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The article discusses several options for the treatment of a rare bleeding disorder called Acquired Von Willebrand Syndrome (AVWS) resulting from reduction in plasma von Willebrand Factor (VWF). According to the author, intravenous immunoglobulin, plasmapheresis and immunomodulatory drugs can be used for treatment instead of desmopressin (DDAVP) and tranexamic acid. AVWS can be found in patients with no previous personal or family history of Von Willebrand Disease (VWD).

The article reports a case of severe haemophilia A and X-linked moyamoya syndrome in a 37-year-old male patient. It is noted that the combined disorder resulted from a single genetic event, a gross deletion of ~250 kb telomeric involving a portion of F8 gene and adjacent genes. Results of the patient's genetic investigation and chromosomal analysis, which include polymerase chain reaction amplification, as well as the clinical features of X-linked moyamoya syndrome are discussed.

Mounting evidence indicates the presence of a near complete biological nitrogen cycle in redox-stratified oceans during the late Archean to early Proterozoic ( c. 2.5-2.0 Ga). It has been suggested that the iron (Fe)- or vanadium (V)-dependent nitrogenase rather than molybdenum (Mo)-dependent form was responsible for dinitrogen fixation during this time because oceans were depleted in Mo and rich in Fe. We evaluated this hypothesis by examining the phylogenetic relationships of proteins that are required for the biosynthesis of the active site cofactor of Mo-nitrogenase in relation to structural proteins required for Fe-, V- and Mo-nitrogenase. The results are highly suggestive that among extant nitrogen-fixing organisms for which genomic information exists, Mo-nitrogenase is unlikely to have been associated with the Last Universal Common Ancestor. Rather, the origin of Mo-nitrogenase can be traced to an ancestor of the anaerobic and hydrogenotrophic methanogens with acquisition in the bacterial domain via lateral gene transfer involving an anaerobic member of the Firmicutes. A comparison of substitution rates estimated for proteins required for the biosynthesis of the nitrogenase active site cofactor and for a set of paralogous proteins required for the biosynthesis of bacteriochlorophyll suggests that Nif emerged from a nitrogenase-like ancestor approximately 1.5-2.2 Ga. An origin and ensuing proliferation of Mo-nitrogenase under anoxic conditions would likely have occurred in an environment where anaerobic methanogens and Firmicutes coexisted and where Mo was at least episodically available, such as in a redox-stratified Proterozoic ocean basin. [ABSTRACT FROM AUTHOR]

Malignant myoepithelioma of the breast (MMB) is a rare and often aggressive disease with poor prognosis. Little is known regarding its optimal treatment and progression. We describe the clinical history of a woman following excision of a benign adenomyoepithelioma which recurred years later as a radioresistant malignant myoepithelioma with high levels of ataxia telangiectasia mutated protein and mutant p53 (Cys135Phe). MMB requires close follow-up and aggressive treatment. If adjuvant radiotherapy is adopted to improve local control, minimal postoperative delay and higher doses than for standard post-mastectomy radiation are recommended. [ABSTRACT FROM AUTHOR]

To compare the apical density of several obturation techniques when used in palatal roots of extracted maxillary molars. Simplifill as recommended, Thermafil, mechanical lateral and WVC (Schilder) obturation techniques created more complete obturation using gutta-percha at the 2 and 4 mm levels than cold lateral, WVC (continuous wave), and Simplifill not used as directed. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to the article about age-related factor IX correction in symptomatic female carriers with haemophilia B Leyden.

Recently published studies on the development and use of recombinant vaccinia virus (VV) vaccines incorporating either the complete envelope (env) gene or only a fragment of the env gene consisting of the coding sequence for the env glycoprotein 51 (gp5l) and part of gp30 of the bovine leukaemia virus (BLV) are described. It has been reported that vaccination of sheep with recombinant VV vaccines containing the complete env gene appears to protect sheep against challenge infection with BLV. The evidence for this protection is based on the lack of persistence of high titres of anti-gp51 antibodies compared with unvaccinated BLV infected controls, on the enhanced CD4 proliferative responses to specific BLV gp51 synthetic peptides in the vaccinated sheep, and on the inability to detect BLV pro-virus by polymerase chain reaction in the vaccinated sheep after 4 months following challenge infection compared with continual detection in unvaccinated sheep over a 16 month trial period. It has been suggested that cell-mediated immune responses may be an important aspect of protective immunity against BLV infection and it has been reported that large tracts of amino acid sequences within the env and poi genes are highly conserved in different isolates from different countries which is of importance in designing peptide derived vaccines. [ABSTRACT FROM AUTHOR]

Immunoglobulin and T cell receptor gene probes have been used to investigate cell lineage and monoclonality in lymphoid malignancies. In the present study we have used T cell receptor β-and γ-chain gene probes to screen for abnormal rearrangements of these genes in B lymphoblastoid cells from patients with ataxia-telangiectasia (A-T). No rearrangement of either gene was observed but deletion of a β-chain gene allele is described for one A-T ceil line. Expression of mRNA hybridizing to the β-chain gene probe was demonstrated for two A-T homozygotes (brother and sister) as well as for their mother (heterozygote). This transcript was found to be truncated in all three cases. [ABSTRACT FROM AUTHOR]

In order to elucidate whether natural infection of BLV in cattle might induce humoral immunological responses, changes in IgG1, IgG2 and IgM concentrations in the sera of infected cattle were determined. Twelve BLV-infected cattle were used. Cattle of different breeds were classified serologically and haematologically into BLV+PL+, BLV+PL− and BLV-free groups. Ig concentrations in the sera of the three groups were quantitated using a commercial single radial immunodiffusion assay. The findings were compared to those of BLV-free cattle. The serum IgM concentrations were significantly lower in cattle with PL (P< 0.001) than in BLV+PL− and BLV-free cattle. The IgM concentrations tended to be lower in BLV+PL− than those of BLV-free cattle. There were no significant differences in IgG1 and IgG2 serum concentrations between the three experimental cattle groups. IgG1 was the predominant subtype in the sera of all cattle groups. [ABSTRACT FROM AUTHOR]

This study was designed to determine the relative infectivity of lymphocytes and secretions from BLV-infected cattle with and without persistent lymphocytosis (BLV+PL+ and BLV+PL−). Ninety-seven sheep of mixed sex and age were assembled into 21 experimental groups. The recipient sheep were inoculated intravenously with serial dilutions of whole blood, saliva or nasal secretions from BLV+PL+ and BLV+PL− donor cows. Between 200 to 20,000 cells from single and mixed BLV+PL+ or single and mixed BLV+PL− donor cattle were used for inoculation. A very small number of BLV-infected lymphocytes (200 cells) was sufficient to induce BLV infection in sheep inoculated with diluted whole blood from BLV+PL+ cattle. The inoculation of whole blood (containing up to 20,000 lymphocyte cells) from BLV+PL− cattle did not induce BLV infection in recipient sheep. Saliva and nasal secretions also failed to bring about BLV transmission. Zusammenfassung Eine experimentelle Infektion von Schafen mit bovinem Leukämievirus: eine Untersuchung über die Infektiosität von Blut, Speichel- und Nasensekreten Die relative Infektiosität von Lymphozyten und Sekreten von mit BLV infizierten Rindern mit persistierender oder ohne persistierende Lymphozytose (BLV+PL+ und BLV+PL-) wurde untersucht. Siebenundneunzig Schafe gemischten Geschlechts und Alters wurden in 21 Versuchsgruppen eingeteilt. Die Empfängertiere wurden mit gleichmäßig abgestuften Serienverdünnungen aus Ganz-blut, Speichel- und Nasensekreten, die von Rindern mit BLV+PL+ gewonnen wurden, intravenös inokuliert. Zwischen 200 und 20 000 Zellen von Rindern mit entweder einer einfachen oder gemischten BLV+PL+ bzw. BLV+PL- Infektion wurden auf die Schafe überimpft. Eine sehr geringe Anzahl von mit BLV infizierten Lymphozyten (200 Zellen) reichte aus, um bei Schafen, die mit verdünntem Ganzblut von Rindern mit BLV+PL+ geimpft wurden, eine BLV-Infektion hervorzurufen. Eine Impfung mit Ganzblut (mit bis zu 20 000 Lymphozytenzellen), gewonnen von Rindern mit BLV+PL-, rief bei den Empfängertieren keine Infektion mit BLV hervor. Auch nach Impfung mit Speichel- und Nasensekreten fand keine Übertragung von BLV statt. [ABSTRACT FROM AUTHOR]

Twelve sheep were experimentally infected with a phytohemagglutinin (PHA) treated short term culture of lymphocytes from a cow naturally infected with BLV at the PL stage. Five of 12 (42%) BLV infected sheep had histologically confirmed lymphosarcoma 10-16 months after infection. The PBL's were increased to leukemic levels 3-21 weeks before death due to lymphoblastic leukemia. Lymphocyte proliferation and appearance of immature lymphocytes and lymphoblastic cells in the blood were a characteristic feature of tumour development following inoculation with an Australian strain of BLV. In contrast to a number of previous studies the peripheral lymph nodes of all infected sheep were clinically normal throughout the experimental period but at death gross tumours were evident in the mesentric lymph nodes and the heart in all cases. All the other lymph nodes, liver, spleen, kidney and lung were histologically infiltrated with lymphoid tumour cells. Gross tumours were present in the abomasum (1 out of 5) in the urinary tract (2 out of 5) and in the uterus (1 out of 2). The majority of the tumour cells isolated from the various tissues were centroblastic demonstrating that the malignant leukemia in experimentally infected sheep was of a multicentric centroblastic type. The central nervous system was not involved in any case. Zusammenfassung Über die Bildung eines Lymphosarkoms bei Schafen, die mit Rinderleukämievirus (BLV) experimentell infiziert wurden Zwölf Schafe wurden mit einer mit Phytohämagglutinin (PHA) behandelten kurzzeitigen Lymphozytenkultur, gewonnen von einer mit Rinderleukämievirus (BLV) natürlich infizierten Kuh in dem präleukotischen Stadium, experimentell infiziert. 10-16 Monate nach der Infektion zeigten fünf von 12 mit Rinderleukämievirus (BLV) infizierten Schafen (42 %) ein histologisch nachweisbares Lymphosarkom. Auf Grund einer lymphoblastischen Leukämie erreichten die periphären Blutlymphozyten (PBL) 3-21 Wochen vor dem Eintritt des Todes leukämische Werte. Die Vermehrung von Lymphozyten und das Erscheinen unreifer Lymphozyten sowie lymphoblastischer Zellen im Blut waren, nach Impfung mit einem australischen BLV-Stamm, für eine Tumorbildung charakteristisch. Im Gegensatz zu mehreren früheren Untersuchungen blieben die Lymphknoten aller infizierten Schafe während des gesamten Zeitraumes klinisch normal, aber bei allen Tieren wurden nach dem Tode große Tumoren in den Mesenteriallymphknoten und im Herzen festgestellt. Die anderen Lymphknoten, die Leber, die Milz, die Nieren und die Lungen waren alle mit lymphotischen Tumorzellen histologisch infiltriert. Große Tumoren konnten im Labmagen (1 von 5), im Harnapparat (2 von 5) und in der Gebärmutter (1 von 2) nachgewiesen werden. Die meisten Tumorzellen, die aus verschiedenen Geweben isoliert wurden, waren zentroblastisch. Dies zeigte, daß karzinogene Leukämie bei experimentell infizierten Schafen multizentrisch und zentroblastisch war. Das zentrale Nervensystem war nicht betroffen. [ABSTRACT FROM AUTHOR]

- A group of 65 patients with superficial bladder carcinoma was followed for 2 years and tumour recurrence rate was correlated both with transferrin receptor status of the initial primary tumour and with the results of voided urine cytology. Nine of 24 patients with transferrin receptor negative tumours had recurrences compared with 30 of 41 patients with transferrin receptor positive tumours. This difference was highly significant. Urine cytology at presentation was also predictive of further tumour formation: of 30 patients who were transferrin receptor positive and had positive urine cytology, 25 developed recurrences. [ABSTRACT FROM AUTHOR]

- An immunohistochemical profile of cells in the mucosa and lamina propria of adult human bladders is described. Urothelial cells were HLA-DR-ve, ACP + ve and ATPase-ve. All lymphocytes in this layer were of the suppressor/cytotoxic T subgroup and dendritic cells with an identical phenotype to Langerhans cells were seen. In the lamina propria most lymphocytes were of the suppressor/cytotoxic type with some helper/inducer cells. An occasional natural killer cell and Langerhans cell was identified and large macrophage cells with a common phenotype to antigen presenting interdigitating (ID) cells were noted. Fibronectin staining was dense in the vicinity of basement membranes merging to form fine interconnecting latticework-like structures elsewhere in the lamina propria. [ABSTRACT FROM AUTHOR]