小儿皮肌炎合并消化道大出血4例临床分析

X N Wang, X L Wang, J R Zhang, X Yao, Y Liu, J G Li
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引用次数: 0

摘要

目的:探讨小儿皮肌炎(JDM)并发严重胃肠出血的临床特点、治疗及预后。方法:回顾性分析我院2017年1月至2025年1月收治的4例合并严重胃肠出血的JDM患者。资料包括人口统计学,临床表现,实验室和影像学检查,疗程和结果进行了回顾。结果:4例患者(男2例,女2例),发病年龄分别为6.1、6.2、10.0和8.0岁。所有患者均出现皮疹和疲劳,并被诊断为严重难治性JDM(抗nxp2抗体强阳性)。JDM诊断后18、12、51、2个月发生严重胃肠道出血。两例确诊的胃肠道感染是由受污染的食物引起的。腹痛为首发胃肠道症状,所有病例均出现黑便,血红蛋白水平降至60 g/L以下(病例1-4分别降至38、59、60、43 g/L)。所有患者均表现为肠壁增厚。活动出血部位包括十二指肠(3例,靠近十二指肠乳头2例,弥漫性十二指肠渗出1例)。急诊内镜止血3例。一例弥漫性十二指肠出血患者在介入栓塞失败后需要额外的糖皮质激素脉冲治疗。3例患者在积极治疗后病情稳定,1例因十二指肠穿孔死亡。结论:抗nxp2抗体阳性的JDM患者容易累及胃肠道,特别是慢性病例。十二指肠出血是常见的,血管糜烂和深部粘膜溃疡构成危及生命的危险。对于出现腹痛、肠壁增厚的抗nxp2抗体阳性患儿,应尽早进行早期内镜检查,发现消化道病变,及时治疗。严重消化道出血患者通常需要积极治疗基础疾病,并结合内镜止血治疗。治疗及时,预后较好。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Clinical analysis of 4 cases of juvenile dermatomyositis complicated with massive hemorrhage of digestive tract].

Objective: To investigate the clinical characteristics, treatment and prognosis of juvenile dermatomyositis (JDM) complicated by sever gastrointestinal hemorrhage in children. Methods: A retrospective analysis was conducted on 4 JDM patients with sever gastrointestinal hemorrhage admitted to our hospital, from January 2017 to January 2025. Data including demographics, clinical manifestations, laboratory and imaging findings, treatment courses, and outcomes were reviewed. Results: The cohort comprised 4 patients (2 males, 2 females), with onset ages of 6.1, 6.2, 10.0 and 8.0 years. All presented with rash and fatigue and were diagnosed with severe refractory JDM (strongly positive anti-NXP2 antibodies). Sever gastrointestinal hemorrhage occurred 18, 12, 51, and 2 months after JDM diagnosis. Two cases had confirmed gastrointestinal infections due to contaminated food. Abdominal pain was the initial gastrointestinal symptom and black stool was observed in all cases, hemoglobin levels dropped below 60 g/L (case 1-4 decreased to 38, 59, 60 and 43 g/L respectively). All patients exhibited intestinal wall thickening. Active bleeding sites included the duodenum (3 cases: 2 cases near the duodenal papilla, 1 cases with diffuse duodenal oozing). Emergency endoscopic hemostasis was performed in 3 cases. One patient with diffuse duodenal bleeding required additional glucocortieoid pulse therapy after failed interventional embolization. Three patients stabilized following aggressive treatment of JDM, while 1 case died due to duodenal perforation. Conclusions: Anti-NXP2 antibody-positive JDM patients are prone to gastrointestinal involvement, particularly in chronic cases. Duodenal bleeding is common, with vascular erosion and deep mucosal ulcers posing life-threatening risks. For children with positive anti-NXP2 antibodies who present with abdominal pain and thickened intestinal walls, early endoscopic examination should be conducted as soon as possible to detect digestive tract lesions and provide timely treatment. Patients with severe digestive tract bleeding usually require active treatment for the underlying disease combined with endoscopic hemostasis therapy. Under timely treatment, the prognosis is relatively favorable.

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来源期刊
CiteScore
1.30
自引率
0.00%
发文量
14916
期刊介绍: Chinese Journal of Pediatrics is the only high-level academic journal in the field of pediatrics in my country, supervised by the China Association for Science and Technology and sponsored by the Chinese Medical Association. It was founded in 1950. The purpose of the journal is to combine theory with practice, with emphasis on practice; to combine basic and clinical, with major clinical; to combine popularization with improvement, with emphasis on improvement. It is to promote academic exchanges in the field of pediatrics in my country; to serve the development and improvement of my country's pediatric medicine; to serve the training of pediatric medical talents in my country; and to serve the health of children in my country. Chinese Journal of Pediatrics is mainly composed of columns such as monographs, clinical research and practice, case reports, lectures, reviews, conference (symposium) minutes, clinical pathology (case) discussions, international academic exchanges, expert explanations, and new technologies.
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