Extraglomerular Vascular Involvement of Glomerulopathy with Fibronectin Deposits

Hara, Masayuki; Kusaba, Tetsuro; Ono, Kenshi; Masuzawa, Naoko; Nakamura, Itaru; Urata, Noriko; Shiraishi, Hiroaki; Hara, Shigeo; Konishi, Eiichi; Matoba, Satoaki; Shiotsu, Yayoi; Tamagaki, Keiichi

doi:10.2169/internalmedicine.6558-20

Cited by 2 publications

(2 citation statements)

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“…Влияние возраста, высокоуглеводной и высокожировой диеты между увеличением концентрации фибронектина и диабетической нефропатией [18], повышением концентрации фибронектина в плазме крови и сахарным диабетом 1-го типа [19], а также гипертензией, нефропатией [20], ожирением и триглицеридемией у пациентов с сахарным диабетом 2-го типа [21].…”

Section: Discussionunclassified

The effect of age and a high-fat, high-carbohydrate diet on the development of arterial hypertension and kidney disease in the experiment

et al. 2022

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Aim. To identify the structural foundations of the pathogenesis of arterial hypertension and kidney disease associated with a high-fat, high-carbohydrate diet and age.Materials and methods. The study was carried out on male Wistar rats aged 60 and 450 days. The animals were divided into 4 groups: group 1 (n = 14) – intact rats (60 days old) fed with a standard diet for 90 days; group 2 (n = 14) – rats (aged 60 days) receiving a high-fat, high-carbohydrate diet for 90 days; group 3 (n = 14) – intact rats (aged 450 days) receiving a standard diet for 90 days; group 4 (n = 14) – rats (aged 450 days) fed with a high-fat, high-carbohydrate diet for 90 days. Clinical and instrumental research methods, enzyme-linked immunosorbent assay, and immunohistochemistry and histology techniques were used in the study.Results. Feeding 60-day-old animals with a high-fat, high-carbohydrate diet resulted in an increase in body weight and abdominal fat, a rise in systolic blood pressure, and moderately pronounced histologic changes in the kidneys. In intact 450-day-old rats, age-related changes prevailed: changes in the myocardial mass, an increase in TGF-β1, morphological changes in the renal tubules and glomeruli. In 450-day-old rats receiving a high-fat, highcarbohydrate diet, the most pronounced increase in both systolic and diastolic blood pressure, a significant rise in serum fibronectin, and destructive changes in the renal tissue were noted.Conclusion. Functional and biochemical signs of arterial hypertension and morphological changes in the kidneys were the most pronounced in 450-day-old rats fed with a high-fat, high-carbohydrate diet.

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Section: Discussionunclassified

The effect of age and a high-fat, high-carbohydrate diet on the development of arterial hypertension and kidney disease in the experiment

et al. 2022

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“…In this study group, overall dominant mutations in FN1 accounted for 40% of GFND cases. However, the other 60% of familial GFND cases did not show mutations in the pathogenic gene FN1 (7). In addition, family members had different symptoms even if they had the same gene mutation.…”

Section: Introductionmentioning

confidence: 98%

Bortezomib-containing regiment in treating glomerulopathy with fibronectin deposits combined with monoclonal gammopathy of undetermined significance: a case report and literature review

Zhang

Wei

et al. 2022

Ann Transl Med

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Background: Glomerulopathy with fibronectin deposits (GFND) is a newly recognized rare glomerular disease. As its onset can be stably inherited in affected families without sex differences and fibronectin 1 (FN1) mutations can be detected in 40% of patients' families, GFND is considered to be an autosomal dominant genetic disease. The main clinical manifestations are proteinuria, progressive renal failure, edema, hypertension, hematuria, and type 4 renal tubular acidosis. The diagnosis was confirmed by renal biopsy, and there was no specific treatment. Monoclonal gammopathy refers to the existence of monoclonal immunoglobulin (MIg) produced by monoclonal plasma cells in serum. When MIg damages the kidney by direct deposition or indirect mechanisms, it is defined as monoclonal gammopathy of renal significance (MGRS). The principle of treatment is to inhibit plasma cells from producing MIg.Case Description: We report the efficacy of a case of GFND combined with monoclonal gammopathy of undetermined significance (MGUS) treated with a bortezomib-containing regimen. A 44-year-old female patient was admitted to the hospital for "edema of both lower extremities for 1 month and aggravation for 5 days". In May 2018, after exertion, the patient developed edema of both lower extremities, accompanied by foamy urine with no obvious deepening of urine color or decreased output, no gross hematuria, and gradual aggravation with fatigue. Conclusion:After treatment, the edema of patient subsided, urinary protein decreased significantly, and serum albumin increased near to normal. It is achieving a very good therapeutic effect and long-term event-free survival.The treatment is safety and there are no obvious toxic side effects. It provides a new idea for the treatment of GFND.

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Extraglomerular Vascular Involvement of Glomerulopathy with Fibronectin Deposits

Cited by 2 publications

References 14 publications

The effect of age and a high-fat, high-carbohydrate diet on the development of arterial hypertension and kidney disease in the experiment

The effect of age and a high-fat, high-carbohydrate diet on the development of arterial hypertension and kidney disease in the experiment

Bortezomib-containing regiment in treating glomerulopathy with fibronectin deposits combined with monoclonal gammopathy of undetermined significance: a case report and literature review

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