Background: Takayasus arteritis (TA) is a rare disease which appears to be m ost common in East Asia. However, the disease has been reported to be worldwide. The clinical features of the disease can show variations ...Background: Takayasus arteritis (TA) is a rare disease which appears to be m ost common in East Asia. However, the disease has been reported to be worldwide. The clinical features of the disease can show variations in different geographi cal areas. The aim of this study is to evaluate clinical, laboratory and radiolo gical features and the outcome of patients with TA in our hospital. Methods: The hospital files of patients who were followed with the diagnosis of TA between t he years 1973 and 2003 in Hacettepe University Hospital were retrospectively eva luated. Results: Male/female ratio was 5/40, and the mean age was 34 years (18- 59). Constitutional symptoms were present in 71%of the patients. Claudication a nd pallor of the extremity, decreased extremity pulsations, arterial hypertensio n, and arterial bruits were present in 44%, 56%, 58%, and 27%of the patients , respectively. Aortic valv-ular insufficiency, abdominal aortic aneurism, and cardiomegaly were present in four, one, and four patients, respectively. The ini tial complaint of six patients was cerebrovascular events. The distribution of t he patients according to the angiographic findings was as follows, 56%Type I, 18%Type II , 22%Type III, and 4%Type IV arteritis. The need for vascular surgical interve ntions were significantly less common in patients who were treated with immunosu ppressives plus alternate dose steroids (6%) compared to patients who were trea ted only with antiaggregant agents (33%). Conclusions: The demographic and angi ographic findings of our patients were similar to previous observations from Jap an and Italy, and disclose distinct clinical features in comparison to other Asi an countries. Alternate-day glucocorticoids plus cytotoxic drugs may be benefic ial and safe in patients with TA.展开更多
Background: Arcuate ligament vascular compression syndrome has not been described previously in the pediatric or pediatric surgical literature. However, it is mentioned in the literature of vascular and general surger...Background: Arcuate ligament vascular compression syndrome has not been described previously in the pediatric or pediatric surgical literature. However, it is mentioned in the literature of vascular and general surgery and in journals of radiology and orthopedics. In this review, the intraoperative pathological anatomy and the principles of treatment for 8 children will be presented. Methods: The chart records and the anatomical sketches that were documented by the surgeon immediately after each procedure were analyzed retrospectively. In addition, preoperative courses and long-term follow-up (range, 3- 18 years) were evaluated by a defined program. Results: The diagnosis of celiac artery compression by an arcuate ligament was suspected in children presenting with a history of several years of recurrent acute abdominal pain associated with a typical arterial bruit in the midline of the epigastric region. Conclusions: Other diseases with recurrent abdominal pain and an arterial bruit must be excluded before making the decision for an operative intervention. Duplex ultrasound and angiography are possibly helpful tools to establish the respective diagnosis, but in the patients of the present series, these techniques neither confirmed compression of the celiac axis nor demonstrated decreased perfusion of the superior mesenteric artery. However, as the clinical symptoms clearly announce the disease, these diagnostic measures are not mandatory.展开更多
文摘Background: Takayasus arteritis (TA) is a rare disease which appears to be m ost common in East Asia. However, the disease has been reported to be worldwide. The clinical features of the disease can show variations in different geographi cal areas. The aim of this study is to evaluate clinical, laboratory and radiolo gical features and the outcome of patients with TA in our hospital. Methods: The hospital files of patients who were followed with the diagnosis of TA between t he years 1973 and 2003 in Hacettepe University Hospital were retrospectively eva luated. Results: Male/female ratio was 5/40, and the mean age was 34 years (18- 59). Constitutional symptoms were present in 71%of the patients. Claudication a nd pallor of the extremity, decreased extremity pulsations, arterial hypertensio n, and arterial bruits were present in 44%, 56%, 58%, and 27%of the patients , respectively. Aortic valv-ular insufficiency, abdominal aortic aneurism, and cardiomegaly were present in four, one, and four patients, respectively. The ini tial complaint of six patients was cerebrovascular events. The distribution of t he patients according to the angiographic findings was as follows, 56%Type I, 18%Type II , 22%Type III, and 4%Type IV arteritis. The need for vascular surgical interve ntions were significantly less common in patients who were treated with immunosu ppressives plus alternate dose steroids (6%) compared to patients who were trea ted only with antiaggregant agents (33%). Conclusions: The demographic and angi ographic findings of our patients were similar to previous observations from Jap an and Italy, and disclose distinct clinical features in comparison to other Asi an countries. Alternate-day glucocorticoids plus cytotoxic drugs may be benefic ial and safe in patients with TA.
文摘Background: Arcuate ligament vascular compression syndrome has not been described previously in the pediatric or pediatric surgical literature. However, it is mentioned in the literature of vascular and general surgery and in journals of radiology and orthopedics. In this review, the intraoperative pathological anatomy and the principles of treatment for 8 children will be presented. Methods: The chart records and the anatomical sketches that were documented by the surgeon immediately after each procedure were analyzed retrospectively. In addition, preoperative courses and long-term follow-up (range, 3- 18 years) were evaluated by a defined program. Results: The diagnosis of celiac artery compression by an arcuate ligament was suspected in children presenting with a history of several years of recurrent acute abdominal pain associated with a typical arterial bruit in the midline of the epigastric region. Conclusions: Other diseases with recurrent abdominal pain and an arterial bruit must be excluded before making the decision for an operative intervention. Duplex ultrasound and angiography are possibly helpful tools to establish the respective diagnosis, but in the patients of the present series, these techniques neither confirmed compression of the celiac axis nor demonstrated decreased perfusion of the superior mesenteric artery. However, as the clinical symptoms clearly announce the disease, these diagnostic measures are not mandatory.