Objective To summarize the experience of open heart operation on neonates with critical and complex congenital heart diseases and evaluate the methods of perioperative management. Methods From May 2001 to January 2003, 12 patients of neonates with congenital heart diseases underwent emergency operation. Their operating ages ranged from 6 to 30 days, the body weights were 2.8 to 4.5 kg. Their diagnoses included D-transposition of the great arteries in 4 cases, ventricular septal defect with atrial septal defect in 5 cases, complete atrioventricular septal defect, obstructed supracardiac total anomalous pulmonary venous drainage and cardiac rhabdomyomas in 1 case respectively. 12 cases were operated under moderate or deep hypothermic cardiopulmonary bypass. Results All cases were observed in ICU for 2-11 days and discharged 7-19 days after operation. The postoperative complications included low cardiac output, mediastinal infection, respiratory distress syndrome, systemic capillary leak syndrome and acute renal failure. All cases were cured and the follow-up (from 6 months to 2 years) showed satisfactory outcome. Conclusion A particular cardiopulmonary bypass and proper perioperative management is very important to ensure the successful outcome. Peritoneal dialysis is an effective and safe method for treating acute renal failure after cardiac operation in neonates.
【摘要】目的 探討成人先天性膽總管囊腫的診斷和治療。方法 對我科收治的7例成人先天性膽總管囊腫患者的資料進行回顧性分析。術前B超診斷正確率為91.9%(34／37)。6例行囊腫切除和肝總管空腸Roux-Y吻合術，1例因癌變行胰十二指腸切除術。結果 共隨訪29例，隨訪率為78.4%，平均隨訪43個月，未發現惡變者。結論 B超應作為先天性膽總管囊腫的首選檢查，囊腫切除和肝總管空腸Roux-Y吻合術應作為首選術式，早期診斷，早期根治性治療，是先天性膽總管囊腫診治的關鍵。
ObjectiveTo analyze the trend of disease burden changes in congenital birth defects in China from 1990 to 2019. MethodsUsing the global burden of disease study 2019 (GBD 2019), we analyzed the morbidity, mortality, and disability-adjusted life years (DALYs) of congenital birth defect diseases and their corresponding age-standardized rates and average annual percentage change (AAPC) to analyze the changes in the disease burden of congenital birth defects in China and compared them with global data from 1990 to 2019. ResultsIn 2019, the age-standardized incidence, mortality, and DALY in China were 147.41/100 000, 4.62/100 000, 480.95/100 000, respectively. Compared with 1990, the age-standardized incidence rate, age-standardized mortality rate, and age-standardized DALY rate increased by 12.08% and decreased by 70.38% and 66.82%, respectively. In recent years, although the age-standardized incidence of congenital birth defect disease in China is on the rise and higher than the global level, the disease burden is roughly on the decline and lower than the global level, which is closely related to earlier intervention and treatment of the disease resulting in a lower standardized mortality rate. ConclusionThe age-standardized mortality rate of children with congenital birth defects in China showed a decreasing trend from 1990 to 2019, and the burden of disease ranged from slightly higher than global to lower than global levels, but the age-standardized incidence rate was significantly higher, but the age-specific incidence rate has increased significantly.
Congenital deformities of the diaphragm include (1) Hiatus hernia; (2) Congenital diaphragmatic hernia; (3) Eventration of deaphragm. Fifty-one cases were seen by the authors in the past 30 years. Each type of the pathologic feature and the experiences in the surgical repair were suggested in this paper. Choice of an appropriate surgical procedure on the basis of its pathoanatomic and pathophysiologic features was emphasized in order to enhance the efficacy of treatment.
【Abstract】Objective To evaluate the distribution of nerve growth factor receptor( P75 NGFR) in congenital choledochal cyst(CCC) and its clinical implication. Methods Specimens from 18 children with CCC and normal choledochal specimens from 9 controls were immuno-stained with P75 NGFR antibody. Results Extensive P75 NGFR staining was found in the nerve fibres of normal comnon bile duct，bly staining of ganglion cells were observed on the normal specimens. There was very little immunoreactive fibre in the CCC. Conclusion The abnormal distribution of P75 NGFR in the aganglionic choledochal suggests that abnormal P75 NGFR is related to the occurrance of the CCC.