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1.
Int. j. cardiovasc. sci. (Impr.) ; 36: e20210040, 2023. tab, graf
Article in English | LILACS-Express | LILACS | ID: biblio-1421770

ABSTRACT

Abstract Takayasu arteritis (TA) is a rare type of vasculitis that affects mainly the aorta and its major branches. It is highly similar to giant cell arteritis (GCA), and differentiation between them may not be achieved even by histological examination. Arterial hypertension is typical of TA and is caused by stenosis of the renal arteries. Here we report the case of a 59-year-old woman, with a history of dyslipidemia and anemia, seen in the Internal Medicine department for resistant hypertension. Evaluation of secondary causes led to stenosis of the renal arteries. Assessment of target organ involvement was performed by computed tomography angiograph which revealed ectasia of the aortic arch and ascending aorta, tortuous course of the brachiocephalic trunk and the proximal portion of the right common carotid artery; positron-emission tomography which showed diffuse increased uptake in the ascending aorta, compatible with large vessels vasculitis. The patient was submitted to aortic valve replacement with a biological prosthesis combined with myocardial revascularization (Bentall-De Bono procedure). Aortic biopsy specimens showed anatomical and pathological features of GCA and TA. Due to persistently uncontrolled hypertension, prednisone 60 mg was initiated,with significant improvement in patient's condition.

2.
Chinese Journal of General Practitioners ; (6): 728-731, 2023.
Article in Chinese | WPRIM | ID: wpr-994761

ABSTRACT

Clinical data of 15 patients diagnosed with acute renal infarction (ARI) in Affiliated Zhongshan Hospital of Dalian University from Jan 2011 to Dec 2021 were retrospectively analyzed. Of the included 15 patients, there were 14 cases of cardiac origin and 1 case of antiphospholipid syndrome. We found that there were 12 cases of atrial fibrillation, 2 cases of atrial premature beats, 12 cases of elevated level of D-dimer, 15 cases of elevated level of LDH, 11 cases of positive urine occult blood and positive urine protein. Among the 15 patients, catheter-directed thrombolysis was performed in 4 cases, of which 3 cases were revascularized successfully, intravenous thrombolysis in 2 cases and alone anticoagulation therapy in 9 cases. It is suggested that CECT or CTA can assist the early diagnosis of ARI especially in patients with acute onset and persistent abdominal pain with high risk factors of thromboembolism, high levels of LDH, microscopic hematuria and/or proteinuria. Despite prolonged embolic ischemia, try to reconstruct blood flow to save the kidney as much as possible. Late standardized anticoagulant therapy is of critical importance to prevent recurrent embolic episodes.

3.
Chinese Journal of General Practitioners ; (6): 1288-1294, 2021.
Article in Chinese | WPRIM | ID: wpr-911765

ABSTRACT

Objective:To investigate the diagnostic value of contrast-enhanced ultrasonography (CEUS) in evaluating the severity of renal artery stenosis.Methods:Eighty six patients with suspected renal artery stenosis admitted to Shanxi Provincial People′s Hospital from January 2018 to July 2021 were enrolled in the study. All patients underwent digital subtraction angiography (DSA), Doppler ultrasonography (DUS) and CEUS examinations. With DSA results as gold standard the sensitivity, specificity, accuracy, positive predictive value (PPV) and negative predictive value (NPV) of CEUS and DUS in the diagnosis of renal artery stenosis were analyzed. The consistency between CEUS and DSA in the diagnosis of renal artery stenosis was evaluated. The accuracy of DUS and CEUS in diagnosis of renal artery stenosis was assessed by the area under receiver operating characteristic (ROC) curve (AUC) and compared between groups.Results:Among 153 renal arteries from 86 patients examined by DSA, 101 showed the stenosis ≥30% (49 left and 52 right renal arteries), and there were 31, 45, 20 and 5 renal arteries with grade I, II, III and IV stenosis. The sensitivity and specificity of DUS in the diagnosis of renal artery stenosis were 74.3% (75/101) and 76.9% (40/52),respectively. DUS showed 21, 42, 20 and 4 renal arteries with grade Ⅰ, Ⅱ, Ⅲ and Ⅳ stenosis, respectively. The sensitivity and specificity of CEUS in the diagnosis of renal artery stenosis were 88.1% (89/101) and 86.5%(45/52), respectively. CEUS showed 26, 43, 23 and 4 renal arteries with grade Ⅰ, Ⅱ, Ⅲ and Ⅳ stenosis, respectively. There was a significant difference between DUS and DSA in grading of renal artery stenosis (χ2=4.447, P=0.03),and there was a moderate consistency between DUS and DSA ( Kappa=0.480). There was no significant difference between CEUS and DSA in grading renal artery stenosis (χ2=0.842, P=0.36) with a good consistency ( Kappa=0.730). The AUC of CEUS in diagnosis of renal artery stenosis was higher than that of DUS [0.873 (95% CI: 0.808-0.938) vs. 0.756 (95% CI:0.673-0.839); Z=4.361, P<0.01]. Conclusion:The study suggests that contrast-enhanced ultrasonography may be used as a diagnostic method for grading renal artery stenosis.

4.
Chinese Journal of Geriatrics ; (12): 836-841, 2021.
Article in Chinese | WPRIM | ID: wpr-910925

ABSTRACT

Objective:To establish and validate a prognostic model of a contrast-enhanced ultrasound scoring(CEUS)system for evaluating renal artery stenosis(RAS)in the elderly.Methods:This was a single-center retrospective study.A total of 324 elderly RAS patients admitted to Beijing Hospital from October 2017 to July 2020 were randomly assigned into the model group(n=174)and the validation group(150)in a 1∶1 ratio.Clinical and imaging data of patients on admission including general conditions, previous medical history, blood pressure, blood creatinine, renal artery stenosis and cortical blood perfusion in the affected kidney and renal function(GFR)at 1-year follow-up were collected.Univariate and multivariate logistic regression was used to establish a model of the CEUS scoring system.The receiver operating characteristic(ROC)curve and area under the ROC curve(AUC)were used to evaluate prediction accuracy.Clinical application value of the CEUS scoring system model was evaluated via decision curve analysis using a nomogram.Results:Baseline clinical and radiomic data had no significant difference between the model group and the validation group( P>0.05). Multivariate logistic regression analysis results showed that age( OR=1.242, 95% CI: 1.081-1.427, P<0.01), diabetes( OR=1.545, 95% CI: 1.107-2.156, P<0.05), blood pressure( OR=1.328, 95% CI: 1.056-1.670, P<0.05), renal function( OR=2.374, 95% CI: 1.216-3.887, P<0.01)and cortical blood perfusion parameter( OR=2.646, 95% CI: 1.553-6.369, P<0.01)were risk factors for the deterioration of renal function during 1 year follow-up.Based on these results, a nomogram for the CEUS scoring system model was drawn, and its consistency index, the C-Index, was 0.725(95% CI: 0.653-0.776). The AUC of the CEUS scoring system was 0.824 and the Youden index was 0.711 in the model group, with a specificity of 0.774 and a sensitivity of 0.837.The AUC of the CEUS scoring system was 0.853 and the Youden index was 0.715 in the validation group, with a specificity of 0.684 and a sensitivity of 0.889.There was no significant difference in ROC curve between the two groups( D=1.387, P>0.05). In addition, calibration charts of the two models showed that the calibration curve of the CEUS scoring system was close to the standard curve, with no statistically significant difference( P>0.05). Conclusions:The CEUS scoring system model can be used to predict the risk of worsening renal function in elderly RAS patients during 1-year follow-up.

5.
Chinese Journal of General Surgery ; (12): 38-41, 2020.
Article in Chinese | WPRIM | ID: wpr-870411

ABSTRACT

Objective To investigate the safety and mid-term efficacy of percutaneous endovascular angioplasty(PTA) and stent implantation for transplantation renal arterial stenosis (TRAS).Methods Retrospective analysis was performed on 18 patients with TRAS admitted to of department vascular surgery,PLA General Hospital from Jan 2011 to Dec 2018.Results PTA and stent implantation were performed in all 18 patients via ipsilateral or contralateral femoral artery including 4 cases of PTA alone,8 cases of PTA plus stent,6 cases of stent implanted directly.Three of the 18 patients underwent ipsilateral femoral artery catheterization and 15 underwent contralaterally.A total of 14 stents were implanted,all of were balloon expanding stents,of which 2 were drug-coated stents,and the technical success rate was 100%.The average dosage of contrast agent was 64 ml,the stenosis rate of renal artery before interventional treatment was 50%-99%,and that after interventional treatment reduced to 10%-30%.The systolic blood pressure decreased from (157.2 ± 43.0) mmHg preoperatively to (129.8 ± 8.6) mmHg postoperatively.The SCr level decreased from (258.8 ± 214.7) μ mol/L to (176.3 ± 101.1) μmol/L.Preoperative urea nitrogen decreased from (15.7 ± 1.6) mmol/L to (10.6 ± 1.1) mmol/L postoperatively (P < 0.05).Mean postoperative follow-up time was 42.4 months (3-93 months).17 cases were cured,1 case was ineffective,1 case suffered restenosis after 30 days,and was given remedy PTA plus stenting.Conclusions TRAS is a vascular factor leading to grafted renal failure,the endovascular treatment of TRAS is safe,effective and has good mid-term result.

6.
Chinese Journal of Interventional Imaging and Therapy ; (12): 46-51, 2019.
Article in Chinese | WPRIM | ID: wpr-862189

ABSTRACT

Objective" To investigate the value of balanced steady-state free precession (B-SSFP) MRA for diagnosing renal artery stenosis (RAS) with Meta-analysis. Methods PubMed, EMbase and Cochrane Library databases were searched. English studies assessed B-SSFP MRA diagnostic performance in RAS patients were reviewed. Quality assessment was conducted by adopting the Quality Assessment of Diagnostic Accuracy Studies-2 checklist. Statistical analysis was adopted using the software of Meta Disc 1.4 and Stata 12.0. The summary ROC (SROC) curve was drawn, and pooled AUC, sensitivity, specificity, diagnostic odds ratio were calculated. Results A total of 12 articles including 21 results were enrolled, which had highly heterogeneous by using random effects models. In B-SSFP diagnosing RAS, meta-analysis showed the pooled sensitivity was 0.85 (95%CI [0.81, 0.88], I2=56.8%) and pooled specificity was 0.96 (95% CI [0.95, 0.97], I2=85.3%), the area under the SROC curve was 0.964 4, the pooled diagnostic odds ratio was 137.94 (95%CI [84.75, 224.50]). Regression analysis showed that heterogeneity may be derived from the year of publication (P=0.038). Conclusion: B-SSFP MRA is accurate and reliable for diagnosing RAS, and may be the first choice in patients with renal insufficiency.

7.
Chinese Journal of Cardiology ; (12): 628-633, 2019.
Article in Chinese | WPRIM | ID: wpr-805710

ABSTRACT

Objective@#To evaluate the characteristics of renal cortical blood perfusion assessed by contrast-enhanced ultrasound (CEUS) in elderly patients with renal artery stenosis (RAS) and its relationship with renal function.@*Methods@#Ninety-three elderly patients diagnosed with RAS, who were admitted in Beijing Hospital during June 2017 and December 2018, were retrospectively enrolled. According to the degree of RAS, 186 renal arteries were divided into normal renal artery group (n=79), mild RAS group (30% to 49%, n=59), moderate RAS group (50% to 70%, n=33), and severe RAS group (70% to 99%, n=15). Renal cortical blood perfusion and renal glomerular filtration rate (GFR) were measured by CEUS and radionuclide renal dynamic imaging. According to the renal GFR, 186 kidneys were divided into normal renal function group (GFR≥35 ml/min, n=42) and mild renal insufficiency group (35 ml/min>GFR≥25 ml/min, n=51), moderate renal insufficiency group (25 ml/min>GFR≥15 ml/min, n=75) and severe renal insufficiency group (GFR<15 ml/min, n=18). The renal cortical blood perfusion time-intensity curve (TIC) and related parameters were analyzed, including the area under the curve (AUC), the slope of the ascending branch (A), the peak intensity (PI), the peak time (TTP) and the mean transit time (MTT), the kidneys of different RAS groups and patients with different renal function groups were analyzed. Pearson correlation analysis was used to evaluate the correlation between renal cortical blood perfusion parameters and renal GFR.@*Results@#(1) Renal cortical blood perfusion and GFR: CEUS showed that parameter A of TIC was significantly reduced, while TTP was prolonged in the mild renal artery stenosis group compared with the normal renal artery group (both P<0.05), GFP was similar between the two groups. Cortical perfusion parameters, such as AUC, A, PI and GFR were significantly lower, while TTP and MTT were significantly prolonged in the moderate and severe renal artery stenosis group than in the normal and mild stenosis groups (all P<0.05). Compared with the moderate stenosis group, AUC, A, PI and GFR were significantly lower while TTP, MTT were significantly prolonged in the severe renal artery stenosis group (all P<0.05). (2) TIC showed that the renal perfusion parameters, AUC, PI and A were significantly lower, while TTP was significantly longer in the mild renal dysfunction group than in the normal renal function group (all P<0.001). The changes aggravated in proportion with renal dysfunction. (3) Correlation between perfusion parameters and GFR: Pearson correlation analysis showed that the AUC (r=0.774, P<0.05), A (r=0.815, P<0.05) and PI (r=0.772, P<0.05) were positively correlated with GFR; serum creatinine level (r=-0.841, P<0.05), renal function grading (r=-0.731, P<0.05), TTP (r=-0.803, P<0.05) and MTT (r=-0.741, P<0.05) were negative correlated with GFR. The degree of stenosis was negatively correlated with GFR (r=-0.427, P<0.05).@*Conclusion@#Cortical perfusion parameters differ significantly among patients with various degree of RAS and renal dysfunction. The renal cortical blood perfusion parameters are correlated with renal GFR.

8.
Chinese Journal of Geriatrics ; (12): 565-568, 2019.
Article in Chinese | WPRIM | ID: wpr-745559

ABSTRACT

Objective To investigate the renal cortical perfusion parameters assessed by contrast-enhanced ultrasound(CEUS)in elderly patients with moderate-to severe renal artery stenosis (RAS),and relationship of those with glomerular filtration rate(GFR).Methods A total of 59 elderly patients with unilateral moderate to severe RAS(50%-99%)admitted into Beijing Hospital during June 2017 to December 2018 were consecutively enrolled in the cross sectional and self-control study.Based on the GFR assessed by radionuclide renal dynamic imaging,patients were divided into the mild-(35>GFR≥25 ml/min,n=18),moderate-(25 > GFR≥ 15 ml/min,n =37) and severe renal dysfunction(GFR< 15 ml · min-1,n =4) groups.CEUS evaluated-renal cortical perfusion parameters,including area under curve (AUC),ascending slope (A),derived peak intensity (DPI),time to peak (TTP) and mean transit time(MTT),were compared among different levels of severity of renal dysfunction groups.Pearson correlation analysis was used to evaluate the relationship between CEUS imaging-evaluated renal cortical perfusion parameters and radionuclide renal dynamic imaging-assessed GFR.Results Compared with the mild renal dysfunction group,the moderate renal dysfunction group showed a reduced AUC and A(P<0.05),and the severe renal dysfunction group showed more markedly reduced AUC,A,DPI,TTP and MTT (P < 0.05).Compared with the moderate renal dysfunction group,the severe renal dysfunction group showed a reduced AUC,A and GFR(P <0.05),while no significant differences in TTP and MTT were found.As far as relationship between renal cortical perfusion parameters and GFR were concerned,Pearson correlation analysis demonstrated that AUC(r =0.804),A(r =0.813) and DPI(r =0.733) were significantly and positively correlated with GFR(all P < 0.05).However,renal function (r =-0.721),TTP(r =-0.744) and MTT(r =-0.743)were negatively correlated with GFR(P<0.05).And the degree of renal artery stenosis was poorly correlated with GFR (r =-0.462).Conclusions For elderly patients with unilateral moderate to severe RAS,contrast-enhanced ultrasonography can display the changes of renal cortical blood perfusion parameters along with the aggravation of renal function impairment.AUC and A are strongly positively correlated with GFR.The degree of renal artery stenosis is poorly correlated with renal function.And more studies are needed to confirm these findings.

9.
Chinese Journal of Ultrasonography ; (12): 953-957, 2018.
Article in Chinese | WPRIM | ID: wpr-707751

ABSTRACT

Objective To explore the value of color Doppler ultrasonography in diagnosis of transplant renal artery stenosis( TRAS) of children . Methods The results of ultrasound examination and clinical data of 82 kidney transplant children with suspected TRAS were retrospectively analyzed . According to the results of the CTA ,the enrolled children were divided into a stenosis group with 21 cases and a non-stenosis group with 61 cases . The differences of ultrasound hemodynamic parameters between the two groups were compared . The hemodynamic parameters and cutoff values of prediction of TRAS were determined according to the ROC curve ,and the corresponding sensitivity and specificity were calculated . Logistic regression was used to screen TRAS independent predictors . Results Compared with the non-stenosis group ,the peak systolic velocity ( PSV ) of the renal aorta ,the renal aorta PSV/interlobular artery PSV ( post PSV ratio ) and acceleration time ( AT ) of interlobular artery in the stenosis group were significantly higher ,but the PSV of the interlobular artery was lower ( all P < 0 .05 ) . Differences of indicators between the two groups can be used to predict TRAS . Through the ROC curve the peak systolic velocity of the renal aorta and the interlobular artery were 218 .5 cm/s and 25 .5 cm/s ,respectively ,the post PSV ratio was 7 .2 ,and the AT of intervein arterial was 67 .5 ms ,the sensitivity and specificity were the highest ,which could be used as the ultrasound screening threshold . The PSV of the interlobular artery can be used as an independent predictor of TRAS . Conclusions Analysis of hemodyamic parameters of transplanted kidney arteries by Doppler ultrasonography might be useful in predicting TRAS in children .

10.
Journal of Chinese Physician ; (12): 1456-1460, 2018.
Article in Chinese | WPRIM | ID: wpr-706011

ABSTRACT

Objective To observe the therapeutic effects and adverse effect of different lipid-lowering regimens in Atherosclerosis renal artery stenosis (ARAS) rat model and select the optimal treatment.Methods 90 Wister rats were randomly divided into sham operation group (group F) and operation group.The operation group was divided into normal feed group (group N) and high-fat feed group (group T).We established the rat model in operatino group by narrowing the renal artery with acupuncture needle and 8 weeks of high-fat feeding.According to the different lipid-lowing therapeutic schedules,the T group rats were randomly divided into five groups:the blank group (T0 group),the Xuezhikang group (T1 group),the rosuvastatin group (T2 group),the regular dose of rosuvastatin combined with Xuezhikang (T3 group),the low dose of rosuvastatin combined with Xuezhikang (T4 group).Mter 6 weeks of intervention,the serum lipids [total cholesterol (TC),triglyceride (TG),high density lipoprotein-cholesterol (HDL-C),low density lipoproteincholesterol (LDL-C)],liver function [alanine aminotransferase (ALT),aspartate aminotransferase (AST)],kidney function [blood urea nitrogen (BUN),serum creatinine (Scr)] and pathological changes of aorta and kidney were detected before and after treatment.Results (1) The blood pressure,BUN and Scr were significantly increased in N group and T group compared with F group (P < 0.05).TC,TG,LDL-C were significantly increased (P < 0.05) and HDL-C were significantly decreased (P < 0.05) in T group compared with N,T group.According to the pathological section,the aorta in T group had visible atherosclerosis change,the renal tubules were atrophied and decreased.All above,the rat model of ARAS was successfully established.(2) After 6 weeks of drug intervention,compared with prior treatment,TC,TG,LDL-C,BUN and Scr were significant decrease (P < 0.05),while HDL-C was significantly increased (P < 0.05) in all treatment groups,and the most remarkable change was in T3,T4 group,T3 group was better than T4 group but with no significant difference between T3 and T4 group (P > 0.05).We found that ALT and AST were significantly increased in T2 and T3 group (P < 0.05) after treatment.Compared with T0 group,pathological lesions of aorta and kidney were reduced best in T3 and T4 group.Conclusions Low dose of rosuvastatin combined with Xuezhikang can achieve ideal lipid-lowing effects.At the same time,it has a protective effect on kidney,and has little damage to liver function.

11.
Chinese Journal of Interventional Imaging and Therapy ; (12): 217-220, 2018.
Article in Chinese | WPRIM | ID: wpr-702395

ABSTRACT

Objective To investigate the efficacy and safty of percutaneous transluminal angioplasty (PTA) of renal artery stenosis resulting from fibromuscular dysplasia.Methods A total of 32 patients with fibromuscular dysplasia complicated with hypertension in level 2 to 3 or refractory hypertension were enrolled,who underwent PTA.The blood pressure,types of medication,restenosis,survival rates and complications were observed after treatment.Results Technical success rate of PTA was 93.94% (31/33).The blood pressure decreased from (189.6±26.0)mmHg/(121.6± 21.7)mmHg before operation to (136.6±8.0)mmHg/(81.1±11.5)mmHg after operation (t=9.117,7.745,both P< 0.001).No renal artery rupture,interlayer,branch blockage,thrombosis and other related complications and postoperative adverse events occurred.All patients were followed up for 5 months to 100 months,with an average of (40.4±26.1) months.Survival rate was 100% (32/32).Conclusion PTA is safe and effective for treating renal artery stenosis from fibromuscular dysplasia.

12.
Journal of the Korean Child Neurology Society ; (4): 113-118, 2018.
Article in English | WPRIM | ID: wpr-728854

ABSTRACT

Posterior reversible encephalopathy syndrome (PRES, or posterior leukoencephalopahty syndrome) is a neurological condition caused by reversible cortical/subcortical vasogenic brain edema secondary to hypertension, cytotoxic drugs, immunosuppressants, autoimmune diseases, renal disease, eclampsia or pre-eclampsia. It is characterized by acute neurological symptoms such as headache, seizures, visual disturbances, and impaired levels of consciousness. Brain imaging usually reveals bilateral, cortical/subcortical vasogenic edema. Completely unilateral PRES constituted only 2.6% of the cases in a previous study. Here we report the case of a pediatric patient with completely unilateral PRES. A 13-year-old boy was admitted with acute gastroenteritis. On the fourth day of hospitalization, he started to complain of headache and vomiting. He then developed generalized tonic-clonic seizure 3 times. His blood pressure was 180/121 mmHg during the first seizure, 188/112 mmHg during the second seizure and 152/92 mmHg during the third seizure. T2-weighted imaging with fluid attenuation by inversion recovery (T2 FLAIR) demonstrated high-signal intensity in the cortical gyri of the left frontal, parietal, and occipital lobes. Follow-up magnetic resonance imaging (MRI) was performed 2 weeks after the seizure onset, which indicated a significant improvement in the patient's condition. Abdominal pelvic computed tomography (CT) and renal CT angiography showed abnormal narrowing of the left renal artery. In summary, we present a case report of unilateral PRES secondary to renovascular hypertension due to left renal arterial obstruction.


Subject(s)
Adolescent , Female , Humans , Male , Pregnancy , Angiography , Autoimmune Diseases , Blood Pressure , Brain Edema , Consciousness , Eclampsia , Edema , Follow-Up Studies , Gastroenteritis , Headache , Hospitalization , Hypertension , Hypertension, Renovascular , Immunosuppressive Agents , Magnetic Resonance Imaging , Neuroimaging , Occipital Lobe , Posterior Leukoencephalopathy Syndrome , Pre-Eclampsia , Rabeprazole , Renal Artery Obstruction , Renal Artery , Seizures , Vomiting
13.
Chinese Journal of Nephrology ; (12): 831-837, 2017.
Article in Chinese | WPRIM | ID: wpr-666292

ABSTRACT

Objective To summarize the clinical features of 9 cases with mutations in PKHD1 gene for a better understanding of its phenotype.Methods Clinical data of nine cases with mutations in PKHD1 gene were summarized from January 2011 to December 2016 in our center,including clinical manifestations,laboratory findings,imaging data and family investigation.Next generation sequencing was used to screen 4000 genes in case 1 to 4 and whole exons in case 5 to 9.Significant variants detected by next generation sequencing were confirmed by conventional Sanger sequencing.Segregation analysis was performed using parental DNA samples.Relevant literature was reviewed.Results Among these 9 cases,5 are male,4 are female.The average age of onset was 2.6 years old (ranging from 0.5-5.2 years).Renal ultrasound revealed that all 9 cases had cysts in bilateral kidney,7 cases with enlarged kidney,1 case with normal size kidney,1 case with normal size kidney,and 1 case with bilateral renal atrophy.Two cases with renal artery stenosis,1 case with focal narrowing in left main branch and 1 case with vesico-ureteral reflux were found.Among the 9 cases,3 cases had homozygous mutations,and 6 cases had compound heterozygous mutations,including 1 nonsense mutation,1 frameshift mutation and 15 missense mutations.There were 2 cases with 3 heterozygous mutations,2 c.5935C > T mutations and 2 eases with C.5869G > A mutations.A total of 10 new mutations were identified.Conclusion Patients with mutations in the PKHD1 gene had normal size kidney,or even atrophic kidney.Renal artery stenosis,vesicoureteral reflux and bronchial stenosis were all first reported in patients with mutations in PKHD1 gene.The novel mutations,c.274C > T,c.9059T > C,c.8996delG,c.281C > T,c.10424T > A,c.7092T > G,c.4949T > C,c.5869G > A,c.6197A > G and c.1877A > G further expanded the mutation spectrum of PKHD1 gene.

14.
Chinese Journal of Medical Ultrasound (Electronic Edition) ; (12): 232-235, 2017.
Article in Chinese | WPRIM | ID: wpr-641192

ABSTRACT

Objective To study the changes of Young's modulus value of the renal cortex,vertebral body and sinus when there was a different degree of acute renal artery stenosis.Methods 10 Japanese white rabbits were used for this study.The left renal artery was dissected.An ultrasound probe was placed on the left kidney,Young's modulus of the renal cortex,vertebral body and sinus were recorded using shear wave ultrasound elastic imaging technique during three stages:when the renal artery was intact (0% stenosis),tied with a suture (> 50% stenosis),and ligated (100% stenosis).Renal tissue elasticity with different degrees of renal artery stenosis were compared.Pathological study was carried out in the kidneys,each with either 0%,> 50% or 100% renal artery stenosis.Results The mean values of Young's modulus from the renal cortex,vertebral body and sinus with different degrees of renal artery stenosis were as follows:0% stenosis,(16.31 ±1.70) kPa,(13.38± 1.63) kPa,and (12.75±2.26) kPa;> 50% stenosis,(14.16±2.34) kPa,(11.49±2.70) kPa,and (10.72±2.56) kPa;100% stenosis,(11.35 ± 1.48) kPa,(8.39± 1.29) kPa,and (7.08 ± 1.52) kPa.The mean value of Young's modulus from the renal cortex was significantly higher than those from the renal vertebral body and sinus (P < 0.05).The mean values of Young's modulus from different parts of the kidney decreased progressively with an increase in renal artery stenosis (P < 0.05).The pathological study demonstrated that with the increase in the severity of renal artery stenosis,glomerular volume,renal interstitial capillary diameter and red blood cells decreased.When renal artery stenosis reached 100%,there was patch degeneration and inflammatory cell infiltration within the renal interstitium,and mild degeneration of tubular epithelial cells with obscure cell borders.Conclusions The value of Young's modulus from the renal cortex was higher than those from the renal pyramid and sinus in rabbits.The values of Young's modulus from the renal cortex,pyramid and sinus decreased with an increase in the severity of renal artery stenosis,which suggest that,in the study of the elasticity of the kidney,the renal artery blood flow dynamic state can obviously affect the value of the elasticity of the kidney.

15.
Journal of Peking University(Health Sciences) ; (6): 158-163, 2017.
Article in Chinese | WPRIM | ID: wpr-509416

ABSTRACT

Objective:To investigate the significance of filtration fraction (FF) and renal artery stenting in the treatment of atherosclerotic renal artery stenosis.Methods:In the study,42 cases of renal artery stenosis were treated with 52 renal artery stent implantation.Percutaneous transluminal renal angioplasty and stent (PTRAS) of the patients' health side kidney,ipsilateral kidney (renal) glomerular filtration rate (GFR),renal effective renal plasma flow effective renal plasma flow (ERPF),kidney filtration fraction changes of preoperative and postoperative serum creatinine (SCR) and the changes in the patients with blood pressure (SBP) and the changes after taking antihypertensive drugs were observed and analyzed.Results:The 52 cases of renal artery stent implantation were all successful.Preoperative ipsilateral GFR was significantly lower than that of normal side (t =-3.989,P =0.000);preoperative ipsilateral ERPF was significantly lower than the contralateral side (t =-4.926,P =0.000).On both sides,the overall FF values were equal (t =1.273,P =O.207).Postoperative ipsilateral renal GFR was increased,but there was no statistical difference (t =-1.411,P =0.164).Postoperative ipsilateral renal ERPF was increased significantly (t =-4.954,P =0.000),and FF lower (closer to the normal value (t =3.274,P =0.002).Postoperative side GFR was significantly reduced (t =2.569,P =0.000),the contralateral ERPF was significantly reduced (t =3.889,P =0.001),and FF had no significant change (t =-0.758,P =0.454).Postoperative side GFR was lower than that of the contralateral (t =-3.283,P =0.002) and postoperative side ERPF was still lower than that of the contralateral (t =-3.351,P =0.001),but on both sides,the FF values were equal (t =-0.361,P =0.719).Preoperative FF was relatively normal in the patients with kidney,and the postoperative FF value change was small (t =O.799,P =O.430);preoperative FF was significantly higher in the patients with kidney,and the postoperative FF value was lower than the preoperative (normal value,t =5.299,P =0.000).Postoperative overall serum creatinine was significantly decreased (t =2.505,P =0.016);but for the patients with unilateral renal artery stenosis,the changes in serum creatinine had no statistical difference (t =1.228,P =0.299);and for the patients with bilateral renal artery stenosis and serum creatinine compared with the preoperative,the changes were decreased significantly (t =2.518,P =0.030);postoperative blood pressure (SBP) was significantly decreased compared with that before operation (t =8.945,P =0.000);antihypertensive drugs taken were decreased significantly compared with the preoperative (t =5.280,P =0.000).Conclusion:For the patients with renal artery stenosis,FF is a useful index to understand the pathophysiological process of renal ischemia.Whether preoperative FF is significantly increased or FF is relatively normal,should be regarded as the indications of renal artery stent implantation.

16.
Vascular Specialist International ; : 1-15, 2017.
Article in English | WPRIM | ID: wpr-117391

ABSTRACT

Renal artery stenosis (RAS) is frequently associated with severe comorbidities such as reduced renal perfusion, hypertension, and end-stage renal failure. In approximately 90% of patients, renal artery atherosclerosis is the main cause for RAS, and it is associated with an increased risk for fatal and non-fatal cardiovascular and renal complications. Endovascular management of atherosclerotic RAS (ARAS) has been recently evaluated by several randomized controlled trials that failed to demonstrate benefit of stenting. Furthermore, the Cardiovascular Outcomes in Renal Atherosclerotic Lesions study did not demonstrate any benefit over the revascularization approach. In this review, we summarized the available data from retrospective, prospective and randomized trials on ARAS to provide clinicians with sufficient data in order to produce useful conclusions for everyday clinical practice.


Subject(s)
Humans , Angioplasty , Atherosclerosis , Comorbidity , Hypertension , Kidney Failure, Chronic , Perfusion , Prospective Studies , Renal Artery Obstruction , Renal Artery , Retrospective Studies , Stents
17.
Fortaleza; s.n; 2016. 59 p. ilus, tab.
Thesis in Portuguese | LILACS | ID: biblio-972028

ABSTRACT

INTRODUÇÃO: A estenose da artéria do enxerto renal (EAER), complicação vascular mais comum pós-transplante (Tx) renal, pode levar à hipertensão resistente, piora da função renal e até perda do enxerto. OJETIVOS: Investigar a prevalência e fatores associados à EAER. MÉTODOS: Estudo caso-controle retrospectivo, em população de receptores de Tx realizados de janeiro de 2008 a março de 2014, em um centro de referência em Tx renal no nordeste do Brasil. Foram avaliados como fatores associados à EAER características demográficas e clínicas do receptor e doador, dados relacionados a cirurgia, dados laboratoriais e número de anti-hipertensivos. A análise estatística foi realizada através do programa SPSS 17.0, valores descritivos abaixo de 5% (p < 0,05) foram considerados estatisticamente significativos. RESULTADOS: Foram avaliados 494 de 529receptores, sendo 24 pacientes com EAER, prevalência de 4,8%. Média do tempo do diagnóstico 89,9dias pós-Tx. Fatores associados a EAER foram número de anti-hipertensivos ≥ 2 e enxerto com duas ou mais artérias (p< 0,05). Houve redução significativa namédia da PAS (147,1 ± 23,7para 127,8 ± 15,2mmHg, p=0,001) e da PAD (86,6 ± 13,0para 77,6 ± 9,4mmHg, p=0,001) após a correção da EAER, bem como na creatinina sérica (de 2,8 ± 2,4 para 1,9 ± 1,8mg/dL, p=0,04)...


INTRODUCTION: The transplant renal artery stenosis (TRAS)is the most common vascular complication post-kidney transplant (Tx) that can lead to resistant hypertension, impaired renal function and even loss of the graft. OBJECTIVES: To investigate the prevalence and factors associated with TRAS. METHODS: A retrospective case-control study was carried out in a population of Tx recipientsfrom January 2008 to March 2014 in a renal Tx reference center in northeastern Brazil. Demographic and clinical characteristics of the recipient and donor,data related to the surgery, laboratory data and number of antihypertensive drugs were assessed as factors associated with TRAS. Statistical analysis was performed using SPSS 17.0. RESULTS: A total of 494 of 529 recipients wereassessed, of which 24 had TRAS.The prevalence of TRASwas 4.8% (24 patients), of which 23 had a deceased donor. Twelve patients (50%) were males, mean age of 46.7 ± 13.5 years (range 17-78 years).Meantime of diagnosis was 89.9 days post-Tx. The risk factors associated with TRASwere number of antihypertensivedrugs≥ 2 (OR: 17.0; CI: 4.1 to 70.4, p = 0.001) and grafting with two or more arteries (OR 8.9, CI: 1.4 -56.6, p = 0.021). There was a significant reduction in mean SBP (147.1 ± 23.7 to 127.8 ± 15.2 mmHg,p = 0.001) and DBP (86.6 ± 13.0 to 77.6 ± 9.4mmHg, p = 0.001) after TRASrepair,as well as in serum creatinine (2.8 ± 2.4 to 1.9 ± 1.8 mg / dL, p = 0.04)...


Subject(s)
Humans , Kidney Transplantation , Renal Artery Obstruction , Creatinine
18.
Korean Journal of Pediatrics ; : S84-S87, 2016.
Article in English | WPRIM | ID: wpr-201854

ABSTRACT

Midaortic syndrome (MAS) is a rare vascular disease that commonly causes renovascular hypertension. The lumen of the abdominal aorta narrows and the ostia of the branches show stenosis. MAS is associated with diminished pulses in the lower extremities compared with the upper extremities, severe hypertension with higher blood pressure in the upper rather than lower extremities, and an abdominal bruit. The clinical symptoms are variable, and recognition in children with hypertension can aid early diagnosis and optimal treatment. Hypertension with MAS is malignant and often refractory to several antihypertensive drugs. Recently, radiologic modalities have been developed and have led to numerous interventional procedures. We describe the case of a 3-year-old boy presenting with left ventricular hypertrophy whose severely elevated blood pressure led to the diagnosis of idiopathic MAS. This case highlights the importance of measuring blood pressure and conducting a detailed physical examination to diagnose MAS. This is the first reported case of idiopathic MAS diagnosed in childhood in Korea.


Subject(s)
Child , Child, Preschool , Humans , Male , Antihypertensive Agents , Aorta, Abdominal , Aortic Coarctation , Blood Pressure , Constriction, Pathologic , Diagnosis , Early Diagnosis , Hypertension , Hypertension, Malignant , Hypertension, Renovascular , Hypertrophy, Left Ventricular , Korea , Lower Extremity , Physical Examination , Renal Artery Obstruction , Upper Extremity , Vascular Diseases
19.
Chinese Circulation Journal ; (12): 122-126, 2016.
Article in Chinese | WPRIM | ID: wpr-487070

ABSTRACT

Objective: To evaluatethe efficacy of renal artery stenting combining optimal drug therapy in patients with atherosclerotic renal artery stenosis. Methods:This is a prospective cohort study for patients who received percutaneous renal artery stenting in our hospital from 2011-09 to 2013-03. All patients had conifrmed diagnosis of atherosclerotic renal artery stenosis combing hypertension and/or renal functional damage. Thepatients received optimal drug therapy for anti-platelet and blood pressure, lipids, glucose controlling, and they were followed-up for at least 12 months to observe the improvement of blood pressure and renal function. Results:There were 149 patients at the mean age of (61.54 ± 9.63) years and 185 renal artery stenosis with stent implantation;the average stenosis rate was (83.11±7.30)%and the success rate of operation was 99.32%(148/149). During follow-up period, the patients had increased estimated glomerular ifltration rate (GFR) compared to base line from (76.49 ± 22.50) ml/(min·1.73 m2) to (84.09 ± 28.79) ml/(min·1.73 m2), P Conclusion:Renal artery stenting combining optimal drug therapy may improve blood pressure controlling and renal function in strictly selected patients with atherosclerotic renal artery stenosis.

20.
Chinese Journal of Postgraduates of Medicine ; (36): 254-257, 2016.
Article in Chinese | WPRIM | ID: wpr-490694

ABSTRACT

Objective To investigate the incidence of atherosclerotic renal artery stenosis (ARAS), the relationship between ARAS and cerebral artery stenosis, and the risk of ARAS in patients with brain infarction. Methods The clinical data of 1 650 brain infarction patients were analyzed, which were carried out digital subtraction angiography(DSA) of cerebral and renal artery.The incidence of ARAS was counted out, and the relationship was analyzed between the different degree and number of cerebral artery stenosis and the rate of RAS. The demographic characteristics and the common risk factors of atherosclerosis were recorded, and the risk factors of ARAS were analyzed. Results The rate of ARAS in moderate stenosis group and severe stenosis and occlusion group of cerebral artery were all significantly higher than that in mild stenosis group and no stenosis group (all P < 0.01). The rates of ARAS in severe stenosis and occlusion group were significantly significantly higher than those in moderate stenosis group (P < 0.01). The rates of ARAS in 2 branch stenosis group and ≥3 branch stenosis group were both significantly higher than those in no stenosis group and 1 branch stenosis group (P < 0.01). The rates of ARAS of ≥3 branch stenosis group were significantly higher than those in 2 branch stenosis group (P<0.05). The rate of ARAS of 1 branch stenosis group were significantly higher than those in no stenosis group (P < 0.05). Age, hypertension, moderate or more artery stenosis or occlusion, and≥2 branch stenosis was independent risk factor of ARAS. Conclusions The incidence of ARAS increasesd with the increase of the degree of cerebral artery stenosis and the number of branch involved.Older age, hypertension, moderate or more artery stenosis or occlusion, and≥2 branch stenosis is risk factor for ARAS.

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