Kidney Week

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

• AKI Clinical: Outcomes and Biomarkers
  November 03, 2017 | Location: Hall H, Morial Convention Center
  Abstract Time: 10:00 AM - 10:00 AM

Category: Acute Kidney Injury

• 001 AKI: Basic

Authors

• Ashida, Akira, Osaka Medical College, Takatsuki, Osaka-Fu, Japan

Akira Ashida,

• Matsumura, Hideki, Osaka Medical College, Takatsuki, Osaka-Fu, Japan

Hideki Matsumura,

• Sawai, Toshihiro, Shiga university of medical science, Shiga, Japan

Toshihiro Sawai,

• Fujimaru, Rika, Osaka City General Hospital, Osaka, Japan

Rika Fujimaru,

• Fujii, Yuko, Osaka Medical College, Takatsuki, Osaka-Fu, Japan

Yuko Fujii,

• Shirasu, Akihiko, Osaka Medical College, Takatsuki, Osaka-Fu, Japan

Akihiko Shirasu,

• Nakakura, Hyogo, Osaka Medical College, Takatsuki, Osaka-Fu, Japan

Hyogo Nakakura,

• Iijima, Kazumoto, Dept. of Pediatrics, Kobe Univ. Graduate School of Medicine, Kobe, Japan

Kazumoto Iijima,

Background

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.

Methods

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

Results

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.

Conclusion

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.