Verani R, Bernstein J
Department of Pathology, University of Texas Medical School, Houston 77225.
Arch Pathol Lab Med. 1988 Mar;112(3):271-4.
Three children had renal histopathologic findings indicative of glycogen storage disease type I. Glomerular basement membrane (GBM) alterations were present in the three patients, particularly so in the two patients with proteinuria. Thickening, lamellation, and glycogen deposition were the characteristic alterations in the GBM. Glomerulosclerosis was prominent in one patient. We suggest that the GBM alteration is related to the glomerular sclerosis and that both are related to metabolic derangements of glycogen storage disease type I.
三名儿童的肾脏组织病理学检查结果提示为Ⅰ型糖原贮积病。三名患者均存在肾小球基底膜（GBM）改变，尤其是两名蛋白尿患者更为明显。GBM的特征性改变为增厚、分层和糖原沉积。一名患者肾小球硬化明显。我们认为GBM改变与肾小球硬化有关，且二者均与Ⅰ型糖原贮积病的代谢紊乱有关。
