Hypertensive crisis in an 11-year-old girl with kidney and inferior vena cava abnormalities and leg thrombosis: Question
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CLINICAL QUIZ
Hypertensive crisis in an 11-year-old girl with kidney and inferior vena cava abnormalities and leg thrombosis: Questions Ľubica Kováčiková 1 & Mária Chocholová 1 & Milan Ilčík 2 & Žofia Varényiová 1,3 & Tomáš Seeman 3 & Ľudmila Podracká 1 Received: 11 August 2020 / Revised: 22 September 2020 / Accepted: 13 October 2020 # IPNA 2020
Keywords Child . Hypertensive crisis . Kidney hypoplasia . Venous thrombosis . Inferior vena cava abnormalities
Case summary An 11-year-old girl was admitted to the hospital suffering from severe headache, eye pain, diplopia, loss of appetite, and vomiting lasting for 3 days. At the emergency department, the patient was somnolent with blood pressure (BP) of 180/ 130 mmHg. Electrolyte and biochemical analysis did not reveal any abnormal findings. Fundoscopy showed bilateral papilledema. Therefore, a brain computed tomography (CT) was performed with no signs of any pathologic lesions. Echocardiogram revealed left ventricular hypertrophy (LVH). Kidney ultrasound revealed hypoplastic left kidney (length 5.3 cm, < 5th centile [1]), while the right kidney showed compensatory hypertrophy (length 11.2 cm, > 95th centile [1]). The blood flow to the left renal artery measured by a Doppler ultrasound was not detectable. Therefore, kidney CT angiography was performed, which demonstrated severely stenotic left renal and left polar renal artery without segmental stenosis and an absence of the infrarenal segment of the inferior vena cava (IVC) (Fig. 1) with a collateral drainage through azygous and hemiazygos veins. DMSA kidney scan showed severe The answers to these questions can be found at https://doi.org/10.1007/ s00467-020-04830-x. * Ľudmila Podracká [email protected] 1
Department of Pediatrics, Medical Faculty, Comenius University and National Institute of Children’s Diseases, Limbova 1, 833 40 Bratislava, Slovakia
2
Department of Pediatric radiology, Medical Faculty, Comenius University and National Institute of Children’s Diseases, Bratislava, Slovakia
3
Department of Pediatrics, 2nd Faculty of Medicine and Faculty of Medicine, Charles University in Prague and Motol University Hospital, Prague, Czech Republic
hypofunction of the left kidney (14%). The glomerular filtration rate (GFR) calculated by Schwartz equation [2] was 81 ml/min/ 1.73 m2, urinalysis was negative, and 24-h urine protein excretion was 67 mg/m2/day; microalbuminuria was 2.07 mg/mmol. Pharmacological therapy of hypertensive crisis was immediately initiated using continuous intravenous urapidil [3]. Simultaneously, the patient was administered amlodipine and hydrochlorothiazide orally. Despite the intensive antihypertensive management, an invasive blood pressure (BP) measurement revealed that the BP ranged 150–215/105–150 mmHg. Amlodipine was replaced by nifedipine, and oral metoprolol was added to the therapy. Subsequently, systolic BP dropped to the range of 130–145 mmHg, and the intravenous therapy with urapidil was discontinued. Because of persisting diplopia and vomiting, magnetic resonance angiogr
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