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17例先天性肺囊性腺瘤样畸形病例系列:治疗与结果

A series of 17 cases of congenital cystic adenomatoid malformation of the lung: management and outcome.

作者信息

Cacciari A, Ceccarelli P L, Pilu G L, Bianchini M A, Mordenti M, Gabrielli S, Milano V, Zanetti G, Pigna A, Gentili A

机构信息

Department of Pediatric Surgery, University of Bologna, S. Orsola-Malpighi Hospital, Italy.

出版信息

Eur J Pediatr Surg. 1997 Apr;7(2):84-9. doi: 10.1055/s-2008-1071060.

Abstract

During a 5-year-period, 17 cases of congenital adenomatoid malformation of the lung were observed in the Department of Pediatric Surgery of Bologna University-S. Orsola-Malpighi Hospital; 16 cases were diagnosed in utero by ultra-sound. 13 patients had prenatal diagnosis between 21 and 23 weeks of gestation; 2 cases were diagnosed at 25 weeks and another one at 26 weeks. The other case came from another hospital, and the diagnosis was accidentally detected postnatally. In 3 cases the parents decided to terminate the pregnancy 3 cases had spontaneous improvement during the last weeks of gestation, and were perfectly normal at birth. One case was lost to follow-up; all remaining patients underwent resection. Among these patients only one was a nonsurvivor, while 9 have completely recovered. However, one patient developed persistent pulmonary hypertension, which could successfully be treated with drugs, another underwent laser treatment for laryngeal stenosis due to intubation. A special epidemiological information: 13 out 17 cases (76.5%) were female.

摘要

在5年期间,博洛尼亚大学圣奥索拉-马尔皮基医院小儿外科观察到17例先天性肺腺瘤样畸形;16例在子宫内通过超声诊断。13例患者在妊娠21至23周时进行了产前诊断;2例在25周时诊断,另一例在26周时诊断。另一例来自另一家医院,诊断是在出生后偶然发现的。3例中父母决定终止妊娠,3例在妊娠最后几周有自发改善,出生时完全正常。1例失访;其余所有患者均接受了切除术。在这些患者中,只有1例死亡,而9例已完全康复。然而,1例患者出现持续性肺动脉高压,可通过药物成功治疗,另1例因插管导致喉狭窄接受了激光治疗。一项特殊的流行病学信息:17例中有13例(76.5%)为女性。

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