Midterm Outcomes of Angioplasty for Pediatric Renovascular Hypertension

PurposeTo evaluate the midterm outcomes of percutaneous transluminal renal angioplasty (PTRA) for pediatric renovascular hypertension (RVH).Materials and MethodsThe clinical data of patients who underwent PTRA for RVH in the authors’ hospital from 2012 to 2019 were retrospectively analyzed. Postprocedural blood pressure, glomerular filtration rate (GFR) of the affected kidney, restenosis, and complications were closely monitored.ResultsPTRA was performed in a total of 30 children (20 boys and 10 girls), with a mean age of 7.3 years ± 0.7 (range, 40 days to 13.9 years) and a mean weight of 25.0 kg ± 2.3 (range, 3.4–53 kg). The median follow-up period was 26.5 months (range, 1 month to 7.5 years). Technical success was achieved in 26 (86.7%) of the 30 patients. Restenosis developed in 3 patients (10.0%). Only 1 patient underwent stent implantation, and the stent fractured 8 months later, requiring further intervention. There were no other complications. In terms of clinical benefit of blood pressure control after the initial PTRA procedure, 15 patients (50%) were cured and 7 patients (23.3%) showed improvement. There was no significant difference in the etiology, lesion location, and lesion length between patients with clinical benefit and failure (P = .06, P = .202, and P = .06, respectively). GFR of the affected kidney was significantly improved from 19.9 mL/min ± 11.2 to 38.1 mL/min ± 11.9 at the 6-month follow-up after PTRA (P < .001).ConclusionsThe overall results of PTRA for pediatric RVH caused by different etiologies are promising. PTRA not only provided a clinical benefit of blood pressure control in 73.3% of the patients but also significantly improved the function of the affected kidney.     

Histological interpretation of temporal artery biopsies

Temporal artery biopsy is recommended for diagnosis of suspected giant cell arteritis, a systemic vasculitis of older adults. There is currently no formal consensus for histological interpretation of the biopsies. Typical histological findings include a transmural lymphocytic infiltrate with a population of macrophages resulting in destruction of the internal elastic lamina. However, it is a patchy process and multiple tissue levels must be examined. It is important to be aware of various subtle features that may lead to a diagnosis of arteritis, and immunohistochemistry can be helpful in some cases. Some biopsies show unusual features that could raise a differential diagnosis of alternative vasculitides. When there is no evidence of arteritis in a specimen, there are often non-specific features seen in the context of age-related changes. All of these histological patterns require close clinicopathological correlation to ensure correct interpretation.     

翁维良教授活血通络法治疗多发性大动脉炎长时医案举隅

大动脉炎是一种慢性进行性非特异性炎性疾病，以抗炎、抑制免疫、扩血管等内科治疗为主，由于大动脉炎具有反复发作的特点，因此如何控制病情发展、减少受累血管、控制血压、缓解症状成为治疗的重点与难点。文章系统分析翁维良教授应用活血通络法治疗大动脉炎长时医案1则（治疗33 a），通过对其诊治思路、用药经验进行分析，总结出翁教授在活血通络基础上，根据疾病不同发展阶段本虚标实的偏盛，运用搜风通络、清热解毒、清肝平肝治疗大动脉炎的临证经验。     

婴幼儿多发性大动脉炎14例临床特点分析

目的通过分析总结14例婴幼儿多发性大动脉炎(TA)的临床特点,并结合文献复习总结,以提高对该病的认识及诊疗水平。方法回顾性分析2016年7月至2019年5月在首都儿科研究所附属儿童医院住院的TA婴幼儿的临床资料及随访情况,并结合文献,分析总结该病的临床特点。结果14例患儿年龄为1个月23 d^28个月,男6例,女8例。临床表现中最常见的是发热[10例(71.4%)],高血压9例(64.3%),脉弱或无脉5例(35.7%)。按照病变血管部位的临床分型,广泛型11例(78.5%),头臂动脉型3例(21.4%),本组患儿无胸腹主动脉及单纯肺动脉型。14例TA患儿中,12例有颈总动脉、颈动脉、锁骨下动脉及冠状动脉及其分支(前降支、回旋支)受累(85.7%);11例肾动脉受累(78.6%);9例腋动脉受累(64.2%);8例腹主动脉受累(57.1%);6例降主动脉受累(42.9%);6例胸主动脉受累(42.9%);6例肠系膜上动脉受累(42.9%);5例股动脉受累(35.7%);5例肺动脉受累(35.7%);4例肱动脉受累(28.6%)。14例患儿中,误诊11例,诊断不清3例,误诊时间为18 d^2个月。误诊病例中,8例误诊为不典型川崎病。14例患儿中,7例治疗后大部分病变血管范围逐渐减少,受累较轻血管甚至可以完全恢复正常血管状态。4例患儿血管影像学检查较前无明显加重或好转。9例出现高血压患儿应用降压药血压能控制在正常范围,但不能停用降压药。5例查体发现脉弱或无脉患儿均未改善。14例患儿中7例生长发育同正常同龄儿,7例落后于正常同龄儿身高体质量第25百分位。14例患儿随访2~22个月,均规律治疗,未出现复发。结论3岁以内TA累及血管较多,病情严重,误诊率高,经过治疗病情很快能够控制,但容易遗留血管病变,部分患儿预后不佳。     

大动脉炎合并脑梗死49例临床分析

目的 总结大动脉炎合并脑梗死患者的临床特点，分析此类患者脑梗死复发的相关因素。 方法 回顾性分析2010年1月-2020年6月随访超过3年的大动脉炎合并脑梗死患者的临床资料，对 此类患者脑梗死复发的相关因素进行分析。 结果 共纳入49例患者，首次脑梗死中位年龄30.0（22.0～45.0）岁，中位随访时间3.4（3.2～3.7）年。 11例（22.4%）出现复发性脑梗死，两次脑梗死间隔中位时间8.0（5.0～88.0）个月。与脑梗死无复发 患者相比，复发性脑梗死患者合并糖尿病比例（27.3% vs 2.6%，P =0.031）以及首次脑梗死后随访 期间平均TG水平（3.65±0.96 mmol/L vs 1.14±0.54 mmol/L，P =0.001）升高。大动脉炎受累动脉以颈 总动脉最为常见（48/49，98%），其次是椎动脉（40/49，81.6%），受累血管病变性质以狭窄（49/49， 100%）和闭塞（36/49，73.5%）最常见。脑梗死复发组动脉闭塞比例（100% vs 65.8%，P =0.024）和 动脉血栓发生率（45.5% vs 13.2%，P =0.033）均高于脑梗死无复发组。治疗方面，脑梗死复发组抗 血小板治疗患者比例（63.6% vs 97.4%，P =0.007）及接受血管重建术的患者比例（27.3% vs 68.4％， P =0.033）均低于脑梗死无复发组。 结论 大动脉炎合并脑梗死的患者，如合并糖尿病、血脂控制欠佳、主动脉弓分支动脉有闭塞、血 栓形成者，脑梗死复发风险高。     

Aortitis and periaortitis: The puzzling spectrum of inflammatory aortic diseases

Aortitis and periaortitis are inflammatory diseases of the aorta and its main branches; they differ in the extension of inflammation, which is confined to the aortic wall in aortitis, and spreads to the periaortic space in periaortitis. Aortitis is classified as non-infectious or infectious. Non-infectious aortitis represents a common feature of large-vessel vasculitides but can also be isolated or associated with other rheumatologic conditions. Periaortitis can be idiopathic or secondary to a wide array of etiologies such as drugs, infections, malignancies, and other proliferative diseases. Notably, both aortitis and periaortitis may arise in the context of IgG4-related disease, a recently characterised fibro-inflammatory systemic disease. Prompt recognition, correct diagnosis and appropriate treatment are essential in order to avoid life-threatening complications.     

儿童风湿病国际相关诊治指南系列解读之七——儿童大动脉炎分类标准解读

大动脉炎（TA）是由免疫机制介导的慢性、肉芽肿性、炎症性疾病，病变主要累及主动脉及其一级分支。现今儿童TA诊断多采用由欧洲抗风湿病联盟、欧洲儿童风湿病学会和儿科风湿病国际研究组织共同制定的分类标准。影像学检查是诊断TA及评估疾病活动程度的重要方法。欧洲儿童风湿病单中心及访问点所发布的共识对该标准做了进一步补充。     

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??Abstract?? Systemic vasculitis is a group of vasculitis with common inflammation of the blood vessel and involves multiple organs. It can involve all kinds of vessels of the organs??showing different clinical manifestations and lacking specificity. Through summarizing the clinical manifestation and diagnosis of some common systemic vasculitis in children??we aim to improve pediatricians’s awareness of these diseases??in order to early discover??diagnose and improve the prognosis.