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先天性肾病综合征(芬兰型)。

Congenital nephrotic syndrome (Finnish type).

作者信息

Fujinami M, Hane Y, Ito K, Kajita A

出版信息

Acta Pathol Jpn. 1985 Mar;35(2):517-25. doi: 10.1111/j.1440-1827.1985.tb00594.x.

DOI:10.1111/j.1440-1827.1985.tb00594.x
PMID:2992230
Abstract

The case was a one year and four months old boy, who had been diagnosed as congenital nephrotic syndrome on the 4th day of life. He died of septicemia from peritonitis. Large pale kidneys, dilated trunk of pulmonary artery, and thickened left atrial endocardium were observed at autopsy. Renal histology seemed to be compatible with congenital nephrotic syndrome of the Finnish type. Viral antigens of herpes simplex type 1 and varicella-zoster in paraffin-embedded sections were proven to be negative. Besides renal change, increased number of pancreatic islets without hypertrophy was noticed.

摘要

该病例为一名1岁4个月大的男孩,其在出生后第4天被诊断为先天性肾病综合征。他死于腹膜炎引发的败血症。尸检时观察到肾脏苍白肿大、肺动脉主干扩张以及左心房心内膜增厚。肾脏组织学表现似乎符合芬兰型先天性肾病综合征。石蜡包埋切片中单纯疱疹病毒1型和水痘 - 带状疱疹病毒抗原经证实为阴性。除了肾脏病变外,还注意到胰岛数量增加但无肥大现象。

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