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The International Hereditary Thrombotic Thrombocytopenic Purpura Registry: key findings at enrollment until 2017

The result's identifiers

  • Result code in IS VaVaI

    <a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00023736%3A_____%2F19%3A00012821" target="_blank" >RIV/00023736:_____/19:00012821 - isvavai.cz</a>

  • Alternative codes found

    RIV/00843989:_____/19:E0107958

  • Result on the web

    <a href="https://doi.org/10.3324/haematol.2019.216796" target="_blank" >https://doi.org/10.3324/haematol.2019.216796</a>

  • DOI - Digital Object Identifier

    <a href="http://dx.doi.org/10.3324/haematol.2019.216796" target="_blank" >10.3324/haematol.2019.216796</a>

Alternative languages

  • Result language

    angličtina

  • Original language name

    The International Hereditary Thrombotic Thrombocytopenic Purpura Registry: key findings at enrollment until 2017

  • Original language description

    Congenital thrombotic thrombocytopenic purpura is an autosomal recessive inherited disease with a clinically heterogeneous course and an incompletely understood genotype-phenotype correlation. In 2006, the Hereditary TTP Registry started recruitment for a study which aimed to improve the understanding of this ultra-rare disease. The objective of this study is to present characteristics of the cohort until the end of 2017 and to explore the relationship between overt disease onset and ADAMTS13 activity with emphasis on the recurring ADAMTS13 c.4143_4144dupA mutation.

  • Czech name

  • Czech description

Classification

  • Type

    J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database

  • CEP classification

  • OECD FORD branch

    30205 - Hematology

Result continuities

  • Project

  • Continuities

    I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Others

  • Publication year

    2019

  • Confidentiality

    S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Data specific for result type

  • Name of the periodical

    Haematologica

  • ISSN

    0390-6078

  • e-ISSN

  • Volume of the periodical

    104

  • Issue of the periodical within the volume

    10

  • Country of publishing house

    IT - ITALY

  • Number of pages

    9

  • Pages from-to

    2107-2115

  • UT code for WoS article

    000488513600038

  • EID of the result in the Scopus database

    2-s2.0-85065445111