I型糖原贮积病相关肾脏疾病中的转化生长因子-β

Transforming growth factor-beta in renal disease with glycogen storage disease I.

作者信息

Urushihara Maki, Kagami Shoji, Ito Michinori, Yasutomo Koji, Kondo Shuji, Kitamura Akiko, Takahashi Akiyoshi, Kuroda Yasuhiro

机构信息

Department of Pediatrics, School of Medicine, University of Tokushima, and Division of Pediatrics, Tokushima Red Cross Hospital, Komatsushima City, Japan.

出版信息

Pediatr Nephrol. 2004 Jun;19(6):676-8. doi: 10.1007/s00467-004-1456-6. Epub 2004 Apr 3.

DOI:10.1007/s00467-004-1456-6

PMID:15064941

Abstract

We report a 14-year-old patient with Japanese glycogen storage disease I (GSD-I) who was found to have proteinuria. Renal biopsy revealed massive tubular atrophy and interstitial fibrosis with mononuclear cell infiltration, but the glomeruli were almost normal. The epithelial cells of tubules contained periodic acid-Schiff-positive glycogen deposits digested by diastase. In an immunohistological study, transforming growth factor (TGF)-beta expression was increased in tubular epithelial cells compared with a normal control kidney specimen. These data suggest that increased TGF-beta expression is involved in the pathophysiology of renal interstitial fibrosis in a patient with GSD-I.

摘要

我们报告了一名14岁患日本型糖原贮积病I型（GSD-I）的患者，该患者被发现存在蛋白尿。肾活检显示大量肾小管萎缩和间质纤维化，并伴有单核细胞浸润，但肾小球基本正常。肾小管上皮细胞含有经淀粉酶消化后对高碘酸-希夫染色呈阳性的糖原沉积物。在一项免疫组织学研究中，与正常对照肾脏标本相比，转化生长因子（TGF）-β在肾小管上皮细胞中的表达增加。这些数据表明，TGF-β表达增加参与了GSD-I患者肾间质纤维化的病理生理过程。