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Updated: Oct 6, 2025
06:01
A Thrombotic Stroke Model Based On Transient Cerebral Hypoxia-ischemia
Published on: August 18, 2015
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Thrombotic microangiopathy in children
Lilian Monteiro P Palma1, Maria Helena Vaisbich-Guimarães2, Meera Sridharan3
1Department of Pediatrics, Pediatric Nephrology, State University of Campinas (UNICAMP), Rua Tessalia Vieira de Camargo, 126, Cidade Universitaria, Campinas, SP, 13,083-887, Brazil. lilianp@unicamp.br.
Pediatric Nephrology (Berlin, Germany)
|January 18, 2022
Summary
Thrombotic microangiopathy (TMA) presents with anemia, low platelets, and organ damage. This review details pediatric TMA causes, including rare inherited conditions, and offers an approach for timely diagnosis and treatment.
Area of Science:
- Pediatric Hematology
- Nephrology
- Genetics
Background:
- Thrombotic microangiopathy (TMA) is a serious condition involving microangiopathic hemolytic anemia, thrombocytopenia, and organ damage.
- TMA encompasses diverse etiologies with distinct presentations in children versus adults.
Purpose of the Study:
- To review acquired and inherited causes of TMA, emphasizing pediatric-specific considerations.
- To provide a practical clinical approach for evaluating and managing TMA in children.
Main Methods:
- Literature review of acquired and inherited TMA etiologies.
- Focus on differential diagnosis in pediatric populations.
- Development of a pragmatic diagnostic and management strategy.
Main Results:
- Key pediatric TMA causes include shigatoxin-HUS, infection-associated TMA, and rare inherited disorders (e.g., complement-associated HUS, cobalamin defects, DGKE mutations).
- TMA is also a concern post-transplantation (solid organ or stem cell).
- A structured approach aids in early treatment initiation pending definitive diagnosis.
Conclusions:
- Accurate diagnosis of TMA in children requires consideration of both common and rare causes.
- A systematic evaluation facilitates prompt management, improving patient outcomes.
- Early intervention is crucial while awaiting etiological confirmation.
Keywords:
ComplementDGKeHemolytic uremic syndromeHypertensionInfectionThrombotic microangiopathyTransplantMore Related Videos
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