The More Recent History of Hemophilia Treatment

Welcome to the latest of our Eberhard F. Mammen award announcements. As noted in previous editorials (►Table 1), Thieme, the publisher of Seminars in Thrombosis & Hemostasis (STH), has created the "Eberhard F. Mammen Excellence in Thrombosis and Hemostasis Awards" in honor of Eberhard Mammen (►Fig. 1), and in recognition of his contribution to this field and to the journal that he both founded and steered for over three decades. These awards began in 2009, under two categories, "Most Popular Article Awards" and "Young Investigator Awards." Accordingly, 2024 represents 16 years of award presentations (►Table 1). Current details and conditions of the award can be summarized as:➢ Most Popular Article Awards: awarded to the authors of the most popular articles published in STH in the preceding 2 years as captured in the preceding year.The awards are determined by the Editor in Chief on the basis of user statistics from the publisher Thieme e-Journals. Prefaces, Errata, Letters to the Editor, Editorials, Commentaries, and previous award-winning articles are excluded from further consideration of these awards, which currently comprise two categories-one for "Free Access" articles, and another for a "General Category." There are two major cash prizes of US$1,000 for each category. In addition, winners of the "General Category" awards are granted "Free Access" status for these articles thereafter.

Section: “Most Popular” Article Awardsmentioning

confidence: 99%

2024 Eberhard F. Mammen Award Announcements: Part I—Most Popular Articles

Favaloro

2024

“…Although gene therapy has a long history in hemophilia A and B, it has not yet been used in rare bleeding disorders. 98 99 The only attempt was made in our department in 2021. In this, a novel gene editing system, clustered regularly interspaced short palindromic repeat (CRISPR)/CRISPR-associated protein 9 (Cas9) system, was used to correct the most common disease-causing mutation of FXIII deficiency in dermal-fibroblasts in Iran.…”

Section: Management Of Factor XIII Deficiencymentioning

confidence: 99%

The History of Factor XIII Deficiency

Dorgalaleh¹

2023

Despite the early discovery of factor XIII (FXIII) in 1944, the diagnosis of FXIII deficiency was not made until 1960, after all the other coagulation factor deficiencies, most likely due to the normality of routine coagulation testing in FXIII deficiency. Although the first case was detected by the clot solubility test and this test has long since been used to detect FXIII deficiency, the test is no longer recommended by experts. Over the past 60 years, knowledge about FXIII deficiency has expanded considerably, between 1992, when the first variant was identified, and 2022, 197 mutations have been reported. Almost all missense mutations have a similar effect on FXIII, leading to instability and faster degradation of mutant FXIII protein. Therapeutic options have evolved from historical fresh frozen plasma (FFP), old plasma, whole blood, and cryoprecipitate, to plasma-derived and recombinant FXIII concentrates, respectively available since 1993 and 2012. These concentrate products were respectively approved by the Food and Drug Administration in 2011 and 2013. This historical review covers various aspects of FXIII related disorders, including the discovery of the FXIII, associated disorders, molecular basis, diagnosis, and treatment of FXIII deficiency.

“…Consequently, the most popular articles from each category are identified separately. Tables 3 and 4 , respectively, list the top 15 downloaded articles from STH in 2022 (from 2021 and 2022 inclusive), as eligible for either the “free access” ( Table 3 ) 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 or the “General Category” ( Table 4 ) 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 awards. As expected, the majority of the “Free Access” articles were related to COVID-19.…”

Section: “Most Popular” Article Awardsmentioning

confidence: 99%

2023 Eberhard F. Mammen Award Announcements: Part I—Most Popular Articles

Favaloro

2023

Welcome to the latest of our Eberhard F. Mammen award announcements. As noted in previous editorials (►Table 1), Thieme, the publisher of Seminars in Thrombosis and Hemostasis (STH), has created the "Eberhard F. Mammen Excellence in Thrombosis and Hemostasis Awards" in honor of Eberhard Mammen (►Fig. 1), and in recognition of his contribution to this field and to the journal that he both founded and steered for over three decades. These awards began in 2009, under two categories, "Most Popular Article Awards" and "Young Investigator Awards." Accordingly, 2023 represents 15 years of award presentations (►Table 1). Current details and conditions of the award can be summarized as:Most popular article awards: Awarded to the authors of the most popular articles published in STH in the preceding 2 years as captured in the preceding year. The awards are determined by the editor in chief on the basis of user statistics from Thieme e-Journals. Prefaces, Errata, Letters to the Editor, Editorials, Commentaries, and previous award-winning articles are excluded from further consideration of these awards, which currently comprise two categories-one for "Free Access" articles and another for a "General Category." There are two major cash prizes of US$ 1,000 for each category. In addition, winners of the "General Category" awards are granted "Free Access" status for these articles thereafter. Young investigator awards: Best presentation or meeting abstract by a young investigator-as presented or delivered to an international or large regional meeting on a topic related to the fields of thrombosis and hemostasis, and whose subject matter is determined to be in the spirit of Dr.