Autosomal dominant tubulointerstitial kidney disease (ADTKD) is a recently defined entity
that includes rare kidney diseases characterized by tubular damage and interstitial fibrosis …

Uromodulin (also known as Tamm-Horsfall protein) is exclusively produced in the kidney
and is the most abundant protein in normal urine. The function of uromodulin remains …

Recently, there has been an increasing amount of literature published on the effects of 4-
phenylbutyric acid (4-PBA) in various biological systems. 4-PBA is currently used clinically to …

Background Autosomal dominant tubulointerstitial kidney disease (ADTKD) caused by
mutations in the UMOD gene (ADTKD-UMOD) is considered rare and often remains …

Uromodulin, the major protein secreted in normal urine, is exclusively produced in the thick
ascending limb (TAL) cells of the kidney. The exact role uromodulin (UMOD) plays in renal …

Results Thirty-one peer-reviewed publications reporting on 202 patients from 74 families
with 59 different UMOD mutations were included. Median ages at onset of hyperuricemia …

Background: Epidemics of chronic kidney disease of uncertain etiology (CKDu) are
occurring on the Pacific coast of Central America, in Sri Lankan and Indian agricultural …

The clinical characteristics of autosomal dominant tubulointerstitial kidney disease (ADTKD)
include bland urinary sediment, slowly progressive chronic kidney disease (CKD) with many …

Renal cystic diseases (RCDs) can arise from utero to early adulthood and present with a
variety of symptoms including renal, hepatic, and cardiovascular manifestations. It is well …

Autosomal dominant tubulointerstitial kidney disease (ADTKD) is a rare inherited disorder
characterized by progressive loss of kidney function, nonsignificant urinalysis and …