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Biliary and gallbladder diseases in infants and children often present unique diagnostic and therapeutic challenges that require a fundamental understanding of notable biliary diseases and anatomical variations. Surgical and endoscopic approaches that are often the gold standard in adult biliary treatment may be technically challenging and are associated with a high morbidity that may warrant a multidisciplinary treatment approach. This article will provide a comprehensive overview of the biliary conditions where interventional radiology can play a vital role in the diagnosis, management, and treatment. Differences in approach or technique between children and adults will be highlighted.  相似文献   
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Laser-assisted uvulopalatoplasty (LAUP) is an established treatment for snoring. Our institution has been using a modification of the Kamami technique since 1995. Between January 1995 and December 2001, 469 patients underwent LAUP for snoring. A telephone survey of these patients and their partners was conducted retrospectively. One hundred and sixty-eight patients and their partners were interviewed and the findings analysed.Of the 168 patients, 122 had LAUP alone, 42 had LAUP and tonsillectomy and four had LAUP and septoplasty. The median follow-up time was 59 months (range 19 to 98 months).Seventy-four per cent reported improvement at six weeks, 69 per cent reported improvement at nine months and 55 per cent reported improvement at the time of interview. To assess the degree of improvement, patients and their partners were asked to grade any improvement in percentage terms. The mean subjective improvement scores were 57 per cent at six weeks (95 per cent confidence interval (CI) 50-64 per cent), 45 per cent at nine months (95 per cent CI 38-59 per cent) and 30 per cent at the time of interview (95 per cent CI 23-35 per cent). Patients who reported weight gain since surgery did not have a poorer outcome than those who reported no weight change or weight loss. Neither did weight loss predict a better outcome. The procedure was generally well tolerated, with only 30 patients (18 per cent) reporting complications (mainly minor). The benefits of LAUP wane with time and the results are best in the first 12 months following surgery. Our study suggests that 55 per cent of patients selected after sleep studies and sleep nasendoscopy will enjoy long-term benefit.  相似文献   
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Selective muscarinic agonists could be useful in the treatment of neurological disorders such as Alzheimer's disease, schizophrenia, and chronic pain. Many muscarinic agonists have been developed, yet most exhibit at best limited functional selectivity for a given receptor subtype perhaps because of the high degree of sequence homology within the putative binding site, which appears to be buried within the transmembrane domains. Bivalent compounds containing essentially two agonist pharmacophores within the same molecule were synthesized and tested for receptor binding affinity and muscarinic agonist activity. A series of bis-1,2,5-thiadiazole derivatives of 1,2,5,6-tetrahydropyridine linked by an alkyloxy moiety exhibited very high affinity (K(i) < 1 nM) and strong agonist activity. The degree of activity depended on the length of the linking alkyl group, which could be replaced by a poly(ethylene glycol) moiety, resulting in improved water solubility, binding affinity, and agonist potency.  相似文献   
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Optimal treatment of esophageal small‐cell cancer, a rare disease, lacks consensus. Based on its lung small‐cell cancer analog, we hypothesized that chemotherapy with adjuvant radiotherapy would be optimal. This hypothesis was tested by studying the collective published literature. A meta‐analysis of individual patients from 148 articles (1952–2010) explored treatment and outcome of 577 patients with esophageal small‐cell cancer. Hazard function frailty modeling identified optimum therapy after accounting for article‐level and patient‐level heterogeneity. Fifty‐nine percent of publications reported one patient and 25% five or more. Sixty‐six percent of patients were men, mean age was 63 ± 11 years, and 64% had localized disease. One, 3‐, and 5‐year survival was 37%, 14%, and 11%, respectively. Survival variation among articles was substantial (P = 0.004), with survival improving across time (P < 0.0004). Chemotherapy was associated with better survival (hazard ratio [HR] = 0.53, 68% confidence interval [CI] = 0.44–0.65; P = 0.002) than surgery alone, radiotherapy alone, nonstandard therapy, or no therapy. Adding local therapy, either surgery (HR = 0.41, 68% CI = 0.34–0.51; P < 0.0001) or radiotherapy (HR = 0.33, 68% CI = 0.27–0.41; P < 0.0001), to chemotherapy further improved survival. Adding both did not provide further benefit. The strategy of borrowing from consensus treatment of lung small‐cell cancer and analyzing the scarce available esophageal small‐cell cancer literature may be beneficial in the study of rare diseases. It confirmed that chemotherapy should be the mainstay of therapy, with additional benefit from adjuvant therapy with either surgery or radiotherapy; both are not needed.  相似文献   
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