Letter to the Editor
Atypical haemolytic-uraemic syndrome in a young patient with renal, neurological, ocular and cardiovascular involvement
Síndrome hemolítico urémico atípico en un paciente joven con compromiso renal, neurológico, ocular y cardiovascular
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=> array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 1 => array:4 [ "nombre" => "Lina María" "apellidos" => "Serna-Higuita" "email" => array:2 [ 0 => "lm.serna@hotmail.com" 1 => "johnfredynieto@gmail.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" ] ] ] 2 => array:3 [ "nombre" => "Estefanía" "apellidos" => "Calle-Botero" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 3 => array:3 [ "nombre" => "Catalina" "apellidos" => "Ocampo-Kohn" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 4 => array:3 [ "nombre" => "Arbey" "apellidos" => "Aristizabal-Alzate" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 5 => array:3 [ "nombre" => "Mónica" "apellidos" => "Zuluaga-Quintero" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 6 => array:3 [ "nombre" => "Gustavo" "apellidos" => "Zuluaga-Valencia" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Nefrólogo Hospital Pablo Tobón Uribe–Universidad de Antioquia, Medellín, Colombia" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Residente de Medicina Interna Universidad Pontificia Bolivariana, Medellín, Colombia" "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" => "Síndrome hemolítico urémico atípico en un paciente joven con compromiso renal, neurológico, ocular y cardiovascular" ] ] "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" => 1906 "Ancho" => 2492 "Tamanyo" => 304841 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Results of laboratory investigations during treatment.</p> <p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Laboratory parameters during treatment: 13 sessions of plasma exchange (blue arrows); total 4 red cell transfusions (red arrow); eculizumab, started on 29 September 2014 (green arrow). The LDH values are at a scale of 1<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>10<span class="elsevierStyleSup">2</span> and the platelet values are at a scale of 1<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>10<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">4</span></a>.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">We present the case of a 23-year-old man with a 3-month history of headache, loss of vision, loss of appetite, and foamy urine. In the previous week, he had been bedridden, with sensory disturbances, oliguria, and dyspnoea. Upon initial assessment he appeared generally unwell, drowsy, disorientated, tachycardic, hypertensive, and tachypnoeic. He had asterixis, uraemic breath, jugular venous distension, breath sounds with bibasal rales, and oedema of lower limbs. Lab test on admission showed elevated nitrogen products, metabolic acidosis with a wide anion gap, evidence of non-immune microangiopathic haemolytic anaemia and thrombocytopenia (<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>). Further tests were requested, including ADAMTS13 activity, and infection and immune profiles, which were negative (<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>). Treatment was started with haemodialysis, parenteral labetalol, red cell transfusion, and plasma exchange. Once uraemia and hypertensive crisis were controlled, ophthalmological examination revealed a marked loss of visual acuity (RE: 20/400<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>1; LE: 20/150<span class="elsevierStyleHsp" style=""></span>−<span class="elsevierStyleHsp" style=""></span>1) and hypertensive retinopathy. On day 7, haemolytic activity was controlled, which allowed discontinuation of plasma exchanges. However, the patient relapsed 48<span class="elsevierStyleHsp" style=""></span>h later, plasma exchanges were restarted and a definitive diagnosis was made of aHUS (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). Treatment with eculizumab was initiated, and plasma exchanges were stopped. The patient made adequate progress, with recovery of vision (20/30 in both eyes), however, dialysis had to be maintained. Genetic studies to detect complement mutations associated with aHUS were negative (<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>). At 9 month follow-up, the patient remained dialysis-dependent; therefore, a diagnosis was made of end-stage renal disease (ESRD) secondary to aHUS, and the workup for renal transplantation was initiated.</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">Atypical HUS is an extremely rare chronic genetic disease. It is caused by an abnormality in the regulation of complement and can lead to severe sequelae in multiple organs and even death.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1–4</span></a> It is characterised by the triad of microangiopathic haemolytic anaemia, thrombocytopenia, and renal failure, though it can affect any organ.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1,2</span></a> Our patient displayed renal, haematological, neurological, cardiovascular, and ocular involvement; ocular damage is not frequently reported in the literature.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1,2</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">In more than half of the patients with aHUS, it is possible to identify the complement mutation or the mutation in other molecules leading to abnormal complement regulation. However, genetic study is not necessary for diagnosis and initiation of therapy. However genetic studies should be performed to establish prognosis, as some mutations are more aggressive than others. Moreover, genetic study allows more adequate strategy for renal transplantion.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1,2,5</span></a> Up to 30% of patients have no documented genetic mutation,<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">4,6</span></a> as it was the case in our patient.</p><p id="par0020" class="elsevierStylePara elsevierViewall">The treatment of choice for aHUS is eculizumab, a humanised monoclonal antibody that blocks the cleavage of the C5 component of complement, thus preventing the release of the anaphylatoxin C5a and the formation of the membrane attack complex C5b-9. This prevents endothelial damage and the generation of thrombotic microangiopathy.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1,7,8</span></a> As a prerequisite for starting eculizumab, the patient must be vaccinated against meningococcus and start antibiotic prophylaxis directed against meningococcus whilst the vaccine becomes effective. The earlier eculizumab is started in patients with aHUS, the higher the likelihood of recovery and the fewer sequelae.<a class="elsevierStyleCrossRefs" href="#bib0075"><span class="elsevierStyleSup">5,9</span></a> Nonetheless, whilst the diagnosis of aHUS is being established, plasma exchanges can stabilise the patient's haemolytic activity, therefore these should be started early if the clinical picture is one of a thrombotic microangiopathy. It should be kept in mind that blood samples should be obtained for ADAMTS13 activity and autoantibody profile prior to plasma exchange to avoid missing other diagnoses.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1,5,7</span></a> If the definitive diagnosis is aHUS, treatment should be changed to eculizumab, as long-term plasma therapy has not been shown to change the devastating course of aHUS.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1,5,9,10</span></a> In our patient, plasma exchanges were performed initially, achieving control of haemolytic activity; once the diagnosis of aHUS was established, treatment was changed to eculizumab, achieving a good response with no further need for plasma exchange and no further relapses (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). However, the patient was left with the sequela of ESRD.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conclusion</span><p id="par0025" class="elsevierStylePara elsevierViewall">Atypical HUS is a rare genetic disease that affects children and adults, characterised by the presence of non-immune microangiopathic haemolytic anaemia, thrombocytopenia and multi-organ damage. The clinical course can be devastating and endanger patients’ life and quality of life, leaving them with severe sequelae such as ESRD. Treatment with eculizumab must be started early to halt multi-organ damage and avoid death.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Conflicts of interest</span><p id="par0030" class="elsevierStylePara elsevierViewall">Dr Nieto-Ríos and Dr Serna-Higuita have delivered conferences sponsored by Alexion Pharma. The other authors declare no conflicts of interest with the content of this article.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:3 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Conclusion" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Conflicts of interest" ] 2 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2015-04-15" "fechaAceptado" => "2015-07-29" "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Nieto-Ríos JF, Serna-Higuita LM, Calle-Botero E, Ocampo-Kohn C, Aristizabal-Alzate A, Zuluaga-Quintero M, et al. Síndrome hemolítico urémico atípico en un paciente joven con compromiso renal, neurológico, ocular y cardiovascular. Nefrologia. 2016;36:82–85.</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" => 1906 "Ancho" => 2492 "Tamanyo" => 304841 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Results of laboratory investigations during treatment.</p> <p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Laboratory parameters during treatment: 13 sessions of plasma exchange (blue arrows); total 4 red cell transfusions (red arrow); eculizumab, started on 29 September 2014 (green arrow). The LDH values are at a scale of 1<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>10<span class="elsevierStyleSup">2</span> and the platelet values are at a scale of 1<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>10<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">4</span></a>.</p>" ] ] 1 => array:7 [ "identificador" => "tbl0005" "etiqueta" => "Table 1" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "tabla" => array:2 [ "leyenda" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">ALP, alkaline phosphatase; ALT, alanine aminotransferase; ANA, antinuclear antibodies; AST, aspartate aminotransferase; BUN, blood urea nitrogen; C3, complement C3; C4, complement C4; CT, computed tomography; DB, direct bilirubin; ENA, antibodies against the extractable nuclear antigens anti-RNP, -Sm, -Ro, and -La; HCO<span class="elsevierStyleInf">3</span>, serum bicarbonate; HDL cholesterol, high-density lipoprotein cholesterol; HIV, human immunodeficiency virus antibody; HPF, high-power field; IB, indirect bilirubin; LDH, lactate dehydrogenase; LDL cholesterol, low-density lipoprotein cholesterol; MPOAb, anti-myeloperoxidase antibodies; PBF, peripheral blood film; PCO<span class="elsevierStyleInf">2</span>, partial pressure of carbon dioxide; PO<span class="elsevierStyleInf">2</span>, partial pressure of oxygen; PR3Ab, anti-proteinase 3 antibodies; PTH, parathyroid hormone; TB, total bilirubin; VDRL, non-treponemal test for syphilis.</p>" "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><tbody title="tbody"><tr title="table-row"><td class="td" title="table-entry " rowspan="2" align="left" valign="top">Urine cytochemistry</td><td class="td" title="table-entry " align="left" valign="top">Appearance \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">pH \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Density \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Proteinuria \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Red blood cells \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Leucocytes \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Proteinuria \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="left" valign="top">Turbid \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">8 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">1.015 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">>300<span class="elsevierStyleHsp" style=""></span>mg/dL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">>50/HPF \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">21–50/HPF \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">850<span class="elsevierStyleHsp" style=""></span>mg/day \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " rowspan="2" align="left" valign="top">Capillary gases</td><td class="td" title="table-entry " align="left" valign="top">pH \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">HCO<span class="elsevierStyleInf">3</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">PCO<span class="elsevierStyleInf">2</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">PO<span class="elsevierStyleInf">2</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">PaO<span class="elsevierStyleInf">2</span>/FiO<span class="elsevierStyleInf">2</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Lactate \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Base excess \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="left" valign="top">7.24 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">9.6<span class="elsevierStyleHsp" style=""></span>mmol/L \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">22<span class="elsevierStyleHsp" style=""></span>mmHg \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">121<span class="elsevierStyleHsp" style=""></span>mmHg \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">242 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">1.3<span class="elsevierStyleHsp" style=""></span>mmol \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">−15.7<span class="elsevierStyleHsp" style=""></span>mmol/L \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " rowspan="6" align="left" valign="top">Renal and metabolic profile</td><td class="td" title="table-entry " align="left" valign="top">Creatinine \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">BUN \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Uric acid \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Sodium \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Potassium \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Chloride \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Phosphorus \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="left" valign="top">19.7<span class="elsevierStyleHsp" style=""></span>mg/dL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">128<span class="elsevierStyleHsp" style=""></span>mg/dL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">8.5<span class="elsevierStyleHsp" style=""></span>mg/dL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">140<span class="elsevierStyleHsp" style=""></span>mEq/L \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">4.2<span class="elsevierStyleHsp" style=""></span>mEq/L \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">100<span class="elsevierStyleHsp" style=""></span>mEq/L \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">8<span class="elsevierStyleHsp" style=""></span>mg/dL \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="left" valign="top">Corrected calcium \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Ionised calcium \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Magnesium \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Albumin \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Glucose \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Total cholesterol \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">HDL-cholesterol \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="left" valign="top">6.7<span class="elsevierStyleHsp" style=""></span>mg/dl \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">0.65<span class="elsevierStyleHsp" style=""></span>mmol/L \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">1.53<span class="elsevierStyleHsp" style=""></span>mg/dL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">2.8<span class="elsevierStyleHsp" style=""></span>g/dL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">92<span class="elsevierStyleHsp" style=""></span>mg/dL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">108<span class="elsevierStyleHsp" style=""></span>mg/dL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">31<span class="elsevierStyleHsp" style=""></span>mg/dL \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="left" valign="top">LDL-cholesterol \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Triglycerides \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Vitamin D \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">PTH \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Vitamin B<span class="elsevierStyleInf">12</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Ferritin \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Folic acid \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="left" valign="top">63.6<span class="elsevierStyleHsp" style=""></span>mg/dL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">61<span class="elsevierStyleHsp" style=""></span>mg/dL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">19.3<span class="elsevierStyleHsp" style=""></span>ng/mL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">751<span class="elsevierStyleHsp" style=""></span>pg/mL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">286<span class="elsevierStyleHsp" style=""></span>pg/mL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">551<span class="elsevierStyleHsp" style=""></span>ng/mL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">7.7<span class="elsevierStyleHsp" style=""></span>ng/mL \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " rowspan="6" align="left" valign="top">Haematological profile</td><td class="td" title="table-entry " align="left" valign="top">Haemoglobin \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Haematocrit \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Leucocytes \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Neutrophils \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Lymphocytes \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Monocytes \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Basophils \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="left" valign="top">5.1<span class="elsevierStyleHsp" style=""></span>g/dL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">14% \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">6.200<span class="elsevierStyleHsp" style=""></span>mm<span class="elsevierStyleSup">3</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">80.2% \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">11.3% \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">8.1% \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">0.4% \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="left" valign="top">Eosinophils \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Platelets \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Corrected reticulocytes \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">PBF \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">LDH \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Haptoglobin \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">ADAMTS13 activity \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="left" valign="top">0% \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">82,000<span class="elsevierStyleHsp" style=""></span>mm<span class="elsevierStyleSup">−3</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">5.2% \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Schistocytes ++ \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">547<span class="elsevierStyleHsp" style=""></span>U/L \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">10.6<span class="elsevierStyleHsp" style=""></span>mg/dL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">74.5% \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="left" valign="top">PTT \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">PTT control \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">PT \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">INR \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Fibrinogen \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="left" valign="top">30<span class="elsevierStyleHsp" style=""></span>s \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">26.1 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">11.3<span class="elsevierStyleHsp" style=""></span>s \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">1.04 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">435<span class="elsevierStyleHsp" style=""></span>mg/dL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " rowspan="2" align="left" valign="top">Liver profile</td><td class="td" title="table-entry " align="left" valign="top">TB \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">DB \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">IB \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">AST \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">ALT \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">ALP \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="left" valign="top">1.5<span class="elsevierStyleHsp" style=""></span>mg/dL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">0.4<span class="elsevierStyleHsp" style=""></span>mg/dL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">1.1<span class="elsevierStyleHsp" style=""></span>mg/dL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">22<span class="elsevierStyleHsp" style=""></span>U/L \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">18<span class="elsevierStyleHsp" style=""></span>U/L \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">77<span class="elsevierStyleHsp" style=""></span>U/L \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " rowspan="2" align="left" valign="top">Immunological studies</td><td class="td" title="table-entry " align="left" valign="top">C3 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C4 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">ANA \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">ENA \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">MPOAb \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">PR3Ab \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Direct Coombs \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="left" valign="top">106<span class="elsevierStyleHsp" style=""></span>mg/dL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">28.8<span class="elsevierStyleHsp" style=""></span>mg/dL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Negative \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Negative \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Negative \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Negative \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Negative \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="left" valign="top">Complement genetic study \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " colspan="7" align="left" valign="top">No pathological mutations were found for: ADAMTS13, C3, CD46, CFB, CFH, CFHR1, CFHR2, CFHR3, CFHR4, CFHR5, CFI, DGKE, PIGA, THBD</td></tr><tr title="table-row"><td class="td" title="table-entry " rowspan="4" align="left" valign="top">Other investigations</td><td class="td" title="table-entry " colspan="2" align="left" valign="top">Protein electrophoresis</td><td class="td" title="table-entry " align="left" valign="top">HIV \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Hepatitis B \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Hepatitis C \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">VDRL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Renal biopsy \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " colspan="2" align="left" valign="top">Hypoalbuminemia, non-specific elevation of alpha 1 band and B2</td><td class="td" title="table-entry " align="left" valign="top">Negative \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Negative \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Negative \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Negative \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Thrombotic microangiopathy \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " colspan="2" align="left" valign="top">Plain CT head</td><td class="td" title="table-entry " colspan="2" align="left" valign="top">Echocardiography</td><td class="td" title="table-entry " colspan="3" align="left" valign="top">Renal ultrasound</td></tr><tr title="table-row"><td class="td" title="table-entry " colspan="2" align="left" valign="top">Normal</td><td class="td" title="table-entry " colspan="2" align="left" valign="top">Pulmonary hypertension and mild pericardial effusion</td><td class="td" title="table-entry " colspan="3" align="left" valign="top">Small echogenic kidneys with loss of corticomedullary differentiation</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab1381866.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Laboratory results.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 6 | 10 | 16 |
| 2024 October | 55 | 46 | 101 |
| 2024 September | 59 | 27 | 86 |
| 2024 August | 75 | 49 | 124 |
| 2024 July | 58 | 29 | 87 |
| 2024 June | 62 | 37 | 99 |
| 2024 May | 85 | 38 | 123 |
| 2024 April | 58 | 27 | 85 |
| 2024 March | 45 | 22 | 67 |
| 2024 February | 59 | 33 | 92 |
| 2024 January | 46 | 21 | 67 |
| 2023 December | 38 | 24 | 62 |
| 2023 November | 57 | 32 | 89 |
| 2023 October | 37 | 27 | 64 |
| 2023 September | 34 | 21 | 55 |
| 2023 August | 44 | 39 | 83 |
| 2023 July | 36 | 22 | 58 |
| 2023 June | 79 | 19 | 98 |
| 2023 May | 76 | 32 | 108 |
| 2023 April | 50 | 22 | 72 |
| 2023 March | 75 | 21 | 96 |
| 2023 February | 47 | 17 | 64 |
| 2023 January | 41 | 27 | 68 |
| 2022 December | 62 | 44 | 106 |
| 2022 November | 36 | 39 | 75 |
| 2022 October | 53 | 46 | 99 |
| 2022 September | 47 | 40 | 87 |
| 2022 August | 49 | 61 | 110 |
| 2022 July | 30 | 38 | 68 |
| 2022 June | 34 | 43 | 77 |
| 2022 May | 51 | 46 | 97 |
| 2022 April | 38 | 44 | 82 |
| 2022 March | 33 | 35 | 68 |
| 2022 February | 50 | 47 | 97 |
| 2022 January | 43 | 31 | 74 |
| 2021 December | 30 | 38 | 68 |
| 2021 November | 34 | 38 | 72 |
| 2021 October | 47 | 44 | 91 |
| 2021 September | 33 | 30 | 63 |
| 2021 August | 30 | 34 | 64 |
| 2021 July | 62 | 35 | 97 |
| 2021 June | 45 | 22 | 67 |
| 2021 May | 47 | 38 | 85 |
| 2021 April | 95 | 45 | 140 |
| 2021 March | 85 | 32 | 117 |
| 2021 February | 77 | 33 | 110 |
| 2021 January | 67 | 30 | 97 |
| 2020 December | 39 | 25 | 64 |
| 2020 November | 40 | 15 | 55 |
| 2020 October | 35 | 30 | 65 |
| 2020 September | 44 | 22 | 66 |
| 2020 August | 54 | 16 | 70 |
| 2020 July | 50 | 12 | 62 |
| 2020 June | 63 | 13 | 76 |
| 2020 May | 53 | 14 | 67 |
| 2020 April | 49 | 19 | 68 |
| 2020 March | 59 | 21 | 80 |
| 2020 February | 51 | 21 | 72 |
| 2020 January | 50 | 21 | 71 |
| 2019 December | 55 | 27 | 82 |
| 2019 November | 70 | 20 | 90 |
| 2019 October | 38 | 9 | 47 |
| 2019 September | 60 | 20 | 80 |
| 2019 August | 37 | 17 | 54 |
| 2019 July | 31 | 19 | 50 |
| 2019 June | 36 | 17 | 53 |
| 2019 May | 38 | 21 | 59 |
| 2019 April | 59 | 34 | 93 |
| 2019 March | 55 | 21 | 76 |
| 2019 February | 40 | 15 | 55 |
| 2019 January | 31 | 20 | 51 |
| 2018 December | 141 | 36 | 177 |
| 2018 November | 156 | 17 | 173 |
| 2018 October | 113 | 16 | 129 |
| 2018 September | 84 | 11 | 95 |
| 2018 August | 55 | 18 | 73 |
| 2018 July | 41 | 9 | 50 |
| 2018 June | 44 | 15 | 59 |
| 2018 May | 48 | 13 | 61 |
| 2018 April | 66 | 1 | 67 |
| 2018 March | 62 | 15 | 77 |
| 2018 February | 75 | 4 | 79 |
| 2018 January | 52 | 7 | 59 |
| 2017 December | 82 | 8 | 90 |
| 2017 November | 57 | 5 | 62 |
| 2017 October | 58 | 12 | 70 |
| 2017 September | 44 | 10 | 54 |
| 2017 August | 39 | 8 | 47 |
| 2017 July | 47 | 12 | 59 |
| 2017 June | 45 | 14 | 59 |
| 2017 May | 56 | 31 | 87 |
| 2017 April | 42 | 17 | 59 |
| 2017 March | 26 | 11 | 37 |
| 2017 February | 39 | 17 | 56 |
| 2017 January | 20 | 4 | 24 |
| 2016 December | 41 | 11 | 52 |
| 2016 November | 53 | 16 | 69 |
| 2016 October | 75 | 11 | 86 |
| 2016 September | 97 | 2 | 99 |
| 2016 August | 80 | 0 | 80 |
| 2016 July | 131 | 3 | 134 |
| 2016 June | 112 | 0 | 112 |
| 2016 May | 150 | 0 | 150 |
| 2016 April | 108 | 0 | 108 |
| 2016 March | 108 | 0 | 108 |
| 2016 February | 85 | 0 | 85 |
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