Shwachman综合征伴肝硬化及门静脉高压临床病理分析

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。

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

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.