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Davenport M Ong E Sharif K Alizai N McClean P Hadzic N Kelly DA 《Journal of pediatric surgery》2011,46(9):1689-1694
Introduction
Biliary atresia (BA) is a rare, potentially life-threatening condition of the newborn presenting with conjugated jaundice. Typically, it is treated by an initial attempt to restore bile flow (the Kasai portoenterostomy [KP]) as soon as possible after diagnosis and, if this fails, liver transplantation. Since 1999, the treatment of BA has been centralized to 3 centers in England and Wales able to offer both treatment options. The aim of this study was to review the outcome of this policy change and provide a national benchmark.Methods
The management of all infants born within England and Wales during the period January 1999 to December 2009 was assessed using 3 key performance indicators such as median time to KP, percentage clearance of jaundice (≤20 mol/L) post-KP, and 5- and 10-year native liver and true survival estimates. Data are quoted as median (range), and P < .05 was considered significant.Results
A total of 443 infants had confirmed BA; and of these, most were isolated BA (n = 359), with 84 having other significant anomalies (but predominantly BA splenic malformation syndrome). Four infants died before any biliary intervention. Kasai portoenterostomy was performed in 424 infants (median age, 54 [range 7-209] days), and a primary liver transplant was performed in 15. Clearance of jaundice post-KP was achieved in 232 (55%). There were 41 deaths, including 4 (10%) without any intervention, 24 (58%) post-KP usually because of end-stage liver disease and mostly on a transplant waiting list, and 13 (32%) post-LT usually because of multiorgan failure. Overall, the 5- and 10-year native liver survival estimates were 46% (95% confidence interval [CI], 41-51) and 40% (95% CI, 34-46), respectively. The 5- and 10-year true patient survival estimates were 90% (95% CI, 88-93) and 89% (95% CI, 86-93), respectively. Outcome was worse for those with other anomalies (lower clearance of jaundice post-KP [43% vs 57%; odds ratio, 1.7; 95% CI, 1.04-2.8]; P = .02) and an increased mortality overall (eg, at 5 years, 72 [95% CI, 64-83] vs 94 [95% CI, 91-96]; χ2 = 33; P < .0001).Conclusions
National outcome measures in BA appear better than those from previously published series from comparable countries and may be attributed to centralization of surgical and medical resources. 相似文献47.
Malignant peripheral nerve sheath tumor is a rare neurogenic tumor that usually presents in geriatic patients. Typically, it is found in the trunk and extremities and rarely presents in the head and neck region. It may mimic a carotid body tumor when it presents in the neck. We report the first case of malignant peripheral nerve sheath tumor of the vagus nerve in an adolescent boy. He presented with an asymptomatic lateral neck lump that was thought to be a benign schwannoma on preoperative imaging. We describe the diagnostic dilemma and management difficulties in this patient and review the literature. 相似文献
48.
Jazrawi LM Ong B Jazrawi AJ Rose D 《American journal of orthopedics (Belle Mead, N.J.)》2001,30(3):223-224
Synovial chondromatosis is an uncommon disorder with rare occurrence in the elbow. Case reports in the literature for elbow synovial chondromatosis have described presenting symptoms secondary to peripheral nerve compressions or localized bursitis. We discuss a case of synovial chondromatosis of the elbow that presented as an isolated soft-tissue mass over the radial head-more suggestive of a soft-tissue tumor than of synovial chondromatosis. 相似文献
49.
Finucane BT Ganapathy S Carli F Pridham JN Ong BY Shukla RC Kristoffersson AH Huizar KM Nevin K Ahlén KG;Canadian Ropivacaine Research Group 《Anesthesia and analgesia》2001,92(5):1276-1285
We evaluated the safety and efficacy of a 72-h epidural infusion of ropivacaine and measured the impact of adding fentanyl 2 microg/mL to the required infusion rate, on the quality of postoperative pain relief and the incidence of side effects, after colonic surgery. One hundred fifty-five patients scheduled for elective colonic surgery were randomized in this trial. Epidural infusions of ropivacaine 2 mg/mL with fentanyl 2 microg/mL (R + F) and without fentanyl (R) were commenced during surgery and continued for 72 h postoperatively. This was a prospective, randomized, double-blinded, multi-center trial. The median infusion rate required was less in the R + F group (9.3 vs 11.5 mL/h, P < 0.001). Median pain scores at rest and on coughing were lower in the R + F group (P < 0.0001). The incidence of hypotension was more in the R + F group (P = 0.01). Time to readiness for discharge was delayed in the R + F group (median 6.6 vs 5.5 days, P = 0.012). The addition of fentanyl to ropivacaine resulted in decreased infusion rates and enhanced pain control; however, adverse effects were increased and readiness to discharge was delayed. IMPLICATIONS: Epidural infusions of ropivacaine with and without fentanyl were administered to patients to control pain after colonic surgery. Patients who received ropivacaine with fentanyl had better pain control, increased side effects, and delayed readiness to discharge. This study questions the value of adding opioids to epidural infusions of local anesthetics. 相似文献
50.
Philip Peng FRCPC Manon Choiniere PhD Dominique Dion MD MSc Howard Intrater FRCPC Sandra LeFort PhD Mary Lynch FRCPC May Ong FRCPC Saifee Rashiq MSc DA FRCPC Gregg Tkachuk PhD Yves Veillette FRCPC 《Journal canadien d'anesthésie》2007,54(12):977-984
PURPOSE: The objective of this survey was to examine the services offered by multidisciplinary pain treatment facilities (MPTFs) across Canada and to compare access to care at these MPTFs. METHODS: A MPTF was defined as a clinic that advertised specialized multidisciplinary services for the diagnosis and management of patients with chronic pain, having a minimum of three different health care disciplines (including at least one medical speciality) available and integrated within the facility. The search method included approaching all hospital and rehabilitation centre administrators in Canada, the Insurance Bureau of Canada, the Workplace Safety and Insurance Board or similar body in each province. Designated investigators were responsible for confirming and supplementing MPTFs from the preliminary list for each province. Administrative leads at each eligible MPTF were asked to complete a detailed questionnaire regarding their MPTF infrastructure, clinical, research, teaching and administrative activities. RESULTS: Completed survey forms were received from 102 MPTFs (response rate 85%) with 80% concentrated in major cities, and none in Prince Edward Island and the Territories. The MPTFs offer a wide variety of treatments including non-pharmacological modalities such as interventional, physical and psychological therapy. The median wait time for a first appointment in public MPTFs is six months, which is approximately 12 times longer than non-public MPTFs. Eighteen pain fellowship programs exist in Canadian MPTFs and 64% engage in some form of research activities CONCLUSION: Canadian MPTFs are unable to meet clinical demands of patients suffering from chronic pain, both in terms of regional accessibility and reasonable wait time for patients' first appointment. 相似文献