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We report a case of Carmi Syndrome in a neonate. Aim: To share our lessons in diagnosis of the case of Carmi Syndrome. Case Report: Carmi Syndrome is an extremely rare autosomal recessive genetic disorder characterized the coexistence of pyloric atresia and junctional epidermolysis bullosa, and with aplasia cutis congenita in approximately 28% patients. In this case, a full-term male neonate was born to a G4P2+1L1 multipara through cesarean section delivery in hospital in a non-consanguineous marriage with 4000mL of II°meconium-stained amniotic fluid. He was found extensive skin loss over lower legs and other parts, with scattered blisters and bilateral microtia. Plain abdominal X-ray revealed a large gastric air bubble with no gas distally. The mother had an intrauterine fetal loss previously for reasons unknown. The dermatologist diagnosed the newborn with Bart Syndrome, while the pediatric surgeon diagnosed congenital pyloric atresia(CPA). The parents refused further treatment and the neonate passed away about 30 hours after birth. Outcome: The neonate passed away about 30 hours after birth. Conclusion: Lessons from this case:①.Rule out Carmi Syndrome in patients with PA, and differentiate Bart syndrome and Carmi Syndrome in patients with abnormal skin manifestations. ②. For rare and/or severe diseases, multidisciplinary teams(MDTs) should be establish. ③. Genetic counseling and prenatal diagnosis are necessary prior to subsequent childbearings. ④.Termination of pregnancy might be contemplated if certain indicators are revealed. [ABSTRACT FROM AUTHOR]

The objective of the study was to compare outcomes of women with placenta accreta spectrum(PAS) who underwent emergency cesarean hysterectomy with and without prophylactic intravascular balloon catheters. Methods Sixty-three cases who had emergency hysterectomy related to PAS disorders were retrospectively analyzed. Subjects with prophylactic intravascular balloon catheter placement plus hysterectomy were compared with those who had hysterectomy alone. The cases were divided into three groups: infrarenal aortic artery balloon occlusion (IAABC) group (who had IAABC plus hysterectomy, n = 31), internal iliac artery balloon occlusion (IIABOC) group (who had IIABOC plus hysterectomy, n = 17), and hysterectomy alone group (control group n = 15). Outcome measures were not significantly different between the IIABOC group and the control group. IAABC plus hysterectomy resulted in a better outcome than hysterectomy alone, with less amount of estimated blood loss (EBL) during cesarean section (2000 vs 3000 ml, p =.011) and 24 h after cesarean section (2010 vs 4520 ml, p =.004), less operation time(90 vs 106 min, p =.01), and a lower rate of major blood loss(42 vs 93%, p =.029). IAABC plus hysterectomy resulted in better outcomes than IIABOC plus hysterectomy, with less amount of EBL during cesarean section (2000 vs 3000 ml, p =.005) and 24 h after cesarean section (2010 vs 3000 ml, p =.035), a lower rate of major blood loss(42 vs 100%, p <.001), less operation time (90 vs 100 min, p =.038), and less fetus radiation dose（1.9 mGy vs 22.4 mGy, p <.001). IAABC was more effective in emergency cesarean hysterectomy related to PAS disorders. [ABSTRACT FROM AUTHOR]

Objective: The aim of our study was to investigate the predictors of adverse perinatal outcomes in intrahepatic cholestasis of pregnancy (ICP) with dichorionic diamniotic (DCDA) twin pregnancies.Methods: This study was a retrospective study of women diagnosed with ICP and DCDA twin pregnancies in Chengdu's women and children's central hospital. These patients were subdivided into mild and severe ICP groups according to total bile acid (TBA) level. The clinical characteristics and perinatal outcomes were collected and compared between the two groups. Logistic regression analysis was developed to evaluate predictors of adverse perinatal outcomes.Results: About 134 cases were included in the study. Eighty-four cases were in the mild ICP group, and the other 50 cases were in the severe ICP group. Level of alanine aminotransferase (ALT), aspartate aminotransferase (AST), alkaline phosphatase (ALP), total bilirubin (TBIL), and direct bilirubin (DBIL) in the severe ICP group were significant higher than those in the mild ICP group. The rate of delivery before 34 gestational weeks, meconium-stained amniotic fluid, and composite adverse neonatal outcome were higher in the severe ICP group than those in the mild ICP group. After adjusting for confounders, ICP onset gestational age (GA) <30 weeks and AST >200U/l were associated with GA at delivery <34 weeks. ALP >400U/l was an independent risk factor of meconium-stained amniotic fluid. ICP onset GA <30 weeks was an independent risk factor of composite adverse neonatal outcome.Conclusion: ICP onset GA <30 weeks, TBA >40 µmol/l, AST >200U/l, and ALP >400U/l were associated with composite adverse perinatal outcomes in ICP with DCDA twin pregnancies. For those patients with these characteristics, fetal surveillance and treatment should be enhanced. [ABSTRACT FROM AUTHOR]

Background: The purpose of this study is to investigate the role of prophylactic internal iliac artery balloon occlusion combined with uterine artery embolization (UAE) in patients with abnormally invasive placenta. Methods: Forty cases with abnormally invasive placenta who visited our hospital were analyzed retrospectively from May 2014 to September 2015. Twenty cases who had prophylactic balloon occlusion of internal iliac artery with/without UAE were in the study group, while the other 20 cases in the control group. Volume of estimated blood loss and blood transfusion, rate of hysterectomy, surgery duration, postoperative complication and length of hospitalization were compared between two groups. Results: The mean estimated blood loss in the study group (800 ml,500-1800 ml) was less than that in the control group (1875 ml, 500-7600 ml) (p = .01). Only two cases had blood transfusion in the study group, while 12 cases in the control group. No case was performed hysterectomy in the study group, while one case had the operation in the control group. There was no significant difference in surgery duration, postoperative complications and length of hospitalization between two groups. Conclusion: Prophylactic balloon occlusion of internal iliac artery combined with UAE is effective and safe for patients with abnormally invasive placenta. [ABSTRACT FROM AUTHOR]

Background: To evaluate the clinical significance of primary symptoms in women with placental abruption.Methods: A retrospective study of 273 cases of placental abruption was performed. The subjects were classified into two groups according to primary symptoms: 210 cases of the vaginal bleeding group and 63 cases of the abdominal pain group. The clinical features, maternal and neonatal outcomes were compared between two groups.Results: The incidence of preeclampsia and preterm birth in the vaginal bleeding group was significantly lower than abdominal pain group, while the incidence of premature rupture of membrane (PROM) in the former group was higher than that in the latter group. Both fetal and maternal outcomes were significantly poorer in the abdominal pain group than in the vaginal bleeding group in terms of rate of abnormal fetal heart monitoring (FHR), concealed abruption, abruption area over 50%, uteroplacental apoplexy, volume of postpartum hemorrhage, rate of blood transfusion, neonatal asphyxia and acidemia.Conclusions: Primary symptoms of placental abruption were associated with preterm birth, preeclampsia and PROM, which could predict pregnancy outcomes effectively. [ABSTRACT FROM AUTHOR]

Background: To describe the surgical technique and operative outcomes of transvaginal natural orifice transluminal endoscopic surgery (V-NOTES) for sacrocolpopexy with or without robotic surgical system in patients with pelvic organ prolapse (POP)., Methods: Patients with POP undergoing traditional transvaginal natural orifice transluminal endoscopic surgery (TV-NOTES) or robotic transvaginal natural orifice transluminal endoscopic surgery (RV-NOTES) for sacrocolpopexy performed by one surgeon from Sep 2020 to Jan 2023 in our hospital were included in this study. The baseline demographics and operative outcomes were collected and analyzed. In addition, some surgical skills were presented. The operative outcomes of V-NOTES for sacrocolpopexy performed by three beginners were also presented., Results: Eight patients who underwent TV-NOTES, and two patients who underwent RV-NOTES were included in this study. The mean operative time was 180 ± 49 min, and the estimated blood loss was 107 ± 82 ml for these ten cases. Particularly, the operative time of the two patients who underwent RV-NOTES was 275 and 132 min, while the estimated blood loss (EBL) was 100 and 50 ml respectively. During the follow-up period, no mesh exposure and recurrence were observed. In addition, five cases of TV-NOTES for sacrocolpopexy by beginners were all successfully completed., Conclusion: Both TV-NOTES and RV-NOTES appeared to be feasible and safe for sacrocolpopexy., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2023 The Authors.)