Alport syndrome is a hereditary glomerular basement membrane disease.Hematuria,sensorineural deafness and progressive renal function impairment is the main clinical symptoms.Alport syndrome can be associated with ocular abnormalities,and a few of patients were complicated with diffuse leiomyoma.The incidence of Alport syndrome with diffuse leiomyoma is low,and it mainly invades esophagus,the trachea and female genital tract,et al.Patients with Alport syndrome and diffuse leiomyoma have difficulty in swallowing and postprandial vomiting,recurrent bronchitis symptoms and dyspnea.Diffuse leiomyoma is commonly seen in the esophagus.Alport syndrome with huge esophageal and gastric leiomyoma is rarely seen in clinical practice.In this article,the imaging manifestations of Alport syndrome combined with huge esophageal and gastric leiomyoma is summarized to improve the understanding and diagnostic accuracy of this disease.

Objective To summarize the experiences in the diagnosis and treatment of the hepatic hereditary hemorrhagic telangiectasia (HHHT).Methods The clinical data of 15 HHHT patients who were admitted to the Qilu Hospital,People's Hospital of Mengyin,People's Hospital of Liaocheng,Henan Provincial People's Hospital,the Second Hospital of Hebei Medical University,First Affiliated Hospital of Zhejiang University were retrospectively analyzed.The clinical manifestation,features of imaging and laboratory examination were summarized,and the diagnosis,treatment and prognosis of the disease were investigated.Results HHHT patients had nonspecific symptoms in the early stage,and some patients presented with right upper quadrant discomfort,shortness of breath,anemia and liver bruit.The condition of HHHT patients could be worsened by liver cirrhosis or portal hypertension rapidly.The results of color doppler ultrasound and computed tomography showed intrahepatic telangiectasia,arteriovenous fistula and hepatic artery aneurysm in the 15 patients.Digital subtraction angiography was not clear enough for 2 HHHT patients with more than 1 enlarged hepatic arteries,but computed tomographic angiography was feasible.According to the degree and stages of the HHHT,all the 15 patients were divided into asymptomatic HHHT,simple HHHT and complex HHHT.Among the 6 patients who underwent surgical treatment,5 received ligation or banding of the enlarged hepatic arteries with subsequent disappearance of symptoms.Three patients received interventional treatment,and the treatment for 1 patient with complex HHHT was failed,and the patient died 30 months after medical treatment.Six patients were treated by conservative treatment,2 patients of them had no symptoms at the beginning,then they suffered from hepatic dysfunction and ascites at 21 and 35 months,respectively,and 1 of them died 6 months later.Four patients received medical treatment,and the results of color doppler ultrasound and computed tomography showed the pathological changes were aggravated gradually.Conclusions Telangiectasia,intrahepatic arteriovenous fistula and hepatic artery aneurysm are the main imaging characteristics of HHHT,and imaging diagnosis has significant value in the diagnosis of HHHT.HHHT is a progressive disease,early,active and individualized treatment is beneficial to the patients.The outcome of ligation or banding of the hepatic arteries is satisfactory.

Background and objective Gemcitabine and taxanes are effective agents commonly used in advanced squamous lung cancer. hTe best treatment sequence, however, is unclear to our knowledge. So we conducted this retrospective study in order to compare the effcacy and toxicities of ifrst-line Gemcitabine+/-platinum followed by second-line taxanes+/-platinum with the reverse sequence. Methods We totally analyzed 105 patients with stage IIIb-IV squamous lung cancer in our retrospective study. hTere were 49 patients receiving gemcitabine+/-platinum ifrst-line followed by taxanes+/-plati-num second-line (G-T group), and 56 patients receiving taxanes+/-platinum ifrst-line followed by gemcitabine+/-platinum second-line (T-G group). hTe primary endpoint of the study was overall survival (OS), and the secondary endpoints included progression-free survival (PFS), objective response rate (ORR), disease control rate (DCR) and toxicities. Results hTe me-dian OS were 18.5 mo in G-T group and 19.0 mo in T-G group (P=0.520). hTe median PFS1 was 5.0 mo and 4.0 mo with ifrst-line gemcitabine+/-platinum and taxanes+/-platinum, respectively (P=0.584). hTe median PFS2 was 2.7 mo and 2.5 mo with second-line gemcitabine+/-platinum and taxanes+/-platinum (P=0.432). hTe ORR1 of G-T group and T-G group were 36.73%and 33.92%(P=0.577), and DCR1 were 79.59%and 89.29%(P=0.186);the ORR2 of G-T group and T-G group were 4.08%and 5.36%(P=0.085), and DCR2 were 51.02%and 66.07%, respectively (P=0.118). Hematologic toxicities was more frequent in G-T group, the patients experienced more grade 3-4 lower hemoglobin (P=0.027) and thrombocytopenia (P=0.002). Conclusion hTe effcacy of ifrst line gemcitabine+/-platinum followed by second line taxanes+/-platinum and the reverse sequence was similar, and the toxicities was tolerable. Both sequential patterns were effective in advanced squamous lung cancer.

Objective:To investigate the clinical advantages of absorbable barbed wires in laparoscopy combined with choledochoscopy treatment of gallbladder polyps.Methods:The clinical data of 103 patients with gallbladder polyps who received laparoscopy combined with choledochoscopy treatment in The First People's Hospital of Lianyungang from February 2016 to March 2018 were retrospectively analyzed. Sixty-five patients in the observation group underwent gallbladder wall sutures with absorbable barbed wires, and thirty-eight patients in the control group underwent gallbladder wall sutures with common absorbable wires. The operative time, gallbladder wall suture time, intraoperative blood loss, average hospitalization time, and postoperative bile leakage were compared between the two groups.Results:There were no significant differences in intraoperative blood loss and average hospitalization time between the two groups ( P = 0.312, P = 0.114). In the observation group, gallbladder wall suture time and operative time were (5.58 ± 1.14) minutes and (60.71 ± 11.03) minutes, respectively, which were shorter than (6.32 ± 1.04) minutes and (68.24 ± 9.61) minutes in the control group ( t = 3.23, 3.50, P = 0.002, 0.001). No bile leakage occurred in the observation group and bile leakage occurred in four (10.5%) patients in the control group. There was a significant difference in bile leakage between the observation and control groups ( χ2 = 4.57, P = 0.032). Conclusion:Absorbable barbed wires for gallbladder wall sutures during operation in laparoscopy combined with choledochoscopy treatment of gallbladder polyps is safe and feasible. It can markedly shorten gallbladder wall suture time and operative time, decrease the incidence of bile leakage, and has a clinical advantage over common absorbable wires.