Vascular Malformation in the Kidney as a Cause of Severe Hypertension in an Infant
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SCIENTIFIC LETTER
Vascular Malformation in the Kidney as a Cause of Severe Hypertension in an Infant Rina R. Rus 1,2
&
Tomaž Ključevšek 3 & Nika Kojc 4
Received: 25 March 2020 / Accepted: 23 July 2020 # Dr. K C Chaudhuri Foundation 2020
To the Editor: A 3-mo-old girl was admitted to hospital due to vomiting. On admission, she was irritable, mildly dehydrated and had a labial hemangioma. Laboratory tests revealed proteinuria (4+) and microhematuria, while renal function and electrolytes were normal. High blood pressure was detected in the next few days (155/110–190/115 mmHg). She was treated with nicardipine, dihydralazine and captopril. Additionally, hypokalemia (3.5 mmol/L) and hyponatremia (127 mmol/L) occurred and high plasma renin activity (PRA > 216 ηg/L/h) and aldosterone (2.26 nmol/L) were detected. Doppler ultrasound showed a conglomerate of vessels in the right renal hilus (Fig. 1a). Digital subtraction angiography demonstrated aneurismatic dilation of the right renal artery and venous outflow, suggesting high flow arterio-venous (AV) malformation (Fig. 1b). There was decreased relative function of the right kidney (22%) on renal scan with MAG 3. Because of the anatomical situation, nephrectomy of the right kidney was performed instead of percutaneous embolization of AV malformation. Macroscopic examination of the removed kidney showed a thrombosed vascular structure of 1 cm in diameter in the central part of the kidney (Fig. 1 b, c). In addition, there was
* Rina R. Rus [email protected] 1
Department of Nephrology, University Children’s Hospital, University Medical Centre Ljubljana, Ljubljana, Slovenia
2
Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia
3
Clinical Institute of Radiology, University Medical Centre Ljubljana, Ljubljana, Slovenia
4
Institute of Pathology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia
extensive infarction in the upper pole of the kidney. Light microscopy confirmed occlusive thrombi of different ages, with destruction of the vascular wall. The thrombi appeared partially organized, with recanalization, and partially fresh, indicating very recent occlusion (Fig. 1d). Genetic analysis of the most common genes known to cause Rendu-OslerWeber disease was negative. Since the nephrectomy, the girl has had normal blood pressure without therapy, with normal growth and development. Based on angiographic and macroscopic features, we considered the vascular lesion in the kidney to be a congenital AV malformation, which is a rare cause of severe hypertension in infancy [1]. However, hypertension in this case was probably induced by thrombosis of vascular malformation, which caused ischemia of the renal parenchyma and resulted in activation of the renin-angiotensinaldosterone system (RAAS), as was confirmed by high levels of renin in plasma. Additionally, activation of RAAS, together with volume depletion, which stimulate anti-diuretic hormone (ADH) release, can cause an increase in aldosterone secretion, resulting in hyponatremia and hypokal
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