Objective To compare the clinical efficacy of three different therapies,including transjugular intrahepatic portosystemic shunt(TIPS)treatment,endoscopic treatment and medication treatment,combined with transhepatic arterial chemoembolization(TACE)in treating primary liver cancer complicated by portal hypertension and upper gastrointestinal bleeding.Methods A total of 105 patients with primary liver cancer associated with portal hypertension and upper gastrointestinal bleeding,who were admitted to the No.980 Hospital of PLA Joint Logistics Support Forces of China to receive treatment between January 2014 and June 2020,were enrolled in this study.According to the therapeutic scheme,the patients were divided into TIPS+TACE group(TIPS group,n=25),endoscopy+TACE group(endoscopy group,n=30),and medication+TACE group(medication group,n=50).The clinical efficacy,recurrence rate of bleeding,incidence of hepatic encephalopathy,and survival rate were compared between each other among the three groups.Results The differences in the postoperative 6-month,12-month and 24-month recurrence rates of bleeding between each other among the three groups were statistically significant(all P＜0.05).In TIPS group,the portal vein pressure decreased from preoperative(38.47±9.35)mmHg(1 mmHg=0.133 kPa)to postoperative(25.24±5.68)mmHg,the difference was statistically significant(P＜0.05).After treatment,the hemoglobin level in the three groups showed varying degrees of elevation,which in the TIPS group and endoscopy group were better than that in the medication group,the differences were statistically significant(P＜0.05).In all three groups,the differences in the recurrence rate of bleeding between postoperative 6-month value,12-month value and 24-month value were statistically significant(all P＜0.05).The postoperative 6-month,12-month and 24-month recurrence rates of bleeding in the TIPS group were lower than those in the endoscopy group and the medication group,and the differences were statistically significant(P＜0.05).The postoperative 12-month and 24-month recurrence rates of bleeding in the TIPS group were lower than those in the endoscopy group,and the differences were statistically significant(P＜0.05).The postoperative 12-month and 24-month recurrence rates of bleeding in the endoscopy group were lower than those in the medication group(P＜0.05),and the difference in the postoperative 6-month recurrence rate of bleeding between the two groups was not statistically significant(P＞0.05).The postoperative 6-month and 12-month incidences of hepatic encephalopathy in the TIPS group were higher than those in the endoscopy group and the medication group,the differences were statistically significant(P＜0.05),while the differences in the postoperative 6-month and 12-month incidences of hepatic encephalopathy between the endoscopy group and the medication group were not statistically significant(P＞0.05),and the differences in the postoperative 24-month incidence of hepatic encephalopathy between each other among the three groups were not statistically significant(P＞0.05).No statistically significant difference in the 6-month mortality existed between TIPS group and endoscopy group(P＞0.05),and the 6-month mortality of both TIPS group and endoscopy group was remarkably lower than that of the medication group(P＜0.05).The postoperative 12-month mortality and 24-month mortality in TIPS group were lower than those in the endoscopy group and the medication group,and the differences were statistically significant(P＜0.05),but the differences in the postoperative 12-month mortality and 24-month mortality between the endoscopy group and the medication group were not statistically significant(P＞0.05).Conclusion For primary liver cancer associated with portal hypertension and upper gastrointestinal bleeding,TIPS combined with TACE can effectively control tumor progression and prolong survival.(J Intervent Radiol,2024,32:33-37)

OBJECTIVE: To explore the clinical and genetic characteristics of a patient with hypertrophic cardiomyopathy as the initial manifestation of Mucopolysaccharidosis type Ⅲ A (MPS Ⅲ A). METHODS: A female patient with MPS Ⅲ A who was admitted to the Affiliated Hospital of Jining Medical University in January 2022 and her family members (seven individuals from three generations) were selected as the study subjects. Clinical data of the proband were collected. Peripheral blood samples of the proband was collected and subjected to whole exome sequencing. Candidate variants were verified by Sanger sequencing. Heparan-N-sulfatase activity was determined for the disease associated with the variant site. RESULTS: The proband was a 49-year-old woman, for whom cardiac MRI has revealed significant thickening (up to 20 mm) of left ventricular wall and delayed gadolinium enhancement at the apical myocardium. Genetic testing revealed that she has harbored compound heterozygous variants in exon 17 of the SGSH gene, namely c.545G>A (p.Arg182His) and c.703G>A (p.Asp235Asn). Based on guidelines from the American College of Medical Genetics and Genomics (ACMG), both variants were predicted to be pathogenic (PM2_Supporting +PM3+PP1Strong+PP3+PP4; PS3+PM1+PM2_Supporting +PM3+PP3+PP4). Sanger sequencing confirmed that her mother was heterozygous for the c.545G>A (p.Arg182His) variant, whilst her father, sisters and her son were heterozygous for the c.703G>A (p.Asp235Asn) variant. Determination of blood leukocyte heparan-N-sulfatase activity suggested that the patient had a low level of 1.6 nmol/(g·h), whilst that of her father, elder and younger sisters and son were all in the normal range. CONCLUSION The compound heterozygous variants of the SGSH gene probably underlay the MPS ⅢA in this patient, for which hypertrophic cardiomyopathy is an associated phenotype.
Female ; Humans ; Cardiomyopathy, Hypertrophic ; Contrast Media ; East Asian People ; Gadolinium ; Mucopolysaccharidosis III ; Mutation ; Pedigree ; Male ; Middle Aged

Objective To investigate the changes of component and morphology in internal carotid vulnerable plaque,for helping to make clinical intervention strategy individually. Methods A total of 47 patients with internal carotid vulnerable plaques and primary hypertension underwent 2 high-resolution and multi-contrast MRI scans, from March 2008 to April 2014 were retrospectively reviewed. At baseline, the plaque was mainly located at the proximal internal carotid artery,and maximum plaque thickness ≥1.5 mm with intraplaque hemorrhage(IPH)and(or)thin or ruptured fibrous cap.Interscan interval was 0.5 years and above. Patients with carotid occlusion or surgery were excluded. Morphological measurements included maximum plaque thickness, maximum plaque area and cross-sectional vessel area (CSVA) on the level of plaque with maximum thickness. The paired-samples t test was performed to compare the difference of plaque morphology between baseline and follow-up carotid MRI.Results The interscan interval was 1.83 (1.59,1.99)years for 47 internal carotid vulnerable plaques.One case(interscan interval 2.16 years)showed IPH within those 11 plaques without IPH at baseline,and one case(interscan interval 1.42 years)had new incident IPH within those 36 plaques with IPH at baseline. Maximum plaque thickness increased significantly from(3.94±1.44)mm to(4.24±1.68)mm(t=2.30,P<0.05)by 5.14%(-3.83,11.34)% per year. Maximum plaque area increased significantly from(49.19±21.15)mm2to(56.03±24.91)mm2(t=3.87,P<0.01)by 6.67%(-2.26,19.60)% per year.CSVA increased significantly from(66.22±27.51)mm2to(73.68±31.47)mm2(t=4.08,P<0.01)by 5.18%(-1.63,12.34)% per year.Conclusion The progression of component,burden and outer remodeling in the internal carotid vulnerable plaque may be faster in hypertension, therefore reasonable intervention strategy and regular follow-up carotid MRI should be performed.

Objective To assess the different methods and their outcomes of interventional therapy for stenosis or occlusion in superior vena cava and its branches. Methods Sixty patients with stenosis or occlusion of SVC and its branches were retrospectively analyzed after interventional therapy. Among them, 38 were males and 22 were females, with age range from 15 to 72 years old(mean age 58). Seventeen patients were treated by thrombolysis, and the rest 43 patients accepted percutaneous angioplasty and stenting. Before and after that, the pressures within the vein were measured at the inflow side. The paired-t test was used for statistical analysis. Results After treatment, the pressure at the inflow side dropped from (24.8±2.3)mm Hg to (7.1±1.5)mm Hg(1 mm Hg=0.133 kPa), with a significant difference(t=3.232,P＜0.01). The clinical outcomes included complete relief in 27 patients, partial relief in 28 patients and non-relief in 5 patients. No major complications occurred. During 6 months follow up, restenosis occurred in 10 patients, among whom 6 received repeat intervention with good results. The other 4 patients turned to surgery .Conclusion Interventional therapy for stenosis or occlusion in SVC and its branches could recanalize the vessels, restore the blood flow and relief the clinical symptoms.

Objective To study the method and effectiveness of interventional therapy for the stenotic or obstructive inferior vena cava(IVC) caused by hepatic cancer. Methods All together 6 patients were enroled, 4 males and 2 females, with age of 45 to 58 years, average 53 years. The main symptoms including abdominal distention, varices on abdominal walls and edema in lower extremities were caused by compression or involvement of IVC with right hepatic lobe lesion. We performed thrombolysis first and follower by balloon dilation and stent placement. Results IVC were recanalized in all 6 patients with free patent blood flow. The postal venous pressure dropped from 4.4 kPa to 2.1 kPa without major complications. Conclusions Treating the IVC stenosis and obstruction caused by primary liver cancer with interventional method is safe and effective together with improvement of life quality and prolongation of survival time. (J Intervent Radiol, 2006, 15: 537-538)

Objective To evaluate the efficacy and safety of interventional therapy for left iliac vein compression syndrome (LICS). Methods A total of 27 patients with LICS that were diagnosed by colour Doppler or venography,were included in this study. Among the cases,the disease course was shorter than 3 weeks in 13 patients,who presented with acute iliac-femoral vein thrombosis; in the other 14 patients,the disease course was longer than 3 weeks,showing the symptoms of chronic venous thrombosis. Venous puncture was performed via the contralateral femoral vein in 13 cases,and through the ipsilateral popliteal vein in 14. Venous filter was placed in 11 cases before thrombolysis,while in the other 16 cases,no filter was used. All of the patients received catheter-directed thrombolysis,afterwards,7 of them underwent percutaneous transluminal angioplasty,and the other 20 received angioplasty plus stent placement. Results In the 13 patients,who received venous puncture via the contralateral femoral approach,the guide wire could advance through the stenosis successfully in 8 patients but failed in 5. Ipsilateral popliteal vein puncture were then employed and succeeded in all the patients. Ipsilateral popliteal puncture were performed successfully in 14 patients. In 11 patients,inferior vena cava filter was placed before the procedure. In this series,the thrombolysis time was (85?16) h with a dose of (3000?320) thousand units urokinase. The thrombosis was dissolved completely in 13 acute patients and partial dissolution was attained in 14 patients in whom blood flow signal were detected color Doppler. After the operation,venogram showed complete patent iliac veins in all of the 27 cases. The patients were followed up for 6 to 26 months (mean,11 months). During the period,19 patients obtained patent iliac-femoral vein with the symptoms disappeared; 8 patients met the standard of improvement. Conclusions Interventional technique can be the first-line treatment for left iliac vein compression syndrome because of its favorable result and minimal invasion.

Objective To explore the imaging diagnostic method of Merkel cell carcinoma(MCC). Methods Imaging and pathologic features of MCC of 1 female and 44 years old patient were determinded by CT, MRI, pathologic examination and operative observation. Results MCC is a rare subepidermal carcinoma, and most located in the head, neck region and extrimities. Clinically, only a presumptive diagnosis of MCC can be established. The definite diagnosis of MCC can be made only by pathologic examination, especially immunohistological method. MCC is red and brown, and looks like rotten meat. MCC texture is middle with rich blood supply. MCC can invade occipital bone, occipital muscles, dura and cerebellar tissues. Optical microscope observation showed that MCC is located at dermis, and sometimes at subcutaneous tissues. MCC cells are gathered in groups, their sizes are unanimous, and their shapes look like lymphocytes. The cell nuclei of MCC looklike empty bubbles, and have many karyokinesis. The chromatin of MCC is very fine. The cytoplasms of MCC are little, and oxyphil staining. Immunohistological staining showed that both NSE (neuron-specific enolase) and CK(cytokeratin) are positive. The CT imaging of MCC is higher density. The MRI imaging of MCC is lower signals with unclear border in T1w and T2w, but becomes clear in enhanced MRI. Tumor cells invasion to soft tissues and bones could be seen on CT or MRI. Conclusion CT and MRI could clearly show the characteristics of MCC, and provide valuable information for operative treatment of MCC.

Objective To evaluate the effect of transarterial chemoembolization (TACE) before operation, including side effects and the improvment for the suceesfult resection rate.Methods 56 patients with cardiac cancer were divided into two groups: TACE group and resection group. The former included 25 patients (male 14, female 11) undertaken TACE 7-15 days before surgery. The image manifestations, histological changes and side effects together with the time of operation, volume of blood lose during operation and the succesful resection rate were compared with those of the latter. Results 1. TACE induced the necrosis of tumor tissue to some extent and possessed the power to localize the lesion with little side effects. 2. TACE before resection did not only minimize the injury of operation but also improved the resection rate. Conclusion TACE is a safe, effective modality before operation and is worthy for recommendation

Purpose:To eveluate the effect of liver function after TIPSS.Materials and methds:Ananalysis of 17 cases with laboratory data to study the liver function changes after TIPSS (transjugular intrahepatic portosystemic stent shunts).Results:There were 35 percent of cases whose liver function were worse than before,the others showed no change or better after TIPSS. Couclusion:It was important to choose the cases strictly before TIPSS so as to reduce the compli- cation and improve the survival rates.Although some cases liver function went worse after TIPSS but only temporarily.TIPSS can reduce bleeding retes effectively,thus simultaneously seve the lives.It is worthy to extend this procedue.