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IMÁGENES EN PEDIATRÍA
Displasia fibromuscular: una causa rara de hipertensión arterial
Fibromuscular dysplasia: an uncommon cause of arterial hypertension
Nuria Heredia-Torres
Autor para correspondencia
nuri_h_92@hotmail.com

Autor para correspondencia.
, Francisco Antonio Nieto-Vega, Begoña Rodríguez Azor, Verónica Martínez-Rivera
Unidad de Nefrología Pediátrica. UGC Pediatría. Hospital Regional Universitario de Málaga, Málaga, España
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Ni&#241;a de 13 a&#241;os que consult&#243; por astenia&#44; hiporexia&#44; sensaci&#243;n de mareo y dolor abdominal de dos meses de evoluci&#243;n&#46; A la exploraci&#243;n destacaba hipertensi&#243;n arterial &#40;HTA&#41; de 200&#47;135 mmHg&#46; La ecograf&#237;a-Doppler mostr&#243; ri&#241;&#243;n derecho atr&#243;fico&#44; con aneurisma de arteria renal derecha&#44; confirmado con angio-TC &#40;<a class="elsevierStyleCrossRef" href="#fig0005">fig&#46; 1</a>&#41; y arteriograf&#237;a&#46; Present&#243; como lesiones de &#243;rganos diana&#58; da&#241;o renal agudo &#40;creatinina m&#225;xima 1&#44;45 mg&#47;dL&#41;&#44; retinopat&#237;a&#44; miocardiopat&#237;a y microinfartos en sustancia blanca&#46; Se descartaron aneurismas en otras localizaciones mediante angiorresonancia de cuerpo completo&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">La paciente no cumpl&#237;a criterios diagn&#243;sticos de s&#237;ndrome de Marfan&#44; Ehlers-Danlos&#44; Grange&#44; poliarteritis nodosa&#44; esclerosis tuberosa o neurofibromatosis&#46; No hab&#237;a otros signos sugerentes de arteriosclerosis&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">Se decidi&#243; tratar inicialmente mediante angioplastia y colocaci&#243;n de <span class="elsevierStyleItalic">stent</span>&#44; no siendo posible por embolizaci&#243;n espont&#225;nea&#46; Dada la persistencia de HTA y pr&#225;ctica anulaci&#243;n funcional renal derecha&#44; se decidi&#243; nefrectom&#237;a derecha &#40;<a class="elsevierStyleCrossRef" href="#fig0010">fig&#46; 2</a>&#41;&#44; con mejor&#237;a progresiva de tensiones y funci&#243;n renal&#46; Histopatolog&#237;a compatible con displasia fibromuscular &#40;DFM&#41;&#46; Tras dos a&#241;os est&#225; normotensa&#44; sin tratamiento y el filtrado glomerular estimado seg&#250;n la f&#243;rmula de Schwartz modificada es 66 mL&#47;min&#47;1&#44;73 m<span class="elsevierStyleSup">2</span>&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">La DFM es una enfermedad rara en ni&#241;os&#44; de causa y prevalencia desconocidas&#44; caracterizada por la presencia de estenosis segmentarias no inflamatorias en arterias de mediano-peque&#241;o calibre&#44; siendo la arteria renal la m&#225;s frecuentemente afectada<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">1&#44;2</span></a>&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">La ecograf&#237;a-Doppler&#44; pese a su baja sensibilidad&#44; es &#250;til en el abordaje diagn&#243;stico inicial de HTA renovascular&#46; La angio-TC y angio-RM ofrecen mayor sensibilidad&#46; La arteriograf&#237;a renal constituye el <span class="elsevierStyleItalic">gold standard</span><a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">1&#44;3</span></a>&#46; El tratamiento engloba el control tensional y&#44; en casos seleccionados y con HTA refractaria&#44; la angioplastia<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">1&#44;3</span></a>&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicto de intereses</span><p id="par0030" class="elsevierStylePara elsevierViewall">Los autores declaran no tener ning&#250;n conflicto de intereses&#46;</p></span></span>"
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ISSN: 16954033
Idioma original: Español
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