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Background: Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic disorder affecting multiple systems. However, arterial stenosis is a rare manifestation in patients with NF1. Since the symptoms of arterial stenosis caused by NF1 are often atypical and have a high under-diagnosis rate, this can lead to serious complications such as hypertension, ischemic stroke, or even death. The aim of our research is to analyse the clinical characteristics of arterial stenosis in pediatric patients with NF1 and to summarise its diagnosis, treatment and prognosis., Methods: We conducted a retrospective review of data from patients with NF1 treated at Beijing Children's Hospital from 2016 to 2020. Patients diagnosed with arterial stenosis, identified through clinical symptoms, physical examination, arterial ultrasonography, or imaging studies, were included in this study. These patients received symptomatic drug and/or surgical treatments and were followed up regularly. We summarized demographic characteristics, sites of arterial stenosis, clinical manifestations, and treatment outcomes., Result: Among the 258 patients with NF1 treated at our hospital, 12 (4.7%) had arterial stenosis, comprising 9 males and 3 females with a median age of 7 years (range: 1-14 years). Renal artery stenosis was diagnosed in 7 patients (58.3%), while internal carotid artery (ICA) stenosis was diagnosed in 5 patients (41.7%). The predominant symptoms of renal artery and ICA stenosis were renal hypertension and convulsions, respectively. Antihypertensive drugs were effective in 5 patients with renal hypertension; 2 patients required balloon dilatation of the renal artery due to inadequate response to medication. Oral antiepileptic treatment was effective in 3 patients with ICA stenosis, and encephaloduroarteriosynangiosis was effective in the remaining 2 cases. The follow-up period ranged from 2 to 6 years, with a median duration of 3 years. No deterioration or mortality was observed during the follow-up period., Conclusions: Arterial stenosis was present in approximately 4.7% of patients with NF1, predominantly affecting the renal artery and ICA. Renal hypertension and convulsions were the primary symptoms of renal artery and ICA stenosis, respectively. Early diagnosis and intervention can substantially improve the prognosis of these patients., Competing Interests: Declarations. Ethics approval and consent to participate: This retrospective study was approved by the ethics committee of Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health (Code: 2022-E-083-R). All methods were carried out in accordance with relevant guidelines and regulations. And informed consent was obtained from all subjects and/or their legal guardians. Consent for publication: Not applicable. Competing interests: The authors declare no competing interests., (© 2024. The Author(s).)

Renal artery thrombosis is an uncommon but serious cause of acute kidney injury that can result in permanent loss of function. It is frequently misdiagnosed due to its non-specific appearance, which can mimic renal colic and other more common causes of abdominal pain. There is no agreement on the first-line treatment and no prospective studies comparing thrombolysis, anticoagulation and embolectomy. We present a case of a patient with right renal artery thrombosis and acute kidney injury, in whom renal function was recovered with anticoagulation therapy., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Tuberous sclerosis complex (TSC) is a rare autosomal dominant neurocutaneous disease. Arterial hypertension is one of its uncommon complications, which is supposed to be caused by renal cysts or angiomyolipomas. Few studies have been reported in the literature on renal artery stenosis (RAS) as the cause of hypertension in TSC. Hence, we reported a boy who presented with uncontrolled hypertension under five anti-hypertension drugs and was diagnosed with TSC complicated with left RAS. His high blood pressure was relieved by percutaneous transluminal renal angioplasty (PTRA). In one and a half years follow-up, his blood pressure was normal whilst he took four anti-hypertensive drugs. In conclusion, children with TCS complicated with hypertension should be carefully screened for RAS, which might be relieved by percutaneous balloon dilatation., (© 2024 Asian Pacific Society of Nephrology.)

Renovascular hypertension (RVH) is a primary cause of secondary hypertension, primarily driven by the activation of the renin-angiotensin-aldosterone system activation. Recently, growing studies suggested accessory renal artery (ARA) might also contribute to RVH. However, the treatment of ARA-related hypertension and whether to take interventional treatment lack consensus. Herein, we report two cases of ARA-related hypertension in our hospital. Imaging studies of both patients showed ARA stenosis. One patient had ARA occlusion well-compensated through tortuous collateral branches, achieving normal blood pressure by medical treatment alone. The other patient had ARA stenosis coexisted with main renal artery stenosis, and revascularization of both arteries led to a significant postoperative reduction in blood pressure. A literature review was conducted to summarize overall treatment strategies for ARA-related hypertension and clarify the relationship between ARA and hypertension. Recent research supported an association between ARA and hypertension. While medical therapy remains the first-line treatment for ARA-related hypertension, interventional procedures should be considered for patients whose blood pressure remains uncontrolled despite conservative management., (© 2024 The Author(s). The Journal of Clinical Hypertension published by Wiley Periodicals LLC.)

Presentation: We present a case of 50-year-old lady who present with 1-month history of uncontrolled hypertension., Diagnosis: Magnetic resonance angiography showed stenosis of the left renal artery just beyond the ostium extending over approximately 7mm in length. Computed tomography angiography show focal narrowing of the proximal renal artery just distal to vessel origin, approximately 60% stenosis., Treatment: Anti-hypertensive medication was initiated in the ward. She was referred to vascular surgeon for renal bypass., Discussion: Renal artery stenosis is common cause of hypertension but may go unrecognised. The focus of this case is on the evaluation and necessity for a complete evaluation of the patient who is presenting with uncontrolled hypertension. To rule out renal artery stenosis, patient should be examined using CT-angiography or, if possible, arteriography., Competing Interests: None declared.

A 63-year-old woman with uncontrolled hypertension despite taking 5 antihypertensive medications was referred for percutaneous renal artery intervention.

Renal artery stenosis is one of the common vascular diseases that cause hypertension in children. However, renal artery aneurysms and abdominal aortic aneurysms, which may be components of mid-aortic syndrome, are rarely associated with renal artery stenosis. Despite its rarity, early diagnosis and treatment are critical to prevent fatal complications. Currently, non-surgical invasive techniques are considered the first choice for treatment, but in some cases, surgery is inevitable. Here, we present a 5-year-old boy with a mid-aortic syndrome. The patient presented with a history of severe headache and epistaxis 5-6 times a day and was diagnosed with hypertension. A 9 × 9 mm saccular aneurysm on the anterior surface of the abdominal aorta at the level of the left renal artery ostium, and a 12 mm aneurysm in the left renal artery after a stenotic segment at the hilum level was detected in the doppler USG and contrast-enhanced imaging techniques. The patient was operated on electively. We used a PTFE patch to repair the abdominal aorta and, saphenous vein which was taken from his father to repair the renal artery. The patient recovered well and was discharged on the 18th day., (© 2023 Asian Pacific Society of Nephrology.)

Peripheral artery (PAD) disease in association with renal artery stenosis is an important association which predicts the severity of the disease. An increase in the number of vessels affected by peripheral artery disease increases the chances of renal artery stenosis. In our case, the patient had primarily presented with anginal chest pain with complaints of claudication which on further investigation was diagnosed to be a triple vessel coronary artery disease along with bilateral subclavian and bilateral renal stenosis. On detailed history taking, risk factors like hypertension and chronic smoking was found to be present in our case which predisposed to peripheral artery disease secondary to atherosclerosis which was diagnosed on further investigations. Although the association of renal artery stenosis is not very rare in cases of severe peripheral vascular diseases, the presence of a triple vessel coronary artery disease in synchrony is what makes it unique. Take away lesson from this case report is importance of early diagnosis of dyslipidemia causing atherosclerosis and its complications. Multiple atherosclerotic lesions in synchrony i.e, bilateral renal artery stenosis with bilateral subclavian artery stenosis with coronary artery triple vessel atherosclerotic disease like in our case and its severity should create awareness among health care individuals and early treatment measures including lifestyle modifications should be considered to avoid such drastic events., Competing Interests: No competing interests were disclosed., (Copyright: © 2024 Toshniwal S et al.)

An 18-year-old man with past medical history of secondary arterial hypertension diagnosed at age 15 presented with an abdominal magnetic resonance imaging (MRI) that exhibited left renal artery stenosis.

Atherosclerotic renovascular disease (ARVD) is the most common type of renal artery stenosis. It represents a common health problem with clinical presentations relevant to many medical specialties and carries a high risk for future cardiovascular and renal events, as well as overall mortality. The available evidence regarding the management of ARVD is conflicting. Randomized controlled trials failed to demonstrate superiority of percutaneous transluminal renal artery angioplasty (PTRA) with or without stenting in addition to standard medical therapy compared with medical therapy alone in lowering blood pressure levels or preventing adverse renal and cardiovascular outcomes in patients with ARVD, but they carried several limitations and met important criticism. Observational studies showed that PTRA is associated with future cardiorenal benefits in patients presenting with high-risk ARVD phenotypes (i.e. flash pulmonary oedema, resistant hypertension or rapid loss of kidney function). This clinical practice document, prepared by experts from the European Renal Best Practice (ERBP) board of the European Renal Association (ERA) and from the Working Group on Hypertension and the Kidney of the European Society of Hypertension (ESH), summarizes current knowledge in epidemiology, pathophysiology and diagnostic assessment of ARVD and presents, following a systematic literature review, key evidence relevant to treatment, with an aim to support clinicians in decision making and everyday management of patients with this condition., (© The Author(s) 2023. Published by Oxford University Press on behalf of the ERA.)

Atherosclerotic renovascular disease is the most frequent cause of renovascular hypertension and its prevalence increases with age and in specific subset of patients, such as those with end-stage chronic kidney disease, heart failure, and coronary artery disease. Besides hypertension, atherosclerotic renovascular disease is responsible for several clinical manifestations, including life-threatening conditions, such as recurrent flash pulmonary edema, rapidly progressive chronic kidney disease, or acute kidney injury. Atherosclerotic renovascular disease is usually part of a more diffuse atherosclerotic process and requires a combination therapy including antihypertensive, antiplatelet and lipid-lowering agents, as well as optimization of antidiabetic treatment, if needed. Besides medical therapy, percutaneous renal angioplasty was supposed to be the most effective therapy for atherosclerotic renovascular disease, by leading to blood flow restoration. However, despite an apparently solid rationale, several randomized clinical trials failed to confirm the favorable effects of percutaneous renal angioplasty on blood pressure control, kidney function, cardiovascular and renal outcomes, previously reported in observational, retrospective and single-center cohorts, switching off the enthusiasm for this procedure. Several studies' limitations may partly account for this failure, including heterogeneity of diagnostic techniques, overestimation of the degree of renal artery stenosis, inappropriate timing of revascularization, multiple protocol revisions, frequent crossovers, and most importantly exclusion of patients at higher likelihood to respond to angioplasty. The purpose of this review is to summarize studies' potential weaknesses and provide guidance to the clinician for identification of patients who may benefit most from revascularization., Competing Interests: Disclosures None.

Renal artery entrapment (RAE) by hypertrophic diaphragmatic crura is an extremely rare cause of renovascular hypertension (RVH). Here, we report the case of a 9-year-old boy diagnosed with RVH caused by right RAE by a hypertrophic diaphragmatic crus and successfully managed with close medical monitoring. Diagnosis of this entity is easily overlooked if the optimal views are not obtained during imaging, which depends on a high index of suspicion. We would like to remind clinicians to keep this rare condition in mind when evaluating children with RVH., (Copyright © 2024 Copyright: © 2024 Saudi Journal of Kidney Diseases and Transplantation.)

Takayasu arteritis (TAK) is classified as large vessel vasculitis, and continuous inflammation of the vessel results in aneurysm or stenosis, which leads to various serious complications. Recently, a TAKT [TAK treated with tocilizumab (TCZ)] study showed that subcutaneous TCZ, a humanised anti-interleukin-6 receptor monoclonal antibody, is an effective treatment in patients with TAK above 12 years of age; however, the effectiveness of TCZ for juvenile TAK under 12 years old remains unclear. Here, we described the case of a 2-year-old girl with TAK, which was successfully treated with intravenous TCZ. She was diagnosed with TAK type V (Numano's angiographic classification system) with aortic aneurysms, bilateral renal arteries stenosis, and atypical descending aortic coarctation based on contrast-enhanced computed tomography findings. Treatment was started with 2 mg/kg/day prednisolone (PSL) and methotrexate instead of methylprednisolone pulse due to renovascular hypertension. She was immediately afebrile and her C-reactive protein level decreased, although it was elevated 4 weeks after starting PSL. Intravenous TCZ of 8 mg/kg/2 weeks was added because the progression of aneurysms or stenosis might lead to a poor prognosis. PSL was steadily reduced under intravenous TCZ. Magnetic resonance imaging showed that aortic aneurysms, renal arteries stenosis, and aortic coarctation ameliorated 4 months after starting TCZ, with the amelioration maintained at 1 year after starting TCZ. Aneurysms and stenosis improved; therefore, TCZ may be effective for the treatment of inflammation of vessels, aneurysms, and stenosis. It is desirable to examine the effect of TCZ on TAK patients under 12 years of age., (© Japan College of Rheumatology 2022. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Primary aldosteronism (PA) is a typical example of low renin hypertension, whereas renal artery stenosis (RAS) is a classic form of high renin hypertension. PA and RAS occurring simultaneously in a patient is challenging to diagnose. We report a 32-year-old woman with a 12-year history of resistant hypertension. She was identified to have elevated plasma aldosterone and renin levels with normal aldosterone/renin ratio (ARR). Imaging examinations identified bilateral adrenal thickening and subtotal occlusion of the anterior segment of the left renal artery. Adrenal venous sampling was performed and indicated the existence of unilateral aldosterone over-secretion. It may suggest that even though RAS led to non-suppressed renin, adrenal venous sampling remains to be an applicable approach to establish the diagnosis of aldosterone-producing adenomas, although the diagnostic value of ARR may be compromised due to non-suppressed renin level. The patient underwent a two-stage treatment. First, stenosis of the left renal artery was dilated by percutaneous transluminal renal balloon angioplasty. Two months later, laparoscopic complete left adrenalectomy was performed. Hematoxylin-eosin staining and CYP11B2 immunostaining suggested that this tumor was an aldosterone-producing adenoma. After the two-stage treatment, her blood pressure decreased to a normal level without antihypertensive drugs. This case report raises our awareness of the simultaneous occurrence of RAS and PA. Under this condition, ARR could lead to a false-negative PA. Adrenal venous sampling is warranted to achieve a confirmed diagnosis. For subjects with complex etiologies of secondary hypertension, multi-stage treatment may be required.

The study consists in determining the effect of renal artery stenting in patients with arterial hypertension in combination with renal artery stenosis on the dynamics of clinical and geriatric status in terms of such indicators as the number of drugs taken, cognitive status, determined by the MMSE scale, prevalence of the frequency of falls, psycho-emotional status by the Beck scale, the quality of sleep on the VAS scale, the index of age discrimination according to the E.Palmore ageism questionnaire, the quality of life according to the SF 36 questionnaire. The study involved 72 elderly patients with arterial hypertension in combination with renal artery stenosis. According to the results of the study, as a result of stenting of the renal arteries, there is a decrease in the number of drugs taken, an improvement in the cognitive status on the MMSE scale, a decrease in the frequency of falls, an improvement in the psycho-emotional status on the Beck scale, an improvement in the quality of sleep on the VAS scale, a decrease in the level of age discrimination, an increase in the quality of life according to questionnaire SF-36 on all scales.

A 2-month-old girl presented with malignant arterial hypertension revealing bilateral renal artery stenosis secondary to neurofibromatosis type 1 (NF1). Life-supporting care was initiated immediately. High-dose peripheral vasodilator therapy induced life-threatening toxicity; vascular surgery was therefore performed. Technical difficulties due to the young age and low body weight of the patient resulted in fatal bleeding. Renovascular disease is an important cause of pediatric hypertension. NF1-associated renovascular hypertension in young pediatric patients is rare, and its highly specialized management is best delivered via a multidisciplinary approach. The long-term prognosis remains poor., Competing Interests: Conflicts of interest None., (Copyright © 2022 French Society of Pediatrics. Published by Elsevier Masson SAS. All rights reserved.)

Renovascular hypertension in most cases requires endovascular treatment and/or surgery. This is technically much more difficult in small children and there is very limited published knowledge in this age group. We here present treatment and outcome of young children with renovascular hypertension at our institution. Children below 2 years of age, with renovascular hypertension between January 1998 and March 2020 were retrospectively reviewed. Demographics and treatment modalities were noted. Primary outcome was blood pressure within a week after the procedures and at last available visit. Sixty-six angiographies were performed in 34 patients. Median age at time of first angiography was 1.03 (interquartile range (IQR) 0.4-1.4) years and systolic blood pressure at presentation 130 (IQR 130-150) mm Hg. Thirty-eight percent (13/34) of children were incidentally diagnosed and 18% (6/34) presented with heart failure. Twenty-six (76%) children had main renal artery stenosis and 17 (50%) mid-aortic syndrome. Seventeen (50%) children showed intrarenal, six (18%) mesenteric, and three (9%) cerebrovascular involvement. Twenty patients underwent 45 percutaneous transluminal angioplasty procedures and seven children surgeries. In 44% of the 16 patients who underwent only percutaneous transluminal angioplasty blood pressure was normalized, 38% had improvement on same or decreased treatment and 19% showed no improvement. Complications were seen in 7.5% (5/66) of angiographies. In four of the seven (57%) children who underwent surgery blood pressure was normalized, two had improved (29%) and one unchanged (14%) blood pressure., Conclusion: In small children with renovascular hypertension below the age of 2 years, percutaneous transluminal angioplasty caused significant improvement in blood pressure with low complication profile. Surgery can be recommended where percutaneous transluminal angioplasty and medical treatments failed., What Is Known: • Renovascular hypertension is diagnosed in all age groups from a few weeks of life until adulthood. • Both angioplasty and surgery are significantly more difficult to perform in small children and the published information on short and long-term outcome in these children is very scarce., What Is New: • Children below the age of two years can safely and successfully undergo selective renal angiography and also safely be treated with angioplasty. • We here present a large group of babies and infants where angioplasty and in some cases surgery effectively and safely improved their blood pressure., (© 2022. The Author(s).)

Renovascular disease is a major causal factor for secondary hypertension and renal ischemic disease. However, several prospective, randomized trials for atherosclerotic disease failed to demonstrate that renal revascularization is more effective than medical therapy for most patients. These results have greatly reduced the generalized diagnostic workup and use of renal revascularization. Most guidelines and review articles emphasize the limited average improvement and fail to identify those clinical populations that do benefit from revascularization. On the basis of the clinical experience of hypertension centers, specialists have continued selective revascularization, albeit without a summary statement by a major, multidisciplinary, national organization that identifies specific populations that may benefit. In this scientific statement for health care professionals and the public-at-large, we review the strengths and weaknesses of randomized trials in revascularization and highlight (1) when referral for consideration of diagnostic workup and therapy may be warranted, (2) the evidence/rationale for these selective scenarios, (3) interventional and surgical techniques for effective revascularization, and (4) areas of research with unmet need.

Transplant renal artery stenosis (TRAS) constitutes 75% of all vascular complications in kidney transplant recipients, being a significant source of graft dysfunction and loss. TRAS is a heterogeneous disease with different risk factors and causes. The incidence differs greatly, and it is likely it will increase because of the aging population of potential recipients and donors of renal grafts and the expanding use of extended-criteria donors. Prompt diagnosis and treatment of TRAS can prevent irreversible allograft dysfunction and loss. Current evidence of risk factors, diagnostic challenges, and therapeutic options are presented in this short review., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background We investigated the early postoperative effect of percutaneous transluminal renal angioplasty on ambulatory blood pressure (BP) and the circadian characteristics of natriuresis and autonomic nerve activity. Methods and Results A total of 64 patients with hypertension with hemodynamically significant renal artery stenosis (mean age, 60.0±21.0 years; 31.3% fibromuscular dysplasia) who underwent angioplasty were included, and circadian characteristics of natriuresis as well as heart rate variability indices, including 24-hour BP, low-frequency and high-frequency (HF) components, and the percentage of differences between adjacent normal R-R intervals >50 ms were evaluated using an oscillometric device, TM-2425, both at baseline and 3 days after angioplasty. In both the fibromuscular dysplasia and atherosclerotic stenosis groups, 24-hour systolic BP (fibromuscular dysplasia, -19±14; atherosclerotic renal artery stenosis, -11±9 mm Hg), percentage of differences between adjacent normal R-R intervals >50 ms, HF, brain natriuretic peptide, and nighttime urinary sodium excretion decreased (all P <0.01), and heart rate increased (both P <0.05) after angioplasty. In both groups, revascularization increased the night/day ratios of percentage of differences between adjacent normal R-R intervals >50 ms (both P <0.01) and HF, and decreased those of low frequency/HF (all P <0.05) and nighttime urinary sodium excretion (fibromuscular dysplasia, 1.17±0.15 to 0.78±0.09; atherosclerotic renal artery stenosis, 1.37±0.10 to 0.99±0.06, both P <0.01). Multiple logistic regression analysis indicated that a 1-SD increase in baseline low frequency/HF was associated with at least a 15% decrease in 24-hour systolic BP after angioplasty (odds ratio, 2.30 [95% CI, 1.03-5.67]; P <0.05). Conclusions Successful revascularization results in a significant BP decrease in the early postoperative period. Intrarenal perfusion might be a key modulator of the circadian patterns of autonomic nerve activity and natriuresis, and pretreatment heart rate variability evaluation seems to be important for treatment success.

Objective: The indication of percutaneous renal transluminal angioplasty (PTRA) in fibromuscular dysplasia (FMD) is mainly based on renal artery stenosis (RAS) due to atherosclerosis criteria, which are not specific to FMD. Consequently, the selection of patients who could benefit from this treatment and its effectiveness remain uncertain. The aims of this study were to: (1) report the effects of PTRA guided by trans-stenotic pressure measurements on hypertension 7 months after treatment; (2) assess the impact of pressure measurement to guide treatment efficacy in comparison to visual angiographic parameters; and (3) evaluate the reproducibility and accuracy of the stenosis measurement using a 4F catheter in comparison to a pressure guidewire., Methods: This prospective multi-centric study analyzed 24 patients with hypertension with RAS due to FMD that required PTRA. Clinical, duplex ultrasound, and angiographic indices were collected, and patients were followed up for 7 months (±1 month). Angiographic indices were measured twice both by a pressure guidewire and a 4F catheter. Assessment of procedural and clinical success of angioplasty was performed for all patients., Results: Twenty-three patients (96%) had procedural success (considered as a post-PTRA translesional systolic gradient ≤10 mmHg or reduced by at least 80%) with a significant decrease in the systolic gradient after angioplasty (26.50 mmHg; [interquartile range, 16.75-38.75] vs 0.00 [interquartile range, 0.00-2.00]; P < .01). Three patients (12%) had complications, including two renal artery dissections and one partial renal infarction. Twenty-one patients (88%) were clinical responders to angioplasty at follow-up. Visual stenosis assessment showed a poor correlation with systolic gradient measurement before and after PTRA (R from -0.05 to 0.41; P = 0.06-0.82). High correlations were found between pressure measurements made by a 4F catheter and guidewire (R from 0.64 to 0.89; P ≤ .003)., Conclusions: In patients selected by clinical indicators and duplex ultrasound, reaching a translesional systolic gradient ≤10 mmHg or reduced by at least 80% after angioplasty, promotes a high success rate for PTRA in hypertension due to FMD RAS., (Copyright © 2021 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Renal artery thrombosis can cause acute occlusion of unilateral or bilateral renal arteries,and kidney failure would be induced if it is not diagnosed and treated in time.Therefore,rapid and correct treatment is especially important for renal artery thrombosis.Due to the lack of specificity of clinical manifestations,this disease in commonly misdiagnosed or missed and thus has a low early diagnosis rate.Here we report a case of acute renal artery thrombosis to improve the diagnosis and treatment.

The diagnosis and management of atherosclerotic renovascular disease (ARVD) is complex and controversial. Despite evidence from the ASTRAL (2009) and CORAL (2013) randomized controlled trials showing that percutaneous renal artery revascularization did not improve major outcomes compared with best medical therapy alone over 3-5 years, several areas of uncertainty remain. Medical therapy, including statin and antihypertensive medications, has evolved in recent years, and the use of renin-angiotensin-aldosterone system blockers is now considered the primary means to treat hypertension in the setting of ARVD. However, the criteria to identify kidneys with renal artery stenosis that have potentially salvageable function are evolving. There are also data suggesting that certain high-risk populations with specific clinical manifestations may benefit from revascularization. Here, we provide an overview of the epidemiology, diagnosis, and treatment of ARVD based on consensus recommendations from a panel of physician experts who attended the recent KDIGO (Kidney Disease: Improving Global Outcomes) Controversies Conference on central and peripheral arterial diseases in chronic kidney disease. Most focus is provided for contentious issues, and we also outline aspects of investigation and management of ARVD that require further research., (Copyright © 2021 The Authors. Published by Elsevier Inc. All rights reserved.)

We report a case of acute ischemic nephropathy in a patient with severe renal artery stenosis and bradycardia due to sick sinus syndrome. An 83-year-old Japanese woman with a history of hypertension was diagnosed with sick sinus syndrome and scheduled for pacemaker implantation. Four days prior to admission for the procedure, she experienced sudden-onset severe right flank pain that persisted for 1 day. On the day of admission, her serum creatinine level increased from 1.35 mg/dL, measured 2 weeks earlier, to 7.04 mg/dL. Laboratory examinations showed elevated C-reactive protein and lactate dehydrogenase levels. A computed tomography scan showed a severely atrophied left kidney, suggesting that it was non-functioning. Doppler ultrasonography of the right renal artery showed an extended acceleration time, suggesting proximal stenosis. Magnetic resonance imaging showed no enhancement in the proximal portions of the right renal artery, consistent with severe stenosis or occlusion. The patient developed severe bradycardia with lightheadedness; as a result, pacemaker implantation was performed on post-admission day 7. On day 10, digital subtraction angiography revealed diffuse severe stenosis of the right renal artery; intravascular ultrasonography suggested plaque rupture. Percutaneous transluminal renal angioplasty (PTRA) was performed and a drug-eluting stent was placed. On day 11, hemodialysis was performed owing to deteriorating renal function. The patient's renal function dramatically improved shortly thereafter. This case highlights the importance of PTRA for select patients, as it can potentially save some patients from chronic dialysis, and outlines the possible implications of bradycardia in the pathogenesis of ischemic nephropathy., (© 2021. Japanese Society of Nephrology.)

Hyponatremic-hypertensive syndrome (HHS) is an uncommon disorder rarely seen in children. Herein, we report a 19-month-old boy with HHS. He had severe hypertension, polyuria, polydipsia, vomiting, and seizure at presentation. Laboratory findings revealed hyponatremia, hypokalemia, metabolic alkalosis, proteinuria, hypercalciuria, high levels of renin and aldosterone, and renal artery stenosis. All symptoms resolved after nephrectomy. Clinicians should be aware of this syndrome because prompt recognition can be lifesaving.

Objective: Renal artery revascularization has been performed to improve blood pressure control and to cure hypertension in patients with renal artery fibromuscular dysplasia (RAFMD). We herein conducted a systematic review and meta-analysis of studies assessing outcomes associated with the treatment of hypertensive RAFMD patients via endovascular angioplasty in order to offer an up-to-date overview of the relative costs and benefits of this approach to revascularization in RAFMD patients., Methods: We systematically searched the PubMed and Embase databases and the Cochrane Central Register for Controlled Trials to identify relevant studies published as of January 15, 2020. Key outcomes of interest in these studies included technical success, the incidence of perioperative complications, cure rates, and overall improvement rates., Results: In total, we identified 36 relevant studies of 1916 total repairs conducted in 1191 patients. Of these included studies, 33 were retrospective, while 3 were prospective. The overall technical success rate across these studies was 94.3%. Rates of total, major, and minor complications in these pooled studies were 12.9%, 4.6%, and 7.4%, respectively. Pooled rates of cured hypertension and improved hypertension following angioplasty, defined according to study-specific criteria, were 37.0% [95% CI: 27.0%-47.0%] and 80.0% [95% CI: 75.0% to 84.0%], respectively, although these rates varied highly among studies. Cure rates for studies used current clinical definitions for substantial variations across studies. Cure rates in studies using current definitions of cured hypertension (blood pressure <140/90 mm Hg without treatment) were just 18.1% following angioplasty. Cure rates fell markedly with increasing mean patient age (OR associated with an increase in mean age of 10 years: -0.24 [95% CI: -0.44 to -0.04, P = 0.019] and with mean known duration of hypertension (OR associated with an increase in mean hypertension duration of 5 years: -0.09 [95% CI: -0.12 to -0.05, P = 0.001])., Conclusions: These findings suggest that endovascular treatment yielded moderate benefits to RAFMD patients, with substantial variation across studies. The blood pressure outcome was strongly influenced by patient age., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Background: Atherosclerotic renal artery stenosis (ARAS) is frequently related to ischemic nephropathy, secondary hypertension, and end-stage renal failure. Thus, this study aimed to explore whether certain circulating long noncoding RNAs (lncRNAs) may be used as potential specific ARAS biomarkers., Methods: In the present study, a microarray analysis was performed to screen for lncRNAs in renal artery tissue from four ARAS patients and four non-ARAS individuals. To identify specific lncRNAs as candidate potential biomarkers of ARAS, we used the following criteria: the fold change was set to >3.0 (compared with non-ARAS tissues), and p value cutoff was set at .05. According to these criteria, six lncRNAs were identified from 1150 lncRNAs. After validation by quantitative PCR (qPCR), these lncRNAs were independently validated in blood from groups of 18 ARAS patients, 18 non-ARAS individuals, and 18 healthy volunteers, furthermore, the predictive value of lncRNA PR11-387H17.6 was further assessed using blood from groups of 99 ARAS patients, 49 non-ARAS individuals, and 50 healthy volunteers. A receiver operating characteristic (ROC) curve analysis was performed to assess the performance of these lncRNAs as biomarkers., Results: In the ROC analysis, the area under the curve (AUC) of PR11-387H17.6 was 0.733, with 52.5% sensitivity and 84.8% specificity in predicting the occurrence of ARAS. After considering the risk factors, the AUC of PR11-387H17.6 was 0.844, and the optimal sensitivity increased from 52.5% to 74.5%, although the specificity decreased from 84.8% to 81.9%. In the multivariable logistic analysis, PR11-387H17.6 was an independent predictor of major adverse events (OR: 3.039; 95% CI: 1.388-6.654; p = .006)., Conclusions: PR11-387H17.6 is a potential diagnostic biomarker of ARAS. The lncRNA levels in blood cells are regulated in ARAS. Thus, further investigations of the role of lncRNAs in ARAS are warranted.

A 16-year-old patient presented with abdominal pain and sustained hypertension. Thorough evaluation including renography with and without captopril and renal vein renin sampling were normal. Duplex ultrasound, however, raised suspicion of a renal artery stenosis. This was confirmed by computed tomography angiography which showed a severe branch artery stenosis with post-stenotic dilatation consistent with focal fibromuscular dysplasia (FMD). As the hypertension was resistant to 3 classes of antihypertensive treatment, percutaneous transluminal renal angioplasty (PTRA) was offered. The procedure had immediate effect on the blood pressure. Without medication the patient remains normotensive 4 years after and the abdominal pain has only sporadically returned. The presented case illustrates the challenging process of diagnosing FMD-related renal branch artery stenosis as well as the potential benefits of PTRA in this patient group.

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., (© 2021. IPNA.)

Renal artery stenosis is caused by atherosclerosis and fibromuscular dysplasia and is associated with ischemic nephropathy, renovascular hypertension, and accelerated cardiovascular disease. Routine screening for renal artery stenosis is not recommended but is reasonable in patients who have rapid onset of hypertension, resistant hypertension, progressive renal insufficiency, recurrent pulmonary edema, or repeat admissions for heart failure. Acute mesenteric ischemia is caused by arterial embolism or thrombosis, mesenteric venous thrombosis, or nonocclusive mesenteric ischemia, whereas chronic mesenteric ischemia is most often caused by arterial obstruction. This article reviews the epidemiology, pathophysiology, diagnosis, and management of these two conditions., (Copyright © 2021 Elsevier Inc. All rights reserved.)

We present a case of severe renal artery stenosis that mimicked rapidly progressive glomerulonephritis with acute kidney injury, active urine sediments, and severe hypertension. Simultaneous presence of secondary hyperaldosteronism and hypokalemia prompted renal angiography and subsequent renal artery angioplasty and stenting, leading to rapid resolution of acute kidney injury, proteinuria, and hypertension. This case emphasises the importance and benefits of prompt diagnosis and revascularisation of acute severe renal artery stenosis in a patient with history of atherosclerotic renovascular disease presenting with sudden onset of severe hypertension and acute kidney injury with active urine sediment mimicking rapidly progressive glomerulonephritis., (Copyright © 2021 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Abstract: Transplant renal artery stenosis (TRAS) is the most common (1%-23%) vascular complication following kidney transplantation. The aim of this study was to review our experience with an endovascular approach to treat TRAS.We retrospectively reviewed kidney transplant recipients who underwent percutaneous transluminal angioplasty (PTA) due to TRAS in our institute from January 2009 to December 2015. We analyzed the patient's baseline characteristics, postoperative renal function, blood pressure evolution, and the number of pre- and post-procedure antihypertensive drugs.A total of 21 patients (15 men, 6 women) were treated with the endovascular technique. The predominant presentation was graft dysfunction (76.2%). Stenosis or hemodynamic kinking was located at the anastomosis in 7 (33.3%) patients, proximal to the anastomosis in 13 (61.9%) patients, and distal the anastomosis in 1 (4.8%) patient. PTA without stent placement was performed in 7 patients (33.3%), and PTA with stent placement was performed in 14 patients (67.7%). Serum creatinine levels demonstrated no difference between the pre-procedure level and that on discharge day (1.61 mg/dl [0.47-3.29 mg/dl] vs 1.46 mg/dl [0.47-3.08 mg/dl]; P = .33). The glomerular filtration rate also showed no difference between the pre-procedure value and that on discharge day (53.6 ml/min [22.4-145.7 ml/min] vs 57.0 ml/min [17.56 -145 ml/min]; P = .084). Systolic blood pressure and diastolic blood pressure (DBP) varied from 137 mm Hg (120-160 mm Hg) and 84 mm Hg (70-100 mm Hg) pre-procedure to 129 mm Hg (90-150 mm Hg) and 79 mm Hg (60-90 mm Hg) at discharge, respectively (P = .124 and P = .07). The number of antihypertensive medications significantly decreased from 1.5 (0-6) pre-procedure to 0.5 (0-2) at discharge (P = .023). In our study, there were no technical failures, procedure-related complications or deaths. During the follow-up period, the free-from-reintervention rate was 100%, and graft failures occurred in 2 patients (9.5%) due to rejection.Endovascular procedures for TRAS show a high technical success rate with a low complication rate and a low reintervention rate. PTA showed a trend toward a positive impact on lowering serum creatinine, systolic blood pressure, and diastolic blood pressure and improving estimated glomerular filtration rate, and the number of antihypertensive medications could be significantly reduced after this procedure., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

Sympathetic crash acute pulmonary edema (SCAPE) is a life-threatening injury, which requires early recognition and intervention to prevent mortality. We present a case of 18-year-old woman with no previous comorbidity, presenting with SCAPE who was successfully resuscitated and eventually diagnosed with renal artery stenosis. Pickering syndrome is a rare cause of hypertensive emergency and should be considered in a young patient presenting with SCAPE in emergency department., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

The increased prevalence of chronic kidney disease (CKD) in elderly patients recognizes, as main cause, the long-term exposure to atherosclerosis and hypertension. Chronic ischemic damage due to critical renal arterial stenosis induces oxidative stress and intra-renal inflammation, resulting in fibrosis and microvascular remodelling, that is the histological picture of atherosclerotic renal vascular disease (ARVD). The concomitant presence of a long history of hypertension may generate intimal thickening and luminal narrowing of renal arteries and arterioles, glomerulosclerosis, interstitial fibrosis and tubular atrophy, more typically expression of hypertensive nephropathy. These complex mechanisms contribute to the development of CKD and the progression to End Stage Kidney Disease. In elderly CKD patients, the distinction among these nephropathies may be problematic; therefore, ischemic and hypertensive nephropathies can be joined in a unique clinical syndrome defined as atherosclerotic nephropathy. The availability of novel diagnostic procedures, such as intra-vascular ultrasound and BOLD-MRI, in addition to traditional imaging, have opened new scenarios, because these tools allow to identify ischemic lesions responsive to renal revascularization. Indeed, although trials have deflated the role of renal revascularization on the renal outcomes, it should be still used to avoid dialysis initiation and/or to reduce blood pressure in selected elderly patients at high risk. Nonetheless, lifestyle modifications (smoking cessation, increased physical activity), statins and antiplatelet use, as well as cautious use of renin-angiotensin system inhibitors, remain the main therapeutic approach aimed at slowing the renal damage progression. Mesenchymal stem cells and Micro-RNA are promising target of anti-fibrotic therapy, which might provide potential benefit in ARVD patients, though safety and efficacy profile in humans is unknown too.

Background: Rare cases of concurrent primary aldosteronism (PA) and renal artery stenosis (RAS) have been reported. Methods: In this retrospective case-control study, we selected a cohort of 10 PA with RAS patients and a control group of 20 PA without RAS patients from January 1, 2006, to January 1, 2016.  Results: All patients presented with refractory hypertension, and a nonstatistically significant trend toward lower mean serum potassium was seen in the PA with RAS group (p =.07). PA with RAS patients had lower mean orthostatic aldosterone-to-renin ratios (38.4 ± 41.4 ng dL -1 /ng mL -1  h -1  vs. 87.4.4 ± 38.4 ng dL -1 /ng mL -1  h -1 , respectively; p < .01) and a higher false-negative rate (50% vs. 15%, respectively; p < .05) compared with controls. All misdiagnosed patients had the diagnosis of PA confirmed when we revaluated the repeated screening and confirmative tests because of residual hypertension or hypokalemia after successful revascularization of renal artery stenosis.  Conclusions: PA is easily missed in patients with RAS because of the high false-negative rate for screening tests. RAS patients with residual hypertension after successful renal angioplasty should be monitored for coexisting PA. Reevaluation of screening and confirmatory tests is helpful in establishing the correct diagnoses.

Diabetes mellitus (DM) is also a cause of cardiovascular (CV) disease (CVD). Addressing the atherosclerotic CVD (ASCVD) burden in DM should reduce premature death and improve quality of life. Diabetes mellitus-associated ASCVD can lead to complications in all vascular beds (carotids as well as coronary, lower extremity, and renal arteries). This narrative review considers the diagnosis and pharmacological treatment of noncardiac atherosclerotic vascular disease (mainly in patients with DM). Based on current knowledge and the fact that modern DM treatment guidelines are based on CV outcome trials, it should be noted that patients with noncardiac CVD may not have the same benefits from certain drugs compared with patients who predominantly have cardiac complications. This leads to the conclusion that in the future, consideration should be given to conducting well-designed trials that will answer which pharmacological treatment modalities will be of greatest benefit to patients with noncardiac ASCVD.

Objective: The pathologic nature of pediatric renal artery occlusive lesions causing renovascular hypertension has been the subject of numerous anecdotal reports. This study was undertaken to define the character of childhood renal artery stenoses. A better understanding of this disease is particularly germane, given its unknown etiology and the limited success of certain contemporary treatment options., Methods: Renal artery specimens obtained during open operations in children being treated for renovascular hypertension from 2004 to 2016 were studied. Excluded from study were arteries subjected to earlier open or endovascular operations. Histologic preparations employing hematoxylin-eosin, Movat, Masson trichrome, and Verhoeff-van Gieson stains allowed characterization of the intima, media, and adventitial tissues. External and luminal diameters were measured. Microscopic data were correlated with preoperative arteriographic images. The histologic and morphologic findings were assessed in regard to coexistent nonrenal arterial and aortic lesions as well as known syndromic diseases., Results: Thirty-three stenotic renal arteries from 28 children were subjected to examination. Stenoses involved the proximal-ostial renal arteries (24), central renal arteries (7), and distal segmental renal arteries (2). Ostial stenoses commonly exhibited preocclusive concentric hyperplasia of intimal tissues, frequent internal elastic lamina disruptions, and diminutive and discontinuous media. Central and distal renal stenoses most often exhibited lesser intimal cellular hyperplasia and more noticeable fibrodysplasia of the media and adventitia. The mean external and luminal diameters of the renal arteries having ostial stenoses were smaller than the expected renal artery size for a given age. Abdominal aortic coarctation or hypoplastic aortas occurred in 14 children. Neurofibromatosis type 1 affected four children with ostial renal artery disease and one child with midrenal artery disease, but there were no distinguishing features unique to their stenoses., Conclusions: Pediatric renal artery stenotic disease affects exceedingly small arteries. Ostial lesions frequently exhibit extensive luminal encroachments characterized by cellular hyperplasia of intimal tissues and scant medial smooth muscle. Central and distal renal arterial stenoses were characterized most often by extensive fibrodysplasia of the media and adventitia. The early success and durability of catheter-based angioplasty may be compromised by the cellular abnormalities of pediatric renal artery occlusive disease observed in this investigation., (Copyright © 2020 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

A 41-year-old man with end-stage renal disease received a deceased donor kidney transplant without complication. Maintenance immunosuppression consisted of tacrolimus, mycophenolate and prednisone. Two months after transplantation, his creatinine did not improve beyond 2-2.3 mg/dL, which prompted allograft biopsy. His biopsy showed tubular epithelial injury without rejection, and given concern for possible calcineurin-inhibitor toxicity, his tacrolimus was changed to sirolimus. Renal function improved, but 1 month later he presented to the hospital with seizure activity, severe hypertension, acute kidney injury and MRI findings suggestive of posterior reversible encephalopathy syndrome. Blood pressure was difficult to control, which had not been the case in the immediate posttransplant period, and addition of lisinopril worsened his renal function. Transplant renal artery stenosis was suspected, and allograft ultrasound with doppler confirmed our suspicion. The patient underwent an angiogram, showing 60% stenosis of the mid-distal transplanted renal artery. Interventional radiology successfully stented this lesion, with subsequent improvement in allograft function and blood pressure control. He did not require further intervention in follow-up., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Secondary arterial hypertension (HT) is an increase in blood pressure due to a recognisable cause. It is estimated that the prevalence of people with HT is around 5%-15%. The search for secondary HT in all hypertensive patients is neither feasible nor cost-effective. However, there are a series of signs that force us to discount its diagnosis such as newly diagnosed hypertension before the age of 40, acute worsening of hypertension, resistant hypertension, the presence of extensive organ injury or clinical-analytical alterations, etc. It is important to diagnosis these patients since treatments can be curative, especially in young people. We present the case of a 46-year-old man with hypertensive emergency and subarachnoid haemorrhage due to aneurysmal rupture. The study of secondary causes was diagnostic for renal artery stenosis due to Takayasu arteritis in the scarring stage., (Copyright © 2020 SEH-LELHA. Publicado por Elsevier España, S.L.U. All rights reserved.)

Objective: This study is aimed at exploring the accuracy of contrast-enhanced ultrasound (CEUS) in grading renal artery stenosis., Methods: 122 renal arteries with suspected renal artery stenosis were selected. DSA, DUS, and CEUS were performed for all patients with suspected renal artery stenosis in the research. DSA was selected as the gold standard. The sensitivity, specificity, accuracy, positive predictive value (PPV), and negative predictive value (NPV) of CEUS or Doppler ultrasound (DUS) in the diagnosis of renal artery stenosis were analyzed. The consistency between CEUS and digital subtraction angiography (DSA) was compared. The accuracy of DUS or CEUS in grading renal artery stenosis was assessed by the area under the receiver operating characteristic (ROC) curves and compared between groups., Results: The sensitivity, specificity, accuracy, positive predictive value, and negative predictive value of CEUS in the diagnosis of renal artery stenosis were 88.9%, 87.8%, 88.5%, 93.5%, and 80.0%, respectively. There was no significant difference in grading renal artery stenosis between CEUS and DSA ( X 2 = 0.643, P = 0.424). 77 of the 122 renal arteries were diagnosed with the stenosis rate more than 30% by CEUS. Compared with the results of DSA, the kappa value of CEUS was 0.749 ( P < 0.05)., Conclusion: CEUS is accurate in grading renal artery stenosis, and it may represent the method of choice in diagnosing renal artery stenosis., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2020 Yanhua Cui et al.)

Clinical management of renal artery stenosis has seen a major shift, after randomised clinical trials have shown no group benefit of endovascular intervention relative to optimal medical control. However, the inclusion criteria of these trials have been criticised for focusing on a subset of patients with atherosclerotic renal artery stenosis where intervention was unlikely to be beneficial. Moreover, new imaging and computational techniques have become available, which have the potential to improve identification of patients that will respond to interventional treatment. This review addresses the challenges associated with clinical decision making in patients with renal artery stenosis. Opportunities for novel diagnostic techniques to improve patient selection are discussed, along with ongoing Dutch studies and network initiatives that investigate these strategies.

Background: Despite advances in non-invasive vascular imaging, detection of renal artery stenosis via catheter angiography is the criterion standard for the diagnosis of renovascular hypertension (RVH). However, because of lack of evidence, the utility of various blood tests and imaging modalities remains unclear., Methods: We retrospectively analyzed the utility of blood tests (plasma renin activity [PRA], aldosterone, and renal vein renin [RVR] values) and imaging studies (computed tomography angiography [CTA], kidney ultrasonography [US]) by comparing them with catheter angiography. Ten pediatric patients with RVH at two institutions from January 2008 to December 2017 were recruited. The sensitivities for diagnosing RVH via imaging and blood tests (kidney [US], PRA, and aldosterone) were derived by examining patient records. Furthermore, the sensitivity and specificity of CT angiography were calculated by considering both the affected and non-affected renal arteries of the patients., Results: A high sensitivity for diagnosing RVH via kidney US (89%) and PRA (80%) was observed. The sensitivity and specificity of CTA were 100%, each. RVR sampling did not aid in the diagnosis of RVH; only two of six patients with unilateral RVH showed significant laterality of RVR boundary ratios. Renal scintigraphy facilitated detection of a non-functional kidney (split renal function <5%)., Conclusions: RVH in children could be diagnosed utilizing non-invasive blood and imaging tests, without catheter angiography. We recommend kidney length measurement along with measurement of PRA level, as a simple and highly useful screening test, followed by CTA as a diagnostic test., (© 2020 Japan Pediatric Society.)

Revascularization of atherosclerotic renal artery stenosis may cure hypertension, but paradoxically, improvement in systemic blood pressure in response to successful revascularization may precipitate ischemia in other organs affected by previously silent atherosclerotic disease. We describe bowel ischemia secondary to preexisting celiac artery stenosis after revascularisation. Prior knowledge of multivessel disease facilitated prompt diagnosis and management of this condition., (Copyright © 2020 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)