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The 22q11.2 deletion syndrome is characterized by wide phenotypic variability, frequently involving characteristic craniofacial features, cardiac malformations, and learning difficulties. Skeletal anomalies are also common and include an obtuse angle of the cranial base, retrognathia, and cervical spine abnormalities. Despite these anomalies, sleep-disturbed breathing is not reported frequently in patients with 22q11.2 deletion syndrome. We describe a patient with an obstructive sleep disturbance that was successfully treated with a tonsillectomy followed by mandibular distraction osteogenesis. She also had central sleep apnea, initially attributed to spinal cord impingement from cervical instability. Posterior cervical fusion was associated with a decrease in the number of central apneic events.  相似文献   
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PURPOSE: The aim of the study was to assess the impact of sodium hyaluronate and carboxymethylcellulose membrane (Seprafilm®) on postoperative intestinal obstruction as judged by the rates of bowel obstruction and laparotomy for bowel obstruction. A secondary aim was to assess early postoperative morbidity. METHODS: All patients who had Seprafilm® placed during colorectal surgery between June 1993 and October 1998 were included in the study group and compared with a matched group of patients without Seprafilm®. All patients were assessed for intestinal obstruction and complications by telephone interview and chart review. Statistical tests for independence were used where appropriate; alpha was 0.05 for all tests, and the two groups were tested for case matching. Fisher's exact test was used to compare gender distribution, nature of diagnosis (inflammatoryvs. noninflammatory), and urgency of surgery (electivevs. emergency). The age distribution, number of prior abdominal surgeries, and operative time were compared by Student'st-test. Approximation of Katz test was used for independent proportions to compare the two groups for early postoperative morbidity and overall incidence of intestinal obstruction and surgical enterolysis. The incidence of intestinal obstruction between the two groups was also compared with Kaplan-Meier product limit method and log-rank test. RESULTS: Two hundred fifty-nine patients in whom Seprafilm® was placed were compared with a well-matched control cohort of 179 patients. The two groups did not differ in gender or age. One-half of each group had inflammatory conditions, and approximately 90 percent of each group underwent elective operations. The operative times were similar. Both groups had a similar number of abdominal operations before inclusion (mean = 1.2, both groups). Early morbidity rates were 17.8 percent for the Seprafilm® group and 15.6 percent for the controls, with mortality rates of 0.8 percent and 0.0 percent, respectively. There were 12 intestinal obstructions in 12 patients in the Seprafilm® group and 12 intestinal obstructions in 11 patients in the control group at a follow-up period of 65 months in the Seprafilm® group and 81 months in the control group. Eight of the 12 intestinal obstructions in the Seprafilm® group resolved with conservative management while only 5 of 12 in the control group responded without surgery. Thus the enterolysis rate was 1.5 percent in the Seprafilm® group and 3.9 percent in the control group, demonstrating a trend in favor of Seprafilm®. There were no statistically significant differences in the incidence of either overall or abdominopelvic septic complications between the Seprafilm® (3.4 percent) and control (1.1 percent) groups. CONCLUSION: During short-term follow-up in this nonprospective, nonrandomized study, limited placement of Seprafilm® did not significantly reduce the need for surgical enterolysis for intestinal obstruction or significantly adversely affect the morbidity rate. However, a long-term, prospective, randomized trial is underway to elucidate these issues.Supported by an educational grant from Genzyme Surgical Products, Inc.Presented at the Association of Coloproctology of Great Britain and Ireland, Southport, UK, July 10 to 12, 1999.  相似文献   
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BACKGROUND: The survival of patients with human immunodeficiency virus (HIV) has improved considerably with modern medical management. However, there remains surprisingly little information on treating head and neck neoplasms in HIV-positive patients. OBJECTIVE: To report our recent experience treating oral cavity and oropharyngeal tumors in HIV-positive patients. DESIGN AND PATIENTS: Retrospective analysis of a cohort of 8 HIV-positive patients with Kaposi sarcoma (KS), lymphoma, or squamous carcinoma of the oral cavity or oropharynx who were consecutively treated during a single year with radiation therapy at a tertiary care referral center. Length of follow-up was at least 2 years (mean, 2.5 years). RESULTS: All patients had partial and complete responses to treatment lasting until the last follow-up. However, we found that treatment was considerably better tolerated by patients with non-KS tumors, with fewer acute reactions and significantly less weight loss, despite larger treatment volumes and higher radiation doses, compared with patients with KS. Patients with non-KS tumors received a mean radiation dose of 62.6 Gy to 2636 cm3, yet lost only a mean of 0.1 kg in weight, whereas patients with KS were treated with a mean radiation dose of 19 Gy to a mean volume of 568 cm3, but lost a mean of 5.8 kg during treatment (P = .005) and on average sustained an additional grade of severity on a standard scale of mucosal reaction (P = .01). CONCLUSIONS: Oral cavity and oropharyngeal tumors in HIV-positive patients respond to radiation therapy, but there is a marked difference in the degree of acute reactions to treatment between patients with and without KS. Infection with HIV is not a contraindication when aggressive radiation therapy is needed in select patients.  相似文献   
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This article analyzes turnover in the position of state hospital director. It examines the relationship between role structure, job stress and burnout. Based on this analysis, recommendations are made for the restructuring of a top management team. Buffalo Psychiatric Center, a 750-bed facility operated by the New York State Office of Mental Health, provides the setting for this case study.Diane M. Pinchoff, M.A., is Associate Director of the Buffalo Psychiatric Center, 400 Forest Avenue, Buffalo, N.Y. 14213. Mahmud Mirza, M.D., is Director of the Buffalo Psychiatric Center. Reprint requests should be directed to Diane M. Pinchoff at the above address.  相似文献   
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The population diversity of Bangladeshi begomoviruses and their vector, Bemisia tabaci was analysed by PCR-based detection and partial genome sequencing. B. tabaci adults and plants expressing symptoms of virus infection were collected from locations representing diverse agro-ecological regions of the country. Universal and species-specific primers were used to detect begomoviruses in seven crops (chilli, okra, papaya, pumpkin, sponge gourd, tomato and yardlong bean) and two common weeds (Ageratum conyzoides and Croton bonplandianum). At least five distinct species of tomato leaf curl viruses infected tomato and other host-plants. Phylogenetic analyses of their nucleotide sequences (∼530 bases) from the intergenic region and capsid protein of DNA-A indicated the existence of five distinct clusters of begomoviruses. Begomoviruses infecting tomato, chilli and dolichos have been reported previously, and those infecting Ageratum, Croton, okra, papaya, pumpkin and yardlong bean are described for the first time. Phylogenetic analyses based on mitochondrial cytochrome oxidase I gene sequences of 21 B. tabaci from Bangladesh and other reference sequences grouped them into at least two independent clusters. Some sequences from different countries, e.g., Bangladesh, China, India, Nepal, Pakistan and Thailand were almost identical while others collected from plants within the same field diverged by as much as 15%, indicating high diversity even at the local level. None of the B. tabaci from Bangladesh grouped with the reference B- and Q-biotype sequences, thus these two aggressive biotypes were apparently absent from Bangladesh.  相似文献   
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