8 research outputs found
Ultrafast Magnetic Resonance Imaging for Iron Quantification in Thalassemia Participants in the Developing World The TIC-TOC Study (Thailand and UK International Collaboration in Thalassaemia Optimising Ultrafast CMR)
Thalassemia is the most common monogenetic disorder worldwide, with 60 000
infants with thalassemia major born annually.1 Survival often depends on regular
blood transfusions to correct anemia and to reduce ineffective erythropoiesis, but
these transfusions can result in iron overload and organ failure unless chelation therapy
is undertaken. Serum ferritin levels continue to be used as a guide to chelation but are
unreliable, and the availability of cardiovascular magnetic resonance (CMR) T2* imaging
has transformed patient management by allowing organ-specific quantification of
iron content.
Countries with a high prevalence of thalassemia major have CMR, but magnet
time is expensive and analytic expertise lacking. The aim of TIC-TOC (Thailand and
UK International Collaboration in Thalassaemia Optimising Ultrafast CMR) was to
investigate whether ultrafast CMR mapping could provide reliable immediate diagnoses
of heart and liver iron content, eliminating the need for complex analysis, thus
reducing costs to a level within local resources. The research received approval by
the Institutional Review Board of the Faculty of Medicine at Chulalongkorn University.
All participants provided written informed consent.</p
P1524: EFFECTS OF CORONAVIRUS DISEASE 2019 (COVID-19) VACCINATION ON HEMOGLOBIN LEVELS IN THALASSEMIA PATIENTS
Red Cell Indices and Hemoglobin Profile of Newborn Babies with Both the Sickle Gene and Alpha Thalassaemia in Central India
Laboratory testing for ADAMTS13
ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13), also known as VWF (von Willebrand factor) protease, may be assessed in a vast array of clinical conditions. Notably, a severe deficiency of ADAMTS13 characterises TTP (thrombotic thrombocytopenic purpura), a rare but potentially fatal disorder associated with thrombosis due to accumulation of prothrombotic ultra-large VWF multimers. Although prompt identification/exclusion of TTP can be facilitated by rapid ADAMTS13 testing, the most commonly utilised assays are based on ELISA (enzyme linked immunosorbent assay) and require long turnaround time and have relatively limited throughput. Nevertheless, several rapid ADAMTS13 assays are now available, at least in select geographies. The current mini-review discusses these issues, as well as the potential utility of ADAMTS13 testing in a range of other conditions, including coronavirus disease 2019 (COVID-19). This article is protected by copyright. All rights reserved