Preradiation chemotherapy of children and young adults with malignant brain tumors: results of the German pilot trial HIT'88/'89.

Background: Preradiation chemotherapy could be beneficial in malignant brain tumors, because the blood-brain tumor-barrier is disrupted after surgery, bone marrow recovery--essential for intense chemotherapy--is still intact, and CNS toxicity and ototoxicity of active drugs are lower before irradiation of a child's brain.

Patients And Methods: A neoadjuvant phase 2 and a single arm pilot trial were initiated to investigate the efficacy and toxicity of an intense multidrug regimen before radiotherapy in 147 patients aged between 3 and 29; 9 years with medulloblastoma (94), malignant glioma (22), ependymoma (21), and stPNET (10). They were treated with one or two cycles consisting of procarbazine, ifosfamide/mesna with etoposide, high dose methotrexate/CF, and cisplatin with cytarabine.

Effect of fish-oil-enriched margarine on plasma lipids, low-density-lipoprotein particle composition, size, and susceptibility to oxidation.

We investigated the effect of incorporating n-3 polyunsaturated fatty acids (PUFAs) into the diet on the lipid-class composition of LDLs, their size, and their susceptibility to oxidation. Forty-seven healthy volunteers incorporated 30 g sunflower-oil (SO) margarine/d into their habitual diet during a 3-wk run-in period and then used either SO or a fish-oil-enriched sunflower oil (FO) margarine for the following 4 wk. Plasma concentrations of total cholesterol, triacylglycerols, HDL cholesterol, LDL cholesterol, and apolipoproteins A-I and B did not differ significantly between the groups during intervention.

Favorable outcome of giant cell glioblastoma in a child. Report of an 11-year survival period.

Giant cell glioblastomas are defined as glioblastomas with a marked predominance of bizarre, multinucleated giant cells. They represent about 5% of all glioblastomas and can occur at any site of the central nervous system, but the temporal and frontal lobes are the sites of predilection. Overall, giant cell glioblastomas show a prolonged survival period compared with common glioblastoma multiforme, and survival periods of 7 and 9 years have been reported in adults.

Perioperative fluid and electrolyte management in children undergoing surgery for craniopharyngioma. A 10-year experience in a single institution.

In a retrospective study, the intra- and early postoperative data of 39 children with 46 operations for craniopharyngioma were analyzed. Diabetes insipidus (DI) occurred in 30 out of 32 cases without preoperative evidence of DI. We observed that all children who did not have a pituitary stalk preserved and 5 out of 7 patients with preserved pituitary stalk developed DI within 18 h of surgery.

Molecular analysis of childhood primitive neuroectodermal tumors defines markers associated with poor outcome.

Purpose: The diagnostic and prognostic significance of well-defined molecular markers was investigated in childhood primitive neuroectodermal tumors (PNET).

Materials And Methods: Using microsatellite analysis, Southern blot analysis, and fluorescence in situ hybridization (FISH), 30 primary tumors and six CSF metastasis specimens were analyzed for loss of heterozygosity (LOH) of chromosomes 1q31, 6q, 9q22, 10q, 11, 16q22, and 17p13.1 and/or high-level amplification of the c-myc gene.

A possible role for somatostatin receptor scintigraphy in the diagnosis and follow-up of children with medulloblastoma.

The surgical resection of medulloblastoma (MB), the most frequent malignant brain tumor in children, often remains subtotal. To estimate the response to further treatment the residual tumor is monitored by CT or MRI. The interpretation of both imaging techniques is complicated by disturbances resulting from surgery and radiation.

Cognitive deficit in 7-year-old children with prenatal exposure to methylmercury.

A cohort of 1022 consecutive singleton births was generated during 1986-1987 in the Faroe Islands. Increased methylmercury exposure from maternal consumption of pilot whale meat was indicated by mercury concentrations in cord blood and maternal hair. At approximately 7 years of age, 917 of the children underwent detailed neurobehavioral examination.

Medulloblastomas of the desmoplastic variant carry mutations of the human homologue of Drosophila patched.

Inactivating mutations in the PTCH gene, a human homologue of the Drosophila segment polarity gene patched, have been identified recently in patients with nevoid basal cell carcinoma syndrome. These patients are predisposed to various neoplasias including basal cell carcinomas and medulloblastomas (MBs). To determine the involvement of PTCH in sporadic MBs, which represent the most frequent malignant brain tumors in children, we screened for PTCH alterations in an unselected panel of 64 biopsy samples from 62 patients and four continuous MB cell lines, all derived from patients with sporadic MBs.

Odontogenic classification of craniopharyngiomas: a clinicopathological study of 54 cases.

Based on the striking histological similarity of craniopharyngiomas and some odontogenic tumours, we reclassified a series of 54 craniopharyngiomas (52 adamantinomatous and two papillary variants) according to the WHO classification of odontogenic tumours. Twenty-seven tumours (50%) corresponded histologically to calcifying odontogenic cyst. 13 tumours (24%) to ameloblastoma, and eight (15%) tumours showed features of both calcifying odontogenic cyst and ameloblastoma either within the same specimen or in specimens derived from different resections.

Pleomorphic xanthoastrocytoma: report of six cases with special consideration of diagnostic and therapeutic pitfalls.

Background: Pleomorphic xanthoastrocytoma (PXA) is a rare clinicopathologic entity that occurs typically in young patients. Improved neuroradiologic techniques, especially gadolinium-enhanced magnetic resonance imaging (MRI), reveal a characteristic tumor appearance.

Methods: We present six cases of PXA operated on with unusual clinical course, elucidating different clinical implications.

Quantification, by solid-phase minisequencing, of the telomeric and centromeric copies of the survival motor neuron gene in families with spinal muscular atrophy.

In an analysis of 30 families affected by spinal muscular atrophy (SMA) we have used the solid-phase minisequencing method to determine the ratio between the number of telomeric and centromeric copies of the survival motor neuron gene (SMN and cBCD541 respectively) on normal and SMA chromosomes. This has enabled us to establish haplotypes with regard to SMN and cBCD541, and estimate their frequencies, on both types of chromosomes. Six predominant haplotypes were identified, three for normal chromosomes and three for SMA chromosomes, characterized by having 0, 1, or 2 copies, respectively, of cBCD541.

High-resolution deletion mapping of chromosome arm 17p in childhood primitive neuroectodermal tumors reveals a common chromosomal disruption within the Smith-Magenis region, an unstable region in chromosome band 17p11.2.

Loss of heterozygosity (LOH) on chromosome arm 17p is the most common genetic aberration in childhood primitive neuroectodermal tumors (PNETs). To determine the frequency and extent of 17p deletions, 29 loci on 17p were investigated in 24 tumors by using restriction fragment length polymorphism (RFLP) and microsatellite analysis. LOH on 17p was found in 9 of 24 tumors.

Consensus: trigonocephaly.

Trigonocephaly mostly occurs in isolated synostosis of the metopic suture, but 10-20% of patients are affected by complex syndromes, and in 2-5% the condition is familial. Intracranial hypertension has been observed in a minority of cases, but is never severe. Surgery is performed mainly for cosmetic reasons, following the standard principles of craniofacial surgery.

Molecular analysis of medulloblastomas occurring simultaneously in monozygotic twins.

Unlabelled: We report male monocygotic twins with concordant desmoplastic medulloblastoma diagnosed at the age of 20 months. Both tumours were completely removed. As chromosomal loci 17p13 and 9q31 are frequently altered in medulloblastoma these regions were analysed in both tumours in detail using restriction fragment length polymorphism and microsatellite analysis.

Feasibility and validity of three computer-assisted neurobehavioral tests in 7-year-old children.

Three tests from the computerized Neurobehavioral Examination System (NES) were administered to a group of 917 Faroese children at approximately 7 years of age. The NES Continuous Performance Test (CPT) was modified to use animal silhouettes as stimuli instead of letters. Almost all children completed Finger Tapping (FT), the modified CPT, and Hand-Eye Coordination (HE).

Molecular analysis of the lissencephaly gene 1 (LIS-1) in medulloblastomas.

Although medulloblastoma (MB) represents the most frequent malignant brain tumour in children, the molecular pathogenesis of this tumour is still poorly understood. Microsatellite and RFLP studies have revealed allelic losses on the short arm of chromosome 17 in the region 17p13 in approximately 50% of MBs. A candidate for this putative MB suppressor on chromosome 17p13.

Processing of marine foods.

For the Norwegian fish industry, it is an objective to increase the production of value added products in order to improve profitability. This paper will briefly present four areas of important research tasks in this field. To aid in the identification of the species present in a product, we have applied the method called Random Amplification of Polymorphic DNA (RAPD).

Recurrent bacterial meningitis.

Unlabelled: To characterize recurrent bacterial meningitis in children, we reviewed the charts of all patients treated for more than one episode of bacterial meningitis at the Würzburg University Children's Hospital from 1980 to June 1995. Twenty-five children suffered 2-13 episodes of bacterial meningitis. Most patients were referred from other hospitals to our paediatric neurosurgical service.

High incidence of cystic fibrosis on the Faroe Islands: a molecular and genealogical study.

We have studied the genetics of cystic fibrosis (CF) in The Faroe Islands. Based on the number of affected children born during the period 1954-1993, the incidence of CF at birth is 1:1775, which is more than twice the incidence in the rest of Denmark. We have tested all known CF patients and/or their parents for the presence of delta F508 and found it to be the only CF mutation in this population.

Mechanism of post-segregational killing: secondary structure analysis of the entire Hok mRNA from plasmid R1 suggests a fold-back structure that prevents translation and antisense RNA binding.

The hok/sok system of plasmid R1 mediates plasmid stabilization by killing of plasmid-free cells. The Hok mRNA is very stable and can be translated into Hok killer protein. Translation of the Hok mRNA is inhibited by the small unstable Sok antisense RNA.

Mechanism of post-segregational killing: Sok antisense RNA interacts with Hok mRNA via its 5'-end single-stranded leader and competes with the 3'-end of Hok mRNA for binding to the mok translational initiation region.

The hok/sok system of plasmid R1, which mediates plasmid stabilization by killing of plasmid-free segregants, codes for two RNA species, Hok mRNA and Sok antisense RNA. The lethal expression of hok is inhibited post-transcriptionally by the 67 nt Sok-RNA. In this paper, we analyse the secondary structure of Sok-RNA and the binding of Sok-RNA to Hok mRNA in vitro.

Intracranial neurothekeoma (nerve-sheath myxoma). Case report.

Neurothekeoma is a benign cutaneous nerve-sheath tumor. The authors describe the case of a 16-year-old girl with a parasellar neurothekeoma that was successfully removed. Magnetic resonance imaging with gadolinium administration revealed bright enhancement of the peripheral portions and nonhomogeneous uptake in the central areas, which corresponded histologically to the vascular capsule and the myxoid center.

Abdominal obesity is associated with insulin resistance and reduced glycogen synthetase activity in skeletal muscle.

Insulin resistance is commonly associated with obesity. The present study was performed to investigate the relative importance of total fat mass versus localization of adipose tissue in insulin-stimulated glucose disposal (Rd) and skeletal muscle glycogen synthase (GS) activity in obese individuals. Twenty obese women with an average body mass index (BMI) of 37.