INHERITED FACTOR-H DEFICIENCY AND COLLAGEN TYPE-III GLOMERULOPATHY

被引:90
作者
VOGT, BA
WYATT, RJ
BURKE, BA
SIMONTON, SC
KASHTAN, CE
机构
[1] Department of Pediatrics, Rainbow Babies and Children's Hospital, Cleveland, Ohio
[2] Department of Pediatrics, University of Tennessee, Memphis, Tennessee
[3] Department of Laboratory Medicine and Pathology, University of Minnesota, Minneapolis, Minnesota
[4] Department of Pathology, Minneapolis Children's Medical Center, Minneapolis, Minnesota
[5] Department of Pediatrics, University of Minnesota, Minneapolis, Minnesota
关键词
GLOMERULONEPHRITIS; FACTOR H; COMPLEMENT; BETA-1 H GLOBULIN; COLLAGEN TYPE III GLOMERULOPATHY;
D O I
10.1007/BF00858956
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
A non-immune complex-mediated glomerulonephritis associated with persistent hypocomplementemia occurred in a young boy. Measurement of complement components revealed complete factor H deficiency, inherited as an autosomal recessive trait. Evaluation of the renal lesion revealed extensive deposition of type III collagen suggestive of collagen type III glomerulopathy, a recently identified cause of chronic renal insufficiency in children and adults. This report represents the first association of inherited factor H deficiency with collagen type III glomerulopathy.
引用
收藏
页码:11 / 15
页数:5
相关论文
共 19 条
[1]  
Thompson R., Winterborn M., Hypocomplementemia due to a genetic deficiency of β1H globulin, Clin Exp Immunol, 46, pp. 110-119, (1981)
[2]  
Levy M., Halbwachs-Mecarelli L., Gubler M-C, Kohout G., Bensenouci A., Niaudet P., Hauptmann G., Lesavre P., Factor H deficiency in two brothers with atypical dense intramembranous deposit disease, Kidney Int, 30, pp. 949-956, (1986)
[3]  
Lopez-Larrea C., Dieguez M., Enguix A., Dominguez O., Marin B., Gomez E., A familial deficiency of complement factor H, Biochem Soc Trans, 15, pp. 648-649, (1987)
[4]  
Brai M., Misiano G., Maringhini S., Cutaja I., Hauptmann G., Combined homozygous factor H and heterozygous C2 deficiency in an Italian family, J Clin Immunol, 8, pp. 50-56, (1988)
[5]  
Wyatt R., Forristal J., Davis C., Coleman T., West C., Control of serum C3 levels by B-1 H and C3b inactivator, J Lab Clin Med, 95, pp. 905-917, (1980)
[6]  
Kim Y., Vernier R., Fish A., Michael A., Immunofluorescence studies of dense deposit disease: the presence of railroad tracks and mesangial rings, Lab Invest, 40, pp. 474-480, (1979)
[7]  
Marinozzi V., The role of fixation in electron staining, Journal of the Royal Microscopical Society, 81, pp. 141-154, (1963)
[8]  
Nilsson U., Muller-Eberhard H., Isolation of B-IF-globulin from human serum and its characterization as the fifth component of complement, J Exp Med, 146, pp. 257-270, (1965)
[9]  
Vik D., Munoz-Canoves P., Chaplin D., Tack B., Factor H, Curr Top Microbiol Immunol, 153, pp. 147-162, (1989)
[10]  
Nielsen H., Christensen K., Koch C., Thoen B., Heegaard N., Tranum-Jensen J., Hereditary, complete deficiency of complement factor H associated with recurrent meningococcal disease, Scand J Immunol, 30, pp. 711-718, (1989)