[Moebius syndrome: case presentation].
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Biomedical subjects
Publications and source records attributed to R Barone.
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Six patients with unresectable carcinoma of the esophagus received a combined course of external radiation therapy (1000 rads in four fractions in four days commencing on day 2) combined with constant infusional 5-fluorouracil (20 mg/kg every 24 hours for five days beginning on day 1). This program was repeated every other week to give a total x-ray dose of 6000 rads. This regimen has been well-tolerated by the majority of the patients and resulted in a complete response rate within the x-ray treatment field of 83% (5/6). All patients who showed a demonstrable systemic response to 5-fluorouracil reached complete response. The median survival has not yet been reached at six months with post-treatment survivors alive and without disease (four patients) at one, six, nine, and 22 months. Our previous median survival by x-ray therapy alone was 4 1/2 months. Toxicity consists primarily of hematologic suppression at a subclinical level. Although the length of therapy is substantial (11 weeks), the program appears tolerable and is capable of inducing long-term remissions. The program is currently being studied for dose escalation because neither local nor systemic side effects of a dose-limiting nature have been observed at 20 mg/kg 5-FU.
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Complete absence of C1r and almost complete absence of C1s were found in 4 of 8 living siblings. Two of the 4 suffer from a syndrome that combines discoid lupus erythematosus and nondeforming rheumatoid-like arthritis; one of the siblings has mild nephritis. The other 2 C1 deficient family members are clinically well. Evidence from this and other families suggests that deficiency of C1 components or C4 is associated with higher risk of developing a lupus-like disease than is deficiency of C2.
A Sulfolobus solfataricus beta-glycosidase expressed in Saccharomyces cerevisiae (S beta gly) was immobilized on chitosan activated with glutaraldehyde. The yield of immobilization was evaluated as 80%. Compared to the free beta-glycosidase, the immobilized enzyme showed a similar pH optimum (pH = 7.0), the same increasing activity up to 80 degrees C, improved thermostability, and no inhibition by glucose. Functional studies pointed out that the kinetic constant values for both enzymes were comparable. A bioreactor, assembled with the immobilized S beta gly, was used for glucose production. The values of cellobiose conversion increased on increasing residence time in the bioreactor, following a nonlinear trend. However, the highest glucose production/min was obtained at a flow of 0.5 mL/min.
BACKGROUND: We retrospectively evaluated the accuracy of magnetic resonance (MR) imaging in staging colorectal cancer and assessing local tumor extent, nodal involvement, and distant abdominal and pelvic metastases. METHODS: Forty-eight patients with primary colorectal carcinoma were referred for presurgical abdominal and pelvic MR imaging. MR imaging included T1-weighted, fat-suppressed T2-weighted, and fat-suppressed gadolinium-enhanced spin gradient-echo imaging. The prospective interpretations of the MR examinations were reviewed. MR depiction of local tumor extent, nodal involvement, and distant metastases at 18 anatomic locations was noted and compared with subsequent surgical and histopathologic findings. RESULTS: Overall TNM MR staging agreed with surgical and pathologic staging in 41 (85%) of 48 patients, including 21 (78%) of 27 colon cancers and 20 (95%) of 21 rectal cancers. For depth of tumor penetration, which was evaluable in 44 patients, MR imaging agreed with pathologic results in 38 (86%) of 44 patients, including 22 (88%) of 25 colon cancers and 16 (84%) of 19 rectal cancers. In 42 (95%) of 44 patients, MR images correctly distinguished tumor confined to the bowel wall (T0, T1, and T2) from tumor with transmural tumor extension (T3 and T4). Regional nodal metastases were depicted in 15 of 22 patients (sensitivity, 68%; accuracy, 83%). Nodal metastases were better depicted for rectal cancer in eight of nine patients, compared with colon cancer in seven of 13 patients. Distant metastases were correctly depicted on MR imaging in 13 of 14 patients (sensitivity, 93%; accuracy, 98%). In the site-by-site analysis, MR imaging prospectively depicted 66 of 77 sites of surgically confirmed metastatic tumor in the abdomen and pelvis (sensitivity, 86%; specificity, 99%; accuracy, 98%). CONCLUSION: MR imaging using currently available techniques can effectively image local tumor extent and distant metastases in patients with colorectal carcinoma. Especially for colon cancer, incomplete depiction of nodal metastases in normal-size lymph nodes remains a limitation of cross-sectional imaging studies.
A new approach to describe the complexity of chemical structures is proposed. This index is simple and can be easily programmed. It derives from Whitlock's index and, despite its empirical character, provides results paralleling those obtained with the mathematical Bertz index. It has been applied to follow the strategic progress of some natural product syntheses.
When scleroderma involves the small intestines, malnutrition with resulting immune incompetence and sepsis can occur. Two cases are presented in which patients with scleroderma involving the gastrointestinal tract were treated with cyclic home hyperalimentation, restoring their nutritional status and improving their quality of life.
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Forty three patients, mean age 55.20 +/- 9.27 SD, affected by Schizophrenia Residual Type (DSM IV, RDC criteria) and treated with neuroleptic drugs for a mean of 25.42 years (+/- 4.12 SD) were included into the study. Clinical evaluation was cross-sectional assessed by BPRScale, SAPS, SANS, HRS-D, EPSE. ACS and MMSE. Seventy percent of patients presented a "postpsychotic depression" (42%, mild; 16%, moderate and 12% serious). "Postpsychotic depression" does not seem to be influenced by neuroleptics, but it seems to be a component of residual schizophrenia in patients with a long lasting permanence in a mental hospital.
UNLABELLED: Malignant mesothelioma of the peritoneum is a rare tumor for which the therapeutic approach has not yet been standardized. Cytoreductive surgery and hyperthermic antiblastic peritoneal perfusion (HAPP) may be effective in the treatment of this neoplasm. From 1995 to March 2003, we operated 24 patients with malignant peritoneal mesothelioma. Nineteen times we were able to perform radical surgery and HAPP: 6 CC-0, 10 CC-1 and 3 CC-2 (nodules smaller than 1 cm). HAPP was performed by the original "semi-closed" technique, using MMC+ CDDP in 3 patients, CDDP + doxorubicin in 15 patients, only doxorubicin in 1 patient). RESULTS: Operating mortality was 11% and postoperative morbidity was 26%. 4 patients are DOD (40, 20, and 2 at 2 months); 5 patients are AWD (72, 34, 25, and 2 at 15 months); 8 patients are NED (81, 47, 2 at 20 months, 16, 13, 12 months, 1 patient recently operated). The median survival is 40 months. The high rate of morbidity and mortality is due to the presence of postoperative paraneoplastic syndromes, with alterations of coagulation and onset of ARDS. Therefore the good survival results make the methodic one of the best options in the treatment of the disease.