Amniotic fluid peptides predict postnatal kidney survival in developmental kidney disease

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头颈部脉管性疾病的诊断与治疗

脉管性疾病包括血管瘤和脉管畸形,约60％发生于头颈部.该病虽然治疗方法较多,但由于其发病机制不甚明了,临床治疗仍面临挑战.疾病分类对治疗方法的选择起决定性作用,基于文献和我们的经验,本课题组将脉管性疾病进行分类,提出综合序列治疗的理念及其治疗原则.相关研究成果获上海市科技进步一等奖和国家科技进步二等奖.     

Cervicovaginal agenesis treated with modified Yang-Monti technique in two steps: Case report and literature review

IntroductionMüllerian duct anomalies are rare with less than 200 cases published in the literature. Recently, the implementation of the Yang-Monti principle for the creation of the neovagina has been used in 10 previous published cases. Here, we report the first case of cervicovaginal agenesis treated with the modified Yang-Monti technique in two steps in México.Presentation of caseA 14-year-old female presented to the pediatric consult with a history of primary amenorrhea and a chronic-cyclic pelvic pain. She had normal external genitalia and secondary sexual characteristics, with a small vaginal pouch. Pelvic ultrasonography and magnetic resonance showed the absence of cervix and proximal vagina. The surgical approach was carried out in two steps. In the first, a neovagina was created with the modified Yang-Monti technique. On a second step one year after, the neovaginal dome was anastomosed with the uterus in a diamond shape, using Gore-tex^® in the cervicovaginal anastomosis, and a Foley catheter to prevent stenosis. After an 18-month follow-up, no complications have been observed. She has been taking Drospirenone and Ethinyl Estradiol with regular menstrual cycles.DiscussionThe surgical treatment of cervicovaginal agenesis has evolved. The advantages of the modified Yang-Monti technique lie in the possible diminution of the tension on the vascular pedicle, and the gained length of the neovagina. To perform this procedure in two steps, likely diminishes the risk of neovaginal ischemia and leakage of the anastomosis.ConclusionThe two-steps modified Yang-Monti technique represents a safe alternative for the management of cervicovaginal agenesis.     

Clinical evaluation of anomalous aortic origin of a coronary artery (AAOCA)

The clinical evaluation of patients with an anomalous aortic origin of a coronary artery (AAOCA), a congenital abnormality of the origin or course of a coronary artery that arises from the aorta, is challenging given its first presentation being sudden cardiac arrest in about half of the patients. Symptoms of chest pain, shortness of breath and syncope during exertion should be of concern in evaluating young athletes and nonathletes. The lack of abnormal signs on the physical exam and electrocardiogram further adds to the difficulty in establishing the diagnosis. Additional imaging with echocardiography, computed tomography angiography and/or cardiac magnetic resonance imaging (MRI) is often needed and establishes the diagnosis. High‐risk lesions include origin of the coronary artery from the opposite sinus of Valsalva, intramural course and ostial abnormalities (stenosis, hypoplasia). Functional studies should be performed to assess myocardial perfusion at rest and during stress, such as nuclear imaging, stress echocardiography and stress cardiac MRI.