Two cases of hyponatremic-hypertensive syndrome in childhood with renovascular hypertension.
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.


CONCLUSIONS
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.
Ashida
Akira
A
Department of Pediatrics, Osaka Medical College, 2-7, Daigakumachi Takatsuki, Osaka, 569-8686, Japan. ped006@poh.osaka-med.ac.jp
Matsumura
Hideki
H
Inoue
Nao
N
Katayama
Hiroshi
H
Kiyohara
Yuki
Y
Yamamoto
Takehisa
T
Nakakura
Hyogo
H
Hattori
Motoshi
M
Tamai
Hiroshi
H
D002675:Child, Preschool; D005260:Female; D005352:Fibromuscular Dysplasia / Q000150:complications; D006801:Humans; D006965:Hyperplasia; D006978:Hypertension, Renovascular / Q000150:complications* / Q000209:etiology; D007008:Hypokalemia / Q000150:complications; D007010:Hyponatremia / Q000150:complications* / Q000209:etiology; D008297:Male; D009392:Nephrectomy; D011141:Polyuria / Q000150:complications; D011507:Proteinuria / Q000150:complications; D012078:Renal Artery Obstruction / Q000150:complications / Q000601:surgery; D013577:Syndrome; D017539:Tunica Intima / Q000473:pathology; D017540:Tunica Media / Q000473:pathology