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. 2014 Sep 8;2:97. doi: 10.3389/fped.2014.00097

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Copyright © 2014 Hofer, Rosales, Fischer and Giner.

This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

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Recommendation for evaluation and monitoring of extra-renal manifestations in patients with complement-mediated TMA. Depending on the clinical presentation and possible symptoms, the physicians have to be aware of TMA associated, potentially devastating extra-renal TMA manifestations. A high level of suspicion may be needed to diagnose and treat those complications early in disease course. Thus, the initial laboratory and imaging investigations as well as the monitoring procedures have to reflect this knowledge. However, the indications for highly invasive diagnostic procedures like heart catheterization and MRI/A in small children with the need of total anesthesia have to be proven on an individual basis. The recommendation for MRI/A 2 years after onset in all patients with complement-mediated TMA excludes children with the need for total anesthesia, except those with clinical cerebrovascular symptoms. ALAT, Alanin-aminotransferase; AP, alkaline phosphatase; ASAT, aspartat-aminotransferase; CK, creatine kinase; ECG, electrocardiography; ECHO, echocardiography; EEG, electroencephalography; gamma-GT, gamma-glutamyl transpeptidase; MRI/A, magnetic resonance imaging/angiography; nt-pro-BNP, N-terminal pro brain natriuretic peptide.