Abstract
Thrombotic microangiopathy (TMA) may result from a variety of clinical conditions, including thrombotic thrombocytopenic purpura, Shiga toxin-producing Escherichia coli-associated hemolytic uremic syndrome and complement-mediated hemolytic uremic syndrome. Thrombocytopenic purpura is diagnosed when ADAMTS13 is <10%, while a diagnosis of Shiga toxin-producing Escherichia coli-associated hemolytic uremic syndrome is made with the evidence of infection by Shiga toxin-producing Escherichia coli. Diagnosis of complement-mediated hemolytic uremic syndrome is not dependent on a specific laboratory test and is a diagnosis of exclusion. TMA is a rare disease and finding individuals that have more than 1 concurrent etiology leading to TMA is even more rare. Here we describe the presentation and management of an individual with CFHR1 deletion-associated TMA also found to have a positive stool Shiga toxin. We discuss the significance of Shiga toxin in serving as a trigger for development of TMA in an individual predisposed to development of TMA due to presence of a homozygous deletion in CFHR1.
Original language | English (US) |
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Pages (from-to) | 492-498 |
Number of pages | 7 |
Journal | American Journal of the Medical Sciences |
Volume | 356 |
Issue number | 5 |
DOIs | |
State | Published - Nov 2018 |
Externally published | Yes |
Bibliographical note
Publisher Copyright:© 2018 Southern Society for Clinical Investigation
Keywords
- Complement
- Eculizumab
- Hemolytic uremic syndrome
- Shiga toxin
- Thrombotic microangiopathy