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31.
46,XY disorders of sex development and congenital diaphragmatic hernia: A case with dysmorphic facies,truncus arteriosus,bifid thymus,gut malrotation,rhizomelia, and adactyly
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《Respiratory investigation》2014,52(3):203-208
An elderly woman diagnosed with multiple myeloma (MM) in 2007 had improved with chemotherapy. She had severe kyphosis and a diaphragmatic hernia (DH), but no respiratory symptoms. In 2011, because of thoracic deformity and emaciation, we advised her to continue the previously prescribed domiciliary noninvasive positive pressure ventilation (NPPV) therapy for chronic type II respiratory failure. However, she refused to continue NPPV. She was later admitted for deterioration in respiratory status and carbon dioxide (CO2) narcosis. We believed her low adherence to domiciliary NPPV caused CO2 narcosis; hence, we advised her to continue domiciliary NPPV and she complied. In May 2012, the now 79-year-old patient was admitted for acute exacerbation of chronic respiratory failure and CO2 narcosis. Chest imaging suggested that DH had caused a deterioration of her status. She underwent laparoscopic diaphragmatic hernia repair. Operative findings revealed a retrosternal hernia sac, and she was diagnosed as having a Morgagni hernia (MH). Her respiratory status subsequently improved. We hypothesize that NPPV increased intra-abdominal pressure, thereby worsening the MH and exacerbating respiratory failure. We believe that clinicians should be cautious when prescribing NPPV for MH patients. 相似文献
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Alana J. Coleman Beverly Brozanski Burhan Mahmood Peter D. Wearden Douglas Potoka Bradley A. Kuch 《Journal of pediatric surgery》2013
Background/Purpose
Early clinical predictors for the use of ECMO in patients with congenital diaphragmatic hernia (CDH) are lacking. We sought to evaluate the first 24-h SNAP-II score and highest PaCO2 as predictors of ECMO support and in-hospital mortality in neonates with CDH.Methods
Retrospective review of 47 consecutive neonates with CDH admitted to our institution from January 2007 to December 2010 was performed. Covariates of ECMO use including SNAP-II score and highest PaCO2 within the first 24 h of NICU admission were evaluated.Results
Of the 47 infants in this study, 24 patients were supported with ECMO. The ECMO group had a higher incidence of pulmonary hypertension, higher PaCO2, and higher 24-h SNAP-II scores. Only the SNAP-II score and not highest PaCO2 predicted mortality following multivariate adjustment.Conclusions
The first 24-h SNAP-II score and highest PaCO2 may provide some prognostic value in identifying neonates who undergo ECMO support; however neither measure was independently associated with the use of therapy. Only the SNAP-II score was associated with in-hospital mortality following multivariate adjustment. Additional study is needed to validate these results in a larger data set. 相似文献35.
Julia Wynn Gudrun Aspelund Annette Zygmunt Charles J.H. Stolar George Mychaliska Jennifer Butcher Foong-Yen Lim Teresa Gratton Douglas Potoka Kate Brennan Ken Azarow Barbara Jackson Howard Needelman Timothy Crombleholme Yuan Zhang Jimmy Duong Marc S. Arkovitz Wendy K. Chung Christiana Farkouh 《Journal of pediatric surgery》2013
Purpose
To determine developmental outcomes and associated factors in patients with congenital diaphragmatic hernia (CDH) at 2 years of age.Methods
This is a multicenter prospective study of a CDH birth cohort. Clinical and socioeconomic data were collected. Bayley Scales of Infant Development (BSID-III) and Vineland Adaptive Behavior Scales (VABS-II) were performed at 2 years of age.Results
BSID-III and VABS-II assessments were completed on 48 and 49 children, respectively. The BSID-III mean cognitive, language, and motor scores were significantly below the norm mean with average scores of 93 ± 15, 95 ± 16, and 95 ± 11. Ten percent (5/47) scored more than 2 standard deviations below the norm on one or more domains. VABS-II scores were similar to BSID-III scores with mean communication, daily living skills, social, motor, adaptive behavior scores of 97 ± 14, 94 ± 16, 93 ± 13, 97 ± 10, and 94 ± 14. For the BSID-III, supplemental oxygen at 28 days, a prenatal diagnosis, need for extracorporeal membrane oxygenation (ECMO) and exclusive tube feeds at time of discharge were associated with lower scores. At 2 years of age, history of hospital readmission and need for tube feeds were associated with lower scores. Lower socioeconomic status correlated with lower developmental scores when adjusted for significant health factors.Conclusion
CDH patients on average have lower developmental scores at 2 years of age compared to the norm. A need for ECMO, oxygen at 28 days of life, ongoing health issues and lower socioeconomic status are factors associated with developmental delays. 相似文献36.
Left phrenic nerve stimulation is a well-known complication of cardiac resynchronization therapy (CRT). We report a case where electronic or anatomical repositioning of the left ventricular (LV) electrode (within the coronary sinus tributaries) was not feasible/effective. Surgical graft interpositioning was performed to successfully isolate the phrenic nerve from the previously implanted coronary sinus epicardial LV lead. 相似文献
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经膈肌下抬挤心脏方法对心脏停搏兔复苏的实验研究 总被引:1,自引:0,他引:1
目的 比较标准胸外按压心肺复苏(S-CPR)与经膈肌下心脏按压心肺复苏(D-CPR)对复苏循环效应的影响;评价D-CPR用于CPR的可行性.方法 健康新西兰白兔20只,经呼气末窒息8 min造成心脏停搏(CA)模型.随机分两组,每组10只,分别实施S-CPR和D-CPR;于窒息前平静5 min后开始连续记录升主动脉收缩压(AOS)和舒张压(AOD)、经皮血氧饱和度(Sp02)、右心房收缩压(RASP)和舒张压(RADP)、心电图(ECG)等直至实验结束;计算升主动脉平均动脉压(MAP)、冠状动脉灌注压(CPP);分别观察两组动物的自主循环恢复(ROSC)率及6 h存活率.结果 S-CPR组有5只、D-CPR组有8只动物获得ROSC(ROSC率分别为50%和80%,P=20.05);S-CPR组6 h存活率为40%,D-CPR组为50%.D-CPR组复苏1 rain和5 rain时AOS、AoD、MAP和CPP均高于S-CPR组(P均<0.05);D-CPR组复苏1 rain时MAP、CPP分别是其基础值的54.1%、33.4%.5 min时为60.0%、41.8%,而S-CPR组复苏1 min时AOS、AOD为基础值的37.3%、16.5%,5 min时为38.5%、17.1%#且D-CPR组ROSC后动物血流动力学较S-CPR组变化平稳.结论 D-CPR方法可产生较高的动脉血压和心排血量,并能增加实验动物的ROSC率和短期存活率.D-CPR方法优于S-CPR. 相似文献
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目的探究急诊绿色通道护理对创伤性膈疝患者就治成功率的影响。
方法回顾性分析2015年11月至2019年5月,南京医科大学第一附属医院收治的40例创伤性膈疝患者。按急诊护理方法的不同分为对照组和观察组,各20例。对照组行常规护理,观察组行急诊绿色通道护理。分析2组急诊相关指标、术后康复指标,2组护理后1个月的复发率、救治成功率及并发症发生率。采用SPSS 20.0统计软件进行数据分析。
结果与对照组比较,观察组急诊科抢救时间、辅助检查时间、从救治到送入手术室时间、术后机械通气时间均缩短,术后24 h动脉血二氧化碳分压(PaCO2)水平低于对照组,差异均有统计学意义(均P<0.05);护理后观察组和对照组的复发率分别为5.00%、25.00%,救治成功率分别为100.00%、90.00%,差异无统计学意义(P>0.05);护理后观察组并发症总发生率(10.00%)显著低于对照组(45.00%),差异有统计学意义(P<0.05)。
结论急诊绿色通道护理缩短了创伤性膈疝患者的抢救时间,使患者尽早脱离危险,减少创伤性膈疝患者并发症发生率,可有效改善患者预后。 相似文献
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