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Article in Chinese | WPRIM | ID: wpr-455366


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.

Article in Chinese | WPRIM | ID: wpr-430642


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.