The pathological spectrum associated with the ultrastructural finding of thin glomerular basement membrane: A tertiary medical city experience and review of the literature

Kfoury, Hala; Arafah, Maria

doi:10.1080/01913123.2016.1258021

Cited by 7 publications

(2 citation statements)

References 8 publications

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“…Postulating that, if the thinning of glomerular basement membrane is universally and uniformly present, then a thin basement membrane disease will develop. On the other hand, if the modification is sparse and it only shows partial areas of thinning, then it is just a predisposing condition for subsequent glomerulonephritis (21,22).…”

Section: Discussionmentioning

confidence: 99%

Clinicopathologic and ultrastructural findings of hereditary nephritis; a 16-year single center survey in Iran

et al. 2020

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Introduction: Hereditary nephritis is an umbrella term for a group of congenital childhood diseases including but not limited to Alport syndrome, thin basement membrane disease, and Fabry disease. Objectives: The purpose of this study was a clinicopathologic investigation of Alport syndrome, thin basement membrane disease, and Fabry disease with a focus on the role of electron microscopy and toluidine blue staining in diagnosis. Patients and Methods: In this cross-sectional study, we investigated kidney biopsies with a final diagnosis of either Alport syndrome, thin basement membrane disease or Fabry disease from 2001 to 2016. Electron microscopy and light microscopy were done and the clinical and paraclinical data were extracted from the patients’ medical charts. Electron microscopy role was assessed in terms of necessary, helpful or non-necessary, while correlations between clinical and para-clinical data were determined using appropriate statistical tests. Results: Among the 2865 kidney biopsies, there were 22 patients of hereditary nephritis including 15 (0.52%) Alport syndrome, 5 (0.17%) thin basement membrane disease and 2 (0.07%) Fabry disease diagnosed by electron microscopy. Electron microscopy was essential for the diagnosis of 19 (86.4%) cases, helpful for 3(13.6%) and there was no case for which electron microscopy was non-necessary. The patients’ mean age was 16.1 ± 9.0 years. The most common finding in Alport syndrome was proteinuria (86.7%) followed by hematuria (60.0%). Conclusion: Considering the rate of misdiagnosis of hereditary nephritis using light microscopy and clinical findings alone, electron microscopy study and toluidine blue staining has an essential role in the precise diagnosis in these patients. With regard to the progressive nature of these diseases, prompt diagnosis using electron microscopy is pertinent for therapeutic decisions.

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Section: Discussionmentioning

confidence: 99%

Clinicopathologic and ultrastructural findings of hereditary nephritis; a 16-year single center survey in Iran

et al. 2020

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“…Most of FSGS patients are cortisone-resistant, with a tendency to progress to end-stage renal disease [2]. Podocyte injury (including cytoskeleton impairment, hypertrophy and autophagy) and podocyte loss (including apoptosis and detachment), as well as exposure of glomeruli basement membrane, are critical mechanisms leading to the pathogenesis and progression of FSGS [3][4][5]. In diphtheria toxininduced mouse models, FSGS was observed histologically when the percentage of podocytes loss exceeded 20%.…”

Section: Introductionmentioning

confidence: 99%

Nuclear exclusion of YAP exacerbates podocyte apoptosis and disease progression in Adriamycin-induced focal segmental glomerulosclerosis

Zhuang

et al. 2021

Laboratory Investigation

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Focal segmental glomerulosclerosis (FSGS) is a chronic glomerular disease with poor clinical outcomes. Podocyte loss via apoptosis is one important mechanism underlying the pathogenesis of FSGS. Recently, Yes-associated-protein (YAP), a key downstream protein in the Hippo pathway, was identified as an activator for multiple gene transcriptional factors in the nucleus to control cell proliferation and apoptosis. To investigate the potential role of YAP in the progression of FSGS, we examined kidney samples from patients with minimal change disease or FSGS and found that increases in podocyte apoptosis is positively correlated with the cytoplasmic distribution of YAP in human FSGS. Utilizing an established mT/mG transgenic mouse model and primary cultured podocytes, we found that YAP was distributed uniformly in nucleus and cytoplasm in the podocytes of control animals. Adriamycin treatment induced gradual nuclear exclusion of YAP with enhanced phospho-YAP/YAP ratio, accompanied by the induction of podocyte apoptosis both in vivo and in vitro. Moreover, we used verteporfin to treat an Adriamycin-induced FSGS mouse model, and found YAP inhibition by verteporfin induced nuclear exclusion of YAP, thus increasing podocyte apoptosis and accelerating disease progression. Therefore, our findings suggest that YAP nuclear distribution and activation in podocytes is an important endogenous anti-apoptotic mechanism during the progression of FSGS.

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Lupus nephritis and thin glomerular basement membrane coexistence: case report and review of the literature

et al. 2021

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The pathological spectrum associated with the ultrastructural finding of thin glomerular basement membrane: A tertiary medical city experience and review of the literature

Cited by 7 publications

References 8 publications

Clinicopathologic and ultrastructural findings of hereditary nephritis; a 16-year single center survey in Iran

Clinicopathologic and ultrastructural findings of hereditary nephritis; a 16-year single center survey in Iran

Nuclear exclusion of YAP exacerbates podocyte apoptosis and disease progression in Adriamycin-induced focal segmental glomerulosclerosis

Lupus nephritis and thin glomerular basement membrane coexistence: case report and review of the literature

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