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Review
. 2018 Sep;44(9):1536-1538.
doi: 10.1007/s00134-018-5059-2. Epub 2018 Jan 24.

Understanding thrombotic microangiopathies in children

Bérangère S Joly  1   2 , X Long Zheng  3 , Agnès Veyradier  4   5
Affiliations
Review

Understanding thrombotic microangiopathies in children

Bérangère S Joly et al. Intensive Care Med. 2018 Sep.
No abstract available

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Conflict of interest statement

Conflicts of interest

BSJ is recipient of the MaRIH research grant Alexion 2016; AV is a member of the French Advisory board on Caplacizumab (Ablynx); XLZ is a consultant for Ablynx and a member of the speakers’ bureau for Alexion.

Figures

Fig. 1
Fig. 1
Incidence of child-onset thrombotic microangiopathy syndromes. Haemolytic uraemic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) are the most frequent thrombotic microangiopathy (TMA) reported in children. HUS includes either infection-induced HUS (Shiga-toxin-producing Escherichia coli (STEC-HUS) and Streptococcus pneumoniae) or atypical HUS (aHUS) (dysregulation of the complement alternative pathway, mutation of DGKE (diacyglycerol kinase ε) and cobalamin C (cbl-C) defect). TTP pathophysiology is based on both inherited (congenital TTP) or acquired deficiency (autoimmune TTP) of ADAMTS13. All incidences are expressed as the number of new cases per 106 children (< 18 years old) per year, except for aHUS (*) the incidence of which is provided for both adults and children
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