彩色多普勒超声图指导风湿性心脏病二尖瓣狭窄球囊扩张术91例

目的：应用彩色多普勒超声心动图指导经皮二尖瓣球囊成形术(PBMV)治疗风湿性二尖瓣狭窄的价值、方法：运用彩超对行PBMV的患进行术前术后检查，观察二尖瓣钙化程度及部位、开口形状、瓣口面积、二尖瓣返流(MR)程度、瓣下病变、左房左室大小、左房有无血栓．结果：91例PBMV术后患中，术后MR72例，其中MR新出现19例，MR加重8例、与术前对比，术后二尖瓣开放幅度、二尖瓣口面积、二尖瓣跨瓣压、二尖瓣压差半降时间较术前有明显改善趋势，左房内径、肺动脉内径较术前有缩小趋势．同时，术后左房压、右室压及肺动脉压也有下降趋势。结论：彩超能明确PBMV治疗二尖瓣狭窄的效果。影响：PBMV效果的因素较多，但二尖瓣钙化的程度、部位和瓣膜形态较为重要。     

双能量胸部DR对筛查冠状动脉钙化的意义:与多排螺旋CT的比较性研究

目的探讨胸部双能量DR骨骼像在筛查冠脉钙化中的意义。方法35例患者分别行右前斜位胸部双能量DR检查及多排螺旋CT(MDCT)冠状动脉扫描并记录照射后射线的体表入射剂量ESD(mGy)。2位高年资放射科医师及2位低年资医师分析双能量胸片骨骼像,对LAD、LCX、RCA共105条血管的钙化情况进行评估。CT扫描图像进行钙化积分分析。以钙化积分>300为金标准,对双能量DR的结果进行ROC分析。结果DR胸片平均阳性率27.6%(29/105),其中LAD阳性率48.5%(17/35);LCX22.8%(8/35);RCA 11.4%(4/35)。4位医师的ROC结果显示曲线下面积分别为0.866、0.854、0.725、0.642。胸部DR照射平均体表入射剂量为(0.469±0.22)mGy;多排CT入射剂量为(12.29±1.40)mGy。两者经配对t检验有显著差异。结论DR骨骼片可以检出较明显的冠状动脉钙化,对冠心病的筛查和病情的检测有一定的临床意义。     

主动脉瓣机械瓣置换术后交替脉

报道４例因主动脉瓣关闭不全行主动脉瓣置换术后的患者在术后早期出现脉搏交替现象，此时心电图为整齐的窦性心律，所记录的脉搏图形高低交替，如扪诊桡动脉时则会发现桡动脉搏数为心电图示心率的一半。患者术后均顺利恢复，显示这一现象为一过性的、良性的过程。推测这一现象出现的原因可能是术后早期主动脉瓣不再有回流，因而巨大的左室腔充盈不足，加之左室收缩力尚低，不能在每次收缩时都能射出同等量的血流所致     

糖尿病肾病患者血清TRPM7、Sirtuin-1与钙磷代谢、颈动脉钙化的相关性

目的 探讨糖尿病肾病（DN）患者血清瞬时受体电位通道7(TRPM7)、沉默调节蛋白-1(Sirtuin-1)与钙磷代谢、颈动脉钙化的相关性。方法 选取2020年12月—2022年11月成都大学附属医院收治的97例DN患者作为DN组,另取同期在该院就诊的120例2型糖尿病患者作为对照组。采用实时荧光定量聚合酶链反应检测血清TRPM7的表达,酶联免疫吸附试验检测血清Sirtuin-1水平。采用Pearson法分析DN患者血清TRPM7、Sirtuin-1水平与钙磷代谢的相关性,并通过多因素Logistic逐步回归模型分析DN患者颈动脉钙化的危险因素。结果 DN组血肌酐、尿素氮、血清TRPM7、血磷、颈动脉钙化率高于对照组（P <0.05）。DN组Sirtuin-1、血钙低于对照组（P <0.05）。Pearson相关性分析结果显示,DN患者血清TRPM7与血钙呈负相关（r=-0.247,P=0.000）,与血磷呈正相关（r=0.415,P=0.000）;DN患者血清Sirtuin-1与血钙呈正相关（r=0.367,P=0.000）,与血磷呈负相关（r=-0.505,P=0.00...     

Takayasu arteritis: Follow-up studies for 20 years

We reviewed retrospectively 126 (5 male, 121 female) patients suffering from Takayasu arteritis who had been treated in our clinics from 1971 to 1990. The patients' ages ranged from 19 to 80yrs old (1990) with a mean age of 48.7 ± 11.8 years. HLA typing analysis in 98 patients revealed that 45 patients (47%) were confirmed as carrying the Bw52 antigen, a high result that is statistically significant as compared with that in healthy Japanese. Arteriograms (performed in 75 patients) revealed that 28 patients (37%) were affected in the aorta and its main branches by this disease (type IV by Nasu's classification) and 23 patients (31%) were affected only in the main branches (type I). The C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) improved significantly from 2.55 ± 0.28(+) and 57.0 ± 5.69 mm/hr to 0.53 ± 0.12(+) and 31.2 ± 3.45 mm/hr, respectively after treatment including steroid and antiplatelet therapy (P < 0.01).=" patients=" with=" bw52=" exhibited=" more=" severe=" inflammatory=" conditions=" than=" those=" without=" bw52.=" lung=" scintillations=" performed=" in=" 81=" patients=" showed=" pulmonary=" arterial=" lesions=" in=" 50=" patients=" (62%).=" echocardiograms=" revealed=" aortic=" regurgitation=" (ar)=" in=" 44=" patients=" (35%),=" with=" a=" significant=" difference=" noted=" between=" the=" bw52=" positive=" group=" and=" the=" bw52=" negative=" group=" [29/40=" (73%)=" versus=" 11/47=" (23%),=">P < 0.001].=" patients=" with=" bw52=" were=" prescribed=" higher=" doses=" of=" steroids=">P < 0.05)=" for=" longer=" periods=">P < 0.01)=" than=" those=" without=" bw52.=" of=" 11=" patients=" who=" died=" during=" our=" study=" period,=" 7=" died=" of=" cardiac=" complications,=" all=" of=" whom=" were=" suffering=" from=" ar.=" hla=" analysis=" performed=" in=" 6=" of=" these=" 7=" patients=" revealed=" that=" all=" carried=" the=" bw52=" antigen.=" in=" conclusion,=" the=" retrospective=" survey=" revealed=" that=" patients=" carrying=" the=" bw52=" antigen=" showed=" more=" severe=" inflammatory=" conditions=" and=" progressed=" more=" rapidly=" to=" complications=" and=" the=" fatal=" morbid=" condition,=" as=" compared=" with=" those=" without=" bw52.=" this=" suggests=" the=" important=" role=" of=" gene=" disequilibrium=" with=" this=" hla=">     

Clinical significance of intraoperative cultures of aneurysm walls and contents in elective abdominal aortic aneurysmectomy

To investigate the clinical significance of intraoperative cultures in elective abdominal aortic aneurysmectomy, we cultured the aneuryrymml wall and contents in 90 patients undergoing vascular graft surgery. Prosthetic graft infection was documented in 1 out of 62 patients with negative cultures and in none of 28 patients with positive cultures (x² = 0.4, p > 0.1). Bacterial growth was seen in neither of 2 inflammatory aneurysms, 3 of 14 atherosclerotic aneurysms and 2 of 5 aneurysms without specific features. A retrospective analysis of patients' charts aimed at finding possible risk factors failed to identify any correlation between results of cultures and length of hospitalization before surgery, time interval between angiography and surgery, route of angiography procedure or minutes of surgery before sample collection. We conclude that positive cultures may not imply clinical infection at the time of surgery and that prolonged post-operative organism-specific antibiotic therapy does not appear necessary to prevent graft contamination in patients undergoing elective abdominal aortic aneurysmectomy.     

以造影增强磁共振血管成像术诊断先天性主动脉弓畸形

目的评价造影增强磁共振血管成像术（CE-MRA）对先天性主动脉弓畸形的诊断准确性并与超声心动图等比较。方法413例先天性主动脉弓畸形病例在1999年4月到2004年12月期间做了CE-MRA检查。年龄5d～11岁，平均年龄2.4岁。检查使用1.5T磁共振扫描仪。对比剂gadolinium-DTPA，剂量0.2mmol／kg体质量，手动静脉注射。磁共振检查前均已做心脏超声检查。结果在413例中，动脉导管未闭（PDA）166例，主动脉缩窄（COA）168例，主动脉弓中断（IAA）31例，双主动脉弓等其他先天性主动脉弓畸形48例。全部413例均经手术和／或X线心血管造影证实。CE-MRA的诊断准确率为97.3％（402／413），心脏超声检查的诊断准确率为87.2％（360／413）。结论对于先天性主动脉弓畸形，CE-MRA是可靠的影像学检查方法，不仅优于心脏超声检查，有时CE-MRA甚至能比传统的X线心血管造影术提供更多的诊断信息。     

主动脉弓畸形及其临床意义

目的研究主有及其临床意义。方法：在历时１５年对３２０例主动脉弓的解剖中,发现４例主动脉弓畸形,并对此弓变形及其周围诸结构进行解剖和观察。结果：在４例主动脉弓畸形的病例,,２例为右食管后锁骨下动脉并存右非喉返神经,另２例为右主工存左食管后锁骨下一左背主动脉分离。食管后锁上动脉起始部形成憩中产生食管受压性咽下困难,左锁骨下动脉或左背主动脉经支汛管连接或发自左肺动脉,可形成先天性肺动脉一锁骨下动脉窃血症     

Identification of the three-element windkessel model incorporating a pressure-dependent compliance

A new one-step computational procedure is presented for estimating the parameters of the nonlinear three-element windkessel model of the arterial system incorporating a pressure-dependent compliance. The data required are pulsatile aortic pressure and flow. The basic assumptions are a steadystate periodic regime and a purely elastic compliant element. By stating two conditions, zero mean flow and zero mean power in the compliant element, peripheral and characteristic resistances are determined through simple closed form formulas as functions of mean values of the square of aortic pressure, the square of aortic flow, and the product of aortic pressure with aortic flow. The pressure across as well as the flow through the compliant element can be then obtained so allowing the calculation of volume variation and compliance as functions of pressure. The feasibility of this method is studied by applying it to both simulated and experimental data relative to different circulatory conditions and comparing the results with those obtained by an iterative parameter optimization algorithm and with the actual values when available. The conclusion is that the proposed method appears to be effective in identifying the three-element windkessel even in the case of nonlinear compliance.     

A novel de novo PDGFRB variant in a child with severe cerebral malformations,intracerebral calcifications,and infantile myofibromatosis

The spectrum of clinical consequences of variants in the Platelet derived growth factor receptor beta (PDGFRB) gene is wide. Missense variants leading to variable loss of signal transduction in vitro have been reported in the idiopathic basal ganglia calcification (IBGC) syndrome Type 4. In contrast, gain‐of‐function variants have been reported in infantile myofibromatosis, Penttinen syndrome, and Kosaki overgrowth syndrome. Here, we report a patient harboring a novel postzygotic variant in PDGFRB (c.1682_1684del, p.[Arg561_Tyr562delinsHis]) and presenting severe cerebral malformations, intracerebral calcifications, and infantile myofibromatosis. This observation expands the phenotype associated with PDGFRB variants and illustrates the wide clinical spectrum linked to dysregulation of PDGFRB.