Progress on Dent's disease / 国际儿科学杂志

ABSTRACT

There are many causes of proteinuria in children.Because proteinuria is mainly caused by glomerular diseases,renal tubulopathies are often ignored.Dent's disease is an hereditary renal tubulopathy characterized by low-molecular-weigh proteinuria,hypercalciuria,nephrocalcinosis,nephrolithiasis,and progressive renal failure.Dent's disease is mainly caused by mutations in CLCN5 and OCRL1 genes.Current treatments of the disease are still mainly symptomatic and supportive therapy,including reducing urinary calcium excretion,lightening nephrocalcinosis,and delaying the occurrence of renal insufficiency.