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  • Analytical Chemistry and Spectroscopy  (21)
  • Organic Chemistry  (10)
  • Familial adenomatous polyposis  (8)
  • melanoma  (4)
  • 1
    Electronic Resource
    Electronic Resource
    Spontaneous mutation in familial adenomatous polyposis (1990)
    Springer
    Diseases of the colon & rectum 33 (1990), S. 52-55 
    Details
    ISSN: 1530-0358
    Keywords: Familial adenomatous polyposis ; Spontaneous mutation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A retrospective review of the familial adenomatous polyposis registry at the Cleveland Clinic Foundation revealed an incidence of spontaneous mutation in familial adenomatous polyposis (FAP) of 22 percent of family kindreds. These patients were reviewed retrospectively and compared with the total FAP population followed at The Cleveland Clinic Foundation with respect to the onset of disease, the incidence of carcinoma in the resected colon, and incidence of extracolonic manifestations. Review of the characteristics and presentations of these patients suggested that these individuals may harbor a more severe form of FAP. This may be due, in part, to the delay in diagnosis and, therefore, a higher rate of development of colorectal carcinoma and possibly duodenal adenomas. There is also a demonstrable higher rate of extracolonic manifestations of FAP present in this subset of patients. When selecting the initial type of prophylactic colonic resection the surgeon should bear in mind the increased incidence of extracolonic manifestations of the disease in this group of patients and their potential for complications.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02053203
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  • 2
    Electronic Resource
    Electronic Resource
    Predictive value of congenital hypertrophy of the retinal pigment epithelium as a clinical marker for familial adenomatous polyposis (1990)
    Springer
    Diseases of the colon & rectum 33 (1990), S. 1003-1008 
    Details
    ISSN: 1530-0358
    Keywords: Familial adenomatous polyposis ; Congenital hypertrophy ; Retinal pigment epithelium ; Extracolonic manifestations
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract One hundred forty-eight members of 53 kindreds with familial adenomatous polyposis (FAP) were examined for congenital hypertrophy of the retinal pigment epithelium (CHRPE) and extracolonic manifestations (ECM) to assess the value of CHRPE as a predictive marker for FAP. Based on eye examination results, the families were divided into 2 groups. In a first group of 34 families, all 61 members diagnosed as having polyps and 13 of the 33 patients at risk had 4 or more lesions distributed in both eyes. By contrast, in a second group of 18 families, all 32 polyposis patients and all 18 members at risk had less than 4 lesions. Extracolonic manifestations were present in 26 of 34 families in the first group and in 11 of 18 families in the second group. Data on one family with ambiguous ancestry were reviewed separately. The existence of 4 or more CHRPE lesions distributed in both eyes seems to be a congenital marker for FAP, present in 65.4 percent of families. When present in a family: 1) it is found in all diagnosed patients in that family, 2) can therefore be considered predictive for the development of polyps in other family members who carry the trait, and 3) if confirmed by longer follow-up, may possibly preclude members without the trait from further evaluation and surveillance.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02139213
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  • 3
    Electronic Resource
    Electronic Resource
    Noncytotoxic drug therapy for intra-abdominal desmoid tumor in patients with familial adenomatous polyposis (1992)
    Springer
    Diseases of the colon & rectum 35 (1992), S. 29-33 
    Details
    ISSN: 1530-0358
    Keywords: Intra-abdominal desmoid tumor ; Familial adenomatous polyposis ; Nonsteroidal anti-inflammatory drugs ; Antiestrogen drugs ; Prostaglandin synthesis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Forty of 416 patients with familial adenomatous polyposis were noted to have intra-abdominal desmoid tumors, and a subgroup of 16 were treated with noncytotoxic drug therapy. Drugs used were sulindac (14 patients), sulindac plus tamoxifen (3 patients), indomethacin (4 patients), tamoxifen (4 patients), progesterone (DEPO-PROVERA®; Upjohn Co., Kalamazoo, MI) (2 patients), and testolactone (1 patient). Therapy with these drugs for continuous periods of six months or more resulted in three complete and seven partial remissions. When treated patients were compared with untreated patients (n=12), there were significant benefits for the treated group, both in reduction of desmoid size and in improvement of symptoms, despite the inherent selection bias against this. Sulindac was the only drug used in enough patients to permit independent evaluation of its effect, with one complete and seven partial reductions of tumor size. Some patients had a delayed response to sulindac, with tumor shrinkage occurring after an initial period of tumor enlargement. When using sulindac for the treatment of desmoid tumors, this phenomenon should be considered.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02053335
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  • 4
    Electronic Resource
    Electronic Resource
    Mortality in patients with familial adenomatous polyposis (1990)
    Springer
    Diseases of the colon & rectum 33 (1990), S. 639-642 
    Details
    ISSN: 1530-0358
    Keywords: Familial adenomatous polyposis ; Colorectal carcinoma ; Desmoid tumors ; Periampullary carcinoma
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The authors identified 132 patients who died with a documented diagnosis of familial adenomatous polyposis (FAP). A review of the medical records, autopsy reports, and in-depth discussion with local physicians and well-informed family members was performed. It was impossible, even after the review, to ascertain the exact cause of death in 22 patients. In the remaining patients, the cause of death was as follows: metastatic colorectal carcinoma, 64 patients (58.2 percent), (colon, 49 [44.5 percent], rectal, 15 [13.6 percent]); desmoid tumors, 12 (10.9 percent); periampullary carcinoma, 9 (8.2 percent); brain tumors, 8 (7.3 percent); perioperative mortalities, 5 (4.5 percent); adrenal carcinoma, 1 (0.9 percent); and abdominal carcinomatosis, 1 (0.9 percent). Ten patients died of causes not related to FAP. The major causes of death in 36 patients who underwent prophylactic colectomy were desmoid tumor and periampullary malignancy. This finding underscores the importance of lifelong surveillance and periodic endoscopic evaluation in patients with FAP.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02150736
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  • 5
    Electronic Resource
    Electronic Resource
    The double-stapled ileal reservoir and ileoanal anastomosis (1991)
    Springer
    Diseases of the colon & rectum 34 (1991), S. 487-494 
    Details
    ISSN: 1530-0358
    Keywords: Ileoanal reservoir ; Stapling device ; Mucosal ulcerative colitis ; Familial adenomatous polyposis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Fifteen consecutive patients (nine males and six females) who underwent construction of a double-stapled ileoanal reservoir (DS-IAR) were prospectively evaluated. Mean and maximal resting pressures preoperatively, before ileostomy closure, and at 12 months, were 53 and 84 mm Hg, 39 and 62 mm Hg, and 62 and 81 mm Hg. Mean and maximal squeeze pressures at those same time periods were 96 and 153 mm Hg, 111 and 173 mm Hg, and 95 and 168 mm Hg. There were no significant decreases in either resting or squeeze pressure between preoperative values and those obtained 12 months after surgery. However, the length of the high pressure zone decreased from 3–8 cm preoperatively to 2.3 cm at 12 months. This reflects the sacrifice of the cephalad 1.5 cm of the internal anal sphincter necessary to effect this anastomosis at a mean of 1.4 cm from the dentate line. However, this maneuver did not result in poor continence. Eleven patients whose ileostomies were closed for a mean of 9 months, ranging from 3 to 15 months, were evaluated regarding functional outcome. Only one patient had any incontinence and this patient had incomplete circularstapled tissue rings, which necessitated transanal suture repair of the anastomotic defect. Similarly, three of the four patients who sometimes or rarely use a pad at night had transanal-suture reinforcement. Ten of the 11 patients never wear a pad during the day. No pelvic or perianal sepsis occurred. Stratified squamous epithelium was found in 6 of the 13 distal stapler “donuts” that were examined. In addition, 10 patients underwent biopsy of the tissue immediately caudad to the circular staple line at the time of ileostomy closure; in five, only stratified squamous epithelium was noted. The DS-IAR is associated with excellent objective physiologic and subjective functional results.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02049935
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  • 6
    Electronic Resource
    Electronic Resource
    Evaluation of polymorphic genetic markers for linkage to the familial adenomatous polyposis locus on chromosome 5 (1990)
    Springer
    Diseases of the colon & rectum 33 (1990), S. 740-744 
    Details
    ISSN: 1530-0358
    Keywords: Familial adenomatous polyposis ; Restriction fragment length polymorphism ; Chromosome 5q
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A gene associated with the inherited syndrome, familial adenomatous polyposis (FAP), has been localized to the long arm of chromosome 5 near the 5q21-22 region, and markers that identify genetic polymorphisms near this locus are now available. The authors evaluated several of these markers for linkage to the FAP trait in 11 families entered in the Cleveland Clinic Polyposis Registry. The original probe that established linkage to the FAP locus (C11p11) has limited utility for family studies because of low heterozygosity and distance from the FAP gene. Other probes, however, should be useful for assessing FAP inheritance by restriction fragment length polymorphism analysis, for presymptomatic diagnosis of the disease.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02052318
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  • 7
    Electronic Resource
    Electronic Resource
    Systemic cytotoxic chemotherapy and radiation therapy for desmoid in familial adenomatous polyposis (1991)
    Springer
    Diseases of the colon & rectum 34 (1991), S. 1090-1092 
    Details
    ISSN: 1530-0358
    Keywords: Desmoid tumor ; Familial adenomatous polyposis ; Systemic cytotoxic chemotherapy ; Radiation therapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Forty-two of 416 familial adenomatous polyposis (FAP) patients in the FAP registry at the Cleveland Clinic had desmoid tumors. The role of cytotoxic chemotherapy and radiation therapy in the management of these patients was investigated. Eight intra-abdominal desmoid tumors were treated by systemic cytotoxic chemotherapy. Two had complete remission, and one had partial remission. Five patients died as a result of the desmoid tumor or late complications of chemotherapy. Three intra-abdominal desmoids were treated by radiation therapy with no response. Neither cytotoxic chemotherapy nor radiation therapy is recommended as a first-choice treatment for intra-abdominal desmoid tumors in patients with FAP.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02050067
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  • 8
    Electronic Resource
    Electronic Resource
    Laparoscopic total abdominal colectomy (1992)
    Springer
    Diseases of the colon & rectum 35 (1992), S. 651-655 
    Details
    ISSN: 1530-0358
    Keywords: Laparoscopic colectomy ; Laparoscopy ; Ileoanal reservoir ; Restorative proctocolectomy ; Colonic inertia ; Ulcerative colitis ; Familial adenomatous polyposis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The aim of this study was to prospectively assess the impact of laparoscopy upon the outcome of total abdominal colectomy (TAC). Specifically, patients underwent standard laparotomy with TAC and ileoproctostomy (TAC + IP), TAC and ileoanal reservoir (TAC + IAR), laparoscopically assisted TAC + IP (L-TAC + IP), or laparoscopically assisted TAC + IAR (L-TAC + IAR). Parameters studied included the length of surgery, length of ileus, length of hospitalization, morbidity, and mortality. Five patients underwent standard TAC (Group I), and five underwent L-TAC (Group II). Group I consisted of five patients of a mean age of 32 (range, 24–51) years who had mucosal ulcerative colitis (n=1), familial adenomatous polyposis (n=3), or colonic inertia (n=1). Group II consisted of five patients of a mean age of 33 (range, 17–43) years who had mucosal ulcerative colitis (n=1), familial adenomatous polyposis (n=3), or colonic inertia (n=1). This preliminary prospective study indicates that laparoscopically assisted TAC is feasible. L-TAC resulted in a slightly longer length of ileus and length of hospitalization; these differences were not statistically significant. Moreover, the length of time required for the laparoscopic procedures was 35 percent longer than for the open procedures. Although these results may improve as more cases are performed, dramatic differences in rates of postoperative recovery have not yet been realized. In conclusion, L-TAC, while technically feasible, dose not appear to offer any immediately recognizable benefits to the patient as compared with standard laparotomy.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02053755
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  • 9
    Electronic Resource
    Electronic Resource
    Malignant melanoma metastasis to brain: role of degradative enzymes and responses to paracrine growth factors (1993)
    Springer
    Journal of neuro-oncology 18 (1993), S. 139-149 
    Details
    ISSN: 1573-7373
    Keywords: melanoma ; metastasis ; proteases ; endoglycosidases ; growth factors ; growth factor receptors ; basement membrane ; blood brain barrier ; neurotrophins
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Mouse and human melanoma cells metastatic to the brain express degradative enzyme activities that are used for invasion of brain basement membrane and parenchyma. Compared to poorly metastatic or lung- or ovary-metastatic murine melanoma lines, the brain-metastatic sublines secreted higher levels of a variety of degradative enzymes. Brain-metastatic murine and human melanoma cells also degraded subendothelial basement membrane and reconstituted basement membrane at rates higher than other metastatic melanoma cells. In some cases these degradative activities in mouse and human melanoma cells can be induced by paracrine factors known to be present in the brain parenchyma, such as nerve growth factor (NGF). NGF stimulates the expression of degradative enzymes, such as the endo-Β-glucuronidase heparanase, that are important in basement membrane penetration but this factor does not stimulate melanoma cell growth. The growth of brain-metastasizing melanoma cells appears to be stimulated by other paracrine growth factors, such as paracrine transferrin. Melanoma cells metastatic to brain express higher numbers of transferrin receptors and respond and proliferate at lower concentrations of transferrin than do melanoma cells metastatic to other sites or poorly metastatic melanoma cells. The results suggest that degradation and invasion of brain basement membrane and responses to paracrine neurotrophins and paracrine transferrins are important properties in brain metastasis of murine and human malignant melanoma cells.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01050420
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  • 10
    Electronic Resource
    Electronic Resource
    The role of trophic factors and autocrine/paracrine growth factors in brain metastasis (1995)
    Springer
    Clinical & experimental metastasis 13 (1995), S. 67-88 
    Details
    ISSN: 1573-7276
    Keywords: brain metastasis ; growth factors ; melanoma ; melanotropins ; nerve growth factor ; neurotrophins ; signal transduction ; tumor progression ; tyrosine receptor kinase
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: The brain is a unique microenvironment enclosed by the skull, lacking lymphatic drainage and maintaining i highly regulated vascular transport barrier. To metastasize to the brain malignant tumor cells must attach to microvessel endothelial cells, respond to brain-derived invasion factors, invade the blood-brain barrier and respond to survival and growth factors. Trophic factors are important in brain invasion because they can act to stimulate this process. In responsive malignant cells trophic factors such as neurotrophins can promote invasion by enhancing the production of basement membrane-degradative enzymes (such as type IV collagenase/gelatinase and heparanase) capable of locally destroying the basement membrane and the blood-brain barrier. We examined human melanoma cell lines that exhibit varying abilities to form brain metastases. These melanoma lines express low-affinity neurotrophin receptor p75NTR in relation to their brain-metastatic potentials but the variants do not express trkA, the gene encoding a high affinity nerve growth factor (NGF) tyrosine kinase receptor p140 trkA . Melanoma cells metastatic to brain also respond to paracrine factors made by brain cells. We have found that a paracrine form of transferrin is important in brain metastasis, and brain-metastatic cells respond to low levels of transferrin and express high levels of transferrin receptors. Brain-metastatic tumor cells can also produce autocrine factors any inhibitors that influence their growth, invasion and survival in the brain. We found that brain-metastatic melanoma cells synthesize transcripts for the following autocrine growth factors: TGFβ, bFGF, TGFα and IL-1β. Synthesis of these factors may influence the production of neurotrophins by adjacent brain cells, such as oligodendrocytes and astrocytes. Increased amounts of NGF were found in tumor-adjacent tissues at the invasion front of human melanoma tumors in brain biopsies. Trophic factors, autocrine growth factors, paracrine growth factors and other factors may determine whether metastatic cells can successfully invade, colonize and grow in the central nervous system.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00133612
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