Shwachman综合征伴肝硬化及门静脉高压临床病理分析

Pathological analysis on Shwachman syndrome associated with cirrhosis and portal hypertension : report of 1 case and litera-ture review

目的分析以先天性胰腺腺泡发育不良为主要特征之一的Shwachman 综合征的临床表现。方法报告1例病理诊断为先天性胰腺腺泡发育不良,临床表现为 Shwachman 综合征的病例,并做文献复习。结果Shwachman 综合征的临床表现是随着胰腺功能状况而不同,诊断主要取决于胰腺的外腺泡和血液系统的改变。结论随着小儿年龄的增加,胰腺的功能得到改善,症状可以得到缓解。骨髓累及的程度与预后有关。

ObjectiveTo analyze the clinical manifestation of Shwachman syndrome as one of the major characteristics of con-genital hypoplasia of the panceras.MethodsA case of exocrine pancreas hypoplasia diagnosed pathologically and manifested asShwachman syndrome was reported.ResultsThe clinical manifestations of Shwachman syndrome varied with the status of thepancreatic function. Its diagnosis depended on the changes of the pancreas exoglands and hematological findings. ConclusionsWith the growing of the child, the function of the pancreas improves accordingly and the symptoms relieve. Prognosis of Shwachmansyndrome is associated with bone marrow changes.