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Cerebral cavernous malformation (CCM) is one of the most common types of vascular malformations of the central nervous system, affecting nearly one in 200 people. CCM lesions are characterized by grossly dilated vascular channels lined by a single layer of endothelium. Genetic linkage analyses have mapped three CCM loci to CCM1, CCM2 and CCM3. All three causative genes have now been identified allowing new insights into CCM pathophysiology. We focused on the CCM2 protein that might take place in blood vessel formation; we report here the expression patterns of CCM2 in prenatal development and adult human neocortex by means of immunohistochemistry and Western blot analysis. CCM2 was obviously detected in vascular endothelium and neuroglial precursor cells during development and also observed in arterial endothelium, neurons, some of the glial cells in adult neocortex. The expression patterns suggest that it could be one of the arterial markers whether this is a cause or a consequence of an altered vascular identity. CCM2 might play a role during vasculogenesis and angiogenesis during human brain development. Furthermore, with this study, CCM2 have been described for the first time in developing human neocortex.  相似文献   
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Background Alopecia areata (AA) is an organ‐specific, T‐cell‐mediated autoimmune disease that is characterized by non‐scarring hair loss. Objective We aimed to find the factors that may affect the response to topical therapy in AA. Methods The study included a total of 60 patients with AA and 30 healthy control patients. The AA patients were randomized into two groups. 40 patients used 0.05% clobetasol propionate cream, and 20 patients used petrolatum (placebo). Both groups applied topical treatments to their lesions twice daily for 12 weeks. Results The mean extent of AA was 21.88 ± 16.75% in patients with autoantibodies and 12.16 ± 13.55% in those who were negative for autoantibodies (P = 0.021). Ophiasic pattern and nail involvement were observed more frequently in patients with atopy (P < 0.05). Relapse was more frequent in patients with atopy (P = 0.002) and nail involvement (P = 0.02). Conclusions We observed that the presence of autoantibodies was associated with more extensive AA, and that ophiasic hair loss pattern and nail dystrophy were significantly associated with atopy. Topical clobetasol propionate treatment produced a modest advantage in hair regrowth as compared with placebo. Notably, atopic AA patients have a higher risk of relapse and redevelopment of AA after completing a course of topical corticosteroid treatment.  相似文献   
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The current study presents the case of a 19-year-old male patient who was detected with an increased alanine aminotransferase and aspartate transaminase levels during a preoperative evaluation of the right inguinal hernia operation and was later found to have Abernethy malformation accompanied by multiple regenerative nodular hyperplasia and left intra renal inferior vena cava. Regenerative nodular hyperplasia accompanying these two abnormalities is extremely rare and to the best of our knowledge, such a case has not been reported to date. Abdominal ultrasound (US) and color Doppler US, dynamic abdominal magnetic resonance imaging (MRI), and portography examinations were performed and a type 2 abernethy malformation, partial malrotation of the inferior vena cava, and regenerative nodular hyperplasia were detected. We aimed to discuss the radiological signs of these two accompanying abnormalities with a literature review.  相似文献   
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Background and Aim:  Hepatic alveolar echinococcosis (HAE) involves both the vascular and biliary structures of the liver. Endoscopic retrograde cholangiopancreatography (ERCP) is said to be an alternative for the diagnosis and treatment of biliary complications of HAE. We present here our experience with ERCP in HAE.
Methods:  We followed 13 patients who underwent ERCP for the treatment of biliary complications of HAE in the endoscopy unit of our clinic at Ataturk University School of Medicine, Erzurum between January 2002 and June 2008.
Results:  Eight men and five women were followed up. Mean age was 43.2 (24–64 years). All patients had non-resectable HAE. Indications for ERCP were biliary fistula in seven patients, obstructive jaundice in five patients and cholangitis in one patient. Endoscopic sphincterotomy (ES) was carried out in 12 patients, and in one patient with biliary leakage, a stent was inserted into the right hepatic branch. ERCP findings were dilated common bile duct, irregular narrowing and distortion of the common bile duct and common hepatic duct, communication with the cystic cavity or biliocutaneous fistula and complete disappearance of the biliary tree above the level of the common hepatic duct or hepatic bifurcation. In patients with biliary leakage, biliary drainage decreased only in two patients after ERCP and in patients with obstructive jaundice, the high bilirubin levels decreased in only one patient.
Conclusion:  ERCP showed structural changes of the external biliary tract and ES has a limited effect on these changes and stents can be used in selected cases.  相似文献   
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The aim of this study was to evaluate the association between ischemic childhood stroke and thrombophilia. The prevalence of thrombophilia risk factors in 30 unrelated children with ischemic stroke were compared with 33 age-matched control subjects. Patients and control group were tested for the presence of activated protein C (APC) resistance, antiphospholipid antibodies (APLA), increased factor VIII levels, and for the deficiency of protein C (PC), protein S (PS), and antithrombin. When APCR was detected in patients or in controls, factor V Leiden (FVL) mutation was also tested. Seventeen of 30 patients (56.6%) had at least one thrombophilia marker compared with only 5 of 33 control subjects (15.1%). Three children with ischemic stroke (10%) were affected with a combination of two or more thrombophilia markers whereas none of the children in the control group had a combination of risk factors. Seven of 30 children with ischemic stroke (23.3%) and one of 33 control subjects (3.03%) had APC resistance and in all of them FVL mutation were found. The prevalence of FVL mutation was higher among pediatric stroke patients than among control subjects (p < 0.05). None of the patients but one child from the control group (3.03%) had PS deficiency. Antithrombin and PC deficiencies and the presence of APLA and increased factor VIII levels were more frequent in the pediatric stroke patients than in controls but the difference was not statistically significant (p > 0.05). These data confirm that stroke in children is commonly associated with a combination of multiple risk factors and especially the prevalence of FVL mutation is increased in children with ischemic stroke compared with control subjects.  相似文献   
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Dietary and waterborne exposure to copper oxide (CuO) and zinc oxide (ZnO) nanoparticles (NPs) was conducted using a simplified model of an aquatic food chain consisting of zooplankton (Artemia salina) and goldfish (Carassius auratus) to determine bioaccumulation, toxic effects, and particle transport through trophic levels. Artemia contaminated with NPs were used as food in dietary exposure. Fish were exposed to suspensions of the NPs in waterborne exposure. ICP‐MS analysis showed that accumulation primarily occurred in the intestine, followed by the gills and liver. Dietary uptake was lower, but was found to be a potential pathway for transport of NPs to higher organisms. Waterborne exposure resulted in about a 10‐fold higher accumulation in the intestine. The heart, brain, and muscle tissue had no significant Cu or Zn. However, concentrations in muscle increased with NP concentration, which was ascribed to bioaccumulation of Cu and Zn released from NPs. Free Cu concentration in the medium was always higher than that of Zn, indicating CuO NPs dissolved more readily. ZnO NPs were relatively benign, even in waterborne exposure (p ≥ 0.05). In contrast, CuO NPs were toxic. Malondialdehyde levels in the liver and gills increased substantially (p < 0.05). Despite lower Cu accumulation, the liver exhibited significant oxidative stress, which could be from chronic exposure to Cu ions. © 2014 Wiley Periodicals, Inc. Environ Toxicol 30: 119–128, 2015.  相似文献   
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We report a case of successful reoperation for aortic arch dissection with use of the "arch-first" technique in a patient who had Marfan syndrome. Extracorporeal circulation was initiated via right subclavian artery cannulation, and the chest was entered through a clamshell incision for the best exposure. When the patient was cooled to 18 degrees C, the perfusion was stopped. After the 1st aortic arch anastomosis to a 30-mm Dacron graft, cerebral perfusion was reestablished via the right subclavian artery. The aortic repair was then completed. The cerebral ischemic time was 18 minutes, the aortic cross-clamp time was 69 minutes, and the total extracorporeal circulation time was 334 minutes. The patient was discharged from the hospital on postoperative day 10 with no neurologic impairment. The arch-first technique shortens the duration of brain ischemia. When combined with a clamshell incision, the technique is particularly helpful for reoperation of the aortic arch and thoracic aorta.  相似文献   
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