Le syndrome de Klinefelter : une prédisposition au crime sexuel ?

BackgroundSeveral studies have reported over-representation of psychiatric disorders among patients with Klinefelter’ Syndrome (KS), with forensic complications.ObjectiveConsider determinants of sexual assault in patient with KS.ReviewIn this work, we present the case of Jules, 23 years old, with KS, benefiting from steroid replacement therapy, convicted of rape of a minor and evaluated in this context. We question here the role of his genetic pathology and of his hormonal treatment in this sexual assault.FindingsAccording to evidence from the literature, it is not possible to determine with certainty the fair value of each factor and their impact on the occurrence of the sexual criminal act. Indeed, although the crime rate among KS subjects is higher than in the general population, the majority of them have never been in trouble with the law; moreover, these subjects were no more likely to commit violent sexual acts than were criminals without KS. As for hormonal treatment, it seems that testosterone is better viewed as a facilitator of initiating an aggressive response than as a primary inductor.ConclusionIn conclusion, the onset of sexual violence that accompanied the introduction of hormonal treatment into a patient with KS suggests an effective involvement of steroid replacement therapy, even small, in the criminal act. This must incite clinicians to extreme prudence and to take account of multidisciplinary expertise (psychiatrist, endocrinologist) in order to reconsider the continuation of the treatment in this particular forensic context. Finally, we discuss other factors that can precipitate such a violent act.     

Acro-pectoro-renal field defect with contralateral ureteropelvic junction obstruction

Ureteropelvic junction obstruction was noted in a newborn male infant with acro-pectoro-renal field defect. To our knowledge, this association has not previously been reported. Ultrasonography of the urinary tract should be performed on all children with aplasia of the pectoralis major muscle.     

Management of congenital tracheal stenosis

Objectives: Congenital tracheal stenosis is a rare disease. Various methods for treatment exist but there is still much debate as to the appropriate surgical procedure. We present our surgical experiences of patch tracheoplasty and slide tracheoplasty as viable methods for the treatment of congenital tracheal stenosis. Methods: From 1994 to 2002, 13 patients were diagnosed with congenital tracheal stenosis. Eight patients (7 symptomatic and 1 asymptomatic) had their stenosis corrected, three by means of pericardial patch tracheoplasty, four by slide tracheoplasty, and one by resection and anastomosis. Concomitant operations were performed on six patients to treat congenital cardiovascular disease. Five patients showing no significant symptoms did not undergo tracheal surgery and received only cardiac procedures. A retrospective review of the hospital course, complications, and long-term results was conducted. Results: Among the patch tracheoplasty group, every patient suffered from granulation tissue formation. One patient died of respiratory acidosis and one was hospitalized due to recurrent granulation tissue, which required frequent bronchoscopy. The third patient from this group is free of all symptoms. Among the slide tracheoplasty group, one patient died of anastomosis disruption. The three remaining patients are alive and well. The one patient who received resection and anastomosis is alive without symptoms. Conclusions: Surgical repair of long-segment congenital tracheal stenosis exhibited high mortality and morbidity rates. Every patient that underwent pericardial patch tracheoplasty suffered from troublesome granulation tissue. As slide tracheoplasty provided relatively good results in the short and mid-term follow-up periods, it seems to be a preferred method for the treatment of long-segment congenital tracheal stenosis.     

左主干起源于右冠状动脉窦（附2例报告并文献复习）

目的:分析左主干起源于右冠状动脉窦的临床特征,提高对左主干起源于右冠状动脉窦的认识和诊断.方法:回顾分析2例左主干起源于右冠状动脉窦的临床病例并对文献进行回顾分析.结果:左主干起源于右冠状动脉窦是一种罕见的先天性冠脉畸形,这种畸形可分为良性或恶性,主要根据异常左冠状动脉与主动脉和肺动脉之间的关系.对于可能引起猝死或严重心肌缺血患者,应进行冠状动脉旁路移植术.2例患者左主干起源于右冠状动脉窦,其中1例左主干走行于主动脉与肺动脉之间,1例走行于主动脉前方,这2例未进行预防性外科手术,目前预后良好.结论:左主干起源于右冠状动脉窦是罕见的冠脉畸形,应注意鉴别是良性或是恶性,确定下一步治疗方案.     

The Significance of Anatomical Variations of Hepatic Artery and Multiple Arterial Supplies in Embolization of Liver Tumors

(胡国栋)(李小明)(黄志程)(胡道予)ＴｈｅＳｉｇｎｉｆｉｃａｎｃｅｏｆＡｎａｔｏｍｉｃａｌＶａｒｉａｔｉｏｎｓｏｆＨｅｐａｔｉｃＡｒｔｅｒｙａｎｄＭｕｌｔｉｐｌｅＡｒｔｅｒｉａｌＳｕｐｐｌｉｅｓｉｎＥｍｂｏｌｉｚａｔｉｏｎｏｆＬｉｖｅｒＴｕｍｏｒｓ￥ＨＵ...     

Histopathology of the teeth in segmental odontomaxillary dysplasia: new findings

Histological examination of the deciduous teeth in two cases of segmental odontomaxillary dysplasia (SOMD) showed fibrous enlargement of the pulps, an irregular pulp/dentine interface displaying many pseudoinclusions and pulp stones. There were tubular defects in the coronal dentine from pulp horn to cusp tip, an irregular tubular structure to the circumpulpal dentine of the apical half, a focally deficient odontoblast layer and widespread external resorption. Together with the clinical features of unilateral maxillary enlargement, upper alveolar expansion in the distal segment, increased spacing and delayed eruption of the deciduous molars and absence of premolar teeth, these histological appearances allow distinction of this condition from fibrous dysplasia (FD), segmental hemifacial hypertrophy (SHH) and regional odontodysplasia (ROD).     

The assimilated os sustentaculi

The assimilated os sustentaculi is a projection of bone from the medial aspect of the sustentaculum tali which is surmounted by a similar projection from the medial tubercle of the talus. The accessory joint so formed may develop secondary osteoarthritis changes. This congenital anomaly is very rate. The present report is of 2 additional cases.     

Ebstein畸形82例心血管造影分析

报道我院1958～1992年期间的 Ebstein 畸形82例。平片表现可归纳为球型、方型和不典型4种心脏形态。介绍该畸形的心血管造影方法,所见征象和相应的病理基础。主要造影征象有:1.心脏膈面双切迹征;2.帆样征:3.房化心室:4.三尖瓣闭锁不全。本文结合文献对本病病理,X 线表现进行了系统的讨论,并着重突出了我们的观点。     

椎动脉形态学异常对后循环短暂性脑缺血发作症状持续时间的影响

目的 探讨椎动脉形态学异常对后循环TIA症状持续时间的影响。
方法 回顾性分析2015年10月-2018年3月在中国科学院大学重庆仁济医院神经内科完成头颈部DSA
或CTA的后循环TIA住院患者临床资料，根据症状持续时间分为<10 min组、10～59 min组和≥60 mi n组，
比较椎动脉形态异常各亚型在3组间的差异。通过多因素Logistic回归分析明确椎动脉形态异常与后
循环TIA症状持续时间之间的关系。
结果 共纳入237例患者，其中症状持续时间<10 min组109例（45.99%）、10～59 min组71例（29.96%）
和≥60 min组57例（24.05%）。一般临床资料的比较显示，性别、椎动脉和基底动脉狭窄率≥50%
在3组间差异具有统计学意义（P＜0.05）。椎动脉形态异常中，动脉粥样硬化性椎动脉狭窄（狭窄
率1%～99%）在3组间的差异具有统计学意义（P =0.004），而一侧迂曲、双侧迂曲、一侧优势、一
侧优势合并迂曲和起源异常在3组间差异均无统计学意义；多因素Logi sti c回归分析显示椎动脉
狭窄（OR 2.500，95%CI 1.381～4.525，P =0.002）、基底动脉狭窄率≥50%（OR 12.066，95%CI
1.446～100.668，P =0.021）是TI A症状持续时间延长的独立影响因素。
结论 椎动脉狭窄和基底动脉狭窄率≥50%是影响后循环TIA症状持续时间延长的独立影响因素。