Increased incidence of HL-A 1 and 8 in patients showing IgG or complement coating on their red cells

The incidence of HL-A antigens in patients having a variety of medical conditions, but sharing the common feature of a positive antiglobulin test of the red cells, was determined. There was a significant increase in the antigens HL-A 1 and HL-A 8 and of the 1 and 8 combination when compared with a control group. It is suggested that the presence of these antigens may predispose to autoallergy.     

Alexithymia in young adulthood: a risk factor for pathological gambling

BACKGROUND: Pathological gambling is more prevalent among postsecondary students than among the general adult population. While the prevalence of pathological gambling in this group has risen over the past decade, factors underlying the development of problem gambling among university students remain largely unexplored. One early study found alexithymia to be associated with pathological gambling. The aim of the present study was to further examine the relationship between alexithymia and gambling among postsecondary students. METHODS: The relationship between alexithymia and pathological gambling was examined in 562 postsecondary students who completed the South Oaks Gambling Screen (SOGS) and the 20-item Toronto Alexithymia Scale (TAS-20). RESULTS: Approximately 12% of the sample was classified as alexithymic according to the TAS-20. These individuals were found to have significantly more gambling problems, as measured by the SOGS, than nonalexithymic individuals. Approximately 9% of the sample was classified as pathological gamblers according to the SOGS. These individuals were found to have significantly higher levels of alexithymia, as measured by the TAS-20, than nonproblem gamblers. CONCLUSIONS: Alexithymia is associated with pathological gambling and may be a risk factor among postsecondary students for developing severe gambling problems.     

The Revised Toronto Alexithymia Scale: some reliability, validity, and normative data.

Addressing certain problems with the compositional structure of the self-report Toronto Alexithymia Scale, this paper reports the development of a revised version of the scale as well as results from a preliminary series of studies evaluating its reliability and validity. The Revised Toronto Alexithymia Scale (TAS-R) demonstrated good internal consistency and a stable and replicable two-factor structure that is congruent with the two major dimensions of the alexithymia construct. Evidence of convergent and discriminant validity for the TAS-R was provided by correlations with measures of other constructs. Criterion validity was demonstrated by the ability of TAS-R scores to discriminate between behavioral medicine outpatients designated alexithymic and those designated non-alexithymic on the basis of clinical interview ratings. Normative data are provided for university student, normal adult, and psychiatric outpatient populations.     

Egfl7 knockdown causes defects in the extension and junctional arrangements of endothelial cells during zebrafish vasculogenesis.

The endothelial cell (EC) -specific secreted protein EGFL7 is important for tubulogenesis in newly forming blood vessels. We studied its role in vascular tube formation by a quantitative ultrastructural analysis of Egfl7-knockdown zebrafish embryos. At 24 hours postfertilization, the endothelia of dorsal aorta (DA) and posterior cardinal vein (PCV) were correctly anchored to the hypochord and endoderm, respectively, but failed to expand into the vascular area. This resulted in vessels with reduced or split lumen and open sheets of ECs. Concomitantly, the organization of hematopoietic cells-identified by the presence of previously undescribed membrane tubules-between DA and PCV, and within the vessels, was severely disturbed. Strikingly, ectopic cell junctions occurred across the obstructed vessel lumen, on the luminal EC surfaces, which in control conditions never display junctions of any kind. These data suggest that Egfl7 provides ECs with a cue for their extension into the vascular area and in establishing EC cell polarity.     

In vivo macrophage suppression of delayed hypersensitivity in the guinea-pig.

The nature of the suppressive activity in the peritoneal exudate cells (PEC) of guinea-pigs immunized with dinitrophenyl bovine gamma globulin (DNP50-BGG) was investigated. A method was developed to isolate from the peritoneal exudate large numbers of macrophages. Using density gradient centrifugation on Percoll it was possible to obtain a population of cells which contained over 90% macrophages. This macrophage preparation was found to respond to lymphokine but to be incapable of passively transferring delayed hypersensitivity reactions. When these immune macrophages were transferred into antigen immunized animals, which had been pretreated with cyclophosphamide (CY), the skin reactions were suppressed to the same extent as when the total PEC was transferred. PEC from guinea-pigs immunized with ovalbumin in Freund's incomplete adjuvant did not suppress the skin reactions in CY-pretreated DNP50BGG immunized animals. However, in contrast, macrophages from these animals did suppress the skin reactions in the recipient guinea-pigs indicating that the macrophage suppression was not antigen specific.     

Comparison of three methods for identification ofEnterobacteriaceae

This study compares the ability of three commercial overnight methods, API 20E, Minitek and Enteric-Tek, to accurately and completely identify 368 clinically isolatedEnterobacteriaceae without supplemental tests. Organisms includedEscherichia coli (54 strains),Shigella spp. (7),Edwardsiella tarda (1),Salmonella enteritidis (10),Citrobacter spp. (30),Klebsiella spp. (55),Enterobacter spp. (68),Hafnia alvei (2),Serratia spp. (33),Proteus spp. (64),Morganella morganii (24),Providencia spp. (18), andYersinia enterocolitica (2). Methods were those of the manufactures without supplemental tests. API 20E correctly identified 93.2 % of strains to species and 3.3 % to genus level only, with 3.0 % as part of a spectrum of identifications, and 0.5 % incorrect identifications. Minitek yielded 96.0 % correct identifications to species and 0.5 % to genus level only, with 2.5 % spectrum identifications, and 1.0 % incorrect identifications. Enteric-Tek correctly identified 97.0 % of strains to species level with 3.0 % spectrum identifications. API 20E identification of someSerratia andCitrobacter strains was to genus level only. Problem organisms for Minitek includedEnterobacter agglomerans andSerratia marcescens. A comparison of these three commercial methods shows that all three have the ability to identify most clinically isolatedEnterobacteriaceae without supplemental tests.     

Characterization of Vibrio cholerae protease activities with peptide digest analysis.

A simple method for the analysis of microbial proteases is described that was used to characterize the proteolytic activities of various Vibrio cholerae isolates. This method utilized the unique peptides generated from the degradation of a standard protein by proteases of various specificities. These peptides were analyzed by sodium dodecyl sulfate-polyacrylamide gel electrophoresis. The unique patterns of peptides seen in gels can be used to type proteases according to their relative specificities. Culture supernatants of V. cholerae isolates from a variety of environmental and human sources were analyzed for the presence of a protease previously isolated and characterized in this laboratory from V. cholerae strain CA401. Supernatants from most isolates showing dimethyl casein proteolytic activity exhibited the presence of enzymes similar to the CA401 protease in their peptide digest patterns against bovine serum albumin and in their immunological reactivities. The probable widespread presence of this virulence-associated protease in V. cholerae isolates is discussed.     

Spectrum of CLN6 mutations in variant late infantile neuronal ceroid lipofuscinosis

The neuronal ceroid lipofuscinoses (NCLs) are a group of autosomal recessive neurodegenerative diseases of childhood. CLN6, the gene mutated in variant late infantile NCL (vLINCL), was recently cloned. We report the identification of eight further mutations in CLN6 making a total of 18 reported mutations. These mutations include missense, nonsense, small deletions or insertions, and two splice-site mutations. Ten mutations affect single amino acids, all of which are conserved across vertebrate species. Minor differences in the pattern of disease symptom evolution can be identified. One patient with a more protracted disease progression was a compound heterozygote for a missense mutation and an unidentified mutation. Fifteen CLN6 mutations occur in one or two families only, and families from the same country do not all share the same mutation. Unlike NCLs caused by mutations in CLN1, CLN3, CLN5, and CLN8, there is no major founder mutation in CLN6. However, one mutation (E72X) is significantly more common in patients from Costa Rica than two other mutations present in that same population. In addition, a 1-bp insertion (c.316insC) is associated with families from Pakistan and I154del may be common in Portugal. A group of Roma Gypsy families from the Czech Republic share two disease-associated haplotypes, one of which is also present in a Pakistani family, consistent with the proposed migration of the Roma from the Indian subcontinent 1,000 years ago. All mutations are recorded in the NCL Mutation Database together with their country of origin for use in the development of rapid screening assays to confirm diagnosis and to facilitate carrier testing appropriate to a population.     

Carnitine deficiency, organic acidemias, and Reye's syndrome

Relative carnitine deficiency is important in the pathophysiology of several disorders, including Reye's syndrome and organic acidemias. In acute clinical crises, carnitine serves as a "buffer," trapping toxic acyl compounds. Mitochondrial failure develops in carnitine deficiency when there is insufficient tissue carnitine available to buffer toxic acyl-CoA metabolites. Toxic levels of acyl-CoA impair the citrate cycle, gluconeogenesis, the urea cycle, and fatty-acid oxidation. Carnitine replacement therapy is safe and induces excretion of toxic acyl groups in the urine.