A recurrent fibronectin glomerulopathy in a renal transplant patient: a case report

Otsuka, Yasuhiro; Takeda, Asami; Horike, Keiji; Inaguma, Daijyo; Goto, Norihiko; Watarai, Yoshihiko; Uchida, Kazuharu; Mihatsch, Michael J.; Joh, Kensuke; Morozumi, Kunio

doi:10.1111/j.1399-0012.2012.01644.x

Cited by 30 publications

(18 citation statements)

References 11 publications

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“…Almost all the reports of recurrence of rare renal disease presented details of both histological changes based on protocol biopsies and clinical course. These reports included recurrence of light chain deposition disease, fibronectin nephropathy, atypical HUS caused by complement regulatory factor H disorder, HSPN, IgA nephropathy, C‐ANCA‐associated glomerulonephritis, mixed cryogloburinemic glomerulonephritis, FSGS and others. We strongly encourage learning from these papers for a better understanding of the detailed changes in recurrent glomerular diseases.…”

Section: Significance Of Protocol Biopsy For Early Diagnosis Of Recurmentioning

confidence: 99%

Recurrent glomerular disease after kidney transplantation: An update of selected areas and the impact of protocol biopsy

et al. 2014

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ABSTRACT:Recurrence of native kidney disease following kidney transplantation affects between 10% and 20% of patients, and accounts for up to 8% of graft failures. In a considerable number of recipients with transplant glomerulopathy, it is impossible to distinguish between recurrent and de novo types. An accurate estimate of the incidence of recurrence is difficult due to limitations in the diagnosis of recurrent glomerulonephritis. De novo glomerular lesions may be misclassified if histological confirmation of the patient's native kidney disease is lacking. Asymptomatic histological recurrence in renal allografts may be missed if protocol biopsies are not available. Studies based on protocol biopsy are pivotal to accurately estimate the incidence of recurrence. Many factors are known to influence recurrence of kidney disease after transplantation, including the type and severity of the original disease, age at onset, interval from onset to end-stage renal disease, and clinical course of the previous transplantation. Early recognition of recurrence is possible in several glomerular diseases. Factors such as the existence of circulating permeability factors, circulating urokinase receptor and anti-phospholipase A2 receptor antibody, as well as disorders of complement regulatory proteins like factor I mutation and factor H mutation factors are expected to be useful predictors of recurrence. Peculiar clinical course of atypical haemolytic uremic syndrome after kidney transplantation is an informative sign of recurrent glomerular disease. These factors play pivotal roles in the development of recurrence of certain types of glomerulopathies. Understanding the pathogenesis of recurrent glomerulonephritis is critical to optimize prevention as well as treat individual cases of recurrent glomerulonephritis. Subclinical recurrence of IgA nephropathy after kidney transplantation is well recognized. Only protocol biopsies of clinically silent recipient can provide the accurate prevalence of recurrent IgA nephropathy. The study of recurrent glomerulonephritis will contribute not only to improving long-term graft survival, but also to clarifying the pathogenesis of glomerulonephritis. Protocol biopsy is one the most effective methods for elucidating the pathogenesis of recurrent glomerulonephritis.

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Section: Significance Of Protocol Biopsy For Early Diagnosis Of Recurmentioning

confidence: 99%

Recurrent glomerular disease after kidney transplantation: An update of selected areas and the impact of protocol biopsy

et al. 2014

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“…There have been numerous reports of cases with detailed family histories, but few reports of sporadic cases, such as the present patient [ 5 , 19 , 20 , 22 ]. Eighteen of the 75 reported cases (24%) had no apparent family history of renal diseases.…”

Section: Discussionmentioning

confidence: 99%

Fibronectin glomerulopathy complicated with persistent cloaca and congenital esophageal atresia: a case report and literature review

et al. 2017

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BackgroundFibronectin glomerulopathy is a rare, inherited, autosomal dominant, glomerular disease characterized by proteinuria, microscopic hematuria, hypertension, massive glomerular deposits of fibronectin, and slow progression to end-stage renal failure. Because the incident of fibronectin glomerulopathy is extremely low, the pathophysiology, genetic abnormalities, epidemiology, and mechanisms remain to be elucidated.Case presentationWe report a 21-year-old woman with fibronectin glomerulopathy, who had been diagnosed with persistent cloaca and congenital esophageal atresia at birth. She developed proteinuria and hematuria 7 months before admission. Urinary protein and serum creatinine levels were 3.38 g/gCr and 0.73 mg/dL. Renal biopsy showed severe mesangial widening due to massive deposits, which was positive periodic acid-Schiff and negative methenamine silver. Immunostaining was negative for immunoglobulin but positive for fibronectin. Electron microscopy showed diffuse mesangial granular deposits. Thus she was diagnosed with fibronectin glomerulopathy, despite a negative family history of kidney disease and lack of any known missense mutations of fibronectin 1 gene.ConclusionWe report a patient who developed fibronectin glomerulopathy during the clinical course of extremely rare congenital malformations, including persistent cloaca and congenital esophageal atresia. We describe a case of this condition in detail and summarize the 75 case reports of fibronectin glomerulopathy.

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“…Although GFND is defined as a new disease entity where fibronectin polymer deposits in the glomeruli and several gene mutations have been reported, the critical polymerization of fibronectin is still under investigation. (2,(8)(9)(10)(11)(12)(13)(14)(15). In 37 of the 44 cases (84%), the onset occurs before the age of 50.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2013

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A 67-year-old Japanese man with recurrent nephrotic syndrome and impaired kidney function had a sporadic, elderly-onset case of glomerulopathy with fibronectin deposits. The daily urinary protein, serum albumin, blood urea nitrogen, and creatinine levels were 3.6 g/day, 2.7 g/dL, 19.5 mg/dL, and 1.70 mg/dL, respectively. Kidney biopsy samples were evaluated using electron microscopy and demonstrated membranoproliferative glomerulonephritis-like lesions with massive subendothelial depositions and no fibrillary structures. Immunofluorescent studies showed 1+ staining for IgA, C1q, and fibrinogen. Anti-fibronectin immunostaining demonstrated that the subendothelial deposits were positive for fibronectin. The administration of prednisolone and mizoribine improved the nephrotic syndrome.

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A recurrent fibronectin glomerulopathy in a renal transplant patient: a case report

Cited by 30 publications

References 11 publications

Recurrent glomerular disease after kidney transplantation: An update of selected areas and the impact of protocol biopsy

Recurrent glomerular disease after kidney transplantation: An update of selected areas and the impact of protocol biopsy

Fibronectin glomerulopathy complicated with persistent cloaca and congenital esophageal atresia: a case report and literature review

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

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