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          "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Light microscopic&#44; immunofluorescence and electron studies&#46; &#40;A&#41; Sclerotic glomeruli &#40;HE&#41;&#46; &#40;B&#41; Lobular appearance with cellular mesangial nodules &#40;PASM&#41;&#46; &#40;C&#41; Moderate to severe mesangial hypercellularity &#40;PAS&#41;&#46; &#40;D&#41; Faint staining for IgM &#40;Immunofluorescence&#41;&#46; &#40;E&#41; Massive electron-dense deposits&#46; &#40;F&#41; Fibronectin was strongly positive &#40;immunohistochemistry&#41;&#46;</p>"
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Fibronectin glomerulopathy &#40;FNG&#41; is an uncommon autosomal dominant disease with age-related penetrance&#46; Burgin<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> first reported the diease in 1980&#46; Str&#248;m<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a> verified fibronectin glomerulopathy &#8211; a newly recognized autosomal dominant kidney disease&#46; It has been distinguished from these other disease entities by the presence of glomerular fibrillary deposits that show strong immune reactivity to fibronectin &#40;Fn&#41;&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">Here we report a case of FNG 2017 in our hospital&#46; A 45-year-old Chinese female was hospitalized because of edema for 7 days&#46; She denied history of hair loss&#44; oral ulceration&#44; alopecia&#44; oliguria&#44; joint pain&#44; rashes and menstrual irregularities&#46; She had medical history of malignant hydatidiform mole 8-years ago which had been cured&#46; Her sister had nephritic syndrome for ten years with no biopsy performed&#46; Physical examination showed mild hypertension &#40;158&#47;119<span class="elsevierStyleHsp" style=""></span>mmHg&#41; and leg edema&#46; The laboratory examination showed her daily urinary protein&#44; serum albumin&#44; blood urea nitrogen and creatinine were 4&#46;21<span class="elsevierStyleHsp" style=""></span>g&#47;day&#44; 40&#46;9<span class="elsevierStyleHsp" style=""></span>g&#47;L&#44; 4&#46;39<span class="elsevierStyleHsp" style=""></span>mmol&#47;L&#44; 53<span class="elsevierStyleHsp" style=""></span>&#956;mol&#47;L&#46; The blood tests of liver function&#44; lipid&#44; immunoglobulin&#44; complement&#44; antinuclear antibody spectrum&#44; antineutrophil cytoplasmic antibody&#44; hepatitis B and C serology were all negative&#46; The renal and gynecologic ultrasound was normal&#46; The kidney biopsy was showed in <a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#46; Light microscopy showed one of 25 glomeruli was sclerotic glomeruli&#44; others showed moderate to severe mesangial hypercellularity with a lobular appearance with cellular mesangial nodules that were expanded by matrix&#46; Capillary walls showed stenosis&#44; occlusion and basement membrane thickening&#46; Segmental mesangial insertion and double track formation could be seen&#46; Immunofliorescence only showed faint staining for IgM deposits in a band pattern in the subendothelial spaces and testing of IgG&#44; IgA&#44; C3&#44; Cq1&#44; fibrinogen&#44; albumin completely negative&#46; There was no evidence of amyloid deposits on Congo Red stain&#46; Diffuse strong granular-smudgy staining was observed in mesangial and subendothelial locations with fibronectin&#46; Electron microscopy showed 2 glomeruli that the capillary loops were lobulated&#44; and focal endothelial cells proliferated&#46; The wall of renal capsule is thickened and stratified&#46; Extensive fine granular deposits in the mesangium and in the subendothelial spaces could be seen with a diameter of 10&#8211;12<span class="elsevierStyleHsp" style=""></span>nm fibrils&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">A PubMed and CNKI searching using the terms &#8220;fibronection glomerulopathy&#8221; yielded 35 English and 6 Chinese articles reports of patients with clinical and &#40;or&#41; biopsy characteristics until January 31&#44; 2018&#46; This allowed us to compare the disease clinical features and biopsy features&#46; A total of 86 patients&#44; 52 male&#44; 34 female&#44; aged 3&#8211;88<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">3</span></a> years were reported in these literatures&#46; 25&#37; of patients receive renal replacement therapy&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a> 42 patients among 78 patients observed with nephritic range proteinuria &#40;more than 3&#46;5<span class="elsevierStyleHsp" style=""></span>g&#47;day&#41;&#46; 53 patients among 85 patients suffered from hypertension&#46; 23 of 43 cases which were available Urine Analysis had microhematruia&#46; 23 cases&#8217; serum creatinine &#40;normal values 0&#46;6&#8211;1&#46;3<span class="elsevierStyleHsp" style=""></span>mg&#47;dl&#41; was abnormal except for 18 patients who did not provide renal function&#46; Family history was noted in 61 patients&#46; 57 patients of the literatures were provided with renal biopsy&#46; 47 patients were tested intense staining for the serum fibronectin by immunohisto-chemistry which was the basis for the diagnosis&#46; The Congo red staining is negative&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">FNG is a newly recognized autosomal dominant hereditary renal disease&#46; The deposition of Fn in the glomeruli is the key to FNG&#46; The increasing Fn leads to some glomerular diseases&#46; FNG is considered to be a hereditary disease and associated with mutations in the Fn1 gene&#46;<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">4&#44;5</span></a> By haplotype analysis&#44; p&#46;Pro1472del and p&#46;Leu1974Pro are fundamental mutations&#46;<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">6</span></a> FNG is considered an unimmune glomerulopathy&#46; Diagnosis of FNG is made only by renal biopsy&#46; Light microscopy has no diagnostic value for FNG&#46; The diameter of the special deposits is important to diagnose in electron microscope&#46; Fn staining of immunofluorescence can make a definite diagnosis&#46; The disease is characterized by proteinuria&#44; microscopic hematuria&#44; hypertension and slow progression to end-stage renal failure&#46; Some treatments such as glucocorticoid&#44;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a> immunosuppressant&#44;<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">8</span></a> ACEI&#44; ARB<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">9</span></a> et al&#46; have used for the disease&#44; but none of them is effective&#46; There is still a risk of recurrence after renal transplantation&#46;<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">10</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">Our patient had the distinguishing feature of the disease on the basis of clinical symptoms&#58; hypertension&#44; proteinuria&#44; microhematruia&#44; family history and pathological manifestation&#58; intense staining for the serum fibronectin&#44; Congo red staining negative&#46; At present&#44; there are no specific treatments for FNG&#46; Our patient had received angiotensin receptor blocker &#40;ARB&#41; and Shenyanshu tablets&#46; After 3 months of follow-up&#44; the urine protein was 2&#46;85<span class="elsevierStyleHsp" style=""></span>g&#47;day&#46; The proteinuria became less&#46; However&#44; further observation was needed to the proteinuria and renal function&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflict of interest</span><p id="par0030" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflict of interest&#46;</p></span></span>"
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Letter to the Editor
Fibronectin glomerulopathy: A case report and literature review
Glomerulopatía por fibronectina: informe de un caso y revisión de la literatura
Ting Wang
Autor para correspondencia
21917708@qq.com

Corresponding author.
, Hong Bw
Department of Nephrology, The Third Hospital of Mian Yang (Sichuan Mental Health Center), Mian Yang, Si Chuan, PR, China
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alopecia&#44; oliguria&#44; joint pain&#44; rashes and menstrual irregularities&#46; She had medical history of malignant hydatidiform mole 8-years ago which had been cured&#46; Her sister had nephritic syndrome for ten years with no biopsy performed&#46; Physical examination showed mild hypertension &#40;158&#47;119<span class="elsevierStyleHsp" style=""></span>mmHg&#41; and leg edema&#46; The laboratory examination showed her daily urinary protein&#44; serum albumin&#44; blood urea nitrogen and creatinine were 4&#46;21<span class="elsevierStyleHsp" style=""></span>g&#47;day&#44; 40&#46;9<span class="elsevierStyleHsp" style=""></span>g&#47;L&#44; 4&#46;39<span class="elsevierStyleHsp" style=""></span>mmol&#47;L&#44; 53<span class="elsevierStyleHsp" style=""></span>&#956;mol&#47;L&#46; The blood tests of liver function&#44; lipid&#44; immunoglobulin&#44; complement&#44; antinuclear antibody spectrum&#44; antineutrophil cytoplasmic antibody&#44; hepatitis B and C serology were all negative&#46; The renal and gynecologic ultrasound was normal&#46; The kidney biopsy was showed in <a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#46; Light microscopy showed one of 25 glomeruli was sclerotic glomeruli&#44; others showed moderate to severe mesangial hypercellularity with a lobular appearance with cellular mesangial nodules that were expanded by matrix&#46; Capillary walls showed stenosis&#44; occlusion and basement membrane thickening&#46; Segmental mesangial insertion and double track formation could be seen&#46; Immunofliorescence only showed faint staining for IgM deposits in a band pattern in the subendothelial spaces and testing of IgG&#44; IgA&#44; C3&#44; Cq1&#44; fibrinogen&#44; albumin completely negative&#46; There was no evidence of amyloid deposits on Congo Red stain&#46; Diffuse strong granular-smudgy staining was observed in mesangial and subendothelial locations with fibronectin&#46; Electron microscopy showed 2 glomeruli that the capillary loops were lobulated&#44; and focal endothelial cells proliferated&#46; The wall of renal capsule is thickened and stratified&#46; Extensive fine granular deposits in the mesangium and in the subendothelial spaces could be seen with a diameter of 10&#8211;12<span class="elsevierStyleHsp" style=""></span>nm fibrils&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">A PubMed and CNKI searching using the terms &#8220;fibronection glomerulopathy&#8221; yielded 35 English and 6 Chinese articles reports of patients with clinical and &#40;or&#41; biopsy characteristics until January 31&#44; 2018&#46; This allowed us to compare the disease clinical features and biopsy features&#46; A total of 86 patients&#44; 52 male&#44; 34 female&#44; aged 3&#8211;88<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">3</span></a> years were reported in these literatures&#46; 25&#37; of patients receive renal replacement therapy&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a> 42 patients among 78 patients observed with nephritic range proteinuria &#40;more than 3&#46;5<span class="elsevierStyleHsp" style=""></span>g&#47;day&#41;&#46; 53 patients among 85 patients suffered from hypertension&#46; 23 of 43 cases which were available Urine Analysis had microhematruia&#46; 23 cases&#8217; serum creatinine &#40;normal values 0&#46;6&#8211;1&#46;3<span class="elsevierStyleHsp" style=""></span>mg&#47;dl&#41; was abnormal except for 18 patients who did not provide renal function&#46; Family history was noted in 61 patients&#46; 57 patients of the literatures were provided with renal biopsy&#46; 47 patients were tested intense staining for the serum fibronectin by immunohisto-chemistry which was the basis for the diagnosis&#46; The Congo red staining is negative&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">FNG is a newly recognized autosomal dominant hereditary renal disease&#46; The deposition of Fn in the glomeruli is the key to FNG&#46; The increasing Fn leads to some glomerular diseases&#46; FNG is considered to be a hereditary disease and associated with mutations in the Fn1 gene&#46;<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">4&#44;5</span></a> By haplotype analysis&#44; p&#46;Pro1472del and p&#46;Leu1974Pro are fundamental mutations&#46;<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">6</span></a> FNG is considered an unimmune glomerulopathy&#46; Diagnosis of FNG is made only by renal biopsy&#46; Light microscopy has no diagnostic value for FNG&#46; The diameter of the special deposits is important to diagnose in electron microscope&#46; Fn staining of immunofluorescence can make a definite diagnosis&#46; The disease is characterized by proteinuria&#44; microscopic hematuria&#44; hypertension and slow progression to end-stage renal failure&#46; Some treatments such as glucocorticoid&#44;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a> immunosuppressant&#44;<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">8</span></a> ACEI&#44; ARB<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">9</span></a> et al&#46; have used for the disease&#44; but none of them is effective&#46; There is still a risk of recurrence after renal transplantation&#46;<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">10</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">Our patient had the distinguishing feature of the disease on the basis of clinical symptoms&#58; hypertension&#44; proteinuria&#44; microhematruia&#44; family history and pathological manifestation&#58; intense staining for the serum fibronectin&#44; Congo red staining negative&#46; At present&#44; there are no specific treatments for FNG&#46; Our patient had received angiotensin receptor blocker &#40;ARB&#41; and Shenyanshu tablets&#46; After 3 months of follow-up&#44; the urine protein was 2&#46;85<span class="elsevierStyleHsp" style=""></span>g&#47;day&#46; The proteinuria became less&#46; However&#44; further observation was needed to the proteinuria and renal function&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflict of interest</span><p id="par0030" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflict of interest&#46;</p></span></span>"
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          "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Light microscopic&#44; immunofluorescence and electron studies&#46; &#40;A&#41; Sclerotic glomeruli &#40;HE&#41;&#46; &#40;B&#41; Lobular appearance with cellular mesangial nodules &#40;PASM&#41;&#46; &#40;C&#41; Moderate to severe mesangial hypercellularity &#40;PAS&#41;&#46; &#40;D&#41; Faint staining for IgM &#40;Immunofluorescence&#41;&#46; &#40;E&#41; Massive electron-dense deposits&#46; &#40;F&#41; Fibronectin was strongly positive &#40;immunohistochemistry&#41;&#46;</p>"
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