Kidney (2008) 17:235237 DOI 10.

1007/s00596-008-0038-1

Hyponatremic Hypertensive Syndrome Presenting as Malignant Hypertension in a Four-Year-Old Girl

Ana Teixeira & Vania Machado & Teresa Pontes & Teresa Mota & Alberto Caldas Afonso

Received: 14 July 2008 / Accepted: 16 September 2008 / Published online: 10 October 2008 # Springer Science + Business Media, LLC 2008

Abstract The prevalence of renal and renovascular hypertension in the general population is not known precisely; in children it probably accounts for the majority of cases of secondary hypertension. The symptoms of renovascular hypertension vary, and sometimes it can be asymptomatic. In rare cases, clinical manifestations can include electrolyte disorders including hyponatremia. An uncommon etiology of true hyponatremia of renal origin is the hyponatremic hypertensive syndrome. It has been reported in adults with malignant hypertension, but was considered as an unusual form of presentation of renovascular disease in children. In this report, a four-year-old girl presented with hypertensive encephalopathy related to the hyponatremic hypertensive syndrome caused by right renal artery stenosis. Treatment with percutaneous angioplasty was successful, with total resolution of symptoms. This case emphasizes the importance of routine blood pressure evaluation as a major contribution to the prevention of morbidity and mortality associated with severe forms of hypertension in children. Key words hypertension . hyponatremia . renal artery stenosis . renin

Background The prevalence of renal and renovascular hypertension in the general population is not known precisely; in children it probably accounts for the majority of cases of secondary hypertension [1]. Hyponatremic hypertensive syndrome is a rare condition of severe hypertension characterized by electrolyte abnormalities (hyponatremia, hypokalemia), polyuria and high renin activity. At first reported in adults, it was considered unusual in children [2]. This report describes a four-year-old girl with hypertensive encephalopathy related to the hyponatremic hypertensive syndrome caused by right renal artery stenosis.

Case Report A four-year-old girl presented with headache, drowsiness, and a sudden generalized clonic seizure together with tachycardia and severe hypertension (148/124 mmHg). Initial attempts were made to control seizures with anticonvulsant medication with no success, and the patient was admitted to the Paediatric Intensive Care Unit due to hypertensive encephalopathy. After starting antihypertensive medication and a gradual decrease in the patients blood pressure, the seizures stopped and her consciousness level gradually returned to normal. The patient had no previous history of head trauma or medication intake. When specifically asked, complaints of polyuria and polydipsia during the previous two months were reported. The physical examination showed normal size and reactive pupils, normal fundoscopy and normal skin examination. The meningeal signs were negative and the patient had no fever and no signs of respiratory distress. The cardiac and pulmonary auscultation was normal and the abdominal

A. Teixeira (*) : V. Machado : T. Pontes : A. C. Afonso Department of Paediatrics, Woman and Child Autonomous Unit, Hospital So Joo, Alameda Professor Hernani Monteiro, 4200 Porto, Portugal e-mail: anafteixeira@gmail.com T. Mota Paediatric Intensive Care Unit, Woman and Child Autonomous Unit, Hospital So Joo, 4200 Porto, Portugal

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examination showed no bruit, organomegaly or palpable mass. The initial laboratory examination revealed hyponatremia (sodium of 127 mEq/L), hypokalemia (potassium of 3.2 mEq/L), and metabolic alkalosis (pH of 7.5 and bicarbonate of 26 mEq/L). Serum urea and creatinine were normal. The plasma renin activity was 28.7 ng/mL (normal 0.96.4 ng/mL) and serum aldosterone was 945 pg/mL (normal 5201 pg/mL). The cerebral computed tomography was normal, the echocardiogram showed a mild concentric left ventricular hypertrophy and the renal ultrasound revealed a renal asymmetry with left hypertrophy and hyperechogenicity and right parenchyma atrophy. The renal scintigraphy with dimercaptosuccinic acid revealed reduced uptake by the right kidney and right kidney hypofunction (left 83.2% and right 16.8%) (Fig. 1). The renal artery angiography diagnosed a right renal artery stenosis (Fig. 2). The initial management consisted of the hydroelectrolytic disorder correction and anti-hypertensive treatment with nifedipine and captopril. On the eighth day after admission, the patient underwent a percutaneous transluminal renal artery angioplasty. During the remainder of the hospital stay, there was a progressive decrease and stabilization of blood pressure (around 95th percentile). There was no seizure recurrence and the neurological examination was normal. Three months after discharge, the renal ultrasound and scintigraphy were normal. Six months later, the patient had normal blood pressure and, therefore, the anti-hypertensive treatment was progressively decreased and discontinued. At the last examination, the patient was seven years old, was asymptomatic and had normal blood pressure.
Fig. 1 Renal scintigraphy: Right kidney reduced uptake and hypofunction

Fig. 2 Renal artery angiography: right renal artery stenosis

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Discussion The combination of hypertension and hyponatremia can be observed in a number of disorders including renal failure, renin-secreting tumors and renal artery stenosis. Renal ischemia due to renal artery stenosis results in increased renin secretion, resulting in high circulating angiotensin II, which raises blood pressure and stimulates aldosterone resulting in pressure natriuresis. This combination of hypereninemic hypertension with hyponatremia defines the hyponatremic hypertensive syndrome. In adults, this condition presenting with malignant hypertension has been reported more often but in children, beyond the neonatal age, it is rarely observed. The purpose of this case report is to increase the awareness on hyponatremic hypertensive syndrome presenting with malignant hypertension in children. An elevated renin level is a pathognomonic finding in hyponatremic hypertensive syndrome. The combination of renin-induced hypertension and hyponatremia results in hyponatremic hypertensive syndrome irrespective of etiology. The renal ischemia leads to a sudden rise in arterial pressure that can induce a pressure natriuresis through the contralateral normal kidney, leading to volume depletion that may result in postural hypotension, further renin release from the ischemic kidney, and heightening of the aldosterone response to angiotensin II [3]. Potassium deficiency from hyperaldosteronism may further stimulate renin secretion and intensify this circle [3, 4]. The hyponatremia is presumed to result primarily from sodium depletion due to pressure natriuresis and the direct effect of angiotensin II on the kidney. The stimulation of thirst and release of antidiuretic hormone in response to the dual stimuli of exceedingly high levels of angiotensin II and volume depletion can result in the worsening of the hyponatremic condition. The metabolic alkalosis is also a consequence of the renin-angiotensin-aldosterone system activation [5]. Management of the hyponatremic hypertensive syndrome should be directed toward lowering the blood pressure and toward the suppression of the renin-angiotensin-aldosterone

system [35]. In this case, the patient was started on medical treatment with a calcium-blocker agent and an angiotensin-converting enzyme inhibitor and, after diagnosis of the underlying condition, the renal artery stenosis was surgically corrected by percutaneous transluminal angioplasty. This procedure is a good minimal invasive treatment option, but because of the possibility of renal artery restenosis and appearance of new stenotic lesions it requires long-term follow-up [6]. This case report emphasizes the need for routine monitoring of blood pressure in children to achieve early diagnosis and treat possible reversible disorders that can lead to life-threatening conditions. Also, in children the combination of hypereninemic malignant hypertension with hyponatremia does occur and ruling out renal ischemia due to renal artery stenosis is strongly recommended.

References
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