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目的探讨小儿门静脉海绵样变性(CTPV)的误诊原因及预防对策。方法回顾性分析19例门静脉海绵样变性患儿的临床资料。结果 CTPV主要表现为呕血和或便血,脾肿大,血常规白细胞、血红蛋白、血小板均减少。6例误诊为嗜血细胞综合征,4例误诊为再生障碍性贫血,1例误诊为白血病;2例误诊为遗传代谢病,1例误诊为消化性溃疡,1例误诊为肝炎,1例误诊为麦克尔憩室,3例误诊为肝硬化。15例患儿接受了手术治疗,5例保守治疗。结论小儿门静脉海绵样变性以脾大、脾亢及消化道出血为主要临床表现;误诊率高;查体不仔细,对本病认识不足,过分看重化验检查是误诊的主要原因;仔细体格检查,提高对本病认识,及时做彩色多普勒超声检查可最大程度的避免误诊。
Objective To investigate the misdiagnosis reasons and preventive measures of pediatric portal vein cavernous degeneration (CTPV). Methods The clinical data of 19 children with portal cavernous degeneration were retrospectively analyzed. Results The main manifestations of CTPV hematemesis and or blood in the stool, splenomegaly, white blood cells, hemoglobin, platelets were reduced. 6 cases misdiagnosed as hemophilia syndrome, 4 cases misdiagnosed as aplastic anemia, 1 case misdiagnosed as leukemia; 2 cases misdiagnosed as genetic metabolic disease, 1 case misdiagnosed as peptic ulcer, 1 case misdiagnosed as hepatitis and 1 case misdiagnosed as Michael diverticulum, 3 cases misdiagnosed as cirrhosis. Fifteen children underwent surgical treatment and five received conservative treatment. Conclusions The main cavernous degeneration of portal vein in children with splenomegaly, hypersplenism and gastrointestinal bleeding as the main clinical manifestations; misdiagnosis rate; check the body is not careful, lack of knowledge of the disease, excessive emphasis on laboratory tests is the main reason for misdiagnosis; careful physical examination, Improve awareness of the disease, timely color Doppler ultrasound can be the best to avoid misdiagnosis.