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  • 2000-2004  (2)
  • Key words Pancreatic cancer  (1)
  • Key wordsDCX gene  (1)
  • Subcortical laminar heterotopia  (1)
  • 1
    ISSN: 1437-7772
    Keywords: Key words Pancreatic cancer ; National database ; Multidisciplinary treatment ; Survival
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Background. The Japanese Pancreas Society Registry Committee obtained data on 16 071 patients with pancrea-tic cancer diagnosed from 1981 to 1996. This is the largest multi-institutional retrospective study of the Japanese experience to describe the extent of disease and survival. Methods. Data were summarized according to age and sex and location of the disease. Survival analysis, using the Kaplan-Meier method, was performed according to surgical stage and therapeutic categories. Results. The male : female ratio was 1.6 : 1. The peak age was in the patients' sixties for both sexes. Forty-five per-cent of the primary tumors were located in the head of the pancreas. The survival of the patients depended on the histological diagnosis and surgical stage. The most com-mon histology, invasive ductal carcinoma, had the worst prognosis, and the 5-year survival of patients with stage I, II, III, IVa, and IVb disease was 66%, 55%, 21%, 11%, and 6% respectively. At the time of presentation, only 2.5% of the patients had stage I disease, while more than 70% had stage IV. Conclusions. Improvements in early detection strategies and more effective chemical or biological therapeutic agents, together with pancreatectomy, tested in well organized randomized prospective trials, will be the only way to cure this fatal disease.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1435-232X
    Keywords: Key wordsDCX gene ; Subcortical laminar heterotopia ; Isolated lissencephaly sequence
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract We examined mutations of the doublecortin (DCX) gene, which is responsible for X-linked subcortical laminar heterotopia (SCLH) and lissencephaly, in eight unrelated Japanese patients, four with SCLH and four with isolated lissencephaly sequence (ILS). Polymerase chain reaction (PCR) disclosed a deletion of part of the DCX gene in one male ILS patient. Single-strand conformational polymorphism analysis and subsequent sequence analysis were carried out in the remaining seven patients. One male ILS patient had a nonsense mutation in exon V, which would result in premature termination of the gene product. One female SCLH patient had a missense mutation in exon IV. Our results indicate that in the Japanese, as has been seen elsewhere, abnormality of the DCX gene is the common cause of SCLH and ILS.
    Type of Medium: Electronic Resource
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