Ashida Akira, Matsumura Hideki, Inoue Nao, Katayama Hiroshi, Kiyohara Yuki, Yamamoto Takehisa, Nakakura Hyogo, Hattori Motoshi, Tamai Hiroshi
Department of Pediatrics, Osaka Medical College, 2-7, Daigakumachi Takatsuki, Osaka, 569-8686, Japan.
Eur J Pediatr. 2006 May;165(5):336-9. doi: 10.1007/s00431-005-0048-1. Epub 2006 Jan 13.
We report two children with renovascular hypertension and fibromuscular dysplasia. They initially presented with severe hyponatremia, hypokalemia, polyuria, and transient proteinuria. This combination of symptoms is known to occur in patients with renovascular and malignant hypertension, and is known as hyponatremic-hypertensive syndrome (HHS), although it is considered rare in children. Since in both of our patients, the renal arterial stenosis was very severely or almost totally occlusive, we could not perform percutaneous transluminal renal artery angioplasty, and therefore nephrectomy was the only option. A histological study showed partial or complete occlusion with intimal hyperplasia and medial fibroplasia of intrarenal arteries such as the interlobular arteries.
Both patients showed rapidly progressive renovascular hypertension and loss of function of the affected kidney. In order to preserve renal function in such cases, early invasive intervention appears to be necessary.
