title: Two cases of hyponatremic-hypertensive syndrome in childhood with renovascular hypertension.
abstract: 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.
authors.affiliation: Department of Pediatrics, Osaka Medical College, 2-7, Daigakumachi Takatsuki, Osaka, 569-8686, Japan. ped006@poh.osaka-med.ac.jp
authors.forename: Akira
authors.initials: A
authors.lastname: Ashida
authors.forename: Hideki
authors.initials: H
authors.lastname: Matsumura
authors.forename: Nao
authors.initials: N
authors.lastname: Inoue
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Katayama
authors.forename: Yuki
authors.initials: Y
authors.lastname: Kiyohara
authors.forename: Takehisa
authors.initials: T
authors.lastname: Yamamoto
authors.forename: Hyogo
authors.initials: H
authors.lastname: Nakakura
authors.forename: Motoshi
authors.initials: M
authors.lastname: Hattori
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Tamai
mesh_terms: 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