Objective To evaluate the value of covered self-expending metallic stent in the management of malignant esophageal and duodenal strictures or fistulas and explore the treatment for the associated common complications.Methods With interventional procedure under fluoroscopic guidance,95 self-expending mesh stents were implanted in 87 patients,including esophageal carcinoma(50 cases),esophagogastric anastomotic stenosis(23 cases),gastroduodenal malignant stenosis(5 cases),esophagobronchial fistula(3 cases),esophagogastric anastomotic fistula(6 cases).Results Stent placement was successful in all patients at the first trial,having no instant complication except one acute bleeding during the procedure and all of them with relief of stenostic complaint,restoration of clictelic function and closure of fistulas.The long-term curative effect showed something to do with different kind of disease,and the related treatment.Conclusion Self-expending metallic covered mesh stent is safe,effective and less complication;and could be further promoted with addition of radiotherapy and(or)chemotherapy.

Objective To investigate the keratin 9 gene mutation in epidermolytic palmoplantar keratoderma (EPPK) and its relationship with clinical manifestations. Methods Three Chinese pedigrees with EPPK were studied. Polymerase chain reaction (PCR) was performed to amplify the seven exons encoded by keratin 9. Denaturing high-performance liquid chromatography (DHPLC), DNA sequencing and allele-specific PCR were used to reveal the sequence variation in the PCR products. Results An insertion-deletion mutation in the exon 1 of keratin 9 497delAinsGGCT, was revealed in all 3 EPPK families, resulting in the keratin 9 change from tyrosine166 to tryptophan and leucine (Y166delinsWL). Allele-specific PCR confirmed that the mutation was not a commonly seen polymorphism, but a novel mutation which has not been reported in The Human Intermediate Filament Mutation Database (http://www.interfil.org). Conclusions A new keratin 9 gene mutation, 497delAinsGGCT, is found in these Chinese EPPK pedigrees, which may be the genetic basis of EPPK.

Objective To investigate the multi factor for type 2 diabetes with hyperuricemia and metabolic syndrome for preven‐tion cardiovascular disease .Methods 672 cases diagnosed type 2 diabetes were selected in Beijing Anzhen and Cuigezhuang com‐munities to measure height ,weight ,neck circumference ,waist ,BMI ,blood pressure ,fasting plasma glucose ,HbA1c and lipid pro‐file .Results The prevalence of HUA in 672 cases is 8 .04% .It was significantly lower than coastal area population in Shandong Province .BMI ,neck circumference ,waist ,DBP ,FBG ,2 hPG ,HbA1c ,GLT ,BUN ,blood uric were significantly higher than NUA group .The prevalence of MS and fatty in HUA group is 75 .93% ,77 .78% .While in NUA group is 58 .9% ,56 .8% respectively P=0 .014 ,0 .003 .Logistic regression analysis showed that BMI ,HbA1c ,ALT ,BUN were associated with hyperuricemia in T2DM . Conclusion The prevalence of HUA in Beijing diabetic patients was lower than coastal area population ,but complicated MS and cardiovascular risk factor were high and seriously .

Objective To evaluate the diagnostic and therapeutic value and safety of transcatheter arterial angiography and embolization in patients with endoscopic refractory gastrointestinal bleeding.Methods Thirty-one cases of endoscopic refractory gastrointestinal bleeding were performed DSA and treated with transcatheter arterial angiography and embolization.The safty and efficacy was evaluated.Results Angiographic positive rate of bleeding was 80.65％ (25/31);28 cases was treated with embolization.The success rate of first embolization was 75.00％ (21/28),and the total success rate was 82.14 ％ (23/28) by the second embolization.Seven patients received surgical resection after interventional therapy,including 2 cases of jejunal stromal tumors and 5 cases of gastric malignant tumors.Four cases of gastric cancer patients underwent rebleeding within 30 days after interventional therapy,of which 2 died of heart or lung function failure due to basic diseases.Except for 1 patient of anastomotic bleeding after gastrointestinal anastomosis occurred anastomotic fistula after embolization,who recovery with the support treatment,no other cases occurred serious gastrointestinal ischemic necrosis.Conclusion Interventional diagnosis and treatment for gastrointestinal bleeding hemostasis is effective and safety,and also can achieve good results especially for malignant gastric tumor hemorrhage,which can be used for endoscopic refractory gastrointestinal bleeding patients.

ObjectiveThis clinical study is aimed to investigate whether levetiracetam (LEV) can improve electrocorticogram (EEG) in epileptic children epilepsy patients with better clinical manifestation but abnormal EEG findings.MethodsTotally 39 children from our neurological clinic with partial or complex partial epilepsy seizure were included in present study and assigned equally into three groups receiving different treatment:control group,sodium valproate (VPA) group,and LEV group.Their clinical symptoms had been controlled for over one year by carbamazepine ( CBZ),but EEG results showed clearly abnormal.Epileptiform discharges were observed in routine EEG exams half a month before recruiting.After recruiting,they continued to receive CBZ alone (control group) or co-treated with VPA ( VPA group) or LEV (LEVgroup),respectively.Six months later,EEG was taken again and results were analyzed.ResultsImprovement rate were 9.1％ ( control group),23.1％ ( VPA group),and 66.7％ (LEV group),respectively;Overall statistical difference was reached among three groups ( P＜0.01 ) and between control group and LEV group( P＜0.012 5 ),but no statistical difference between control group and VPA group was reached ( P＞0.0125).ConclusionCo-treatment of LEV in child epilepsy patients receiving CBZ can significantly decrease abnormal EEG discharge frequency during interictal period.

Objective To establish a high performance liquid chromatography method for the determination of matrine in Lefu oral liquid .Methods The HPLC method was performed on a Diamonsil Platisil NH2 column (4 .6 mm × 250 mm ,5μm) with the mobile phase of acetonitrile-isopropyl alcohol-3% phosphoric acid solution (84 ∶ 4 ∶ 12 ) . The flow rate was 1 .2 ml/min .The sample injection volume was 5 μl .The detective wavelength was 205 nm .Results The calibration curve of matrine showed good linear response ranged from 54 .50 to 872 .00 μg/ml with r=0 .999 1 .The average recovery of spiked samples for matrine was 99 .82% while the relative standard deviation for repetitions was 1 .12% .Conclusion The method was simple ,reliable and repeatable ,which could be used for the quantitative determination of matrine of Lefu oral liquid .

Objective: To investigate the molecular genetic and clinicopathologic characteristics, immunophenotypes, diagnostic and differential diagnostic features of myxoid angiomatoid fibrous histiocytoma (MAFH). Methods: Three cases of MAFH were collected from the archives of Zhejiang Provincial People′s Hospital between January 2015 to August 2018. The clinical and radiologic features, histomorphology, immunohistochemistry, molecular genetics and prognosis were analyzed. Results: Patients consisted of 2 women and 1 man aged 37 years, 46 years, and 57 years, respectively. The clinical manifestations of 3 patients were presented as a painless, slowly-enlarged mass with a duration ranging of 2 weeks, 2 months and 50 years. These tumors were located at the deep somatic soft tissue of extremities or limbs (right hip, left forearm, left wrist, respectively) and 2 were preoperatively considered as ganglion cyst or giant cell tumor of tendon sheath by imaging examinations. The diameter of circumscribed mass lesion was ranged from 3.0 to 7.5 cm, which exhibited a gray white to tan and gelatinous cut surface. Extensive hemorrhage and cystic changes were observed in 2 cases. Under low magnification, all tumors showed a dense fibrous pseudo-capsule with a peritumoral lymphoplasmacytic cuff and a multi-nodular growth pattern. Blood-filled cystic spaces were observed in 2 tumors. The myxoid stroma occupied 60.0%, 80.0% and 90.0% area of the entire tumor, respectively. Within the myxoid areas, tumor cells were oval to stellate and arranged in cord-like, microcystic and reticular growth patterns. Transitions of myxoid tumor components to more solid areas with typical histology of angiomatoid fibrous histiocytoma (AFH) were observed at least focally in all the three cases. The tumor cells exhibited minimal atypia and scarce mitoses (1 to 2/50 HPF) without necrosis, and prominently focal intracytoplasmic vacuoles were identified in one case. The results of immunohistochemistry staining showed that, 2/3 cases focally expressed desmin, 2/3 focally expressed epithelial membrane antigen (EMA), and 1/3 focally expressed CD99. The positive index of Ki67 was approximately 1% to 5%. Fluorescence in situ hybridization analysis showed that EWSR1 gene rearrangement occurred in all of the three cases. During the period of follow-up, one case showed local recurrence at 15 months, one case showed postoperative recurrence at 24 months, and the recurrent tumor slowly grew for 120 months until the second resection, without recurrence at the following 2 months. The left case showed a disease-free survival at 32 months. Conclusions MAFH is a rare subtype of AFH with a low-grade behavior and may lead to diagnostic confusions. Carefully searching for the typical AFH histomorphology and combining with EWSR1 gene rearrangement test can help to distinguish MAFH from other mimickers.

Objective:To investigate the clinicopathological features,diagnosis and differential diagnosis of renal angiomyolipoma with epithelial cysts(AMLEC).Methods:Four cases of renal AMLEC diagnosed between January 2014 and June 2019 at the Department of Pathology,Zhejiang Provincial People′s Hospital were subjected to clinicopathological, histological and immunohistochemistry analyses along with a literature review.Results:All the four patients were females and aged from 19 to 52 years (mean 34.5 years). Three cases were accidentally discovered by physical examination, and the medical history was 1 to 6 years. The preoperative imaging Bosniak classification was grade Ⅲ in 3 and grade Ⅳ in 1 case. The maximum diameter of the tumor ranged from 2.5 to 9.0 cm (average 5.0 cm). Histologically, all of the 4 tumors showed three histological components: (1) simple epithelial cysts lined by a layer of cuboidal/low-columnar to occasionally, hobnailcells; (2) a thin, compact subepithelial "cambium-like" layer of cellular, mullerian-like short spindle cell stromas with prominent admixedchronic inflammation; (3) a outermost layer of thick, long-fascicles of smooth muscle-like stromas, often surrounded by dysplastic, tortuous thick-walled blood vessels. There was often a prominent lymphatic channel network in the smooth muscle component forming slit like branched and curvilinear spaces. None of the 4 tumors had fat content.Immunohistochemically, the epithelial cells lining the cysts strongly expressed PAX8 and CK7. The subepithelial "cambium-like" stromas strongly expressed melanocytic markers (HMB45, Melan A, Cathspin K, MiTF) and mullerian markers (ER,PR,CD10), and were negative for smooth-muscle markers(SMA,desmin,calponin). The outermost layer of smooth muscle-like stromas strongly expressed smooth-muscle markers, and were only focally positive for melanocytic and mullerian markers. Follow-up information was obtained in 3 cases, among which no evidence of tumor recurrence or metastasis was found at 3, 5, and 66 months of follow-up, respectively.Conclusions:Renal AMLEC is a rare histological subtype of angiomyolipoma with benign biological behavior, and has characteristic histological and immunophenotypic characteristics.Pathologists should be familiar with the clinicopathological appearances of AMLEC and include it in the differential diagnostic spectrums of renal tumors with biphasic epithelial and mesenchymal features.

Objective:To investigate the clinicopathologic features, diagnosis and differential diagnosis of large nested variant of urothelial carcinoma (LNUC) of urinary bladder.Methods:The clinical and pathologic data of five cases of LNUC of urinary bladder diagnosed between January 2014 and December 2018 at the Department of Pathology, Zhejiang Provincial People′s Hospital were analyzed by immunohistochemistry (IHC) and Sanger sequencing. The relevant literature was reviewed.Results:All five patients were male with a mean age of 63 years (range, 48 to 81 years). The mean tumor size was 3.4 cm (range, 1.7 to 4.7 cm). Histologically, the invasive LNUC tumor cells formed medium to large sized nests of varying shapes, from regular round, bulbous, oval to irregularly fused, branched, dumbbell shaped glands, with mild stromal reaction. In all five cases, focal central necrosis and microcystic changes in the tumor nests were identified. Cytologically, the tumor cells were low grade in four cases; the remaining case was overall low grade with focal high grade areas. Mitoses were scarce. All cases possessed surface urothelial tumors, including three low-grade papillary carcinomas, one high-grade papillary carcinoma and one carcinoma in situ. Three of the LNUC were accompanied by small nested variant of urothelial carcinoma and two by conventional high grade invasive urothelial carcinoma. Perineural involvement and angiolymphatic invasion were each noted in four tumors. Radical cystectomy was performed in four cases with TNM stages as followings: pT3aN0M0 in two cases, pT4aN0M0 and pT4aN1M0 in one case each. The remaining case had transurethral bladder resection and was of pT2 stage. By IHC, all five cases were positive for CK7 and p40; four were positive for GATA3; two were positive for CK20; and the mean Ki-67 proliferation index was 18%. TERT promoter mutation status were successfully performed in three cases, with one showing mutation (C228T) and two were wild type. All patients received postoperative chemotherapy. At a follow-up of 2 to 11 months, one patient died of unrelated causes, two patients developed metastases, and two were alive with no evidence of disease.Conclusions:LNUC is a histologic subtype of urothelial carcinoma with deceptively benign features but aggressive behavior, and appreciation of its unique infiltration patterns can aid in diagnosis and differential diagnosis. LNUC tends to coexist with small nested variant of urothelial carcinoma, suggesting these may represent different manifestations of the same urothelial carcinoma subtype.