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胎粪性肠梗阻:28岁以上的10名患者。

Meconium ileus: ten patients over 28 years of age.

作者信息

Shwachman H

出版信息

J Pediatr Surg. 1983 Oct;18(5):570-5. doi: 10.1016/s0022-3468(83)80361-3.

Abstract

Meconium ileus is the initial clinical manifestation of cystic fibrosis. Once the intestinal obstruction is corrected either medically or surgically the course of disease is that of cystic fibrosis. It is suggested that babies with this disease should be treated in specialized pediatric cystic fibrosis centers to prevent early death or some avoidable complications. Genetic counseling for affected families is recommended when a baby is born with this condition. Usually such infants are born at full term and there is no sex predilection. Prognosis is uncertain and the outlook depends, as it does in cystic fibrosis, on the degree and rate of progression of the pulmonary involvement. This paper presents a series of unusual patients with meconium ileus who have reached adulthood, thus, offering hope that a satisfactory life beyond adolescence is possible in this formerly fatal disease of infancy.

摘要

胎粪性肠梗阻是囊性纤维化的初始临床表现。一旦通过内科或外科手段纠正了肠梗阻,疾病进程就与囊性纤维化相同。建议患有这种疾病的婴儿在专门的儿科囊性纤维化中心接受治疗,以预防早期死亡或一些可避免的并发症。当婴儿患有这种疾病时,建议为受影响的家庭提供遗传咨询。通常这些婴儿足月出生,无性别偏好。预后不确定,前景如同囊性纤维化一样,取决于肺部受累的程度和进展速度。本文介绍了一系列已成年的患有胎粪性肠梗阻的特殊患者,因此,为这种曾是婴儿致命疾病的患者在青春期后有可能过上满意生活带来了希望。

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