Patient one was a 19-year-old woman of periodic fever with urticaria during the first week of life.And there were typicalfacies,oligoarthritis and central nervous system involvement.She also exhibited a persistent elevation of acute phase reactants.The diagnosis of chronic infantile neurological cutaneous and articular syndrome was made.Short-term dosing of tumor necrosis factor-α receptor antagonist was administrated and was substituted by glucocorticoid and methotrexate.Another two males had an earlyonset of increased acute phase reactants during inflammation attacks.One of them had recurrent fever with urticaria-like rash,arthralgia and headache.Muckle-Wells syndrome was diagnosed on basis of NLRP3 gene mutation (c.92A ＞ T).And another had recurrent urticaria-like rash with conjunctivitis,oligoarthritis,hepatosplenomegaly and increased serum IgD.The clinical diagnosis was mevalonate-kinase deficiency.Autoinflammatory diseases should be suspected by physicians and diagnosed with a combination of phenotype and genotype.Biological agents,especially interleukin-1 antagonist,have broad prospects in the therapy of autoinflammatory diseases.

Objective To discuss how to improve judgement and technique for avoiding bile duct injury during laparoscopic cholecystectomy(LC).Methods The clinical data of 39 860 patients treated by LC between October 1992 and October 2005 were analyzed retrospectively.Bile duct injury occured in 46 patients.Results Bile duct repair and T-tube drainage was performed in 26 patients and bile duct end-to-end anastomosis and T-tube drainage was performed in 4 patients,with the T-tube indwelling time for 3~12 months.The Roux-en-Y cholangiojejunostomy was conducted in 11 patients,the ligation of accessory hepatic duct was conducted in 5 pauients,the re-operation for bile duct stenosis in 4 patients,and the re-operation for stenosis after cholangiojejunostomy,in 2 patients.Conclusions Intensive anatomic knowledge and skillful surgical performance can avoid or minimize the incidence of bile duct injury.Early detection and active repair of the bile duct injury for preventing acute inflammation is the most important measure to avoid repeated operations.

Objective To explore the management of unsuspected gallbladder carcinoma (UGC) discovered during laparoscopic cholecystectomy (LC). Methods From January 2000 to May 2007, 15 560 cases of LC were performed in our hospital, UGC was discovered in 28 of the cases. Clinical data of the 28 patients were analyzed retrospectively. Results Among the 28 cases of UGC, 12 were discovered intraoperatively, and 16 were diagnosed postoperatively.Radical cholecystectomy was performed on the 12 cases who were detected during the operation,and 5 of the 16 patients who were diagnosed after the operation.LC was carried out in the remaining 11 cases. Postoperative pathological examination showed TNM stageⅠin 3 cases, stageⅡin 23, and stage Ⅲ in 2. The patients were followed up for 7-66 months with a mean of 22 months. During the period, 19 patients died. The 1-, 3-, and 5-year survival rate of the 17 patients who received radical cholecystectomy were 76.5% (13/17), 23.5% (4/17), and 11.8% (2/17), respectively. Whereas, in the 11 patients who underwent LC, the 1-year survival rate was 45.4%; moreover, none of the 11 survived for more than 2 years. Conclusions For high-risk population, attention should be paid to UGC during operation. Most of the UGCs discovered during LC are early-stage carcinomas, which should be treated with standard radical cholecystectomy, but not LC.

Objective To analyze the multi-slice computed tomography (MSCT) enhanced findings in the patients with primary small intestinal stromal tumor(SIST),and to probe the relationship between the imaging findings and the pathologic risk in order to improve the diagnostic accuracy.Methods Thirty patients with primary SIST confirmed by surgical pathology were enrolled in this study.Characterization and compassion of the clinical manifestations and MSCT enhanced findings were carried out between the pathologic low-and high-risk groups.Furthermore,the relationship was analyzed between the enhanced findings and the pathologic risk.Results Among all 30 patients with primary SIST,the lesion was located at duodenum in 5 patients (16.7％),at jejunum in 16 (53.3％),and at ileum in 9 (30％).14 patients were classified in the low risk group with the lesion with the average length of (3.8±0.9) cm,and other 16 in the high-risk group with lesion with the average length of (7.0 ± 1.4) cm.There were no statistical differences between the low-and high-risk groups in CT value in plain and venous phase,and in added value in arterial,venous and delayed phases.However,the significantly differences were observed in CT value in arterial and delayed phases between two groups (P＜0.05).Conclusion MSCT may effectively evaluate the pathologic risk of primary SIST.There are significant differences of the enhanced findings between the low and high-risk groups,which can provide important apreoperative classification for the therapy.

Objective To analyse the CT features of pulmonary echinococcosis and to assess CT value in diagnosing this disease.Methods CT fingings of pulmonary echinococcosis in 35 cases proved pathologically were analyzed retrospectively.Results Pulmonary granulosus echinococcosis was 33 cases including simple hydatid cyst (11 lesions),datugter cyst hydatid (3 lesions),with calcification (1 lesion) ,rupture (11 lesions),merging infection (4 lesions) and rupture into thorax (3 lesions).Pulmonary alveolar echinococcosis was 2 lesions,CT findings apperared as irregular,peripherally scattered more nodules with small vacuoles or calcification and in combination with hepatic alveolar echinococcosis.Conclusion Pulmonary echinococcosis had characteristic features on CT,CT examination can provide more imaging informations for the treatment of pulmonary echinococcosis

Objective To investigate the effects of β1-adrenergic receptor autoantibodies (β1-AA) on the proliferation of different subtypes of T lymphocytes in patients with heart failure .Methods β1-AA-positive IgG antibodies isolated from patients with heart failure were purified by using affinity chromatog -raphy.CD3 +CD4 +T and CD3 +CD8 +T lymphocytes were sorted by flow cytometry analysis .The prolifera-tion of different subtypes of T lymphocytes was tested by using CCK-8 kit.Tests for lactate dehydrogenase ( LDH) level and cell apoptosis were performed to evaluate T lymphocytes damage .Results The prolifera-tion of activated lymphocytes was inhibited by β1-AA isolated from the serum of patients with heart failure in a concentration dependent manner , but it could be blocked by β1 receptor blocker .The damage of lympho-cytes induced by β1-AA was increased.Moreover, β1-AA promoted the necrosis and apoptosis of CD 3 +CD4 +T and CD3 +CD8 +T lymphocytes and thus inhibited the proliferation of them .Conclusion β1-AA isolated from the serum of patients with heart failure inhibited the proliferation of CD 3 +CD4 +T and CD3 +CD8 +T lymphocytes through increasing the necrosis and apoptosis of them .This study suggests that β1-AA might induce immune disorders in addition to causing pathological changes in heart tissues .

Objective To explore CT manifestion of multiple primary lung cancer (MPLC)and to improve the recognization of MPLC.Methods The CT manifestions of 12 cases with MPLC proved by pathology were retrospectively reviewed.Results All the 12 cases were double primary lung cancer.There were 24 lesions with 21 peripheral and 3 central,the average diameter was (2.2 ± 0.6)cm.Lesions located in contralateral lobes were in 2 patients,and located in ipsilateral lobes were in 10 patients,with 3 located in the same lobe and 7 in the different lobes.There were 7 cases of adenocarcinoma-adenocarcinoma,3 cases of squamous cell carci-noma-adenocarcinoma,1 case of squamous cell carcinoma-squamous cell carcinoma and 1 case of squamous cell carcinoma-carcinoid. 1 1 cases were metachronous and 1 case was synchronous.3 lesions were lump located at hilus of the lung,21 lesions were intrapul-monary nodules,showing masses with lobulated shape,spicules of margin,vascular convergence,vacuole and pleural indentation sign.Most foci displayed moderate intensity enhancement and homogeneous density in triphase enhanced scans,the CT value of le-sions on enhanced images ranged from 20-60 HU.Conclusion MPLC are synchronous and peripheral adenocarcinoma type,all of the lesions have typical CT features of primary lung cancer.

BACKGROUND: Vascular endothelial growth factor (VEGF) can accelerate neovascularization and, as a multifunctional cytokine, performs critical functions via its receptors in angiogenesis.OBJECTIVE: To investigate the expression of VEGF and its receptor FLT-1 and FLK-1 mRNA during the early stage of acute focal cerebral brain ischemia, and examine the relationship between the timing and location of their expressions.DESIGN: Randomized controlled study.SETTING: Department of Neurology of the First Hospital Affiliated to Jilin University and Teaching and Research Section of Pathology, Bethune Medical College of Jilin University.MATERIALS: This study was carried out between June 2001 and April 2002. Totally 130 adult SD rats were selected, with male and female in half, and randomly divided into normal control group (n=10), sham operation group (n=10), and cerebral ischemia group (n=110). The rats in cerebral ischemia group were further divided equally into 11 subgroups and examined at 0, 1, 2, 3, 6, 24, 48 hours and 1, 2, 3, 4 weeks after cerebral ischemia model establishment, respectively.METHODS: Permanent middle cerebral artery occlusion (MCAO) model was established in rats in cerebral ischemia group by ligation of the left common carotid artery, while the rats in the sham operation group received no artery ligation but with identical other treatments. The rats in the control group did not have any treatment. Reverse transcriptional (RT) PCR was used to detect the expression of VEGF and its receptor mRNA at different time points after ischemic model establishment, and neovascularization in the rats'brain was observed.MAIN OUTCOME MEASURES: ① Expression of VEGF and its receptor mRNA and ② neovascularization in the brain tissue at different time points of cerebral ischemia.RESULTS: Data of the 130 mice were statistically analyzed without losses.At 3, 6, 24, and 48 hours of ischemia, the number of cells positive for VEGF expression was 31.13±2.21, 43.11±2.43, 85.41±2.75 and 98.66±1.76 in each vision filed in the surrounding ischemic region, greater than the numbers in the central ischemic region at the corresponding time points (13.32±1.31, 19.40±3.22, 47.63±2.45, 57.32±3.35 in each vision field, respectively, P ＜ 0.05). VEGF mRNA expression gradually decreased since 48 hours after model establishment till recovering the control level by 2weeks. The expression of VEGF receptor FLT-1 mRNA, determined by the number of positive cells in each vision field at 3, 6, 24, and 48 hour after the ischemia in each vision field for FLK-1 mRNA at these time points in the peripheral ischemic regions, higher than those in the central ischemic regions (P ＜ 0.05), which recovered the control level 3 weeks after the ischemia (P ＜ 0.05). At 48 hours and 1 week after the ischemia, the number of microvessels in each vision field was 47.2±2.11 and 199.2±3.45 in the peripheral ischemic region, significantly higher than the number in the central ischemic region (29.4±2.37 and 76.6±4.62, P ＜ 0.05).CONCLUSION: VEGF and its receptors FLT-1 and FLK-1 mRNA are expressed in the neurons, glial cells and endothelial cells during the early stage of acute focal cerebral ischemia, and the expressions are significantly enhanced in response to ischemia, exhibiting temporal and spatial expression patterns.

Objective To investigate the role of nuclear factor-kappa B (NF-κB)signaling pathway in propofol-induced suppression of up-regulation of inducible nitric oxide synthase (iNOS) gene expression in LPSstimulated RAW264.7 cells.Methods RAW264.7 cells were purchased from cell bank of Chinese Academy of Sciences and cultured in DMEM culture medium containing 10％ fetal bovine serum.The cells were seeded in 6 cm diameter dishes (3 ml/dish) or in 6-well plates (2 ml/well) with a density of 5 × 105/ml and randomly divided into 3 groups ( n =18): normal control group (group C),group LPS (group L)and group LPS + propofol (group LP).The cells were incubated with LPS 1 μg/ml in groups L and LP.Propofol 50μmol/L was added to the culture medium at 2 h before LPS in group LP.Cells were harvested at 30 min after being stimulated with LPS.Phosphorylation of IκB kinase(p-IKK) and NF-κB activity were detected by Western blot.The expression of iNOS mRNA was determined after 6 h exposure of the cells to LPS.Results LPS significantly up-regulated the expression of p-IKK and iNOS mRNA and increased NF-κB activity in group L as compared with group C.Propofol pretreatment significantly attenuated the effects of LPS on p-IKK,iNOS mRNA expression and NF-κB activity.Conclusion NF-κB signaling pathway is involved in the propofol-induced suppression of up-regulation of iNOS mRNA expression in LPS-stimulated RAW264.7 cells.

Objective: To investigate the effect of sevoflurane anesthesia on hepatic ischemia-reperfusion injury in patients undergoing partial hepatectomy. Methods: A total of 86 patients undergoing partial hepatectomy were divided into sevoflurane group and isoflurane group according to random number table with 43 patients in each group. Both groups received continuous epidural block combined with general anesthesia, sevoflurane group took sevoflurane combined with remifentanil to maintain anesthesia, while isoflurane group took isoflurane remifentanil to maintain anesthesia. The mean arterial pressure (MAP) and heart rate(HR) of different moments, awakening (open-eye time, extubation time, listening to instruction time, orientation recovery time, time of Aldrete score up to 9 minutes), the serum levels of tumor necrosis factor alpha (TNF-α), interleukin 1 (IL-1), aspartate aminotransferase (AST), alanine aminotransferase (ALT), glutamate dehydrogenase (GLDH), alpha glutathione S transferase (α-GST) levels at different moments were compared between the two groups. Results: The MAP and HR at T₁, T₂, T₃ and T₄ in both groups were significantly lower than those at T₀ (P<0.05). The HR at T₁ and T₄ in sevoflurane group were significantly higher than those in isoflurane group (P<0.05). The open-eye time, extubation time, listening to instruction time, orientation recovery time in sevoflurane group were significantly shorter than the isoflurane group (P<0.05). The levels of serum TNF-α, IL-1, AST, ALT, GLDH and α-GST in the two groups at T₂ and T₃ were significantly higher than those at T₀ (P<0.05), and began to fall back to T₄. The levels of serum TNF-α, IL-1, AST, ALT, GLDH and α-GST in the sevoflurane group at T₂ and T₃ were significantly lower than those in the isoflurane group (P<0.05). Conclusions The hepatic hemodynamics in patients undergoing partial hepatectomy with sevoflurane is more stable better than isoflurane during operation, which has faster postoperative recovery and relieved hepatic ischemia-reperfusion injury.