Letter to the Editor
Glomerular involvement in patient with sickle cell disease
Afectación glomerular en paciente con enfermedad falciforme
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Hipertrofia glomerular.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Roman Hernández-Gallego, Isis Cerezo, Sergio Barroso, Lilia Azevedo, María López, Nicolás Roberto Robles, Juan José Cubero" "autores" => array:7 [ 0 => array:2 [ "nombre" => "Roman" "apellidos" => "Hernández-Gallego" ] 1 => array:2 [ "nombre" => "Isis" "apellidos" => "Cerezo" ] 2 => array:2 [ "nombre" => "Sergio" "apellidos" => "Barroso" ] 3 => array:2 [ "nombre" => "Lilia" "apellidos" => "Azevedo" ] 4 => array:2 [ "nombre" => "María" "apellidos" => "López" ] 5 => array:2 [ "nombre" => "Nicolás Roberto" "apellidos" => "Robles" ] 6 => array:2 [ "nombre" => "Juan José" "apellidos" => "Cubero" ] ] ] ] ] "idiomaDefecto" => "es" "Traduccion" => array:1 [ "en" => array:9 [ "pii" => "S2013251417301244" "doi" => "10.1016/j.nefroe.2016.12.012" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => true "ES2" => true "LATM" => true ] "gratuito" => true "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2013251417301244?idApp=UINPBA000064" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0211699517300152?idApp=UINPBA000064" "url" => "/02116995/0000003700000004/v1_201707260022/S0211699517300152/v1_201707260022/es/main.assets" ] ] "itemSiguiente" => array:20 [ "pii" => "S2013251417301220" "issn" => "20132514" "doi" => "10.1016/j.nefroe.2016.11.015" "estado" => "S300" "fechaPublicacion" => "2017-07-01" "aid" => "295" "copyright" => "Sociedad Española de Nefrología" "documento" => "simple-article" "crossmark" => 0 "licencia" => "http://creativecommons.org/licenses/by-nc-nd/4.0/" "subdocumento" => "cor" "cita" => "Nefrologia (English Version). 2017;37:439-41" "abierto" => array:3 [ "ES" => true "ES2" => true "LATM" => true ] "gratuito" => true "lecturas" => array:2 [ "total" => 4269 "formatos" => array:3 [ "EPUB" => 328 "HTML" => 3376 "PDF" => 565 ] ] "en" => array:11 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Letter to the Editor</span>" "titulo" => "Immuno complex mediated acute glomerulonephritis in a patient with infectious mononucleosis" "tienePdf" => "en" "tieneTextoCompleto" => "en" "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "439" "paginaFinal" => "441" ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Glomerulonefritis aguda por inmunocomplejos en mononucleosis infecciosa" ] ] "contieneTextoCompleto" => array:1 [ "en" => true ] "contienePdf" => array:1 [ "en" => true ] "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" => 532 "Ancho" => 1500 "Tamanyo" => 165295 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">CT scan (A): atelectasis/bibasal condensation with symmetrical effusion, pericardial effusion. Skin biopsy (B): focal intracellular epidermal spongiosis, mild edema and hepatic extravasation; and; dermal vessels occupied by inflammatory cells (increase: lymphocytes and polymorphonuclear cells with eosinophilic isolates).</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "M. Adoración Martín-Gómez, Mercedes Caba Molina, Gracia Cruz Caparros, Encarnación Guerrero Sánchez, Abelardo Caballero González, Mercedes Gómez Morales" "autores" => array:6 [ 0 => array:2 [ "nombre" => "M. 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respectively, under hemodialysis (HD) with or without blood transfusion. rSO<span class="elsevierStyleInf">2</span> ratio is defined as the ratio of rSO<span class="elsevierStyleInf">2</span> value at <span class="elsevierStyleItalic">t</span> (min) during HD and the initial rSO<span class="elsevierStyleInf">2</span> value before HD (rSO<span class="elsevierStyleInf">2</span> at <span class="elsevierStyleItalic">t</span> (min) during HD/initial rSO<span class="elsevierStyleInf">2</span> before HD).</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Kiyonori Ito, Susumu Ookawara, Yuichiro Ueda, Takayuki Uchida, Masaya Kofuji, Hideyuki Hayasaka, Taro Hoshino, Kaoru Tabei, Yoshiyuki Morishita" "autores" => array:9 [ 0 => array:2 [ "nombre" => "Kiyonori" "apellidos" => "Ito" ] 1 => array:2 [ "nombre" => "Susumu" "apellidos" => "Ookawara" ] 2 => array:2 [ "nombre" => "Yuichiro" "apellidos" => "Ueda" ] 3 => array:2 [ "nombre" => "Takayuki" "apellidos" => "Uchida" ] 4 => 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[ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Letter to the Editor</span>" "titulo" => "Glomerular involvement in patient with sickle cell disease" "tieneTextoCompleto" => true "saludo" => "Dear Editor," "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "437" "paginaFinal" => "439" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "Roman Hernández-Gallego, Isis Cerezo, Sergio Barroso, Lilia Azevedo, María López, Nicolás Roberto Robles, Juan José Cubero" "autores" => array:7 [ 0 => array:4 [ "nombre" => "Roman" "apellidos" => "Hernández-Gallego" "email" => array:1 [ 0 => "romanhg78@hotmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "Isis" "apellidos" => "Cerezo" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "Sergio" "apellidos" => "Barroso" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 3 => array:3 [ "nombre" => "Lilia" "apellidos" => "Azevedo" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 4 => array:3 [ "nombre" => "María" "apellidos" => "López" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 5 => array:3 [ "nombre" => "Nicolás Roberto" "apellidos" => "Robles" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 6 => array:3 [ "nombre" => "Juan José" "apellidos" => "Cubero" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Servicio de Nefrología, Hospital Universitario Infanta Cristina, Badajoz, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Anatomía Patológica, Hospital Universitario Infanta Cristina, Badajoz, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "<span class="elsevierStyleItalic">Corresponding author</span>." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Afectación glomerular en paciente con enfermedad falciforme" ] ] "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" => 1307 "Ancho" => 1625 "Tamanyo" => 669401 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Hematoxylin–eosin. Glomerular hypertrophy.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Sickle cell nephropathy is one of the complications of sickle cell disease (SCD); this is due to the polymerization of deoxygenated hemoglobin S in the renal medulla, a place of special physiological conditions (hyperosmolarity, hypoxia and acidosis)<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1,2</span></a> which contributes to the typical obstruction of vessels.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">3</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">Chronic renal failure and proteinuria are the risk factors associated with increased mortality in these patients.<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">4</span></a> Albuminuria is the initial marker of SCD associated glomerulopathy, the most frequent expression of which is focal and segmental glomerulosclerosis (FSG).<a class="elsevierStyleCrossRefs" href="#bib0075"><span class="elsevierStyleSup">5,6</span></a> Renal biopsy is indicated if glomerulopathy is suspected.</p><p id="par0015" class="elsevierStylePara elsevierViewall">We present the case of a 30-year-old white male, with a history of homozygous sickle cell disease that was treated with hydroxyurea and deferasirox, he develops 1–2 annual crises, with no renal repercussions until present. Smoker of 3 cigarettes per day.</p><p id="par0020" class="elsevierStylePara elsevierViewall">The patient was sent for consultation in relation of proteinuria of 2.75<span class="elsevierStyleHsp" style=""></span>g in 24<span class="elsevierStyleHsp" style=""></span>h (albuminuria: 1.5<span class="elsevierStyleHsp" style=""></span>g/24<span class="elsevierStyleHsp" style=""></span>h). The urine density was 1006, pH: 5.5, proteinuria 100<span class="elsevierStyleHsp" style=""></span>mg/dl and a normal sediment. Renal function was normal (Cr: 0.7<span class="elsevierStyleHsp" style=""></span>mg/dl and MDRD<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>60<span class="elsevierStyleHsp" style=""></span>ml/min); Hb: 10.8<span class="elsevierStyleHsp" style=""></span>g/dl; Hto: 30% (TSI: 80%, folic acid: 8.2<span class="elsevierStyleHsp" style=""></span>ng/ml; VitB: 570<span class="elsevierStyleHsp" style=""></span>pg/ml) and CRP: 6.6<span class="elsevierStyleHsp" style=""></span>mg/l. The expanded study with autoimmunity and hepatitis C and B viruses as well as HIV is negative. In abdominal ultrasound, the kidneys do not have morphological abnormality and the spleen was small with diffuse increase of echogenicity suggesting fibrosis after repeated infarctions.</p><p id="par0025" class="elsevierStylePara elsevierViewall">Once proteinuria was confirmed, a renal biopsy was performed that showed glomerular hypertrophy without sclerosis, enlargement of the glomerular capillaries and the presence of sickle cells within the capillaries (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). The tubules have cells with haemosiderin by Perls staining and occasional areas of atrophy associated with fibrosis seen with trichrome staining (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>A and B). Interstitial vessels do not reveal abnormalities and direct immunofluorescence shows absence of deposits.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">The patient is diagnosed of sickle cell glomerulopathy. After one year with valsartan, 80<span class="elsevierStyleHsp" style=""></span>mg/day, proteinuria decreased to 1.6<span class="elsevierStyleHsp" style=""></span>g/24<span class="elsevierStyleHsp" style=""></span>h (albuminuria: 1.1<span class="elsevierStyleHsp" style=""></span>g/24<span class="elsevierStyleHsp" style=""></span>h) and the mean blood pressure was 111/63<span class="elsevierStyleHsp" style=""></span>mmHg by ABPM, values did not allow an increase in doses of ARA-II or double blockage of the renin–angiotensin system due to risk of symptomatic hypotension. At present, renal function is maintained normal with Cr: 0.64<span class="elsevierStyleHsp" style=""></span>mg/dl (MDRD<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>60<span class="elsevierStyleHsp" style=""></span>ml/min, CKD-EPI: 130<span class="elsevierStyleHsp" style=""></span>ml/min), serum phosphorus 5<span class="elsevierStyleHsp" style=""></span>mg/dl (tubular resorption phosphate: 0.9; tubular reabsorption of urate: 0.9) and urinary sediment continues to be normal.</p><p id="par0035" class="elsevierStylePara elsevierViewall">Sickle cell disease is one of the most frequent hereditary hematological diseases, and it is classified as one of the hemoglobinopathies. This disease is caused by a point mutation that changes glutamine by valine in the globin gene on chromosome 11, it generates hemoglobin S (HbS), which polymerizes in long fibers when deoxygenated, this causes a decrease in erythrocyte deformability and produces cell membrane damage.<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a> It includes the homozygous state (HbSS), which is the most severe form and it is most frequently in African, Mediterranean and Indian populations and the heterozygous forms HbSC and HbS-beta-thalassemia.<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">8</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">Renal involvement of sickle cell disease has a great variability. Sickle cell nephropathy can range from difficulty of concentrating urine or hyposthenuria (in our case the urine density was 1006), to renal medullary carcinoma as the most extreme expression of severity.<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">9</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">A supranormal proximal tubular function may be present (the patient has hyperphosphoremia and increased tubular phosphate reabsorption), microhematuria, microalbuminuria, proteinuria of glomerular origin (detected by Dipstick or more reliably by 24<span class="elsevierStyleHsp" style=""></span>h urine albuminuria), hypertension, acute renal failure and chronic renal failure.<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">8</span></a> Renal failure and glomerular proteinuria are risk factors associated with an increase in mortality in patients with sickle cell disease.<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">4,10</span></a> It is estimated an overall mortality of 16–18% attributed to renal disease.</p><p id="par0050" class="elsevierStylePara elsevierViewall">In addition to the more frequent form of glomerulopathy (FSG), it may be manifested as membranoproliferative glomerulonephritis, thrombotic microangiopathy and sickle cell-specific glomerulopathy.<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">2,8</span></a> Biopsy is rarely used to establish the diagnosis; in early sickle cell nephropathy, glomerular hypertrophy is observed and is part of sickle cell disease description in 1960; the most frequent location of glomerular hypertrophy is at the juxtaglomerular level, in addition there is tubular hemosiderin deposits that play a relevant role in the progression of the nephropathy. Other microscopy findings include red blood cell sickling in vasa recta, capillary congestion, mesangial expansion and endothelial lesion expressed as expansion of the lamina rara interna. Renal biopsy is necessary in cases of significant proteinuria (>1<span class="elsevierStyleHsp" style=""></span>g/day) or rapid deterioration of renal function that suggests glomerulonephritis.</p><p id="par0055" class="elsevierStylePara elsevierViewall">We conclude that the appearance of proteinuria in a patient with sickle cell disease should guide the existence of a sickle cell glomerulopathy where the glomerular hypertrophy is the consequence of an increased perfusion. Nephrotic range proteinuria is associated with progression of CKD; it is important to know the type of renal involvement given the high prevalence of glomerulopathy in adults with SCD, for which the renal biopsy can help to mark the evolution and prognosis.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Hernández-Gallego R, Cerezo I, Barroso S, Azevedo L, López M, Robles NR, et al. Afectación glomerular en paciente con enfermedad falciforme. Nefrología. 2017;37:437–439.</p>" ] ] "multimedia" => array:2 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1307 "Ancho" => 1625 "Tamanyo" => 669401 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Hematoxylin–eosin. Glomerular hypertrophy.</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Fig. 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 2593 "Ancho" => 1625 "Tamanyo" => 1222479 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Perls and trichrome staining. 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 6 | 10 | 16 |
| 2024 October | 78 | 34 | 112 |
| 2024 September | 62 | 19 | 81 |
| 2024 August | 80 | 48 | 128 |
| 2024 July | 66 | 23 | 89 |
| 2024 June | 79 | 34 | 113 |
| 2024 May | 91 | 40 | 131 |
| 2024 April | 84 | 32 | 116 |
| 2024 March | 62 | 24 | 86 |
| 2024 February | 49 | 42 | 91 |
| 2024 January | 47 | 23 | 70 |
| 2023 December | 38 | 23 | 61 |
| 2023 November | 46 | 31 | 77 |
| 2023 October | 66 | 30 | 96 |
| 2023 September | 51 | 27 | 78 |
| 2023 August | 61 | 31 | 92 |
| 2023 July | 63 | 20 | 83 |
| 2023 June | 56 | 20 | 76 |
| 2023 May | 69 | 30 | 99 |
| 2023 April | 42 | 16 | 58 |
| 2023 March | 64 | 26 | 90 |
| 2023 February | 46 | 13 | 59 |
| 2023 January | 57 | 20 | 77 |
| 2022 December | 67 | 28 | 95 |
| 2022 November | 76 | 28 | 104 |
| 2022 October | 56 | 40 | 96 |
| 2022 September | 49 | 24 | 73 |
| 2022 August | 55 | 47 | 102 |
| 2022 July | 39 | 42 | 81 |
| 2022 June | 63 | 40 | 103 |
| 2022 May | 51 | 24 | 75 |
| 2022 April | 57 | 33 | 90 |
| 2022 March | 54 | 45 | 99 |
| 2022 February | 47 | 39 | 86 |
| 2022 January | 50 | 38 | 88 |
| 2021 December | 60 | 50 | 110 |
| 2021 November | 58 | 38 | 96 |
| 2021 October | 48 | 29 | 77 |
| 2021 September | 37 | 47 | 84 |
| 2021 August | 33 | 39 | 72 |
| 2021 July | 39 | 33 | 72 |
| 2021 June | 29 | 34 | 63 |
| 2021 May | 46 | 40 | 86 |
| 2021 April | 104 | 64 | 168 |
| 2021 March | 65 | 37 | 102 |
| 2021 February | 34 | 28 | 62 |
| 2021 January | 44 | 24 | 68 |
| 2020 December | 28 | 17 | 45 |
| 2020 November | 33 | 16 | 49 |
| 2020 October | 27 | 21 | 48 |
| 2020 September | 28 | 11 | 39 |
| 2020 August | 37 | 10 | 47 |
| 2020 July | 34 | 12 | 46 |
| 2020 June | 28 | 18 | 46 |
| 2020 May | 39 | 13 | 52 |
| 2020 April | 30 | 16 | 46 |
| 2020 March | 34 | 18 | 52 |
| 2020 February | 42 | 18 | 60 |
| 2020 January | 33 | 21 | 54 |
| 2019 December | 57 | 23 | 80 |
| 2019 November | 35 | 19 | 54 |
| 2019 October | 35 | 12 | 47 |
| 2019 September | 36 | 16 | 52 |
| 2019 August | 38 | 14 | 52 |
| 2019 July | 25 | 17 | 42 |
| 2019 June | 37 | 17 | 54 |
| 2019 May | 30 | 10 | 40 |
| 2019 April | 90 | 29 | 119 |
| 2019 March | 44 | 26 | 70 |
| 2019 February | 37 | 16 | 53 |
| 2019 January | 39 | 29 | 68 |
| 2018 December | 164 | 38 | 202 |
| 2018 November | 285 | 20 | 305 |
| 2018 October | 183 | 25 | 208 |
| 2018 September | 102 | 16 | 118 |
| 2018 August | 64 | 10 | 74 |
| 2018 July | 54 | 10 | 64 |
| 2018 June | 72 | 8 | 80 |
| 2018 May | 67 | 16 | 83 |
| 2018 April | 197 | 11 | 208 |
| 2018 March | 100 | 14 | 114 |
| 2018 February | 65 | 6 | 71 |
| 2018 January | 84 | 13 | 97 |
| 2017 December | 80 | 4 | 84 |
| 2017 November | 62 | 8 | 70 |
| 2017 October | 51 | 8 | 59 |
| 2017 September | 53 | 19 | 72 |
| 2017 August | 57 | 15 | 72 |
| 2017 July | 34 | 6 | 40 |
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