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Images in Paediatrics
Fibromuscular dysplasia: an uncommon cause of arterial hypertension
Displasia fibromuscular: una causa rara de hipertensión arterial
Nuria Heredia-Torres
Corresponding author
nuri_h_92@hotmail.com

Corresponding author.
, Francisco Antonio Nieto-Vega, Begoña Rodríguez Azor, Verónica Martínez-Rivera
Unidad de Nefrología Pediátrica, UGC Pediatría, Hospital Regional Universitario de Málaga, Málaga, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">A girl aged 13 years presented with asthenia&#44; decreased appetite&#44; dizziness and abdominal pain of 2 months&#8217; duration&#46; The chief finding of the physical examination were hypertension &#40;HTN&#41; with a blood pressure of 200&#47;135<span class="elsevierStyleHsp" style=""></span>mmHg&#46; The Doppler ultrasound showed an atrophied right kidney with aneurysms in the right renal artery&#44; confirmed by CT angiography &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41; and arteriography&#46; There was end-organ damage&#58; acute renal failure &#40;peak creatinine&#44; 1&#46;45<span class="elsevierStyleHsp" style=""></span>mg&#47;dL&#41;&#44; retinopathy&#44; cardiomyopathy and transient ischaemic attacks in the white matter&#46; The presence of aneurysms in other locations was ruled out with whole-body magnetic resonance angiography&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">The patient did not meet the diagnosis criteria for Marfan&#44; Ehlers-Danlos or Grange syndrome&#44; polyarteritis nodosa&#44; tuberous sclerosis or neurofibromatosis&#46; There were no other signs suggestive of atherosclerosis&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">The approach selected initially was angioplasty with stenting&#44; which could not be carried out due to spontaneous embolization&#46; Given the persistence of HTN and the near-total dysfunction of the right kidney&#44; the decision was made to perform right nephrectomy &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#44; which achieved progressive improvement of blood pressure and renal function&#46; The histological features were compatible with fibromuscular dysplasia&#46; At 2 years of follow-up&#44; her blood pressure is in the normal range without treatment&#44; and the glomerular filtration rate calculated with the modified Schwartz formula is 66<span class="elsevierStyleHsp" style=""></span>mL&#47;min&#47;1&#46;73<span class="elsevierStyleHsp" style=""></span>m<span class="elsevierStyleSup">2</span>&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Fibromuscular dysplasia is a disease that is rare in children of unknown aetiology and prevalence and characterised by the presence of non-inflammatory segmental stenosis in arteries of small and medium calibre&#44; and it most frequently involves the renal artery&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;2</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">Despite its low sensitivity&#44; Doppler ultrasound is useful in the initial diagnosis of renovascular HTN&#46; Computed tomography angiography and MR angiography offer a greater sensitivity&#46; Renal arteriography is the gold standard of diagnosis&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;3</span></a> Treatment consists in controlling the blood pressure and&#44; in select cases with refractory HTN&#44; angioplasty&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;3</span></a></p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicts of interest</span><p id="par0030" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
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ISSN: 23412879
Original language: English
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