患有IA型糖原贮积病儿童的高血压

Hypertension in a child with type IA glycogen storage disease.

作者信息

Jonas A J, Verani R R, Howell R R, Conley S B

机构信息

Department of Pediatrics, University of Texas Medical School, Houston 77030.

出版信息

Am J Kidney Dis. 1988 Mar;11(3):264-6. doi: 10.1016/s0272-6386(88)80160-4.

DOI:10.1016/s0272-6386(88)80160-4

PMID:3422787

Abstract

Hypertension and proteinuria were observed in a 2-year-old child with type IA (von Gierke's) glycogen storage disease (GSD). She had evidence of hyperfiltration and had elevated selective renal vein renins. On renal biopsy, increased mesangial cell matrix and cellularity were observed with focal thickening and irregularity of the basement membrane. This case may be representative of the early renal findings in type IA GSD.

摘要

在一名患有IA型（冯·吉尔克氏）糖原贮积病（GSD）的2岁儿童中观察到高血压和蛋白尿。她有超滤的证据，并且选择性肾静脉肾素升高。肾活检时，观察到系膜细胞基质和细胞增多，伴有基底膜局灶性增厚和不规则。该病例可能代表IA型GSD的早期肾脏表现。