Fang Wenjuan,Li Fangyuan,Peng Guo,et al.Research advances in children with megacystis-microcolon-intestinal hypoperistalsis syndrome[J].Journal of Clinical Pediatric Surgery,2025,(10):995-1000.[doi:10.3760/cma.j.cn101785-202308005-017]
儿童巨膀胱-小结肠-肠蠕动不良综合征的研究进展
- Title:
- Research advances in children with megacystis-microcolon-intestinal hypoperistalsis syndrome
- Keywords:
- Megacystis-Microcolon-Intestinal Hypoperistalsis Syndrome; Pathological Conditions; Signs and Symptoms; Pathologic Processes; Diagnosis; Therapy; Surgical Procedures; Operative; Child
- 摘要:
- 巨膀胱-小结肠-肠蠕动不良综合征是一种罕见的先天性疾病,以巨膀胱、细小结肠、肠蠕动减弱或消失为特征,预后较差。大多数患儿因严重脓毒血症、终末期肾功能不全、长期营养不良而死亡。目前该病尚无有效的治疗方法,发病机制尚未明确,可能与遗传因素、神经源性、肌源性以及内分泌因素有关。本文就巨膀胱-小结肠-肠蠕动不良综合征的发病机制、临床表现、诊断与鉴别诊断、治疗与结局进行综述。
- Abstract:
- Megacystis-microcolon-intestinal hypoperistalsis syndrome is a rare congenital disease characterized by megacystis,microcolon and weakened or absent intestinal peristalsis with a poor prognosis.Most children die from severe sepsis,end-stage renal dysfunction and long-term malnutrition.Currently there is no effective therapy and its pathogenesis has remained elusive.It may be related to genetic factors,neurogenic,myogenic,hormones and other related aspects.This review summarized the pathogenesis,clinical manifestations,diagnosis and differential diagnosis,treatment status and outcomes of giant bladder-small colon-intestinal dysplasia syndrome.
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备注/Memo
收稿日期:2023-8-3。
基金项目:国家自然科学基金委联合基金(U1904208);深圳市医学重点学科(2020—2024—SZXK035)
通讯作者:李守林,Email:lishoulinsz@126.com