Anasarca improved by extracorporeal ultrafiltration through an internal shunt in a case of severe haemophilia B with inhibitor and steroid-resistant nephrotic syndrome.
A patient suffering from severe haemophilia B with factor IX inhibitor developed steroid-resistant nephrotic syndrome. As a result of switching to activated anti-inhibitor coagulant complex (activated prothrombin complex concentrate) agent, FEIBA, bleeding was controlled and internal shunt placement was successful, leading to control of bleeding during extracorporeal ultrafiltration (ECU) therapy. We report this case where regular ECU therapy became possible and as a result anasarca was controlled.
Satomura
A
A
Division of Nephrology and Endocrinology, Department of Medicine, Nihon University School of Medicine, Tokyo, Japan. atsushis@med.nihon-u.ac.jp
Takahashi
S
S
Fujita
T
T
Matsumoto
K
K
Shichino
H
H
Mugishima
H
H
D000328:Adult; D001779:Blood Coagulation Factors / Q000008:administration & dosage*; D004487:Edema / Q000628:therapy*; D005164:Factor IX / Q000037:antagonists & inhibitors*; D005542:Forearm; D006395:Hemarthrosis / Q000517:prevention & control; D006440:Hemofiltration / Q000379:methods*; D002836:Hemophilia B / Q000150:complications*; D006801:Humans; D008297:Male; D009404:Nephrotic Syndrome / Q000150:complications / Q000188:drug therapy*; D012720:Severity of Illness Index; D013256:Steroids / Q000627:therapeutic use*; D016896:Treatment Outcome
D001779:Blood Coagulation Factors; D013256:Steroids; C025667:prothrombin complex concentrates; D005164:Factor IX