Clinicopathological Analysis of Glomerulopathy with Fibronectin Deposits (GFND): A Case of Sporadic, Elderly-Onset GFND with Codeposition of IgA, C1q, and Fibrinogen

Yoshino, Masabumi; Miura, Naoto; Ohnishi, Takahiro; Suzuki, Keisuke; Kitagawa, Wataru; Nishikawa, Kazuhiro; Imai, Hirokazu

doi:10.2169/internalmedicine.52.0046

Cited by 20 publications

(12 citation statements)

References 16 publications

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“…However, a better understanding of the genetic basis of the diseases has prompted a better classification of BFH into a number of rare glomerulopathies, such as MYH9-associated kidney disease, giant fibronectin glomerulopathy, C3/CFHR5 glomerulonephritis, immunotactoid glomerulonephritis and fibrillary glomerulonephritis. The renal outcome of these entities is worse than was initially described for BFH [47,[72][73][74][75][76].…”

Section: Recently Described Glomerulopathiesmentioning

confidence: 89%

Haematuria as a risk factor for chronic kidney disease progression in glomerular diseases: A review

et al. 2015

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Haematuria has long been considered to be a benign condition associated with glomerular diseases. However, new evidences suggest that haematuria has a pathogenic role in promoting kidney disease progression. An increased risk for end-stage renal disease has been reported in adolescents and young adults with persistent microscopic haematuria. A persistent impairment of renal function has been also reported following macroscopic haematuria-associated acute kidney injury in immunoglobulin A nephropathy. Haematuria-induced renal damage has been related to oxidant, cytotoxic and inflammatory effects induced by haemoglobin or haem released from red blood cells. The pathophysiological origin of haematuria may be due to a more fragile and easily ruptured glomerular filtration barrier, as reported in several glomerular diseases. In this review we describe a number of the key issues associated with the epidemiology and pathogenesis of haematuria-associated diseases, provide an update of recent knowledge on the role of haematuria on renal function outcome and discuss specific therapeutic approaches in this setting. KEY SUMMARY POINTS: 1. Glomerular haematuria is a common observation in a number of renal diseases that may lead to persistent renal injury. 2. Haematuria in children differs from that in adults in specific aspects, particularly in the frequency of glomerular diseases and renal disease outcome. 3. Regular follow-up of renal function in children with isolated microhaematuria may be recommended.

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Section: Recently Described Glomerulopathiesmentioning

confidence: 89%

Haematuria as a risk factor for chronic kidney disease progression in glomerular diseases: A review

et al. 2015

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“…Deposition of immunoglobulins or complement components in the glomeruli is a commonly observed phenomenon in immunofluorescent study. Yoshino et al ( 15 ) reported detection of immunoglobulin, fibrinogen, or complement components in 15 of their 43 cases (35%) ( 16 ). The link between immunoglobulin or complement component deposition and prognosis has not been clearly defined.…”

Section: Discussionmentioning

confidence: 99%

Clinicopathologic Features and Outcomes in Fibronectin Glomerulopathy: A Case Series of 19 Patients

Zhang

Zuo

et al. 2020

Front. Med.

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Aims: To describe the characteristics and prognosis of 19 patients with fibronectin glomerulopathy (FNG) and evaluate prognostic factors associated with poor renal outcomes. Methods: Included in this retrospective study was 19 FNG patients in Nanjing Glomerulonephritis Registry system. Associations between the clinical parameters, pathological features, and renal outcomes were evaluated by Kaplan-Meier survival analysis. Results: Of the 19 FNG patients included in this study, 8 (42.1%) were women. The median age of the 19 FNG patients was 31 (17-71) years, and the median disease duration 48 (1-175) months at diagnosis. At the time of renal biopsy, the mean serum creatinine (Scr) was 1.22 ± 0.16 mg/dl and urinary protein was 6.24 ± 0.97 mg/24 h. Renal biopsy showed a lobular appearance with cellular mesangial nodules expanded by matrix in 14 cases. After a median follow-up period of 87 months (interquartile range 34-114.5 months), 8 FNG patients developed renal function decline, including 7 progressing into end-stage renal disease (ESRD) and 1 presenting with by a 2-fold-increase in Scr. Scr and proteinuria remained stable in the remaining 11 patients. Kaplan-Meier survival analysis showed that nephrotic range proteinuria (P = 0.022) and focal glomerular sclerosis (P = 0.028) were associated with renal function decline. Conclusions: Nephrotic range proteinuria and focal glomerular sclerosis were associated with renal function decline during the follow-up period of the FNG patients in our series. FNG Patients at risk of renal function decline should be identified preferentially and given more progressive and effective therapies to prevent further disease progression.

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“…Its mutations altered the proteindimers assembly into fibrils in the extracellular matrix and produce a disbalance between soluble and insoluble fibronectin, leading to its pathognomonic deposition in mesangium and subendothelial area [66,67] . In addition to fibronectine deposition, it has been reported IgA, C1q and fibrinogen deposits [68] . GFND may present at different ages, although mostly in adolescence or early adulthood.…”

Section: Fibril Deposit Diseasementioning

confidence: 99%

Pathogenesis of glomerular haematuria

Yuste

Gutiérrez

Sevillano

et al. 2015

WJN

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Haematuria was known as a benign hallmark of some glomerular diseases, but over the last decade, new evidences pointed its negative implications on kidney disease progression. Cytotoxic effects of oxidative stress induced by hemoglobin, heme, or iron released from red blood cells may account for the tubular injury observed in human biopsy specimens. However, the precise mechanisms responsible for haematuria remain unclear. The presence of red blood cells (RBCs) with irregular contours and shape in the urine indicates RBCs egression from the glomerular capillary into the urinary space. Therefore glomerular haematuria may be a marker of glomerular filtration barrier dysfunction or damage. In this review we describe some key issues regarding epidemiology and pathogenesis of haematuric diseases as well as their renal morphological findings.

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Clinicopathological Analysis of Glomerulopathy with Fibronectin Deposits (GFND): A Case of Sporadic, Elderly-Onset GFND with Codeposition of IgA, C1q, and Fibrinogen

Cited by 20 publications

References 16 publications

Haematuria as a risk factor for chronic kidney disease progression in glomerular diseases: A review

Haematuria as a risk factor for chronic kidney disease progression in glomerular diseases: A review

Clinicopathologic Features and Outcomes in Fibronectin Glomerulopathy: A Case Series of 19 Patients

Pathogenesis of glomerular haematuria

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