An infant with neonatal adrenoleukodystrophy experienced extreme hypotonia and virtually continuous convulsions at four months of age and died. Light and electron microscopic examination revealed evidence of myopathy and the presence of mitochondrial inclusions. Concentrations of very long-chain fatty acids were elevated in blood and fibroblasts and the oxidation of 14C-labeled fatty acids was defective. Urinary pipecolic acid content was increased. Activity of the peroxisomal dihydroxyacetone phosphate acyltransferase, which catalyzes the first step in plasmalogen synthesis, was decreased.

Acyltransferases↗

PubMedDate not supplied

Isolation of microtubule protein from mammalian brain frozen for extended periods of time.

Microtubule protein (MTP) may be isolated in good yield from frozen brains by cycles of temperature-dependent polymerization and depolymerization. If the brains are frozen quickly and stored at -70 degrees C, the yield of MTP is stable for a period of at least 2 months and the yield is only slightly decreased after nearly a year. Cow as well as rat brains may be stored in this manner, provided appropriate precautions are taken to ensure rapid freezing of the cow brain. This procedure allows brains to be accumulated over a period of time for MTP isolation at a convenient later date.

Animals↗

PubMedDate not supplied

Asparagine concentration in plasma after 2,500 IU/m(2) PEG-asparaginase i.v. in children with acute lymphoblastic leukemia.

UNLABELLED: Polyethylene glycol conjugated asparaginase (PEG-ASNase) can be substituted in cases of hypersensitivity to native Escherichia coli asparaginase. We measured asparagine (asn) levels in plasma after a single dose of 2,500 IU/m(2) i.v. PEG-ASNase (Oncaspar) in consolidation treatment of ALL and compared those with data from the previous protocol COALL-05-92. This protocol was similar to COALL-06-97, except that children had been given 45,000 IU/m(2) C-ASNase instead of PEG-ASNase. PATIENTS AND METHODS: Between May 2000 and December 2001 seventy-one children (38 boys, 33 girls) with newly diagnosed ALL treated according to the multicenter protocol COALL-06-97 were investigated in this study. Four hundred and seventy-four plasma samples (71 patients) were analysed by ion exchange chromatography after column derivatization with o-phthaldialdehyde. For comparison data (350 plasma samples) from 51 patients treated according to the protocol COALL-05-92 were available. The same method for detection of asn in plasma was used. RESULTS: The median asparagine level in plasma after 2,500 IU/m(2) PEG-ASNase i.v. was below the limit of detection for at least 5 weeks in 81 % of the patients. When divided into high risk (HR) and low risk (LR) group, HR patients who had previously received one dose more of C-ASNase showed a markedly shorter depletion than the LR patients compatible with a higher risk of antibody formation and consequent silent inactivation after a higher number of exposures to ASNase. In the previous protocol COALL-05-92 median asn levels in plasma after 45,000 IU/m(2) native C-ASNase i.v. were below the limit of detection for at least 5 weeks in 65 % of the patients. CONCLUSIONS: 2,500 IU/m(2) PEG-ASNase led to an equally long depletion of asn in plasma as did 45,000 IU/m(2) native C-ASNase i.v. used in COALL-05-92.

Adolescent↗

PubMedDate not supplied

[Smoking behavior in adolescents: a comparison of population-representative, regional or selectively assessed data and implications for prevention].

INTRODUCTION: The prevalence of adolescent smoking underlines the necessity of preventive measures, which goals are based on representative data. It is not known whether schools participate in prevention interventions, where smoking constitutes a relatively big or minor problem. OBJECTIVE: This study examines a population of adolescents on (a) different smoking variables and (b) compares them with representative, population based data. METHODS: Survey of n = 324 adolescents of grade 7-10 in 3 schools in Greifswald and surroundings that were ready to participate in a prevention curriculum. RESULTS: In total, 80% of the students under examination indicated to have at least tried smoking in their lifetime. Daily smokers were 31%, 18% were occasional smokers, 39% have indicated that they hat tried to quit without success. These figures vary across age, grade and sex. Smoking prevalence is comparable between schools ready to participate in prevention and regionally assessed data, but much higher than population based data would have estimated. CONCLUSION: The goal of preventive measures can not be solely grounded on representative, population-based data, but needs (a) to be regionally defined and (b) to consider the population actually participating in such prevention interventions. The readiness to participate is not higher in schools where smoking constitutes a comparable minor problem. Preventive measures are applied in schools where the problem is perceived.

Adolescent↗

PubMedDate not supplied

[Pharmacokinetics of etoposide short-term infusions within the scope of the GPOH therapy protocol].

Important interpatient variability of etoposide pharmacokinetics during continuous infusion has been reported and drug level targeting, therefore, been suggested. The current German therapeutic protocols are mainly based on short-term infusion. We determined pharmacokinetic parameters during short-term infusion in 18 children aged between 10/12 and 17 years on different therapeutic schedules in order to investigate interpatient variability. The dosages ranged from 66 to 200 mg/m2. In the subgroup of patients who received 150 mg/m2 (n = 10) the AUC was 106 +/- 15 micrograms.h/ml (range 82-139), clearance 24 +/- 3 ml/min/m2 (18-31), t1/2 3.5 +/- 0.4. To compare kinetic data of all 21 courses in 18 children, standard AUC was calculated (AUC/100 mg/m2). The AUC then was (68 +/- 17 micrograms.h/ml)/(100 mg/m2). Half-life was 3.3 +/- 0.7 h and total clearance 26 +/- 6 ml/min/m2, respectively. In 5 courses in children < 2 years (< 10 kg), pharmacokinetic parameters were within the normal range. In this group dosage was calculated per kg. Dose reduction of 31% (mean) resulted in 22% (mean) lower AUC's. In conclusion, interpatient variability of etoposide pharmacokinetics during short-term infusion is limited. Dose reduction in children < 2 years is not substantiated by our pharmacokinetic data.

Adolescent↗