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Assessment of Kidney Function in Mouse Models of Glomerular Disease
Published on: June 30, 2018
A microscopic study of kidney tissue in Familial Mediterranean Fever patients
Seda M Sari Kiliçaslan1, Cevat Ayvali, Hakki Taştan
1Department of Biology, Ankara University, Tandogan, Ankara, Turkey. skaslan@science.ankara.edu.tr
Abstract:
Familial Mediterranean Fever (FMF) is an autosomal recessive hereditary disease leading mostly to renal failure and nephrotic syndrome. The ultrastructure of kidney has not been fully investigated in FMF associated renal disease. The aim of this study is to provide further evidence on the ultrastructure of kidney in patients with FMF who suffer from renal disease. Renal biopsies obtained from two patients who were diagnosed with FMF renal disease complications were examined. Examination of renal tissue by light and electron microscopy identified degenerations both in tubules and the filtration barrier. Foot processes were partly effaced. Amorphous material was found in thickened glomerular basement membranes. Fibrous material deposits in thick Bowman's capsule wall were also seen. Finally, degeneration in the form of folding of plasma membrane and vacuolization as well as fusion in mitochondria cristae, was observed. Accumulation of tissue remnants in the lumen was also found in tubules.
Insights
Familial Mediterranean Fever (FMF) causes kidney damage. This study reveals cellular changes in FMF renal disease, including tubular and filtration barrier degeneration, offering insights into disease progression.
Area of Science:
- Nephrology
- Genetics
- Cell Biology
Background:
- Familial Mediterranean Fever (FMF) is a genetic disorder often leading to kidney failure.
- The kidney's cellular structure in FMF-related renal disease requires further investigation.
Purpose of the Study:
- To examine the kidney's ultrastructure in patients with FMF-associated renal disease.
- To identify specific cellular and tissue-level changes contributing to FMF nephropathy.
Main Methods:
- Light and electron microscopy were used to analyze renal biopsies.
- Two patients diagnosed with FMF renal disease complications were included.
Main Results:
- Degenerative changes were observed in renal tubules and the glomerular filtration barrier.
- Specific findings include effaced foot processes, thickened glomerular basement membranes with amorphous material, and fibrous deposits in Bowman's capsule.
- Tubular degeneration included plasma membrane folding, mitochondrial abnormalities, and accumulation of cellular debris.
Conclusions:
- The study provides detailed ultrastructural evidence of kidney damage in FMF renal disease.
- These findings highlight significant cellular pathology affecting both tubules and filtration units.
- Further research into these ultrastructural changes may inform FMF nephropathy management.
