Hirschsprung's disease in Japan: analysis of 3852 patients based on a nationwide survey in 30 years
Hirschsprung's disease in Japan: analysis of 3852 patients based on a nationwide survey in 30 years
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DOI：
10.1016/j.jpedsurg.2004.09.052
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发表时间：
2005-01-01
影响因子：
2.4
通讯作者：
Nakatsuji, T
中科院分区：
文献类型：
--
作者：
Suita, S;Taguchi, T;Nakatsuji, T
背景/目的：腹腔镜和经肛门直肠内拖出术的引入引起了先天性巨结肠手术方法的革命。为了研究日本先天性巨结肠病的变化概况，作者进行了一项全国性调查。方法：患者数据分3个阶段收集：第1组，1978年至1982年间的1628名患者;第2组，1988年至1992年间的1121名患者;第3组，1998年至2002年间的1103名患者。结果：各组的发病率分别为1：4697、1：5544和1：5343，男女比例分别为3.0：1、3.4：1和3.0：1。出生时体重低于2500 g的患者在第3组中增加至10.4%，而在第2组中为6.5%，在第1组中为5.5%。第3组中有家族史的患者也增加到6.0%，而第2组为2.8%，第1组为3.0%。随着时间的推移，相关异常的发生率增加，第1组为11.1%，第2组为16.3%，第3组为21.2%。23例患者中有4例发现基因突变。各组无神经节细胞症的程度几乎相同。关于确定性手术，非剖腹手术，包括经肛门直肠内拖出，在第3组中增加到42.6%，但在第1组和第2组中为0%。无造口的初次手术的频率也增加了。第3组的确定性手术年龄降低。术前和术后小肠结肠炎的发生率也随着时间的推移而降低。随着时间的推移，死亡率下降，7.1%，4.9%，3.0%的组1，2，3，Conclusions：作者分析了3852例日本患者超过30年。在过去的10年中，低出生体重、相关异常或有家族史的患者比例增加。因此，无需剖腹手术的初次手术已成为最终手术的首选程序。(C)2005年爱思唯尔公司All rights reserved.
Background/Purpose: The introduction of laparoscope and transanal endorectal pull-through has caused a revolution in the operative procedures for Hirschsprung's disease. To study the changing profile of Hirschsprung's disease in Japan, the authors carried out a national survey.Method: Patient data were collected in 3 phases: group 1, 1628 patients between 1978 and 1982; group 2, 1121 patients between 1988 and 1992; and group 3, 1103 patients between 1998 and 2002, respectively.Results: The incidence was 1:4697, 1:5544, and 1:5343 and the male/female ratio was 3.0:1, 3.4: 1, and 3.0:1 in each group, respectively. Patients weighing less than 2500 g at birth increased to 10.4% in group 3, whereas they were 6.5% in group 2 and 5.5% in group 1. The patients with a family history also increased to 6.0% in group 3, in comparison with 2.8% in group 2 and 3.0% in group 1. The incidence of associated anomalies increased over time, 11.1% in group 1, 16.3% in group 2, and 21.2% in group 3. Mutations of genes were found in 4 of the 23 patients examined. The extent of aganglionosis was almost the same in each group. Regarding the definitive operation, the procedures without laparotomy, including transanal endorectal pull-through, increased up to 42.6% in group 3 but 0% in groups 1 and 2. The frequency that a primary operation without stoma was performed also increased. The age at definitive operation decreased in group 3. The incidence of preoperative and postoperative enterocolitis also decreased over time. The mortality was decreased over time, 7.1%, 4.9%, and 3.0% for groups 1, 2, and 3, respectively.Conclusions: The authors analyzed 3852 Japanese patients over 3 decades. The ratio of patients with a low birth weight, associated anomalies, or a family history was increased in the last 10 years. A primary operation without laparotomy has thus become the procedure of choice for a definitive operation. (C) 2005 Elsevier Inc. All rights reserved.