Multiple Hemolymphangioma of the Visceral Organs: A Case Report and Review of the Literature

Hemolymphangioma is a rare disease with malformation of both lymphatic and vascular vessels. Few cases of hemolymphangioma occurring in the rectum, small intestine, pancreas, esophagus, and other organs have been reported. Nevertheless, multiple hemolymphangioma of the visceral organs are extremely rare. We report a 25-year-old female with a significantly enlarged spleen full of multiple-rounded lesions. Curiously, the splenic flexure and even retroperitoneum had many lesions. The patient recovered well after splenectomy and the pathologic diagnosis of spleen was hemolymphangioma with abnormal lymphatic and blood vessels with polycystic spaces.Usually, it is hard to cure this disease. We should take much more consideration into the diagnosis, treatment, and even pathogenesis, even though it is a benign lesion.     

彩色多普勒超声对肝圆韧带架桥门-腔静脉分流术后桥血管通畅性的评价

目的 探讨彩色多普勒超声对肝圆韧带架桥门-腔静脉分流术后桥血管通畅性的观察,进而对手术疗效进行评价。方法 肝圆韧带架桥门-腔静脉分流术后病人共43例 ,用二维超声及彩色多普勒分别于术后3、12及24个月观察桥血管通畅情况,依据桥血管不同的超声表现,分为桥血管通畅组和桥血管狭窄组两组。结果 术后3个月,有41例血管通畅,2例血管狭窄;术后12个月38例血管通畅,5例血管狭窄;术后24个月30例血管通畅,13例血管狭窄,随着时间的延长桥血管发生狭窄的概率增加,术后3个月与24个月比较,P<0.05。结论 术后彩色多普勒超声监测桥血管是评价手术效果的重要手段。     

Alteration of Bone Density,Microarchitecture, and Strength in Patients with Camurati–Engelmann Disease: Assessed by HR-pQCT

Camurati-Engelmann disease (CED) is a rare autosomal-dominant skeletal dysplasia caused by mutations in the transforming growth factor-β1 (TGFB1) gene. In this study, a retrospective review of patients with CED evaluated at Peking Union Medical College Hospital in Beijing, China, between November 30, 2000 and November 30, 2020 was conducted. Data including demographic data, manifestations, and examination results were characterized. Furthermore, bone geometry, density, and microarchitecture were assessed and bone strength was estimated by HR-pQCT. Results showed the median age at onset was 2.5 years. Common manifestations included pain in the lower limbs (94%, 17/18), abnormal gait (89%, 16/18), genu valgum (89%, 16/18), reduced subcutaneous fat (78%, 14/18), delayed puberty (73%, 8/11), muscle weakness (67%, 12/18), hearing loss (39%, 7/18), hepatosplenomegaly (39%, 7/18), exophthalmos or impaired vision or visual field defect (33%, 6/18), and anemia (33%, 7/18). Twenty-five percent (4/16) of patients had short stature. Serum level of alkaline phosphatase was elevated in 41% (7/17) of patients whereas beta-C-terminal telopeptide was elevated in 91% of patients (10/11). Among 12 patients, the Z-scores of two patients were greater than 2.5 at the femur neck and the Z-scores of five patients were lower than −2.5 at the femur neck and/or lumbar spine. HR-pQCT results showed lower volumetric BMD (vBMD), altered bone microstructure and lower estimated bone strength at the distal radius and tibia in patients with CED compared with controls. In addition, total volume bone mineral density and cortical volumetric bone mineral density at the radius were negatively correlated with age in patients with CED, but positively correlated with age in controls. In conclusion, the largest case series of CED with characterized clinical features in a Chinese population was reported here. In addition, HR-pQCT was used to investigate bone microstructure at the distal radius and tibia in nine patients with CED, and the alteration of bone density, microstructure, and strength was shown for the first time. © 2021 American Society for Bone and Mineral Research (ASBMR).     

Does ablation of atrial fibrillation at the time of septal myectomy improve survival of patients with obstructive hypertrophic cardiomyopathy?

ObjectiveTo evaluate the outcomes after septal myectomy in patients with obstructive hypertrophic cardiomyopathy according to atrial fibrillation and surgical ablation of atrial fibrillation.MethodsWe reviewed patients with obstructive hypertrophic cardiomyopathy who underwent septal myectomy at the Mayo Clinic from 2001 to 2016. History of atrial fibrillation was obtained from patient histories and electrocardiograms. All-cause mortality was the primary end point.ResultsA total of 2023 patients underwent septal myectomy, of whom 394 (19.5%) had at least 1 episode of atrial fibrillation preoperatively. Among patients with atrial fibrillation, 76 (19.3%) had only 1 known episode, 278 (70.6%) had recurrent paroxysmal atrial fibrillation, and 40 (10.2%) had persistent atrial fibrillation. Surgical ablation was performed in 190 patients at the time of septal myectomy, including 148 with pulmonary vein isolation and 42 with the classic maze procedure. Among all patients, operative mortality was 0.4%, and there were no early deaths in patients undergoing surgical ablation. Over a median follow-up of 5.6 years, patients with preoperative atrial fibrillation had increased mortality (hazard ratio, 1.36; 95% confidence interval, 0.97-1.91; P = .070) after multivariable adjustment for comorbidities. When considering the impact of atrial fibrillation with or without surgical treatment, the adjusted hazard ratio for mortality in patients undergoing ablation compared with no ablation was 0.93 (95% confidence interval, 0.52-1.69; P = .824).ConclusionsAtrial fibrillation is present preoperatively in one-fifth of patients with obstructive hypertrophic cardiomyopathy undergoing myectomy and showed a trend toward higher all-cause mortality. Survival of patients undergoing septal myectomy with preoperative atrial fibrillation was similar between those who did and did not receive concomitant surgical ablation.     

儿童支气管哮喘与注意缺陷多动障碍的关系及其危险因素

近年来儿童慢性疾病与神经精神疾病的关系及其致病机制已成为儿科领域研究的热点和难点。支气管哮喘(简称哮喘)是儿童期常见的慢性疾病之一,最新基于人群的大样本报道证实哮喘与儿童最常见的神经发育障碍性疾病注意缺陷多动障碍(ADHD)之间关系密切。现就哮喘和ADHD的具体关系及其危险因素最新研究进展进行综述,以期认识二者之间的关系及其危险因素,便于哮喘患儿的长程临床管理。     

基于中医传承辅助平台挖掘中药复方专利中治疗抑郁的用药规律＊

目的 通过挖掘中药复方专利中治疗抑郁的用药规律，为中药新方研发和临床应用提供参考。方法 登录中国专利公布公告网站，检索治疗抑郁的中药复方专利数据，采用中医传承辅助平台（V2.5）建立数据库，并对其进行频次统计、关联规则、复杂网络及熵层次聚类分析。结果 纳入2003年－2018年治疗抑郁的中药复方专利343项，涉及药物685味，最常用单味药为柴胡，高频药物归经以心、肝两经为主，功效多为安神、补气、活血、理气；最常用药对为白芍-柴胡，最常用角药为当归-白芍-柴胡；置信度最高的关联规则为“甘草，当归，茯苓→柴胡”；核心药物为柴胡、白芍、郁金、茯苓、甘草等; 熵层次聚类分析得到核心组合20个，新处方10首。结论 治疗抑郁的中药复方专利中常见证型为“肝郁脾虚证”，以疏肝解郁健脾为主要治则，同时根据症状辅以安神、益气、理气、活血、燥湿、清热等治法，多以逍遥散为基础方，常配伍心、肝两经药。     

结核分枝杆菌检测技术研究进展

结核是由结核分枝杆菌引起的一种世界性疾病,为最受关注的公共卫生问题之一。虽然近年来结核病的发病率呈下降趋势,但其对人类健康造成的危害仍十分严峻。患者的及时上报是结核病防控的重要环节之一,报告率的上升有助于提高结核的治愈率以及卫生机构对结核患者的管理强度。快速、准确的诊断方法直接影响结核病的报告率,影响疾病传播和患者管理,是控制结核发病的基础。近年来,分子诊断技术发展迅速,为结核病的快速诊断提供了新的思路。分子诊断技术检测速度快,灵敏度、特异度、准确率高,绝大多数技术成本低廉,可同时对多个样本进行检测,实用性较强,具有较好的发展前景。本文对现阶段临床上实际应用的结核诊断技术与近年来出现的新型分子诊断技术进行简要概述。     

Depletion of microglia in developing cortical circuits reveals its critical role in glutamatergic synapse development,functional connectivity,and critical period plasticity

Microglia populate the early developing brain and mediate pruning of the central synapses. Yet, little is known on their functional significance in shaping the developing cortical circuits. We hypothesize that the developing cortical circuits require microglia for proper circuit maturation and connectivity, and as such, ablation of microglia during the cortical critical period may result in a long-lasting circuit abnormality. We administered PLX3397, a colony-stimulating factor 1 receptor inhibitor, to mice starting at postnatal day 14 and through P28, which depletes >75% of microglia in the visual cortex (VC). This treatment largely covers the critical period (P19-32) of VC maturation and plasticity. Patch clamp recording in VC layer 2/3 (L2/3) and L5 neurons revealed increased mEPSC frequency and reduced amplitude, and decreased AMPA/NMDA current ratio, indicative of altered synapse maturation. Increased spine density was observed in these neurons, potentially reflecting impaired synapse pruning. In addition, VC intracortical circuit functional connectivity, assessed by laser scanning photostimulation combined with glutamate uncaging, was dramatically altered. Using two photon longitudinal dendritic spine imaging, we confirmed that spine elimination/pruning was diminished during VC critical period when microglia were depleted. Reduced spine pruning thus may account for increased spine density and disrupted connectivity of VC circuits. Lastly, using single-unit recording combined with monocular deprivation, we found that ocular dominance plasticity in the VC was obliterated during the critical period as a result of microglia depletion. These data establish a critical role of microglia in developmental cortical synapse pruning, maturation, functional connectivity, and critical period plasticity.     

超声心动图对妊娠中晚期下腔静脉压迫综合征的诊断价值

目的应用超声心动图探讨妊娠中晚期下腔静脉压迫综合征的临床特征。 方法选取2016年1月至2018年11月在北京百子湾和美妇儿医院做产检的孕妇42例，依据妊娠中晚期是否有明确下腔静脉压迫症状，将孕妇分为无症状的孕组1（22例）和有症状的孕组2（20例），并纳入年龄相匹配的未妊娠健康女性14名作为对照组。采用超声心动图测量左心室舒张末期径（LVEDD）和收缩末期径（LVESD）、左心室射血分数（LVEF）、右心室基底内径、肺动脉瓣血流速度。分别在平卧位、侧卧位和直立位等不同体位下测量下腔静脉血流峰值速度，计算不同体位峰值速度比值。对以上3组间数据进行单因素方差分析，有统计学意义的采用相应组间两两比较的Bonferroni检验。采用受试者操作特征（ROC）曲线分析下腔静脉血流速度及不同体位下下腔静脉血流速度比值预测下腔静脉受压迫的价值。 结果与对照组相比，孕妇组左侧卧位和平卧位时下腔静脉血流速度明显增快，差异有统计学意义（F=16.921、21.845，P均<0.05），且有症状孕组2血流速度增快更明显。直立位时下腔静脉血流速度在3组间比较，差异具有统计学意义（P<0.05）。ROC曲线分析下腔静脉血流速度增快超过157.5 cm/s，敏感度69%，特异度23%。卧位与立位下腔静脉血流速度比值大于1.96时，诊断下腔静脉压迫征的敏感度、特异度分别为61%和83%。 结论中晚期妊娠超声心动图检测下腔静脉血流速度，以及卧位与立位下腔静脉血流速度比值诊断孕中晚期下腔静脉压迫征有重要价值。