Wynik wyszukiwania w bazie Polska Bibliografia Lekarska GBL

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Tytuł oryginału: The immunophenotypic and immunogenotypic B-cell differentiation arrest in bone marrow of RAG-deficient SCID patients corresponds to residual recombination activities of mutated RAG proteins.
Autorzy: Noordzij Jeroen G., Bruin-Versteeg Sandra de, Verkaik Nicole S., Vossen Jaak M. J. J., Groot Ronald de, Bernatowska Ewa, Langerak Anton W., Gent Dik C. van, Dongen Jacques J. M. van
Źródło: Blood 2002: 100 (6) s.2145-2152, il., tab., bibliogr. 45 poz.
Sygnatura GBL: 301,770

Hasła klasyfikacyjne GBL:
  • genetyka
  • immunologia

    Typ dokumentu:
  • praca kliniczna
  • praca opublikowana za granicą
  • tytuł obcojęzyczny

    Wskaźnik treści:
  • ludzie

    Streszczenie angielskie: The protein products of the recombination activating genes (RAG1 and RAG2) initiate the formation of immunoglobulin (lg) and T-cell receptors, whichare essential for B- and t-cell development, respectively. Mutations in the RAG genes result in severe combined immunodeficiency disease (SCID), generally characterized by the absence of mature B and T lymphocytes, but presence of natural killer (NK) cells. Biochemically, mutations in the RAG genes result either in nonfunctional proteins or in proteins with partial recombination activity. The mutated RAG genes of 9 patients from 7 families were analyzed for their recombination activity using extrachromosomal recombination substrates, rearrangment of endogenous Ig loci in RAG gene-transfected nonlymphoid cells, or the presence of Ig gene rearrangements in bone marrow (BM). Recombination activity was virtually absent in all 6 patients with mutations in the RAG core domains, but partial activity was present in the other 3 RAG-deficient patients, 2 of them having Omenn syndrome with oligocional T lymphocytes. Using 4-color flow cytometry, we could define the exact stage at which B-cell differentiation was arrested in the BM of 5 RAG-deficient SCID patients. In 4 of 5 patients, the absence of recombination activity was associated with a complete B-cell differentiation arrest at the transition from cytoplasmic (Cy) Igć- pre -B-I cells to Cylgć+ pre -B-II cells. However, the fifth patient showed low frequencies of precursor B cells with Cylgć and surface membrane IgM...

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    Tytuł oryginału: Surgical view of the treatment of patients with hepatoblastoma. Results from the First Prospective Trial of the International Society of Pediatric Oncology Liver Tumor Study Group (SIOPEL-1).
    Autorzy: Schnater J. Marco, Aronson Dani‰l C., Plaschkes Jack, Perilongo Giorgio, Brown Julia, Otte Jean-Bernard, Brugieres Laurences, Czauderna Piotr, MacKinlay Gordon, Vos Anton
    Źródło: Cancer 2002: 94 (4) s.1111-1120, il., tab., bibliogr. 20 poz.
    Sygnatura GBL: 311,015

    Hasła klasyfikacyjne GBL:
  • pediatria
  • gastroenterologia
  • onkologia

    Typ dokumentu:
  • praca kliniczna
  • praca opublikowana za granicą
  • tytuł obcojęzyczny

    Wskaźnik treści:
  • ludzie

    Streszczenie angielskie: Background. Surgical resection is the cornerstone of treatment for patients with hepatoblastoma (HB). The Society of Pediatric Oncology Liver Tumor Study Group launched its first prospective trial (SIOPEL-1) with the intention to treat all patients with preoperative chemotherapy and delayed surgical resection. The objective of this article was to assess the assumed surgical advantages of primary chemotherapy. Methods. Between 1990 and 1994, 154 patients age 16 years with HB were registered on SIOPEL-1. The pretreatment extent of disease was assessed, and, after undergoing biopsy, patients were treated with cisplatin 80 mg/mý intravenously over 24 hours and doxorubicin 60 mg/mý intravenously over 48 hours by continuous infusion (PLADO). Generally, tumors were resected after four of a total of six courses of PLADO. Results. One hundred twenty eight patients underwent surgical resection (13 patients underwent primary surgery, and 115 patients underwent delayed surgery after PLADO). A pretreatment surgical biopsy was performed in 96 of 128 patients (75 p.c.). Biopsy complications occurred in 7 of 96 patients (7 p.c.). Twenty-two patients showed pulmonary metastases at the time of diagnosis, and 7 patients underwent thoracotomy. Operative morbidity and mortality were 18 p.c. and 5 p.c., respectively. Complete macroscopic surgical resection was achieved in 106 patients (92 p.c.), including 6 patients who underwent orthotopic liver transplantation. The actuarial 5-year event free survival (EFS) rate for all 154 patients in the study was 66 p.c., and the overall survival (OS) rate was (75 p.c.)...

    3/4

    Tytuł oryginału: Chronic graft-versus-host disease and its management in children.
    Autorzy: Vossen J. M., Breedeveld L., Bakker J. D. J., Mertens B., Well-Sipman M. H. van
    Źródło: Rep. Pract. Oncol. Radiother. 2002: 7 (4) s.133-138, tab., bibliogr. 19 poz.
    Sygnatura GBL: 313,406

    Hasła klasyfikacyjne GBL:
  • pediatria
  • immunologia

    Typ dokumentu:
  • tytuł obcojęzyczny

    Streszczenie angielskie: Chronic graft-versus-host disease is sometimes a severe, disabling and long-lasting complication of allogeneic hematopoietic stem cell transplantation. Its frequency lies between 10 and 40 p.c. of pediatric graft recipients, depending on a number of risk factors. Such factors are the type, i.e. HLA-identical related or not, gender and age of the stem cell transplant donor, original diagnosis of the patient and, most importantly, the occurrence or not of a prior acute graft-versus-host disease. Chronic graft-versus-host disease manifests itself as a collagen vascular autoimmune disease. Its treatment consists of immuno-modulatory and antiinfections drugs, in addition to supportive care and psycho-social support. Medical treatment demands a tailor-made approach and careful and prolonged surveillance.

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    Tytuł oryginału: Down-regulation of nuclear receptor DNA-binding activity by nitric oxide - HNF4 as a model system.
    Autorzy: Vossen Christine, Erard Monique
    Źródło: Med. Sci. Monitor 2002: 8 (10) s.RA217-RA220, il., bibliogr. 22 poz.
    Sygnatura GBL: 313,278

    Hasła klasyfikacyjne GBL:
  • genetyka

    Typ dokumentu:
  • tytuł obcojęzyczny

    Streszczenie angielskie: The numerous physiological roles of nitric oxide (NO) are currently teh focus of intensive research. A major pathway by which NO mediates its biological effects is via S-nitrosylation of cysteine residues, and a growing body of evidence suggests that transcription factors are critical targets for such S-nitrosylation. Here we review the ability of NO to down-regulate the activity of transcription factors, and in particular nuclear receptors. Among the latter, the hepatocyte nuclear factor HNF4 stands out as a key regulator of cytochrome P45 (CYP) gene expression. We report on a series fo experients which show that inflammation-induced NO production decreases CYP mRNA transcription, and that NO suppresses the DNA-binding ability of HNF4. Together these data suggest that cysteine-nitrosylation of teh HNF4 DNA-binding domain is the primary molecular mechanism responsible for teh drop in oxydase activities of hepatic cytochrome P450 enzyems, and the consequent impairment in drug metabolism during inflammation. In order to discuss this hypothesis from a structural perspective, we have built a homology-derived model of the HNFe DNA-binding domain and computer-simulated the S-nitrosylation of its cysteine residues. Finally, bearing in mind the structural conservation of the nuclear receptor DNA-binding domain, we discuss to what extent results from HNF4 can be extended to other nuclear receptors.

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