptance of the ketogenic diet in GLUT1 deficiency syndrome: a 2- to 5-year follow-up of 15 children enrolled prospectively.

BACKGROUND: GLUT1 deficiency syndrome is caused by impaired glucose transport into the brain resulting in an epileptic encephalopathy, developmental delay, and a complex motor disorder. A ketogenic diet provides an alternative fuel to the brain and effectively restores brain energy metabolism. METHODS: Fifteen children with GLUT1 deficiency syndrome were enrolled prospectively for a 2.0 - 5.5-year follow-up of the effectiveness of a 3 : 1 LCT ketogenic diet. Eight patients enrolled were described previously, seven patients were novel. RESULTS: Four novel heterozygous GLUT1 mutations were identified. 10/15 patients remained seizure-free on the ketogenic diet in monotherapy. In 2/15 patients seizures recurred after 2(1/2) years despite adequate ketosis, but were controlled by add-on ethosuximide. In one patient seizures were reduced without complete seizure control. No serious adverse effects occurred and parental satisfaction with the diet was good. 2/15 patients discontinued the diet. CONCLUSION: GLUT1 deficiency syndrome represents a complex childhood encephalopathy that can be treated effectively by means of a ketogenic diet. The response to the diet did not correlate to clinical, biochemical, or genetic features of the disease. In contrast to previous reports, our results indicate that epilepsy is not always completely controlled by a ketogenic diet and can recur in a subset of patients.

Increased frequency of multiple primary melanomas in hereditary familial melanoma.

Multiple primary melanomas can be observed in a frequency of 1-4% in melanoma patients, while they obviously occur more frequently in hereditary familial melanoma. In every 8th patient with an--in itself rare--hereditary familial melanoma a development of two or more primary melanomas has to be expected. Casuistically, a family is reported where a 14-year-old girl died of malignant melanoma, whose mother later on developed two primary malignant melanomas in an interval of 2 years.