The first two Japanese cases of severe type I congenital plasminogen deficiency with ligneous conjunctivitis: Successful treatment with direct thrombin inhibitor and fresh plasma
- 16 April 2009
- journal article
- case report
- Published by Wiley in American Journal of Hematology
- Vol. 84 (6), 363-365
- https://doi.org/10.1002/ajh.21402
Abstract
A 71‐year‐old woman and her elder sister developed ligneous conjunctivitis after ocular surgery. Laboratory tests demonstrated that the proband and her sister had 6.6% and 8.1% of plasminogen activity, and 1.2 and 1.4 mg/dl of antigen, respectively. Thus, they were diagnosed as having severe type I plasminogen deficiency, for the first time, in Japan. DNA sequencing and PCR‐RFLP analyses revealed that these two cases are homozygotes of a novel A‐to‐G mutation at the obligatory splicing acceptor site in intron‐C. Both cases were satisfactorily treated with a direct thrombin inhibitor, topical Argatroban, and topical plasma obtained from their healthy family members. Am. J. Hematol. 2009.This publication has 10 references indexed in Scilit:
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