Abstract: FR-PO134

Clinical Presentation and Outcome in a Series of 258 Japanese Pediatric Patients with Thrombotic Microangiopathy: A Nationwide Survey during 2012-2015

Session Information

Category: Acute Kidney Injury

  • 001 AKI: Basic


  • Ashida, Akira, Osaka Medical College, Takatsuki, Osaka-Fu, Japan
  • Matsumura, Hideki, Osaka Medical College, Takatsuki, Osaka-Fu, Japan
  • Sawai, Toshihiro, Shiga university of medical science, Shiga, Japan
  • Fujimaru, Rika, Osaka City General Hospital, Osaka, Japan
  • Fujii, Yuko, Osaka Medical College, Takatsuki, Osaka-Fu, Japan
  • Shirasu, Akihiko, Osaka Medical College, Takatsuki, Osaka-Fu, Japan
  • Nakakura, Hyogo, Osaka Medical College, Takatsuki, Osaka-Fu, Japan
  • Iijima, Kazumoto, Dept. of Pediatrics, Kobe Univ. Graduate School of Medicine, Kobe, Japan

Thrombotic microangiopathy (TMA) includes hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP). As recent studies have shown that HUS has various pathogenesis, we conducted the present study to clarify in detail the epidemiological characteristics of pediatric patients with TMA classified according to etiology.


This survey evaluated 258 Japanese pediatric patients who were diagnosed as having TMA and followed up between 2012 and 2015.


The primary diseases responsible for TMA were categorized as TTP, Shiga toxin-producing Escherichia coli associated HUS (STEC-HUS), atypical HUS, and secondary TMA. In these four categories, the most frequent primary disease was STEC-HUS which was present in 64.3% of the patients, followed in order by atypical HUS (15.5%), secondary TMA (10.1%), and TTP (5.8%).
About 40% of patients with TMA required renal replacement therapy during the acute phase. The final outcomes in terms of renal functions were normal renal function with normal urinalysis parameters in 95 patients, and CKD stage I in 62. However, in 31 patients chronic renal insufficiency (CKD stage II to V) persisted, including 4 patients with end-stage kidney disease (CKD stage V). Seventeen patients suffered recurrence of TMA, and 8 patients died. Among extrarenal complications at the final outcome point, hypertension and neuro-psychological complications were most frequent.


This study of epidemiological and demographic information for Japanese pediatric patients with TMA over the period 2012-2015 has confirmed the relative proportions of the primary underlying diseases reported previously. Pediatric patients with TMA should be followed up with monitoring of laboratory data including urinalysis, as in this series various symptoms remained at the end of the observation period.