完全型雄激素不敏感综合征2例临床特点及处理

雄激素不敏感综合征(androgen insensitivity syndrome,AIS)又称为睾丸女性化综合征(testicular feminization syndrome,TFS),是一种X连锁遗传病,是男性假两性畸形中较常见的类型,可分为完全型AIS和不完全型AIS,其原因主要是雄激素受体(androgen receptor,AR)基因的突变导致其对雄激素产生抵抗和不应答。本文回顾南京医科大学附属妇产医院2例CAIS患者的临床资料及诊疗过程,以期能进一步提高对该病的认知及诊治水平。     

“Cheese Wire” Fenestration of Dissection Intimal Flap to Facilitate Thoracic Endovascular Aortic Repair in Chronic Dissection

Thoracic endovascular aortic repair (TEVAR) for aneurysmal chronic dissection is often complicated by retrograde filling of the false lumen and dissected distal landing zone. A “cheese wire”-style fenestration of the dissection intimal flap can create a landing zone facilitating TEVAR. This technique successfully aided TEVAR in 3 patients with an average age of 57.3 years. Complications included type III endoleak requiring relining and renal artery occlusion requiring stent placement. Average duration of clinical follow-up was 19 ± 4 months. Imaging follow-up was 8 ± 10 months. All patients have survived for more than 1 year without aneurysm enlargement.     

Who should do endovascular repair of complex aortic aneurysms and how should they do them?

BackgroundCure of aneurysms which involve the aorta at the level of the visceral arteries and the thoracoabdominal segment remains a considerable surgical enterprise with a relatively high mortality and morbidity despite improvements of the surgical procedure and anesthetic technique. Fenestrated and branched endovascular stent grafts are currently available offering an attractive less invasive option especially for most frail patients. These grafts are relatively recent, technically more demanding to insert than the current stent graft for infrarenal aneurysm and besides, given the relative low frequency of the disease, they are much less used by practitioners. Thus, unconditional widespread of this sophisticated technique may not necessarily benefit patients.MethodsWe reviewed our experiences and articles regarding this concern, 1) who should perform this new technique and 2) in what kind of setting.ConclusionBased on the combined complexities of 1) patients selection, 2) proper planning and manufacturing of the graft, 3) the need for outstanding imaging and operating facilities, 4) and the required endovascular skill of physicians involved in the procedure, we feel that only highly specialized centers should be allowed to perform this complex procedure.     

Voluntary ambulation using voluntary upper limb muscle activity and Hybrid Assistive Limb® (HAL®) in a patient with complete paraplegia due to chronic spinal cord injury: A case report

Context: We sought to describe our experience with the Hybrid Assistive Limb® (HAL®) for active knee extension and voluntary ambulation with remaining muscle activity in a patient with complete paraplegia after spinal cord injury.

Findings: A 30-year-old man with complete paraplegia used the HAL® for 1 month (10 sessions) using his remaining muscle activity, including hip flexor and upper limb activity. Electromyography was used to evaluate muscle activity of the gluteus maximus, tensor fascia lata, quadriceps femoris, and hamstring muscles in synchronization with the Vicon motion capture system. A HAL® session included a knee extension session with the hip flexor and voluntary gait with upper limb activity. After using the HAL® for one month, the patient’s manual muscle hip flexor scores improved from 1/5 to 2/5 for the right and from 2/5 to 3/5 for the left knee, and from 0/5 to 1/5 for the extension of both knees.

Conclusion/clinical relevance: Knee extension sessions with HAL®, and hip flexor and upper-limb-triggered HAL® ambulation seem a safe and feasible option in a patient with complete paraplegia due to spinal cord injury.     

Spigelian hernias: a prospective analysis of baseline parameters and surgical outcome of 34 consecutive patients

Most of the papers published on spigelian hernia are either case reports or small retrospective series. In this prospective multicenter study, we aimed to outline the specific features of spigelian hernias and patients’ characteristics more clearly. Surgeons enrolled patients to be entered into the database as they diagnosed and treated the hernias at will. The baseline and surgical outcome parameters were noted in each patient. A painful mass was the main presenting complaint in half of 34 patients. Accurate preoperative diagnosis was possible in 31 patients. Open intraperitoneal mesh repair was the preferred technique. The mean hospital stay and time until return to normal daily activities were 4.1 and 15.6 days. Although a rare condition, diagnosis of a spigelian hernia is not difficult once remembered. Its surgical repair seems to cause few complications and is very well tolerated by the patient.     

睾丸鞘膜瓣在尿道修复中的应用

目的探讨睾丸鞘膜瓣覆盖技术在尿道畸形和尿道瘘修复中的效果。方法2002年起对38例尿道下裂手术和术后尿道瘘,采用睾丸鞘膜下组织蒂鞘膜瓣覆盖修复。结果术后随访半年至1年,除1例尿道上裂术后瘘修复后再次发生尿道瘘外,其余均获成功,未再出现尿道瘘或尿道狭窄,阴茎外观满意,勃起正常。结论采用该方法可有效防止尿瘘再发生,提高手术成功率且易于获取,对睾丸无不良影响。     

全腹膜外补片植入术中补片不固定的实验研究

目的评价全腹膜外补片植入术中补片不固定的安全性和有效性。方法将50只SD大鼠建立疝缺损(3cm2)模型,按随机数字表分为2组,使用聚丙烯补片(12 cm2)采用腹膜外补片植入术进行修复。甲组为补片固定组,乙组为补片不固定组。大鼠于术后不同时间分批处死,记录补片的挛缩度、腹壁抗张强度以及组织细胞学变化。结果所有大鼠术后腹腔均无粘连。2组大鼠在术后7 d、14 d、30 d、60 d和90 d补片的挛缩度分别为6.7%±1.3%vs 7.3%±1.5%、12.3%±1.3%vs 12.4%±1.6%、14.0%±2.0%vs 14.5%±1.3%、17.4%±2.1%vs 18.4%±1.5%和18.9%±2.2%vs19.5%±2.6%(P>0.05);2组大鼠的腹壁抗张强度分别为(288.4±8.4)mm Hg vs(286.6±10.2)mm Hg、(300.4±11.2)mm Hg vs(298.8±11.4)mm Hg、(305.0±11.7)mm Hg vs(303.3±16.1)mm Hg、(309.0±10.8)mm Hg vs(307.6±8.9)mm Hg和(311.8±9.8)mm Hg vs(310.0±8.6)mm Hg(P>0.05);2组大鼠的组织细胞学变化一致。结论当补片面积是大鼠疝缺损的4倍以上时,不固定的腹膜外补片植入术是安全有效的。     

膝以下严重开放性创伤的修复

[目的]研究膝以下严重开放性创伤的修复。[方法]自1995年3月～2005年3月收治膝以下严重开放性损伤153例，Ⅰ期修复106例，Ⅱ期修复47例，7种内固定方法，11种皮瓣修复方法。[结果]153例中随访132例，时间6个月～5年，平均17个月，术后功能优良率85．7％，皮瓣成活率良好。皮瓣局部坏死25例，骨不连4例，慢性感染8例，骨髓炎3例。[结论]改进的腓肠神经营养血管皮瓣和皮瓣桥手术扩大了皮瓣的应用范围，减少了并发症的发生。本研究为膝以下严重开放性损伤的修复提供了良好的方法；熟练的显微外科技术和各种组织瓣的合理应用是修复成功的关键。