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"titulo" => "Fibronectin glomerulopathy: A case report and literature review" "tieneTextoCompleto" => true "saludo" => "Dear Editor," "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "74" "paginaFinal" => "76" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "Ting Wang, Hong Bw" "autores" => array:2 [ 0 => array:4 [ "nombre" => "Ting" "apellidos" => "Wang" "email" => array:1 [ 0 => "21917708@qq.com" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:2 [ "nombre" => "Hong" "apellidos" => "Bw" ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Department of Nephrology, The Third Hospital of Mian Yang (Sichuan Mental Health Center), Mian Yang, Si Chuan, PR, China" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "<span class="elsevierStyleItalic">Corresponding author</span>." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Glomerulopatía por fibronectina: informe de un caso y revisión de la literatura" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 2077 "Ancho" => 1800 "Tamanyo" => 647157 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Light microscopic, immunofluorescence and electron studies. (A) Sclerotic glomeruli (HE). (B) Lobular appearance with cellular mesangial nodules (PASM). (C) Moderate to severe mesangial hypercellularity (PAS). (D) Faint staining for IgM (Immunofluorescence). (E) Massive electron-dense deposits. (F) Fibronectin was strongly positive (immunohistochemistry).</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Fibronectin glomerulopathy (FNG) is an uncommon autosomal dominant disease with age-related penetrance. Burgin<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> first reported the diease in 1980. Strøm<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a> verified fibronectin glomerulopathy – a newly recognized autosomal dominant kidney disease. It has been distinguished from these other disease entities by the presence of glomerular fibrillary deposits that show strong immune reactivity to fibronectin (Fn).</p><p id="par0010" class="elsevierStylePara elsevierViewall">Here we report a case of FNG 2017 in our hospital. A 45-year-old Chinese female was hospitalized because of edema for 7 days. She denied history of hair loss, oral ulceration, alopecia, oliguria, joint pain, rashes and menstrual irregularities. She had medical history of malignant hydatidiform mole 8-years ago which had been cured. Her sister had nephritic syndrome for ten years with no biopsy performed. Physical examination showed mild hypertension (158/119<span class="elsevierStyleHsp" style=""></span>mmHg) and leg edema. The laboratory examination showed her daily urinary protein, serum albumin, blood urea nitrogen and creatinine were 4.21<span class="elsevierStyleHsp" style=""></span>g/day, 40.9<span class="elsevierStyleHsp" style=""></span>g/L, 4.39<span class="elsevierStyleHsp" style=""></span>mmol/L, 53<span class="elsevierStyleHsp" style=""></span>μmol/L. The blood tests of liver function, lipid, immunoglobulin, complement, antinuclear antibody spectrum, antineutrophil cytoplasmic antibody, hepatitis B and C serology were all negative. The renal and gynecologic ultrasound was normal. The kidney biopsy was showed in <a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>. Light microscopy showed one of 25 glomeruli was sclerotic glomeruli, others showed moderate to severe mesangial hypercellularity with a lobular appearance with cellular mesangial nodules that were expanded by matrix. Capillary walls showed stenosis, occlusion and basement membrane thickening. Segmental mesangial insertion and double track formation could be seen. Immunofliorescence only showed faint staining for IgM deposits in a band pattern in the subendothelial spaces and testing of IgG, IgA, C3, Cq1, fibrinogen, albumin completely negative. There was no evidence of amyloid deposits on Congo Red stain. Diffuse strong granular-smudgy staining was observed in mesangial and subendothelial locations with fibronectin. Electron microscopy showed 2 glomeruli that the capillary loops were lobulated, and focal endothelial cells proliferated. The wall of renal capsule is thickened and stratified. Extensive fine granular deposits in the mesangium and in the subendothelial spaces could be seen with a diameter of 10–12<span class="elsevierStyleHsp" style=""></span>nm fibrils.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">A PubMed and CNKI searching using the terms “fibronection glomerulopathy” yielded 35 English and 6 Chinese articles reports of patients with clinical and (or) biopsy characteristics until January 31, 2018. This allowed us to compare the disease clinical features and biopsy features. A total of 86 patients, 52 male, 34 female, aged 3–88<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">3</span></a> years were reported in these literatures. 25% of patients receive renal replacement therapy.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a> 42 patients among 78 patients observed with nephritic range proteinuria (more than 3.5<span class="elsevierStyleHsp" style=""></span>g/day). 53 patients among 85 patients suffered from hypertension. 23 of 43 cases which were available Urine Analysis had microhematruia. 23 cases’ serum creatinine (normal values 0.6–1.3<span class="elsevierStyleHsp" style=""></span>mg/dl) was abnormal except for 18 patients who did not provide renal function. Family history was noted in 61 patients. 57 patients of the literatures were provided with renal biopsy. 47 patients were tested intense staining for the serum fibronectin by immunohisto-chemistry which was the basis for the diagnosis. The Congo red staining is negative.</p><p id="par0020" class="elsevierStylePara elsevierViewall">FNG is a newly recognized autosomal dominant hereditary renal disease. The deposition of Fn in the glomeruli is the key to FNG. The increasing Fn leads to some glomerular diseases. FNG is considered to be a hereditary disease and associated with mutations in the Fn1 gene.<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">4,5</span></a> By haplotype analysis, p.Pro1472del and p.Leu1974Pro are fundamental mutations.<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">6</span></a> FNG is considered an unimmune glomerulopathy. Diagnosis of FNG is made only by renal biopsy. Light microscopy has no diagnostic value for FNG. The diameter of the special deposits is important to diagnose in electron microscope. Fn staining of immunofluorescence can make a definite diagnosis. The disease is characterized by proteinuria, microscopic hematuria, hypertension and slow progression to end-stage renal failure. Some treatments such as glucocorticoid,<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a> immunosuppressant,<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">8</span></a> ACEI, ARB<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">9</span></a> et al. have used for the disease, but none of them is effective. There is still a risk of recurrence after renal transplantation.<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: hypertension, proteinuria, microhematruia, family history and pathological manifestation: intense staining for the serum fibronectin, Congo red staining negative. At present, there are no specific treatments for FNG. Our patient had received angiotensin receptor blocker (ARB) and Shenyanshu tablets. After 3 months of follow-up, the urine protein was 2.85<span class="elsevierStyleHsp" style=""></span>g/day. The proteinuria became less. However, further observation was needed to the proteinuria and renal function.</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.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:2 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Conflict of interest" ] 1 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "multimedia" => array:1 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 2077 "Ancho" => 1800 "Tamanyo" => 647157 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Light microscopic, immunofluorescence and electron studies. (A) Sclerotic glomeruli (HE). (B) Lobular appearance with cellular mesangial nodules (PASM). (C) Moderate to severe mesangial hypercellularity (PAS). (D) Faint staining for IgM (Immunofluorescence). (E) Massive electron-dense deposits. (F) Fibronectin was strongly positive (immunohistochemistry).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:10 [ 0 => array:3 [ "identificador" => "bib0055" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Familail glomerulopathy with giant fibrillar deposits" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:3 [ 0 => "M. Burgin" 1 => "E. Hofmann" 2 => "F.W. 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Nozu" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1007/s00467-016-3368-7" "Revista" => array:6 [ "tituloSerie" => "Pediatr Nephrol" "fecha" => "2016" "volumen" => "31" "paginaInicial" => "1459" "paginaFinal" => "1467" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/27056061" "web" => "Medline" ] ] ] ] ] ] ] ] 6 => array:3 [ "identificador" => "bib0085" "etiqueta" => "7" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Fibronectin non-amyloid glomerulopathy" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:3 [ 0 => "J.L. Yong" 1 => "M.C. Killingsworth" 2 => "S.T. 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Mazbar" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:5 [ "tituloSerie" => "Am J kidney" "fecha" => "2012" "volumen" => "60" "paginaInicial" => "839" "paginaFinal" => "842" ] ] ] ] ] ] 9 => array:3 [ "identificador" => "bib0100" "etiqueta" => "10" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "A recurrent fibronectin glomerulopathy in a renal transplant patient: a case report" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:3 [ 0 => "Y. Otsuka" 1 => "A. Takeda" 2 => "K. Horike" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1111/j.1399-0012.2012.01644.x" "Revista" => array:7 [ "tituloSerie" => "Clin Transplant" "fecha" => "2012" "volumen" => "26" "numero" => "Suppl. 24" "paginaInicial" => "58" "paginaFinal" => "63" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/22747478" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/20132514/0000004100000001/v1_202103250823/S201325142100016X/v1_202103250823/en/main.assets" "Apartado" => array:4 [ "identificador" => "35436" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Letters to the Editor" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/20132514/0000004100000001/v1_202103250823/S201325142100016X/v1_202103250823/en/main.pdf?idApp=UINPBA000064&text.app=https://revistanefrologia.com/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S201325142100016X?idApp=UINPBA000064" ]
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2023 June | 49 | 25 | 74 |
2023 May | 58 | 48 | 106 |
2023 April | 35 | 18 | 53 |
2023 March | 52 | 27 | 79 |
2023 February | 32 | 18 | 50 |
2023 January | 33 | 36 | 69 |
2022 December | 48 | 23 | 71 |
2022 November | 46 | 33 | 79 |
2022 October | 48 | 60 | 108 |
2022 September | 31 | 32 | 63 |
2022 August | 31 | 45 | 76 |
2022 July | 37 | 40 | 77 |
2022 June | 19 | 34 | 53 |
2022 May | 30 | 22 | 52 |
2022 April | 26 | 41 | 67 |
2022 March | 35 | 54 | 89 |
2022 February | 32 | 45 | 77 |
2022 January | 40 | 39 | 79 |
2021 December | 22 | 43 | 65 |
2021 November | 39 | 42 | 81 |
2021 October | 41 | 66 | 107 |
2021 September | 35 | 40 | 75 |
2021 August | 31 | 47 | 78 |
2021 July | 35 | 35 | 70 |
2021 June | 20 | 25 | 45 |
2021 May | 51 | 58 | 109 |
2021 April | 96 | 104 | 200 |
2021 March | 8 | 15 | 23 |