All heart transplant patients in our clinic received intravenous immunoglobulins as a prophylaxis against cytomegalovirus infections or reactivations. Serum was sampled from 160 heart transplant patients within 4 months after surgery. In 98 samples (61%) hepatitis C virus (HCV)-specific antibodies could be detected by a "second generation" enzyme immunoassay. Of these HCV antibody-positive patients 89 were tested for a second time. At this time, 5-11 months later, in 66 patients (74%) the HCV antibody had disappeared. In the 23 still positively reacting patients, immunoglobulins were given in the last 6 months before serum sampling. Nine commercial immunoglobulin preparations were tested for HCV-specific antibodies and the presence of HCV RNA. Seven preparations were anti-HCV positive with titres in the range of 64-256, whereas reverse transcription and polymerase chain reaction did not detect HCV RNA in any immunoglobulin preparation. Passive antibody transfer rather than a HCV infection is the cause of HCV antibody detection in our patients. The presence of HCV antibodies in high concentrations in commercial immunoglobulin preparations may only be explained by an extremely high proportion of anti-HCV-positive single donations in the plasma pools used for immunoglobulin production. The passive HCV antibody transmission prevents anti-HCV serological monitoring of patients treated with these preparations. Additionally, there are reports on the transmission of hepatitis non-A, non-B via immunoglobulin preparations. Therefore, we recommend an anti-HCV screening of plasma donors.

Background: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that affects motor neurons. In Europe, disease-causing genetic variants have been identified in 40–70% of familial ALS patients and approximately in 5% of sporadic ALS patients. In Norway, the contribution of genetic variants to ALS has not yet been studied. In light of the potential development of personalized medicine, knowledge of genetic causes of ALS in a population is becoming increasingly important. The present study provides clinical and genetic data on familial and sporadic ALS patients in a Norwegian population-based cohort. Methods: Blood samples and clinical information from ALS patients were obtained at all 17 neurological departments throughout Norway during a 2-year period. Genetic analysis of the samples involved expansion analysis of C9orf72 and exome sequencing targeting 30 known ALS-linked genes. The variants were classified using genotype-phenotype correlations and bioinformatics tools. Results: A total of 279 ALS patients were included in the study. Of these, 11.5% had one or several family members affected with ALS, whereas 88.5% had no known family history of ALS. A genetic cause of ALS was identified in 31 individuals (11.1%), among which 18 (58.1%) were familial and 13 (41.9%) were sporadic. The most common genetic cause was the C9orf72 expansion (6.8%), which was identified in 8 familial and 11 sporadic ALS patients. Pathogenic or likely pathogenic variants of SOD1 and TBK1 were identified in 10 familial and 2 sporadic cases. C9orf72 expansions dominated in patients from the Northern and Central regions, whereas SOD1 variants dominated in patients from the South-Eastern region. Conclusion: In the present study, we identified several pathogenic gene variants in both familial and sporadic ALS patients. Restricting genetic analysis to only familial cases would miss more than 40 percent of those with a disease-causing genetic variant, indicating the need for genetic analysis in sporadic cases as well.

Stepwise treatment of ortho-hydroxy-arenonitriles with 1,1¢-carbonyl-di-(1,2,4-triazole) and O-substituted hydroxylamines in the presence of Et 3 N gave 4-methoxy(aralkoxy)imino-benzo[1,3]oxazin-2-ones and 4-methoxy(aralkoxy)imino-naphtho[2,3e][1,3]oxazin-2-ones.

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