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年轻和年老的sphha/sphha小鼠的临床病理特征。患有先天性溶血性贫血的突变体。

Clinicopathologic features of young and old sphha/sphha mice. Mutants with congenital hemolytic anemia.

作者信息

Maggio-Price L, Russell R, Wolf N S, Alpers C E, Engel D

机构信息

Division of Animal Medicine, School of Medicine, University of Washington, Seattle 98195.

出版信息

Am J Pathol. 1988 Sep;132(3):461-73.

Abstract

A colony of mice with congenital hemolytic anemia, sphha/sphha, were evaluated over a 3-year period. Prominent findings included decreased survivability, reticulocytosis, increased peripheral blood leukocytes, extramedullary hematopoiesis in liver and spleen, lymphoid hyperplasia and membranoproliferative glomerulonephritis. Older (12 to 21 months) anemic animals had elevated serum levels of IgG1 and IgA. There was deposition of C3, IgG, IgM, and IgA in renal glomeruli of both control and anemic mice, but deposition of IgM and IgA was more prominent and widely distributed in anemic animals and correlated with mesangial expansion and the presence of electron dense deposits in the mesangium and in glomerular capillary walls. Prominent renal tubular hemosiderosis was noted in young and old anemic mice. The relation between the hemolytic anemia and glomerular disease is unclear but these mice may be an animal model useful for exploration of changes attendant with chronic hemolysis and evaluation of renal disease that accompanies hemolytic anemia.

摘要

对一群患有先天性溶血性贫血(sphha/sphha)的小鼠进行了为期3年的评估。主要发现包括生存能力下降、网织红细胞增多、外周血白细胞增多、肝脏和脾脏的髓外造血、淋巴样增生以及膜增生性肾小球肾炎。年龄较大(12至21个月)的贫血动物血清IgG1和IgA水平升高。对照小鼠和贫血小鼠的肾小球中均有C3、IgG、IgM和IgA沉积,但IgM和IgA的沉积在贫血动物中更为显著且分布更广泛,并且与系膜扩张以及系膜和肾小球毛细血管壁中电子致密沉积物的存在相关。在年轻和年老的贫血小鼠中均观察到明显的肾小管含铁血黄素沉着症。溶血性贫血与肾小球疾病之间的关系尚不清楚,但这些小鼠可能是一种有用的动物模型,可用于探索慢性溶血伴随的变化以及评估溶血性贫血伴发的肾脏疾病。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/95b7/1880761/642cab013bc9/amjpathol00132-0074-a.jpg

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