congenital defect

  • 文章类型: Journal Article
    背景:胆总管囊肿是一种罕见的先天性异常,表现为胆道树的囊性扩张。这项研究展示了我们在青少年和成人中巨大胆总管囊肿的5年经验,主要关注其临床表现,手术挑战,务实的解决方案。
    方法:对58名在三级医院接受治疗的青少年和成人胆总管囊肿患者进行了回顾性观察研究。巨大胆总管囊肿定义为最大直径≥10cm的囊肿。人口概况,临床表现,并对这些患者的手术结果进行分析。
    结果:共有12例巨大胆总管囊肿患者在我科进行了为期5年的治疗。巨大胆总管囊肿患者的中位年龄为20岁(范围,13-30岁),男女比例为1:2。巨大的胆总管囊肿更有症状,12个中有8个出现经典的腹痛三联征,质量,和黄疸。一名巨大胆总管囊肿患者患有转移性胆管癌。11例患者接受了手术囊肿切除术。巨大囊肿的手术具有挑战性,需要进行技术修改以进行安全切除。
    结论:巨大胆总管囊肿是一种罕见的实体,在生命的第一个十年后很少遇到。这些囊肿提出了独特的手术挑战,需要修改标准手术技术。
    BACKGROUND: Choledochal cyst is a rare congenital anomaly manifesting as cystic dilatation of the biliary tree. This study presents our 5-year experience with giant choledochal cyst in adolescents and adults, focusing primarily on its clinical presentation, operative challenges, and pragmatic solutions.
    METHODS: A retrospective observational study was conducted on 58 adolescent and adult choledochal cyst patients who were managed at a tertiary care hospital. Giant choledochal cyst is defined as cyst with a maximum diameter of ≥ 10 cm. Demographic profile, clinical presentation, and surgical outcome of these patients were analyzed.
    RESULTS: A total of 12 patients with giant choledochal cyst were managed in our department in a duration of 5 years. The median age of patients with giant choledochal cyst was 20 years (range, 13-30 years) and male to female ratio was 1:2. Giant choledochal cysts were more symptomatic and 8 out of 12 presented with classic triad of abdominal pain, mass, and jaundice. One patient with giant choledochal cyst had metastatic cholangiocarcinoma. Eleven patients underwent surgical cyst excision. Surgery of the giant cyst was challenging and required technical modifications for safe excision.
    CONCLUSIONS: Giant choledochal cyst is an unusual entity that is rarely encountered beyond first decade of life. These cysts pose unique surgical challenges and require modification of the standard operative technique.
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  • 文章类型: Journal Article
    Despite the developmental toxicity reported in animals, few epidemiologic studies have investigated the potential effects of prenatal exposure to pyrethroid pesticides (PYRs) on fetal growth. A birth cohort study was conducted to examine the association between prenatal exposure to PYRs and birth outcomes, and a nested case-control study was conducted in this cohort to evaluate the effects of PYR on congenital defects. The assessment of PYR exposure was based on self-reported household pesticide use and urinary PYR metabolite levels. We found that pregnant women in this region were ubiquitously exposed to low-level PYRs, although few reported household pesticide use. Women who often ate bananas or cantaloupes had a higher level of urinary 3-(2,2-dibromovinyl)-2,2-dimethylcyclopropane-1-carboxylic acid (DBCA), and the number of fruit types consumed by pregnant women was positively related to the concentrations of 3-phenoxybenzoic acid (3PBA) and total PYR metabolites (P < 0.05). Increased urinary 4-fluoro-3-phenoxybenzoic acid (4F3PBA), DBCA, and total PYR metabolites were associated with increased birth weight, length, and gestational age, and with decreased risk of small for gestational age (SGA) and/or premature birth. However, maternal household pesticides use was related to congenital anomalies. Thus, although prenatal exposure to low-dose PYRs promoted the fetal growth, the beneficial effects of fruit intake may outweigh the adverse effects of pesticide exposure. This study provided us an insight into the biological mechanisms for the effect of prenatal PYR exposure on fetal development, and suggested that further investigations in a larger study population with low-dose PYR exposure is needed.
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