POLYCYSTIC KIDNEY-DISEASE - A TRULY PEDIATRIC PROBLEM

被引：19

作者：

OGBORN, MR

机构：

[1] Department of Pediatrics and Child Health, University of Manitoba, Winnipeg, Manitoba

来源：

PEDIATRIC NEPHROLOGY | 1994年 / 8卷 / 06期

关键词：

AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE; AUTOSOMAL RECESSIVE POLYCYSTIC KIDNEY DISEASE;

D O I：

10.1007/BF00869116

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

Polycystic kidney disease (PKD) represents the most common inherited cause of chronic renal failure. PKD is a relatively uncommon cause of chronic renal failure or mortality in childhood and adolescence, but is nevertheless often responsible for symptoms of renal disease. Current research into the pathogenesis of PKD suggests that disturbance of the normal regulation of growth and development of tubular epithelium is intrinsic to cyst formation and growth. Features of cystic epithelium that are analogous to earlier stages of renal development include altered composition of the extracellular matrix, abnormal cell proliferation, and the persistence of a secretory pattern of fluid and electrolyte transport. The potential for early diagnosis and intervention in PKD makes it an area of great interest to the pediatric nephrologist. Animal and in vitro studies have achieved modification of cyst growth by reduction of dietary protein, use of amiloride and its analogs, antagonism of the epidermal growth factor receptor system, antiinflammatory therapy, and most recently with the use of taxol, an agent that inhibits microtubule assembly. PKD may represent an area in which childhood diagnosis and intervention will have a significant impact on the prevalence of chronic renal failure in adult life.

引用

页码：762 / 767

页数：6

共 64 条

[1]

Grantham J.J., Fluid secretion, cellular proliferation and the pathogenesis of renal epithelial cysts, J Am Soc Nephrol, 3, pp. 1843-1857, (1993)

[2]

Cole B.R., Autosomal recessive polycystic kidney disease, The cystic kidney, pp. 327-350, (1990)

[3]

Kaplan B.S., Fay J., Shah V., Dillon M.J., Barratt T.M., Autosomal recessive polyeystic kidney disease, Pediatr Nephrol, 3, pp. 43-49, (1989)

[4]

Blyth H., Ockenden B.G., Polycystic disease of kidneys and liver presenting in childhood, J Med Genet, 8, pp. 257-284, (1971)

[5]

Kaplan B.S., Kaplan P., Chadarevian J.P., Jerquier S., O'Regan S., Russo P., Variable expression of autosomal recessive polycystic kidney disease and congenital hepatic fibrosis within a family, Am J Med Genet, 29, pp. 639-647, (1988)

[6]

Shaikewitz S.T., Chapman A., Autosomal recessive polycystic kidney disease: issues regarding the variability of clinical presentation, J Am Soc Nephrol, 3, pp. 1858-1862, (1993)

[7]

Gabow P.A., Autosomal dominant polycystic kidney disease, The cystic kidney, pp. 295-326, (1990)

[8]

Sedman A., Bell P., Manco-Johnson M., Schrier R., Warady B.A., Heard E.O., Butler-Simon N., Gabow P., Autosomal dominant polycystic kidney disease in childhood: a longitudinal study, Kidney Int, 31, pp. 1000-1005, (1987)

[9]

Milutinovic J., Schabel S.I., Ainsworth S.K., Autosomal dominant polycystic kidney disease with liver and pancreatic involvement in early childhood, Am J Kidney Dis, 13, pp. 340-344, (1989)

[10]

Fick G.M., Johnson A.M., Strain J.D., Kimberling W.J., Kumar S., Manco-Johnson M.L., Duley I.T., Gabow P.A., Characteristics of very early onset autosomal dominant polycystic kidney disease, J Am Soc Nephrol, 3, pp. 1863-1870, (1993)

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