Glomerular Pathology in Dent Disease and Its Association with Kidney Function

Abstract

These associations suggest a potential role for glomerular pathology, specifically involving the podocyte, in disease progression, which deserves further study. Furthermore, Dent disease should be suspected in boys and men who have unexplained proteinuria with focal global glomerulosclerosis and segmental foot process effacement on renal biopsy.

Nephrocalcinosis: A Diagnostic Conundrum

“…Focal global glomerulosclerosis, which is noted in 85% of Dent disease, is the predominant histopathologic finding, followed by effacement of foot processes. 13 Atypical presentations as seen in our patient have been reported with focal global glomerulosclerosis. 14 Making an accurate diagnosis of Dent disease is important to help avoid potentially toxic immunosuppressive medications.…”

Dent disease: Same <i>CLCN5</i> mutation but different phenotypes in two brothers in China

“…However, it was a pity that our younger brother did not have a renal biopsy, and therefore the severity of his tubular atrophy was unknown. There was a report on renal pathology and renal function, which showed that higher percentages of globally sclerotic glomeruli, foot process effacement, and interstitial inflammation were associated with a lower estimate of glomerular filtration rate (eGFR) at biopsy, whereas foot process effacement was associated with steeper annual eGFR decline (23). However, no correlation between phenotype and tubular atrophy has been reported in Dent disease.…”

An integrated iterative annotation technique for easing neural network training in medical image analysis

“…To qualitatively prove the effectiveness and extendibility of our method, we show its extension to multi class detection by segmenting glomerular nuclei types [39,40], interstitial fibrosis and tubular atrophy (IFTA) [41,42], as well as differentiating sclerotic and non-sclerotic glomeruli [43] in mouse kidney and human renal biopsies. Figure 4 shows the glomeruli detection network from Figure 2 adapted for nuclei detection.…”