Doubly virus-inactivated factor VIII concentrate in haemophilia A.
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Biomedical subjects
Publications and source records attributed to J Corral.
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BACKGROUND: The discovery of the hepatitis C virus (HCV) largely responsible for the non A non B hepatitis, and the antiHCV antibody allows epidemiologic data and risk factors of infection related to the same to be known. The aim of the authors was to know the prevalence of the antiHCV in health care staff in relation with a group of extrahospitalary workers. METHODS: A transversal seroepidemiologic study was carried out in physicians, nurses and auxiliaries with seniority of more than one year in the medical, surgical, emergency, and ICU departments and laboratories of the general hospital of reference (1200 beds). RESULTS: Of the 874 participants (93% of the candidates) 19 (2%) had HCV antibodies detected by ELISA; with positive RIBA II and positive LIA test in 14 (1.6%) of them. In 11 sera ARN of HVC was detected by PCR. Of 547 extrahospitalary workers of the control group, 2 (0.4%) had positive markers against HCV (p < 0.05). The GPT of the seropositive individuals was normal in four and equal to or less than 70 U/l in six. Seven cases (50%) also had serologic data of contagion with HCV. In 10 (1.1%) of the health care staff no risk factor other than hospitalary work was found. CONCLUSIONS: Working in departments attending HCV carriers represents an increase in risk which must be taken into account with regard to accidental exposure to fluids of patients admitted to these areas.
Simple goitre is defined as an enlargement of the thyroid gland that is not the result of an inflammatory or neoplastic process and is not associated with thyrotoxicosis or myxoedema; the cause is unknown in most cases. Structural or regulatory defects in the proteins involved in thyroid metabolism might be involved in the functional abnormality that brings about the disorder. We have found a mutation within exon 10 of the thyroglobulin gene in 25 of 56 members of three families affected by simple goitre; 14 of the gene carriers had the disorder. DNA sequencing showed a mis-sense mutation within thyroglobulin gene exon 10, resulting in a glutamine to histidine substitution. Thus, some cases of non-endemic simple goitre are associated with a mutation at the thyroglobulin locus.
Autosomal dominant cerebellar ataxia type 1 (ADCA1) is a clinical and genetic heterogeneous neurodegenerative disorder which leads to progressive cerebellar ataxia. One defective gene responsible for the disease was first localised to 6p (SCA1, spinocerebellar ataxia type 1) and the mutation has been more recently characterised. We have analysed the CAG-repeat mutation responsible for the SCA1 phenotype in a large Spanish kindred with 41 affected members, in which positive linkage with D6S89 was previously shown. All (10) clinically affected members analysed were heterozygous with one disease allele being between 41 to 57 CAG repeats, and the other in the normal range, from 6 to 39 repeats. Nine clinically unaffected individuals who were between the ages of 18 and 40, were found to have expansions of the CAG repeat (41 to 59), and 22 other 'at risk' individuals were found to have inherited the SCA1 gene with copies of the CAG repeat in the normal range. We have also observed that affected fathers passed on the mutated SCA1 gene with larger increases in the number of CAG repeats than affected mothers did. In one case a decrease in the number of CAG repeats (51 to 50) was detected in the transmission from the affected mother, and in two cases no change was observed in the transmission of a 41 allele repeat by a mother. As in the other disorders in which knowledge of the mutation has been obtained, analysis of the repeat expansion dramatically changes diagnosis of SCA1.
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We have studied N-acetyl-beta-D-glucosaminidase and alpha-D-mannosidase activities in human sera from 35 control subjects, 47 normo- and hyperinsulinemic obese persons, and 12 diabetic patients after a fasting period of 12 h and at 30, 60, 90, and 120 min after an oral glucose overload. The results show a significantly higher activity of these 2 enzymes in obese subjects and diabetic patients, of similar magnitude, especially in those obese persons with a higher grade of obesity. Moreover, the activity of these glycosidases decreases in a similar way in all these 3 groups after the oral glucose overload.
Glycosidases are lysosomal enzymes that participate in the catabolism of glycoproteins and other glycoconjugates, and in some way may modify their activity in situations in which carbohydrate metabolism could be altered, such as the case of obesity. Using a fluorometric assay, a study was made of four glycosidase activities: N-acetyl-beta-D-hexosaminidase (NAG), alpha-mannosidase and alpha- and beta-glucosidase in the serum, pancreas, liver and kidney of 22 Zucker fa/fa genetically obese rats and of 23 fa/? controls, both with ages ranging between 13 and 15 weeks. After 12-14 hours fast and prior anaesthesia with sodium pentobarbital intraperitoneally, blood and the afore-mentioned organs were removed for enzymatic study of the serum and the organs after homogenization and centrifugation. In the serum a statistically significant increase in alpha-mannosidase (p < 0.0001) and alpha-glucosidase (p < 0.02) activities was found in the fa/fa obese rats as compared with the controls. No statistically significant differences were found in serum hexosaminidase activity between the two groups, and no serum beta-glucosidase enzymatic activity was detected. In liver, a decrease was observed in hexosaminidase (p < 0.002) and alpha-glucosidase (p < 0.01) activities in the obese rats as compared with the controls. In whole pancreas an increase was found in alpha-glucosidase activity in the obese rats with respect to the controls (p < 0.001), with no statistically significant differences in the hexosaminidase, alpha-mannosidase and beta-glucosidase activities.(ABSTRACT TRUNCATED AT 250 WORDS)
BACKGROUND: Whether Q fever responds better to doxycycline or erythromycin is unknown. METHODS: The efficacy of doxycycline and erythromycin in the treatment of pneumonia due to Q fever was assessed in a prospective, randomised, double blind study of 82 patients with a diagnosis of pneumonia and features suggestive of Q fever infection; 48 proved to have Q fever. Of the 48, 23 received doxycycline 100 mg twice a day and 25 patients received erythromycin 500 mg six hourly, both for 10 days. RESULTS: Both treatment groups had similar demographic characteristics. Fever showed a more rapid reduction in the doxycycline group (3(1.6) days versus 4.3(2) days). Side effects were observed in two patients receiving doxycycline compared with 11 patients receiving erythromycin (p less than 0.01). No differences were observed in other clinical or radiological measures. By day 40 the chest radiograph was normal in 47 of 48 patients. CONCLUSION: The results demonstrate the self limiting and benign nature of most cases of pneumonia due to Q fever. Doxycycline was more effective than erythromycin.
The penis carcinoma is a neoplasia representing 0.7% of all male neoplasias, with an annual incidence between 1 and 2 cases per 100,000 inhabitants/year. The present study compiles the authors' experience in treating 22 patients presenting this neoplasia in the period between 1977-90. The procedures carried out included local resection, partial amputation, radical penectomy with lymphadenectomy plus radiotherapy, and palliative surgery plus radiotherapy all based on staging made following TNM grading. Therapy complications, follow-up, and survival results are explained. There were 5 occasions of spinocellular carcinoma co-existing with other neoplasias: 1 colon adenocarcinoma, 1 prostate carcinoma, 1 cleaved small cell centrocytic diffuse lymphoma, and 2 skin epithelioma in other sites, and relevant literature was reviewed. A background of psoriasis treated with psoralens plus UV was present in 4 cases, and caution was advised towards genital protection in patients undergoing UV radiations whether for therapeutic, recreational or cosmetic reasons.
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As part of a survey of the expression of homeobox-containing genes in human hematopoietic cells, we identified a novel gene (PL1) expressed only in cells of the myelomonocytic lineage (Shen et al., Proc. Natl. Acad. Sci, USA 86, 8536, 1989). On Northern gel analysis, major transcripts of 3.0 and 2.2 kb length are observed. Alternatively spliced homeobox-containing cDNAs, corresponding to the major transcripts, have been cloned from two myeloid leukemia cell libraries. The two cDNAs share the homeodomain and 3' flanking region but have unique 5' flanking regions. The longer transcript, would encode a 496 amino acid homeobox-containing protein, while the shorter message would encode a 94 amino acid homeobox-containing protein lacking the extended amino-terminal region. These two transcripts are differentially expressed in human leukemia cell lines. The larger transcript is exclusively expressed in cells with myelomonocytic features, while the smaller transcript is expressed in a variety of hematopoietic cell types. PL mRNA is also detectable in normal human bone marrow by RNAse protection. Neither transcript is expressed in uninduced teratocarcinoma cells or in the adult human tissues surveyed. The homeodomain is identical to the genomic sequence for Hox 1H, a newly identified member of the Hox 1 locus (Acampora et al. Nucl. Acids Res. 17, 10385, 1989). The PL1 gene was localized to chromosome 7 using chromosome specific blots and sublocalized to region pI4-21 by in situ hybridization of chromosomal spreads, confirming its location within the Hox 1 complex.
In a group of 92 women with genital condylomata, 15 (16.3%) human immunodeficiency virus-positive patients were found, whereas no case was detected in a control group of 100 women. The relative risk was greater than 19.28. Human immunodeficiency-positive status was associated with other parameters: lower age and parity, major frequency of induced abortions, and sexually transmitted diseases. Thus although human immunodeficiency-positive status seems to be a true risk factor in relation to the altered immunologic state, an indirect association cannot be discarded. Such patients should be screened closely for human papillomavirus infection and cervical cancer. Among human immunodeficiency-positive women, a more resistant behavior of human papillomavirus-associated lesions was detected (recurrence-persistence of 41.7% versus 12%), a fact that might also be in relation to the immunodepressed status.
We have studied phenotypic and clinical features in a consecutive series of 45 patients with chronic myelogenous leukaemia (CML) in blast crisis (BC). In addition, in 22 of these patients we have analysed the genotypic characteristics including immunoglobulin, T-cell receptor (TCR) and major breakpoint cluster region (M-bcr) gene organization. The granulomonocytic and megakaryoblastic lineages are the most commonly involved in these BC of CML (33% and 33% of cases, respectively); only 18% of our cases displayed a lymphoid phenotype. Moreover, both morphological and immunophenotypic studies revealed the frequent coexistence of two or three cell populations, especially when the megakaryoblast component is involved. The lymphoid BC displayed the highest incidence of complete remissions although this was not associated with a longer survival. Only minor differences between the different myeloid subgroups were observed. Immunoglobulin heavy chain (IgH) gene rearrangement was found in five of the six lymphoid BC and in one myeloid BC. Only one case showed k light chain gene rearrangement. In all but one myeloid BC the TCR-beta gene was in germline configuration. The TCR-gamma gene was rearranged in all lymphoid and one myeloid BC, while TCR-delta gene rearrangement was detected in 67% and 16% of the lymphoid and myeloid BC, respectively. Most of the lymphoid BC (4/5) had the M-bcr breakpoint in subregion 3, while the myeloid BC had the breakpoint either in subregion 2 or 3. No differences between the different myeloid phenotypic subgroups were observed in relation to breakpoint.
The influence of the pineal gland on the histopathologic changes induced in mammary glands by 7, 12-dimethylbenz(a)-anthracene (DMBA) has been studied. To achieve the maximum expression of pineal action in animals, 28-day-old female Sprague-Dawley rats were subjected to one of the following treatments: blindness + olfactory bulbectomy (BA), blindness + underfeeding (BU), or blindness + cold exposure (BC). Half of the rats in each group were also pinealectomized. Animals that were intact or only pinealectomized served as controls. Each animal received a single intragastrical dose of DMBA (20 mg) 4 weeks after performing the treatments mentioned above. At 28 weeks of age animals were sacrificed, and mammary tumors as well as mammary glands without macroscopic lesions were collected. Animals of the BU group failed to develop any tumors throughout the 20-week control period. In the remaining groups, tumors were found; the relation between number of adenocarcinomas (AC) and fibroadenomas was similar, always with a greater incidence of ACs. Some ACs showed areas with changes in the ductal epithelium that are considered signs of tumoral regression; pinealectomized animals had the lowest incidence of ACs with signs of regression. Ductule hyperplasia, considered as a premalignant lesion, was more frequent in pinealectomized rats than in controls and in animals with enhanced pineal function (BA, BU, and BC). Some nontumoral mammary glands showed dysplastic lesions; these lesions were less frequent in BU rats than in controls. We conclude that suppression of pineal function sensitizes the ductule cells to the initiation of neoplastic processes and hinders the development of regressive changes.
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Addition of increasing amounts of zinc to a cold microtubule protein solution results in the disappearance of 30 S oligomer found in the absence of that cation and in the appearance of new tubulin oligomers, 90 S and 23 S. When a microtubule protein solution is warmed in the presence of zinc, tubulin-sheets are assembled. We have tested the influence of microtubule associated proteins and the zinc:tubulin ratio on the polymerization process. Depletion of microtubule associated proteins results in wider and longer tubulin-sheets than those polymerized in the presence of microtubule associated proteins. However by increasing zinc concentration wider but shorter tubulin-sheets were found. These results suggest that microtubule associated proteins and zinc could promote nucleation of tubulin-sheets, but zinc also promotes lateral tubulin-tubulin interaction. This interpretation was confirmed when microtubule protein was assembled at a low zinc:tubulin ratio. In such conditions composite structures of microtubules and zinc tubulin-sheets are formed. These composite structures are consequence of a lateral attachment of a zinc tubulin-sheet on a microtubule protofilament.
Pyruvate decarboxylase activity in the dimorphic fungus Mucor rouxii increased 25- to 35-fold in yeastlike and mycelial cells grown in the presence of glucose as compared to the activity observed in mycelial cultures grown in the absence of glucose.
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