Japanese journal of pediatric nephrology
Online ISSN : 1881-3933
Print ISSN : 0915-2245
ISSN-L : 0915-2245
Case Reports
A case of aHUS with successful treated refractory hypertension for renin-angiotensin inhibitor, ischemic colitis for octreotide acetate
Sakina KugeTakuji Enya Yuichi MorimotoKohei MiyazakiTomoki MiyazawaHiroki KondouNaoki SakataMitsuru OkadaKeisuke Sugimoto
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JOURNAL OPEN ACCESS

2022 Volume 35 Issue 1 Pages 27-34

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Abstract

Atypical hemolytic uremic syndrome (aHUS) is characterized by thrombotic microangiopathy secondary to uncontrolled activation of the complement system. Extra-renal manifestations of aHUS affect the prognosis of this condition; therefore, optimal management of extra-renal manifestations of aHUS is important. A 3-year-old girl admitted with hematuria, proteinuria, and generalized edema was diagnosed with aHUS. We report a pediatric case of life-threatening extra-renal manifestations of aHUS, including gastrointestinal bleeding, refractory hypertension, pulmonary edema, and reversible cerebral atrophy in a patient who was successfully treated with multidisciplinary treatment. In addition to plasma exchange and eculizumab combination therapy, we administered renin-angiotensin system inhibitors for refractory hypertension and octreotide acetate for body fluid management in ischemic enteritis. Notably, multi-organ involvement in patients with extra-renal manifestations of aHUS can result in serious complications in the absence of appropriate treatment; therefore, systemic management with multidisciplinary treatment is important for improved prognosis.

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© 2022 The Japanese Society for Pediatric Nephrology

この記事はクリエイティブ・コモンズ [表示 - 非営利 - 継承 4.0 国際]ライセンスの下に提供されています。
https://creativecommons.org/licenses/by-nc-sa/4.0/deed.ja
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