EndotheLiopathies: Hemolytic Uremic Syndrome, Thrombotic Thrombocytopenic Purpura, and Preeclampsia

Kuang-Yu Jen, Z. G. Laszik

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Microvascular endotheLial cell injury plays a central role in the pathogenesis of a set of diseases known as endotheLiopathies. Typically, such diseases demonstrate prominent renal manifestations since the kidneys are richly vascularized. Morphologically, endotheLiopathies classically demonstrate features of thrombotic microangiopathy on renal biopsy and include diseases such as hemolytic uremic syndrome (HUS), thrombotic thrombocytopenic purpura (TTP), and preeclampsia. Recent advances have elucidated several mechanisms by which endotheLial injury may occur leading to the development of endotheLiopathies. More specifically, direct cytotoxic endotheLial injury is caused by exposure to Shiga or Shiga-Like toxins following gastroenteritis in classic HUS, while atypical HUS has been traced to endotheLial perturbation due to overactivation of the alternative complement pathway. In TTP, abrogated ADAMTS13 activity leads to the accumulation of uncleaved large multimers of von Willebrand factor, which in turn causes inappropriate platelet aggregation and endotheLial damage. In preeclampsia, soluble circulating factors Likely derived from the placenta act as antagonists to the glomerular endotheLial cell microenvironment, inciting endotheLial cell injury. Understanding the molecular mechanisms by which these diseases develop and progress will greatly aid in the design and implementation of effective treatments specific to each endotheLiopathy.

Original languageEnglish (US)
Title of host publicationPathobiology of Human Disease
Subtitle of host publicationA Dynamic Encyclopedia of Disease Mechanisms
PublisherElsevier Inc.
Pages2767-2787
Number of pages21
ISBN (Electronic)9780123864567
ISBN (Print)9780123864574
DOIs
StatePublished - Jan 1 2014
Externally publishedYes

Keywords

  • ADAMTS13
  • Alternative pathway
  • Complement
  • EndotheLiopathy
  • EndotheLium
  • Hemolytic uremic syndrome
  • Microangiopathy
  • Preeclampsia
  • Shiga toxin
  • Thrombotic thrombocytopenic purpura
  • VEGF
  • Von Willebrand factor

ASJC Scopus subject areas

  • Medicine(all)

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  • Cite this

    Jen, K-Y., & Laszik, Z. G. (2014). EndotheLiopathies: Hemolytic Uremic Syndrome, Thrombotic Thrombocytopenic Purpura, and Preeclampsia. In Pathobiology of Human Disease: A Dynamic Encyclopedia of Disease Mechanisms (pp. 2767-2787). Elsevier Inc.. https://doi.org/10.1016/B978-0-12-386456-7.05406-X