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In Table 4, current "state-of-the-art" screening for various cancers is summarized. It is fair to say that, unfortunately, only a minority of the tumors do have proven screening methods that lead to a lower disease-specific mortality. Given the relative lack of improvement in survival rates over the last few decades, this area should be a priority for research endeavors.  相似文献   

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PURPOSE: To describe the spectrum of malignancies in human immunodeficiency virus (HIV)-infected children and the clinical outcome of patients with these tumors. METHODS: We retrospectively surveyed the Children's Cancer Group (CCG) and the National Cancer Institute (NCI) for cases of cancer that occurred between July 1982 and February 1997 in children who were HIV seropositive before or at the time of cancer diagnosis. We used Kaplan-Meier survivorship curves, hazard function estimates, and Cox proportional hazards models to evaluate survival. RESULTS: Sixty-four children (39 boys, 25 girls) with 65 tumors were reported. Thirty-seven children (58%) acquired HIV infection vertically (median age at cancer diagnosis, 4.3 years); 22 children (34%) acquired HIV through transfusion of blood or blood products (median age at cancer diagnosis, 13.4 years). Forty-two children (65%) had non-Hodgkin's lymphoma (NHL). Eleven children (17%) had leiomyosarcomas (or leiomyomas), which are otherwise exceptionally rare in children. Other malignancies included acute leukemia (five children), Kaposi's sarcoma (KS; three children), Hodgkin's disease (two children), vaginal carcinoma in situ (one child), and tracheal neuroendocrine carcinoma (one child). Median survival after NHL diagnosis was 6 months (range, 1 day to 89 months) and after leiomyosarcoma was 12 months (range, 10 days to 19 months). The average monthly death rate after NHL diagnosis was 12% in the first 6 months, which decreased to about 2% thereafter. In contrast, the monthly death rate after leiomyosarcoma diagnosis increased from 5% in the first 6 months to about 20% thereafter. CONCLUSION: After NHL, leiomyosarcoma is the second leading cancer in children with HIV infection. Both cancers have high mortality rates; improved outcome for NHL, in particular, may depend on earlier diagnosis and therapy.  相似文献   

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Five women after precocious menopause and 1 patient with primary ovarian failure showed a simultaneous elevation of plasma gonadotropin and prolactin. The hypersecretion of plasma prolactin was still present 6 months after ovarian failure. After 12-18 months of observation while FSH and LH concentration remained elevated, prolactin concentrations normalized in 5 women and decreased in 1.  相似文献   

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As elaborated by Nobel Laureate Harold Varmus (Director of the National Institutes of Health, Bethesda, MD, USA) in his Keynote address, future studies must not only continue to expand our repertoire of genetic determinants of cancer susceptibility by identifying new cancer susceptibility genes, but most also now begin to address the function of these genes and their roles in the process of tumor development. Key areas for future investigation in this field, identified at the meeting, were: (1) understanding how susceptibility genes are altered in cancer cell physiology, (2) elucidating the function of these genes in the biochemistry of cellular signaling pathways; and (3) improving our ability to use this information to extrapolate basic oncology research to the clinic. At this meeting, the fields of molecular carcinogenesis, DNA repair and human genetics were very successfully integrated. These fields have converged in the areas of cell cycle control and genetic susceptibility, and this meeting capitalized on this convergence to highlight recent progress in these areas. Key areas for future research were also emphasized, including the need to apply the rapidly emerging information from these fields towards developing novel therapeutic modalities to treat and prevent cancer.  相似文献   

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Parkinson's disease (PD) has no cure and is a progressive neurological disorder with treatment aimed at the maintenance of function and limitation of the symptoms. No extensive studies of the disease's impact on health-related quality of life (HRQoL) have been conducted. The purpose of this study was to assess the potential usefulness of the Medical Outcomes Study Short Form (SF-36) and the Functional Status Questionnaire (FSQ) in Parkinson's disease research. This cross-sectional study of 193 PD patients who visited two hospital-based neurology clinics used self-administered in-clinic and take-home questionnaires to ascertain the demographic and environmental characteristics of the subjects and to gain health profile measures from the SF-36 and the FSQ. The two health profiles provide important HRQoL information supplementary to the traditional signs and symptoms evaluated by the Unified Parkinson's Disease Rating Scale (UPDRS). Many of the HRQoL measures discriminate progressive stages of disease in this study group and distinguish those with complications of therapy from subjects without complications.  相似文献   

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There are many examples of different types of cancers that have been prevented by appropriate measures in the past. Most of them were related to occupational, iatrogenic or accidental factors, often as the outcome of heavy exposure of humans to specific carcinogenic agents. Cancer is a disease of DNA, and is generally associated with multiple genetic alterations, these being produced in the typical case by exposure to various carcinogens, each of which exists at minute concentrations. Thus, the impact of carcinogenic factors, xenobiotics and autobiotics, is due to their actions in concert. However, a single mutation yielding genomic instability exerts a disproportionately large influence by resulting in a large number of secondary mutational events. Epigenetic changes can also not be disregarded especially from the view point of prevention of neoplasia. The occurrence of multiple primary cancers among survivors of initial primaries, and the presence of hereditary groups with a high risk of cancer development provide a strong stimulus for establishment of effective approach for cancer prevention, which should be, in principle, multi-faceted. Therefore, a holistic approach is essential with improvement in life style including choosing a balanced diet and avoidance of cigarette smoking and other sources of carcinogens, as integral elements.  相似文献   

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We report the first case published in Spain of a palliative anatomic correction associated with aortic arch repair in a neonate with (S,D,L)-transposition of the great arteries, multiple ventricular septal defects, and severe hypoplasia of the right ventricle with subaortic obstruction and hypoplasic aortic arch with coarctation. A one stage palliative surgery on cardiopulmonary bypass was performed with reconstruction of the aortic arch and an arterial switch procedure which obtained a satisfactory result. The principle of this operation is to switch the subaortic obstruction into a subpulmonary obstruction and reconstruct a large natural aortic root from the principal ventricle. The right ventricle-pulmonary artery continuity may promote growth of the right ventricle with the possibility of a future biventricular repair. We conclude that this operation, when used by surgical teams experienced with arterial switch surgery, is the best treatment for the complex newborn group with single ventricles or severe ventricular disbalance, ventriculoarterial discordance and stablished subaortic stenosis.  相似文献   

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The results of superselective intra-arterial chemotherapy with Mitomycin C (SIAC) in cases of hepatic neoplasms continue to be poor. Survival time was related to the percentage of hepatic replacement (PHR) but only 19% of the patients with Stage I tumours (PHR < 25%) survived for over 5 years and all the others died within 4 years. The patients with hepatic metastases from colorectal cancer achieved a significantly better cumulative 5-year survival figure than those with hepatocellular cancer (P < 0.05). The median survival times for patients with hepatic metastases from colorectal cancer, hepatocellular cancer and gallbladder cancer were 15 months, 6 months and 13 months, respectively. The overall response rate was only 27% (26/97), that for primary liver cancer 20% (7/35), that for hepatic metastases from colorectal cancer 22% (8/37) and that for gallbladder cancer 44% (11/25) and the patients who responded to SIAC (n = 27) had a significantly better cumulative 5-year survival rate (P < 0.005). Cessation of SIAC was necessary in 74% (72/97) of the cases, because of tumour progression in 53% (51/97), major complications in 19% (18/97) and patient refusal in 3% (3/97). The results of this trial may be regarded as disappointing, and we are going to use SIAC for Stage I tumours only. Resection of the tumour continues to provide the only chance of a permanent cure with these patients.  相似文献   

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Medical imaging defines the local, regional and metastatic spread of cancers of the oesophagus, guiding the therapeutic approach and, in particular, the indication for surgery. CT scan and echoendoscopy are currently the reference examinations. MRI provides complementary elements. After treatment, imaging is performed to detect operative complications and recurrence.  相似文献   

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The accumulation of nicotinic acetylcholine receptors (AChRs) at neuromuscular synapses is triggered by agrin, a protein that is synthesized by both nerve and muscle. Nerve-derived agrin, which contains an amino acid insert at a conserved splice site in the carboxy-terminal part of the protein, induces AChR aggregation and causes tyrosine phosphorylation of the AChR beta subunit. In contrast, agrin isoforms synthesized by muscle cells lack such an insert and have no effect on AChR distribution. In order to identify possible functional roles of muscle-derived agrin we have analyzed further the effect of various fragments of recombinant agrin on AChR phosphorylation. A carboxy-terminal fragment of muscle agrin, c95A0B0, reduced AChR gamma and delta subunit phosphorylation when added to C2C12 myotubes in culture. Although c95A0B0 had no effect on AChR beta subunit phosphorylation when added alone, it inhibited AChR beta subunit phosphorylation and AChR aggregation by the nerve-specific agrin isoform c95A4B8. We conclude that muscle-derived agrin can influence, both directly and indirectly, AChR phosphorylation. Such changes may play a role in the formation, maintenance, or function of the neuromuscular junction.  相似文献   

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Biotinidase deficiency is an autosomal recessively inherited disorder that results in the inability to recycle the vitamin biotin. The disorder can cause neurologic and cutaneous abnormalities that can be treated effectively with pharmacologic doses of biotin. We identified 21 mutations that cause profound biotinidase deficiency in 37 symptomatic children (30 different probands and 7 siblings), as well as provide relevant biochemical and clinical information for each child. The two most common mutations (G98:d7i3 and R538C) were found in 31 of 60 alleles (52%), whereas the remainder of the alleles are accounted for by the 19 other unique mutations. Serum samples were available from 18 children, of these 11 had no detectable cross-reacting material (CRM) to antibody prepared against normal human serum biotinidase, three had reduced quantities of CRM and four had normal quantities of CRM in serum. All of these mutations result in complete absence of biotinyl-transferase activity in serum. Two polymorphisms were also identified in normal individuals. It is apparent that a child who inherits any of these mutations, either in the homozygous state or in combination, can develop the clinical features of the disorder if untreated. There are, however, no clear genotype/phenotype correlations that would allow for the prediction of the type, severity, or age of onset of symptoms.  相似文献   

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In 1992 the first Hereditary Cancer Center in Poland has been organized in Szczecin. DESIGN: One of its goals is application of appropriate management in families with hereditary ovarian cancer. The aims of our program include studies of: incidence, clinical characterization, DNA diagnostic tests and efficiency of screening for early detection of hereditary ovarian tumors in North-West Poland. MATERIAL: Our program includes 234 families with 258 cases of ovarian cancers. RESULTS: Site-specific familial aggregation of ovarian cancer was diagnosed in 16 (6.84%) families, breast-ovarian cancer syndrome in 27 (11.54%), Lynch II syndrome in 5 (2.14%) families, undefined cancer family aggregation in 12 (5.13%) families, sporadic ovarian cancers diagnosed age of 44 in 56 (23.93%) families and other sporadic ovarian cancers in 118 (50.4%). In 17 patients with ovarian cancer from families with breast-ovarian cancer syndrome constitutional BRCA-1 gene mutations were studied by sequencing DNA on automated sequencer of PCR products for all 24 exons. In 1 patient constitutional mutation in exone 11 was detected. We found also multiply polymorphic changes. 124 women-members of 116 families with diagnosed hereditary predisposition for ovarian cancer have been studied for asymptomatic tumors by intravaginal USG and evaluation of CA 125 marker every 6 month beginning from 20-25 years of age. Up to now we found 5 cases of benign serous cystadenomas, 3 cases of cystadenomas of borderline malignancy and 1 cases of serous cystadenocarcinoma. CONCLUSIONS: It seems, that particular surveillance program in women from families with hereditary cancers can be the effective way of detection of early ovarian tumors. Clinical characterization of hereditary ovarian cancers in North-West Poland and other countries is similar.  相似文献   

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Body/mind medicine is rapidly becoming one of the catalysts that will integrate conventional and complementary health care. Interactive Imagery, in particular, offers many potent healing opportunities for nurses and patients in the perioperative continuum. Within this holistic approach, nurses play a pivotal part in providing to patients self-care tools that can be applied in and out of the hospital. Imagery adds value to the health care system by offering nurses and other health care practitioners a way to empower themselves and their patients. It is harmless, time and cost-effective, and often fun, and it focuses on patient satisfaction.  相似文献   

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