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.     

面部色素痣的外科治疗

面部色素痣较常见于鼻、颊、额、颧区，部分病变累及一侧面部的多个区域。这些病变手术切除后的缺损主要采用全厚皮片移植、直接拉拢缝合或邻近皮瓣整复。     

原发性色素性结节状肾上腺皮质病(附4例报告)

目的：探讨原发性色素性结节状肾上腺皮质病(PPNAD)的临床表现和诊断治疗方法。方法：总结4例PPNAD的临床资料，4例均有库欣综合征的临床表现，内分泌检查结果提示为功能自主性肾上腺皮质肿瘤，但影像学检查并未发现肾上腺肿瘤。结果：4例患者均行单侧肾上腺全切除术，手术标本均表现为肾上腺大小正常或轻度增大，外表和切面上见黑色或深褐色小结节，结节间皮质萎缩，光镜下见组成结节的细胞体积大，脑质嗜伊红染色、颗粒状，部分细胞脑质中的颗粒状色素颗粒具脂褐质染色特征。结论：PPNAD在青少年中是一种引起库欣综合征的罕见病因，双侧肾上腺切除术是治愈本病的方法。     

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

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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).     

Relative frequency of solitary melanocytic lesions of the oral mucosa

Background: Solitary pigmented lesions of melanocytic origin are uncommon in the oral mucosa. These lesions include the oral and labial melanotic macule, oral melanocytic nevus, oral melanoacanthoma, oral melanoma and atypical melanocytic proliferation. The purpose of the study was twofold: to report a large series of solitary melanocytic lesions from one source, and to determine the relative frequency of these lesions. Methods: The study was based on a systematic search of the files of the Pacific Oral and Maxillofacial Pathology Laboratory, University of the Pacific, San Francisco for solitary pigmented melanocytic lesions (benign and malignant) accessed during the years 1984–2002. Results: Of the 89 430 biopsies accessed during the 19‐year period, 773 (0.83%) cases of solitary pigmented melanocytic lesions in the oral mucosa were identified. Oral and labial melanotic macules were the most common melanocytic lesions comprising 86.1% of the entire group and 0.7% of the total number of accessed biopsies. The vermilion border and gingiva were the most common sites (31.1% and 31.0% respectively). Oral melanocytic nevi comprised 11.8% of the entire melanocytic group and 0.1% of the total number of biopsies. The most common site was the palate (44%). Intramucosal nevi were the most common (64%), followed by compound nevi (16.5%) and common blue nevi (16.5%). Junctional nevi were uncommon (3.0%). Oral melanoacanthoma comprised only 0.9% of the entire melanocytic group and 0.008% of the total number of biopsies. Oral melanoma and atypical melanocytic proliferation were the least common lesions each comprising 0.6% of the entire melanocytic group and 0.006% of the total number of biopsies. The most common site for oral melanoma was the palate (60%). Conclusion: The palate was the most common location for both melanocytic nevi and oral melanoma. Thus, all melanocytic lesions in the palate should be viewed with caution and biopsy is recommended to rule out melanoma. Further studies are required to elucidate the entity of oral atypical melanocytic proliferation.