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婴儿期胆汁淤积性肝病是婴儿期最常见的疾病,病因很多,可分为肝内胆汁淤积性肝病与肝外胆汁淤积性肝病。其中,胆道闭锁与肝内胆汁淤积性肝病二者鉴别诊断十分困难,但十分重要。因为胆道闭锁的预后与手术年龄相关,若在60 d以后手术,成功率即逐渐减少,预后较差。故应早期对婴儿期胆汁淤积性肝病进行鉴别诊断,此讨论中鉴别诊断方法国内可借鉴应用。         本病例临床上表现持续性黄疸,粪便浅黄色,肝脏肿大,血清γ-GT显著升高,肝胆B超、十二指肠检查、肝胆MRCP及核素肝胆显像均支持胆道闭锁的诊断。但病理检查系胆管缺如,基因检测为JAG1基因突变,可明确诊断为Alagille综合征。         此综合征诊断主要依据临床和实验室综合评估,临床上具有慢性胆汁淤积、心脏杂音、特殊面容、蝴蝶椎骨和角膜后胚胎环5项中3项,且肝组织病理检查肝内小叶间胆管数量减少或缺如者,即可诊断。本患儿仅有心脏杂音而无其他表现,最终依靠基因诊断确诊,因此,对γ-GT升高、胆汁淤积性肝病患儿应行基因检查以便早期诊断与治疗。  相似文献   

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OBJECTIVES: The purpose is to quantify objective tissue resistances before healing of prostheses used in pelvic floor surgery. PATIENTS AND METHODS: We measured tissue resistances offered to five types of meshes by four classical surgical routes. We also tested the incidence of the modification of the width of meshes on tissue resistance. This study was realized on frozen cadavers, by pull on prostheses just after implantation. Tests are realized with a dynamometer and results obtained in Newton. RESULTS: In the Retzius space, TVT offers a better resistance than IVS or LIFT meshes. We did not bring to the fore a significant difference between four routes for 1 cm wide prosthesis. The increase of width of Prolene meshes improves their resistance in tissues and thus quality of their fixation. We underscore a better resistance of the trans sacrospinous route with regard to the trans muscular one. DISCUSSION AND CONCLUSION: The increase of resistance is bound to the increase of the contact area between the prosthesis and the tissues. This increase of resistance should be taken into account in prolapse surgery: constraints are stronger than for stress incontinence. The posterior arms of meshes have to measure more than 1 cm wide and be set up through the sacrospinous ligament rather than through the elevator muscles. The type of knitting of prostheses is important and influences the resistance in tissues in the immediate postoperative time. Manufacturers should be interested in conceiving specific meshes for the tension-free fixation, with special and adapted mechanical properties.  相似文献   

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ObjectivesThis study aimed to determine the conditions and way of delivery in women with congenital uterine malformations.Patients and methodsThis retrospective study included a cohort of patients with malformed uterus giving birth in Lille University Hospital between 2000 and 2010, features such as way of delivery, labour, foetal presentation were compared with those patients without uterine malformation.ResultsPatients with uterine anomalies had significantly higher rates of breech présentation (36.51% vs 4.52%; P < 0.01) and cesarean section (55.26% vs 18.70%; P < 0.01) compared to the group of women with a normal uterus.Discussion and conclusionOur results suggest that women with uterine malformation should be informed that they have an increased risk of caesarean section due to more frequent malpresentations.  相似文献   

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Desire for child in psychotic patients: what risks for the child? Here are two very different questions. When mentally ill patients express the desire to have a child, professionals are apprehensive regarding the child's future and express eugenics temptation. Nevertheless, it is a topical question as neuroleptic drugs efficiency as well as mental health policy encourage them to live as close to normality as possible. Therefore, they have sexual relations, they have children. Since 1975, children born from psychotic mothers have been considered by the World Health Organisation (WHO) to be at high risk. This means they justify close support measures and prevention. The child runs 3 risks: a risk to develop a mental illness partly hereditary, a risk regarding his or her development, risks related to suffering, as these children are confronted with the illness of their parents. The major role played by environmental factors guide prevention behaviours; in particular towards parents-to-be psychotic adult patients and during the pregnancy, a privileged moment for preventive strategies consistent with the Perinatal Plan 2005–2007. A clinical label will provide an example of the difficulty to establish a mother-child link observed with a psychotic woman, because the imaginary child overrides the real baby. To conclude the emphasis should be put on multidisciplinary and evolutionary strategies. These imply some optimism.  相似文献   

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We report a case of 31-year-old woman presented with a chronic pelvic pain and a 3-cm left ovarian cyst on ultrasonography. At the time of laparoscopy, we observed two blue peritoneal lesions at the pelvic insertion of left round ligament. Histopathological examination of a biopsy showed a pigmentation due to a decorative tattoo of the left thigh.  相似文献   

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