Burgos Carmen Mesas, Frenckner Björn
Department of Pediatric Surgery, Karolinska Institutet, Stockholm, Sweden.
Department of Pediatric Surgery, Karolinska Institutet, Stockholm, Sweden.
J Pediatr Surg. 2017 Apr;52(4):522-525. doi: 10.1016/j.jpedsurg.2016.09.061. Epub 2016 Sep 23.
Improvements in the clinical management of CDH have led to overall improved reported result from single institutions. However, population-based studies have highlighted a hidden mortality.
To explore the incidence in Sweden and to address the hidden mortality for CDH during a 27-year period in a population-based setting.
This is a population based cohort study that includes all patients diagnosed with CDH that were registered in the National Patient Register, the Medical Birth Register, the Register of Congenital Malformations and the Register for Causes of Death between 1987 and 2013. The mortality rates were calculated based on the number deaths divided by the number of live born cases. The hidden mortality was defined as the number of CDH cases that were not born (because of TOP or IUFD), cases of neonatal demise during birth or demise the same day of birth in patients who were in peripheral institutions and who never reached tertiary centers.
In total, 861 CDH patients were born in Sweden between 1987 and 2013, which corresponds to an incidence of 3.0 born CDH per 10,000 live births. When adding the cases of TOP and IUFD, the total incidence of CDH in Sweden was 3.5/10,000 live born. The mortality rate between 1987 and 2013 was 36%: 44% during the first time period 1987-1999 and 27% in the later period 2000-2013. The hidden mortality in the second period was 30%, resulting in a total mortality rate of 45%.
The incidence of CDH during a 27-year period remained unchanged in the population. However, we observed a decrease in the prevalence because of the increasing numbers of TOP. A significant hidden mortality exists, with overall mortality rate of 45% for CDH in this population.
II (cohort).
先天性膈疝（CDH）临床管理的改善使得单一机构报告的总体结果有所改善。然而，基于人群的研究凸显了一个隐藏的死亡率。
探讨瑞典的发病率，并在基于人群的环境中研究27年间CDH的隐藏死亡率。
这是一项基于人群的队列研究，纳入了1987年至2013年间在国家患者登记处、医疗出生登记处、先天性畸形登记处和死亡原因登记处登记的所有诊断为CDH的患者。死亡率根据死亡人数除以活产病例数计算得出。隐藏死亡率定义为未出生的CDH病例数（由于终止妊娠或死胎）、出生时新生儿死亡病例数或在外围机构出生且从未到达三级中心的患者在出生当天死亡的病例数。
1987年至2013年间，瑞典共出生861例CDH患者，相当于每10000例活产中有3.0例出生时患有CDH。加上终止妊娠和死胎病例，瑞典CDH的总发病率为每10000例活产中有3.5例。1987年至2013年的死亡率为36%：1987 - 1999年第一个时间段为44%，2000 - 2013年后期为27%。第二个时间段的隐藏死亡率为30%，导致总死亡率为45%。
27年间人群中CDH的发病率保持不变。然而，由于终止妊娠数量增加，我们观察到患病率有所下降。存在显著的隐藏死亡率，该人群中CDH的总体死亡率为45%。
II（队列研究）。
