Percutaneous transhepatic stenting by wallstents of portal vein and bile duct stenoses caused by immunoblastic sarcoma in a liver transplantation

Posttransplant lymphoproliferative disorders are infrequent tumors related to chronic immunosuppressive therapy. We present a liver transplant recipient who developed such a tumor in the porta hepatis that provoked obstruction of the entire portal triad. Treatment consisted of systemic chemotherapy, percutaneous dilatation, and placement of Wallstent endoprostheses across both biliary and portal vein stenoses. The patient died 3 weeks later of pneumonia and sepsis. At necropsy, the tumor was completely necrosed and the prostheses in both the common bile duct and the portal vein were patent.     

氟骨症性椎管狭窄症的手术治疗

１９８７～１９９０年手术治疗氟骨症性椎管狭窄症１００例。包括颈椎９例，胸椎８例，腰、骶椎８３例。术后随访１～４年，复查结果：临床治愈９３例（９３％）：显效３例（３％）、有效３例（３％）、无效１例（１％）。手术原则是彻底减压，因氟骨症具有椎骨及其骨周组织严重退变、异化和骨化的临床病理特点，故在胸、腰椎减压术后，一般不影响其稳定性，在颈椎施行双开式或单开式椎管扩大加植骨融合术，则可收减压、稳定及防止或减少椎板间隙瘢痕狭窄的效果。     

椎板后移回植的椎管扩大术治疗椎管狭窄症

作者报告棘突劈开、椎板后移回植的椎管扩大术治疗椎管狭窄症１８２例，使椎管容积扩大３４．８％，横径扩大３．８ｍｍ，矢径扩大６．１ｍｍ。随访２年９个月，优良率８６．６％，椎板愈合率９６．９％。本文探讨少数椎板迟延愈合的原因和预防措施，如斜切椎板、缝合固定和腰背筋膜与棘上韧带悬吊缝合等。术后卧床１０～１２周，用石膏型保护。作者认为本法能有效的预防术后脊柱不稳，顽固性腰痛等并发症。     

腰椎间盘突出合并椎管狭窄的手术治疗

报告手术治疗腰椎间盘突出症合并退行性腰椎管狭窄症１０４例。术后随访９０例，随诊时６个月～６年。优良率８６．５％，无腰椎不稳和腰椎滑脱等并发症。为获得优良效果，作者强调在处理椎间盘突出的同时必须彻底解除侧隐窝及神经根管，黄韧带肥厚对神经根的压迫，才能彻底根除症状     

Molecular breakpoint analysis and relevance of variable mosaicism in a woman with short stature,primary amenorrhea,unilateral gonadoblastoma,and a 46,X,del(Y)(q11)/45,X karyotype

Congenital hydrocephalus associated with aqueductal stenosis and/or agenesis of the corpus callosum has been described in newborn males with mutations in L1CAM, a gene that encodes a neural cell adhesion molecule. These males usually have severe mental retardation and may have spastic paraplegia and adducted thumbs. In contrast, Hirschsprung disease, or absence of ganglion cells in the distal gut, has rarely been described in such individuals. We report a male infant who had severe hydrocephalus identified in the prenatal period with evidence of aqueductal stenosis and adducted thumbs at birth. He developed chronic constipation, and rectal biopsy confirmed the diagnosis of Hirschsprung disease. Molecular testing of the L1CAM gene revealed a G2254A mutation, resulting in a V752M amino acid substitution. A common polymorphism in RET, but no mutation, was identified. Our patient represents the third example of coincident hydrocephalus and Hirschsprung disease in an individual with an identified L1CAM mutation. We hypothesize that L1CAM‐mediated cell adhesion may be important for the ability of ganglion cell precursors to populate the gut, and that L1CAM may modify the effects of a Hirschsprung disease–associated gene to cause intestinal aganglionosis. © 2002 Wiley‐Liss, Inc.     

胸主动脉夹层动脉瘤腔内隔绝术手术入路的探讨

目的 探讨为胸主动脉夹层动脉瘤(TAD)行腔内隔绝术(EVE)选择合适的导入动脉。方法 以彩超，CTA或MRA为检查手段，评估导入动脉(股动脉、髂总动脉、腹主动脉下段等)的直径大小(≥8mm)、有否硬化斑块、狭窄、是否被夹层累及、有否扭曲及其程度，从而选择具体的手术入路。结果 本组37例TAD行EVE术所选择经股动脉手术入路23例、经髂总动脉手术入路14例。未选择经腹主动脉下段手术入路。即时操作成功率为100％。结论 合理的选择导入动脉作为手术入路，是EVE手术治疗TAD顺利完成的要点。     

Turbulent stress measurements downstream of three bileaflet heart valve designs in pigs

Objective: Mechanical heart valves can cause thromboembolic complications, possibly due to abnormal flow patterns that produce turbulence downstream of the valve. The objective of this study was to investigate whether three different bileaflet valve designs would exhibit clinically relevant differences in downstream turbulent stresses. Methods: Three bileaflet mechanical heart valves (Medtronic Advantage^®, CarboMedics© Orbis™ Universal and St. Jude Medical^® Standard) were implanted into 19 female 90 kg pigs. Blood velocity was measured during open chest conditions in the cross sectional area downstream of the valves with 10 MHz ultrasonic probes connected to a modified Alfred^® Pulsed Doppler equipment. As a measure of turbulence, Reynolds normal stress (RNS) was calculated at three different cardiac output ranges (3–4, 4.5–5.5, 6–7 L/min). Results: Data from 12 animals were obtained. RNS correlated with increasing cardiac outputs. The highest instantaneous RNS observed in these experiments was 47 N/m², and the mean RNS taken spatially over the cross sectional area of the aorta during systole was between 3 N/m² and 15 N/m². In none of the cardiac output ranges RNS values exceeded the lower critical limit for erythrocyte or thrombocyte damage for any of the valve designs. Conclusions: Reynolds normal stress values were below 100 N/m² for all three valve designs and the difference in design was not reflected in generation of turbulence. Hence, it is unlikely that any of the valve designs causes flow induced damage to platelets or erythrocytes.     

代谢综合征患者肾动脉狭窄的SCTA与DSA对比研究

目的探讨代谢综合征(MS)患者中肾动脉狭窄的发生率及无创性筛检的必要性与可靠性。方法45例MS患者预行肾动脉螺旋CT血管造影(SCTA),对所检出的14例肾动脉狭窄者行肾动脉数字减影血管造影(DSA)对照,并对SCTA及DSA结果进行对比分析。结果45例MS患者中SCTA显示肾动脉正常31例,其余14例患者存在单侧或双侧肾动脉狭窄,DSA对照显示肾动脉正常6条,肾动脉狭窄22条,与SCTA显示的情况基本符合。结论MS患者中肾动脉狭窄的发生率约31.1%,对可疑患者应常规予以肾动脉SCTA检查。     

主动脉夹层50例临床分析

目的 探讨主动脉夹层(AD)的临床表现，以便早期诊断和治疗，降低死亡率。方法 对50例AD患者的临床表现、影像学检查、治疗经过及死亡原因进行回顾性分析。结果 50例行心脏超声检查，确诊者43例(90％)；21例行CT检查。21例诊断为主动脉夹层(100％)；行磁共振显像(MRI)27例，均确诊为主动脉夹层(100％)。经治疗好转出院42例(84％)，1例并发脑梗死且留有一侧肢体活动障碍后遗症。7例死亡(占14％)。结论 早期诊断，有效治疗是降低死亡率的关键，CT、MRI可提供可靠的确诊依据。