Hypertension as a prominent manifestation secondary to renal artery lesions in pediatric Behcet's disease.

OBJECTIVE: Hypertension caused by vascular Behcet's disease (BD) is an important prognostic factor of paediatric BD. However, much less is known about its clinical features. The objective of this study was to investigate the clinical characteristics of paediatric vascular BD complicated by hypertension. METHODS: A retrospective study was carried out in paediatric BD patients complicated by hypertension treated in the Children's Hospital Capital Institute of Paediatrics from Jan 2009 to Dec 2022. RESULTS: Of 65 BD patients, 6 (9.2%) were complicated by hypertension, 5 patients were female, and the median ages of onset and diagnosis were 9.8 years and 11.3 years, respectively. Three patients were found to have cardiac involvement and hypertensive retinopathy secondary to hypertension. Five of the 6 patients with hypertension had right renal artery involvement, and all of them were treated with glucocorticoids and immunosuppressants. Four patients were treated with biological agents. One patient with severe renal artery stenosis underwent unsuccessful vascular interventional therapy. After 3-6 years of follow-up, five patients were found to have renal atrophy, and one patient was at stable condition. CONCLUSION: Hypertension in paediatric BD is mainly caused by renal artery involvement. Early recognition and treatment of vascular involvement in BD is important to prevent poor prognosis.

A systematic review and meta-analysis on the efficacy of preoperative renal artery embolization prior to radical nephrectomy for renal cell carcinoma: Is it necessary?

INTRODUCTION: Radical nephrectomy for Renal Cell Carcinoma (RCC) is still the treatment of choice for all stages except for stage I and IV, which need patient selectivity. The purpose of Renal Artery Embolization (RAE) pre-operative before radical nephrectomy is to facilitate resection, reduce bleeding, and reduce the time to surgery, but the necessity of this procedure is still debatable. This study investigates the efficacy of pre-operative Renal Artery Embolization (PRAE) before radical nephrectomy for RCC patients. METHODS: The systematic searches based on PRISMA guidelines were conducted in Pubmed, Scopus, Web of Science, Medrxiv, and ScienceDirect databases with pre-defined keywords. Both analyses, quantitative and qualitative, were performed to assess blood loss, transfusion rate, surgical time, Intensive Care Unit (ICU) stay, and hospital stay. RESULTS: A total of 921 patients from 8 eligible studies were included. The blood loss was significantly lower in the PRAE group compared to the control group (p = < 0.00001; SMD -20 mL; 95%CI -0.29, -0.12). There is no statistically significant difference between RAE and without RAE in the transfusion rate nephrectomy (p = 0.53, OR 0.65; 95% CI 0.16, 2.57), mean operative time (p = 0.69; SMD 5.91; 95% CI -23.25, 35.07), mean length of hospital stay (p = 0.05; SMD 0.56; 95% CI 0.00, 1.12), and mean length of stay in the ICU (p = 0.45; SMD 11.61; 95% CI -18.35, 41.57) Conclusions: PRAE before radical nephrectomy significantly reduces blood loss in RCC patients but is similar in the surgical time, transfusion rate, and length of hospital stay and ICU stay.

Non-contrast magnetic resonance angiography for systemic artery evaluation in Kawasaki disease.

BACKGROUND: Kawasaki disease (KD) is a systemic vasculitis; systemic arteries other than the coronary arteries should therefore also be evaluated. This study investigated the feasibility of evaluating coronary aneurysms, systemic artery aneurysms (SAAs), and cerebrovascular diseases in patients with KD using non-contrast magnetic resonance angiography (NC-MRA). METHODS: Coronary artery protocols, including coronary magnetic resonance angiography (MRA) and vessel wall imaging, were performed in 57 examinations of 28 patients. Systemic artery protocol, including SAA scans and head MRA, along with coronary artery protocol, were performed in 42 examinations of 42 patients. The image quality of the SAAs was evaluated on a 4-point scale. Examination time and sedation dosage were compared between the protocols. The presence of SAAs and cerebrovascular disease was also evaluated. RESULTS: The image quality score of SAAs was 4 (interquartile range [IQR]: 4-4) for the aorta, 4 (IQR: 3-4) for the subclavian artery, 4 (IQR: 3-4) for the renal artery, and 3 (IQR: 3-4) for the iliac artery. No differences were found between examination time (47.0 [IQR: 43.0-61.0] min vs. 51.0 [IQR: 45.0-60.0] min, p = 0.48) and sedative dose (4.63 [IQR: 3.93-5.79] mg/kg vs. 4.21 [IQR: 3.56-5.71] mg/kg, p = 0.37) between the protocols. Systemic artery protocol detected SAAs in three patients (7.1%), and cerebrovascular disease was not detected. CONCLUSIONS: Evaluating the coronary and systemic arteries in patients with KD using NC-MRA on a single examination was possible without compromising examination time or sedation dose. The systemic artery protocol was useful in finding SAAs.

[Modern aspects of diagnostics and treatment of renal infarction].

One of the causes of acute kidney injury is the renal artery thrombosis. Clinical manifestations depend on the level of thrombus. This pathology is characterized by non-specific clinical manifestations in the early period, the complexity of differential diagnosis, often delayed verification of the diagnosis and unfavorable prognosis in case of prolonged (5-7 days) anuria. There is no generally accepted protocol for the diagnosis and treatment of renal artery thrombosis. To clarify the diagnosis, intravenous urography, radionuclide renography, and contrast-enhanced computed tomography are recommended. Until recently, patients with suspected renal artery thrombosis were treated with anticoagulant therapy and renal replacement therapy with hemodialysis, which is required constantly as renal function was usually irreversibly impaired. Surgical treatment is effective only in the first hours. The outcome is often unfavorable, the probability of hemorrhagic complications is high. Due to the rare frequency of detection and verification of renal infarction, no consensus has been reached regarding the diagnosis or treatment of this condition.

Characterization of renal artery variation in patients with clear cell renal cell carcinoma and the predictive value of accessory renal artery in pathological grading of renal cell carcinoma: a retrospective and observational study.

OBJECTIVE: To explore the characteristics of renal artery variation in patients with renal cell carcinoma and to evaluate the predicting value of accessory renal artery in the pathological grading of renal cell carcinoma. METHODS: The clinicopathological data of patients with clear cell renal cell carcinoma diagnosed in the Department of Urology of the First Hospital of Shanxi Medical University from September 2019 to March 2023 were retrospectively analyzed. All patients underwent visual three-dimensional model reconstruction from computed tomography images. All kidneys were divided into two groups: the affected kidney and the healthy kidney, and the incidence of renal artery variation in the two groups was analyzed. Then, according to the existence of accessory renal artery in the affected kidney, the patients were divided into two groups, and the relationship between accessory renal artery and clinicopathological features of patients with clear cell renal cell carcinoma was analyzed. Finally, univariate and multivariate logistic regression analyses were performed to determine the predictors of Fuhrman grading of clear cell renal cell carcinoma, and the predictive ability of the model was evaluated by the receiver operating characteristic curve. RESULTS: The incidence of renal artery variation and accessory renal artery in the affected kidney was significantly higher than them in the healthy kidney. The patients with accessory renal artery in the affected kidney had larger tumor maximum diameter, higher Fuhrman grade and more exophytic growth. The presence of accessory renal artery on the affected kidney and the maximum diameter of tumor are independent predictors of high-grade renal cell carcinoma. The receiver operating characteristic curve suggests that the model has a good predictive ability. CONCLUSION: The existence of accessory renal artery on the affected kidney may be related to the occurrence and development of clear cell renal cell carcinoma, and can better predict Fuhrman grade of clear cell renal cell carcinoma. The finding provides a reference for the future diagnostic evaluation of RCC, and provides a new direction for the study of the pathogenesis of RCC.

Graft Arterial Dissection and Thrombosis After Kidney Transplantation With Undiagnosed Fibromuscular Dysplasia From a Deceased Donor: Case Report and Review.

BACKGROUND: Fibromuscular dysplasia (FMD), a relatively frequent arterial deformity with an estimated prevalence of 2% to 6% has been sporadically reported during deceased donor kidney donations. Only 8 case reports are available in the previous literature. CASE PRESENTATION: In our work, implantation of 2 kidneys from the same deceased donor with macroscopically evident and later histologically confirmed FMD are presented, one of which ended up as acute arterial complication. Renal arteries were cut short to allow safe implantation, but arterial dissection and thrombosis led to graft loss in the early perioperative period in the latter case. CONCLUSIONS: Although resection of the arterial segments affected by FMD as a routine may allow implantation, macroscopically healthy-looking arteries might still be affected and thus carry elevated postoperative risk. The aim of our case report is to make proposal for an onsite diagnosis of FMD in case of clinical suspicion.

A novel NF1 mutation in a pediatric patient with renal artery aneurysm.

BACKGROUND: Neurofibromatosis type 1 (NF1) is a neurocutaneous syndrome, due to heterozygous pathogenic variants in NF1 gene. The main clinical manifestations are multiple café au lait spots, axillary and inguinal freckling, cutaneous and plexiform neurofibromas, optic glioma, Lisch nodules and osseous lesions, such as sphenoid and tibial dysplasia. Vasculopathy is another feature of NF1; it consists of stenosis, aneurysms, and arteriovenous malformations, frequently involving renal arteries. CASE PRESENTATION: We report on a 9-year-old girl with a novel mutation in NF1 gene and renal artery aneurysm, treated by coil embolization and complicated with hypertension. CONCLUSION: Vasculopathy is a complication of NF1, affecting from 0.4 to 6.4% of patients with NF1. Among the vascular abnormalities, renal artery aneurysm is a rare manifestation, with only a few cases regarding adult patients and no pediatric reports described in current literature. The finding of a vascular abnormality in a specific site requires the evaluation of the entire vascular system because multiple vessels could be involved at the same time.

Renal arteriovenous fistula complicated with malignant hypertension: a case report.

Renal arteriovenous fistula (RAVF) is a rare vascular disease and is usually presented with severe hypertension. Renin-angiotensin-aldosterone system (RAAS) activation was proposed to play a key role in RAVF-induced hypertension but the data was inconsistent. We reported a case of RAVF presented as malignant hypertension, which was detected by contrast-enhanced ultrasonography and successfully managed by interventional embolization. A 35-year-old male was presented with a headache and blurred vision. His blood pressure was up to 220/110 mmHg, with significantly elevated serum creatinine and proteinuria. Hypertensive target organ impairments were noted. A RAVF was detected by contrast-enhanced renal ultrasonography. He underwent renal artery angiography and renal arteriovenous fistula embolization. RAAS activation was also evaluated by separate renal vein sampling. The patient's blood pressure and target-organ damage improved after RAVF embolization and blood pressure control. This is a rare case of renal arteriovenous fistula with malignant hypertension. Contrast-enhanced ultrasonography can be useful for diagnosis.

Can Aortic and Renal Arteries Calcium Scores Be New Factors to Predict Post-Operative Renal Function After Nephron Sparing Surgery?

INTRODUCTION: This study aims to investigate whether the calcification of renal arteries and aorta may be risk factors for developing chronic kidney disease (CKD) after Nephron sparing surgery (NSS). MATERIALS AND METHODS: The patients that underwent either open or laparoscopic NSS from 2000 to 2019 in 4 different centers were retrospectively assessed. Of these patients, 328 had a non-contrast-enhanced computer tomography. Calcium scores of the renal arteries and abdominal aorta were measured in the non-contrast-enhanced images with the calcium score plugin (version 2.0) of Horos™. Univariate and multivariate logistic regression analysis was performed to determine significant risk factors for developing CKD at the last check-up. Roc curve analysis was performed to determine the optimal cut-off values of age and abdominal aorta calcium scores. RESULTS: A total of 302 patients, of which 52 (16,6%) with CKD and 252 (83,4%) without CKD at the last check-up, were included in the analysis. The mean warm ischemia duration was significantly higher in patients with CKD (18,79±6,72 vs 16,38±5,57 minutes, p=0,016). The mean size of the tumor diameter and the number of the patients with ≥stage T1b were higher in the group with CKD (p=0,024 and 0,005, respectively). The median calcium scores of the aorta and renal arteries were higher in the group with CKD (p<0,001 and p<0,001, respectively). In multivariate analysis, age >60 years (OR:3,65, p=0,022), calcium score of the aorta (OR:4,07, p=0,029), tumor diameter (OR:1,03, p=0,026) and pre-operative CKD stage (OR:10,13, p<0,001) found the be significant factors for predicting last check-up CKD. CONCLUSION: The calcium score of the aorta may be used as an additional risk factor to predict post-operative CKD risk after NSS with sensitivity over 80%.

Renal Functional and Perioperative Outcomes of Retroperitoneal Robot-Assisted Versus Laparoscopic Partial Nephrectomy with Segmental Renal Artery Clamping.

Background: Retroperitoneal approach and segmental renal artery clamping in partial nephrectomy are techniques that facilitate postoperative recovery and renal function preservation. This study aimed to compare the renal function preservation and perioperative outcomes of robot-assisted partial nephrectomy (RAPN) and laparoscopic partial nephrectomy (LPN) with these techniques. Materials and Methods: Clinical parameters of 43 patients who had undergone retroperitoneal RAPN from March 2017 to December 2019 were retrospectively collected and compared with those of 52 patients who had undergone retroperitoneal LPN at the same period in our institution. Differences in operating time, warm ischemia time, estimated blood loss, complications, postoperative hospital stay, as well as renal function loss were compared between the two groups. Results: Background characteristics between RAPN and LPN groups such as age, gender, BMI, and tumor characteristics were comparable. All RAPNs and LPNs were successfully completed without conversion to open surgery or nephrectomy. No significant difference in operating time, estimated blood loss, complications, and postoperative hospital stay was observed between RAPN and LPN groups. The warm ischemia time in RAPN group was slightly shorter than that of LPN groups (P = .054). Compared with the LPN group, the RAPN group was significantly associated with less glomerular filtration rate reduction and renal volume loss rate (P = .042 and P = .013, respectively). Conclusions: The perioperative outcomes were comparable between the two groups. However, compared with LPN, RAPN had superiority in preserving renal function in our series.

Pulmonary and Systemic Hemodynamics following Multielectrode Radiofrequency Catheter Renal Denervation in Acutely Induced Pulmonary Arterial Hypertension in Swine.

OBJECTIVE: We aimed to assess the effects of renal denervation (RDN) on systemic and pulmonary hemodynamics in a swine model of thromboxane A2- (TXA2-) induced pulmonary arterial hypertension (PAH). METHODS: The study protocol comprised two PAH inductions with a target mean pulmonary artery pressure (PAP) of 40 mmHg at baseline and following either the RDN or sham procedure. Ten Landrace pigs underwent the first PAH induction; then, nine animals were randomly allocated in 1 : 1 ratio to RDN or sham procedure; the second PAH induction was performed in eight animals (one animal died of pulmonary embolism during the first PAH induction, and one animal died after RDN). In the RDN group, ablation was performed in all available renal arteries, and balloon inflation within artery branches was performed in controls. An autopsy study of the renal arteries was performed. RESULTS: At baseline, the target mean PAP was achieved in all animals with 25.0 [20.1; 25.2] mcg of TXA2. The second PAH induction required the same mean TXA2 dose and infusion time. There was no statistically significant difference in the mean PAP at second PAH induction between the groups (39.0 ± 5.3 vs. 39.75 ± 0.5 mmHg, P > 0.05). In the RDN group, the second PAH induction resulted in a numerical but insignificant trend toward a decrease in the mean systemic blood pressure and systemic vascular resistance, when compared with the baseline induction (74 ± 18.7 vs. 90.25 ± 28.1 mmHg and 1995.3 ± 494.3 vs. 2433.7 ± 1176.7 dyn∗sec∗cm -5, P > 0.05, respectively). No difference in hemodynamic parameters was noted in the sham group between the first and second PAH induction. Autopsy demonstrated artery damage in both groups, but RDN resulted in more severe lesions. CONCLUSIONS: According to our results, RDN does not result in significant acute pulmonary or systemic hemodynamic changes in the TXA2-induced PAH model. The potential chronic effects of RDN on PAH require further research.

Modified arteriosclerosis score predicts the outcomes of diabetic kidney disease.

BACKGROUND: The significance of renal arteriosclerosis in the prediction of the renal outcomes of diabetic kidney disease (DKD) remains undetermined. METHODS: We enrolled 174 patients with DKD from three centres from January 2010 to July 2017. The severity and extent of arteriosclerosis were analysed on sections based on dual immunohistochemical staining of CD31 and α-smooth muscle actin. An X-tile plot was used to determine the optimal cut-off value. The primary endpoint was renal survival (RS), defined as the duration from renal biopsy to end-stage renal disease or death. RESULTS: The baseline estimated glomerular filtration rate (eGFR) of 135 qualified patients was 45 (29 ~ 70) ml/min per 1.73 m2, and the average 24-h urine protein was 4.52 (2.45 ~ 7.66) g/24 h. The number of glomeruli in the biopsy specimens was 21.07 ± 9.7. The proportion of severe arteriosclerosis in the kidney positively correlated with the Renal Pathology Society glomerular classification (r = 0.28, P < 0.012), interstitial fibrosis and tubular atrophy (IFTA) (r = 0.39, P < 0.001), urine protein (r = 0.213, P = 0.013), systolic BP (r = 0.305, P = 0.000), and age (r = 0.220, P = 0.010) and significantly negatively correlated with baseline eGFR (r = - 0.285, P = 0.001). In the multivariable model, the primary outcomes were significantly correlated with glomerular class (HR: 1.72, CI: 1.15 ~ 2.57), IFTA (HR: 1.96, CI: 1.26 ~ 3.06) and the modified arteriosclerosis score (HR: 2.21, CI: 1.18 ~ 4.13). After risk adjustment, RS was independently associated with the baseline eGFR (HR: 0.97, CI: 0.96 ~ 0.98), urine proteinuria (HR: 1.10, CI: 1.04 ~ 1.17) and the modified arteriosclerosis score (HR: 2.01, CI: 1.10 ~ 3.67), and the nomogram exhibited good calibration and acceptable discrimination (C-index = 0.82, CI: 0.75 ~ 0.87). CONCLUSIONS: The severity and proportion of arteriosclerosis may be helpful prognostic indicators for DKD.

Severity of arterial and/or arteriolar sclerosis in IgA nephropathy and the effects of renin-angiotensin system inhibitors on its prognosis.

IgA nephropathy (IgAN) patients often suffer from arterial and/or arteriolar sclerosis (AAS); however, it is unclear whether these features are associated with a poor prognosis. This retrospective cohort study aimed to analyse the prognosis of IgAN patients with AAS and assess whether treatment with renin-angiotensin system inhibitors (RASI) improved their survival. The study included 678 IgAN patients, who were grouped into AAS0 (n = 340; AAS absent) and AAS1 (n = 338; AAS present) groups. Each patient's clinical, laboratory, and histological backgrounds and 20-year renal prognosis were analysed. In the AAS1 group, the impact of RASI initiated during the follow-up period on the renal prognosis was also evaluated after adjustments for background characteristics. IgAN patients with AAS had significantly higher age, blood pressure, body mass index, total cholesterol, uric acid levels, and proteinuria than patients without AAS; they also had more severe histological findings, decreased renal function, and lower survival rates than those without AAS (64.0 versus 84.7%, p < 0.001). Multivariate Cox regression analysis incorporating clinical and histological findings and treatments revealed AAS as an independent factor for disease progression (hazard ratio: 2.23, p = 0.010). Participants in the AAS1 group treated with RASI during follow-up had a significantly higher renal survival rate than those who were not (75.5 versus 44.3%, p = 0.013). In conclusion, AAS was found to be associated with serious clinical, laboratory, and histological findings and poor prognosis. RASI initiated during the follow-up period was found to improve renal prognosis.

[Treatment of Renal Artery Perforation with Covered Stent after Percutaneous Biopsy of Pancreatic Head]. / Behandlung einer Nierenarterienperforation mit gecovertem Stent nach perkutaner Pankreaskopfbiopsie.

HISTORY AND CLINICAL FINDING: In a 67-year-old female patient with upper abdominal pain, computed tomography showed a partly calcified swelling of the pancreatic head and wall thickening of the duodenum. EXAMINATIONS: Inpatient physical examination findings were normal. Laboratory showed increased pancreatic enzymes (amylase 210 U/l [Standard range: 28-100 U/l], lipase 2115 U/l [Standard range: 23-300 U/l]) and inflammation values (CRP 11.7 mg/l [Standard range: < 5.0 mg/l]), otherwise largely normal laboratory parameters. In the esophago-gastro-duodenoscopy, biopsy of swollen, partly stenosing mucous membrane areas in the duodenum was performed. DIAGNOSIS: Histology revealed partial erosive duodenitis, no evidence of a malignant tumor. If chronic calcifying pancreatitis was suspected, a sonographically guided percutaneous biopsy of the pancreatic head was performed to exclude a tumor. TREATMENT AND COURSE: Post-biopsy, the patient developed abdominal pain and temporary circulatory instability with nausea/vomiting and a drop in Hb to 7.5 g/dl [Standard range: 12.3-15.3 g/dl]. The sonographic suspicion of a retroperitoneal hematoma was confirmed by computed tomography. The cause was a haemorrhage from a renal artery perforation on the right side, which could be stopped by immediate angiographic intervention with a covered stent. CONCLUSION: After percutaneous biopsy, vascular perforation must always be considered. Computed tomography provides a reliable and quick diagnosis. Minimally invasive percutaneous insertion of a covered stent is the therapy of choice in the case of a renal artery accessible to stents.

Renal Outcome and New-Onset Renal and Extrarenal Dissections in Patients With Nontrauma Renal Artery Dissection Associated With Renal Infarction.

[Figure: see text].

Moyamoya disease with refractory hypertension associated with peripheral arterial stenosis in the renal parenchyma.

Moyamoya disease (MMD) has long been known to be associated with hypertension. While renal artery stenosis (RAS) is considered one of the causes of hypertension with MMD, most hypertension causes remain unexplained. A boy with MMD was diagnosed with renovascular hypertension (RVH) due to left-sided RAS by angiography. Although nephrectomy on the affected side for unilateral RVH was performed, hypertension poorly improved. Histopathological examination of the resected specimens revealed that the vascular lumen not only of the renal artery but also of peripheral vessels in the renal parenchyma was narrowed. He developed end-stage renal disease caused by multiple wasp stings and received a kidney transplant from a living donor with his remaining right kidney resected. His hypertension improved dramatically just after the operation. In histopathological findings, the narrowed vascular lumen was also observed in the resected right renal parenchyma similar to that in the left kidney. In our case, these pathological findings were the same as those of major vessels previously reported in MMD patients. Immunohistochemical staining with anti-renin antibody on bilateral intrinsic kidneys was strongly revealed in the Juxtaglomerular apparatus. He has been normotensive with the minimum amount of amlodipine since transplantation and resection of his intrinsic right kidney. This is the first report to show the possibility that peripheral arterial stenosis in the renal parenchyma due to MMD would result in refractory hypertension. If MMD patients have hypertension of unknown origin without significant RAS, it should be considered that the etiology may be peripheral arterial stenosis in the renal parenchyma.

Renovascular hypertension in pediatric patients: update on diagnosis and management.

Renovascular hypertension (RVH) is defined as an elevated blood pressure caused by kidney hypoperfusion, generally as a result of anatomic stenosis of the renal artery with consequent activation of the Renin Angiotensin-Aldosterone System. The main causes include genetic and inflammatory disorders, extrinsic compression, and idiopathic alterations. RVH is often asymptomatic and should be suspected in any child with refractory hypertension, especially if other suggestive findings are present, including those with severe hypertension, abdominal bruit, and abrupt fall of glomerular filtration rate after administration of angiotensin-converting enzyme inhibitors or angiotensin-receptor blockers. There is a consensus that digital subtraction angiography is the gold standard method for the diagnosis of RVH. Nevertheless, the role of non-invasive imaging studies such as Doppler ultrasound, magnetic resonance angiography, or computed tomographic angiography remains controversial, especially due to limited pediatric evidence. The therapeutic approach should be individualized, and management options include non-surgical pharmacological therapy and revascularization with percutaneous transluminal renal angioplasty (PTRA) or surgery. The prognosis is related to the procedure performed, and PTRA has a higher restenosis rate compared to surgery, although a decreased risk of complications. This review summarizes the causes, physiopathology, diagnosis, treatment, and prognosis of RVH in pediatric patients. Further studies are required to define the best approach for RVH in children.