Results 11 to 20 of about 1,912 (211)

Diagnostic utility of bleeding assessment tools in congenital fibrinogen deficiencies [PDF]

Haemophilia, Volume 29, Issue 3, Page 827-835, May 2023., 2023
Background: The assessment of clinical history is crucial before referring a patient for further laboratory testing. Bleeding assessment tools (BAT) are developed to standardize clinical evaluation.
Azarkeivan, Azita, Baghaipour, Mohammad Reza, Jazebi, Mohammad, Menegatti, Marzia, Moazezi, Somayeh, Mohsenian, Samin, Palla, Roberta, Peyvandi, Flora, Seidizadeh, Omid   +8 more
core   +2 more sources

The β-Chain Mutation p.Arg17Stop Impairs Fibrinogen Synthesis and Secretion: A Nonsense Mutation Associated With Hypofibrinogenemia. [PDF]

J Clin Lab Anal
We reported the first case of a FGB p.Arg17Stop mutation with a heterozygous gene status, and conducted research on the pathogenic mechanisms related to the fibrinogen Bβ (p.Arg17Stop) mutation. This mutation significantly impairs both the synthesis and secretion of fibrinogen.
Qian C, Huang C, Luo Q, Qin K, Wu Y, Liao L, Zhang Q, Xiang L, Yan J.   +8 more
europepmc   +2 more sources

PB2654: RARE BLEEDING DISORDERS: LONG-TERM EXPERIENCE FROM A HEMOPHILIA CENTER [PDF]

Hemasphere, 2023
HemaSphere, Volume 7, Issue S3, August 2023.
Meimaridis A, Chissan S, Kotsiou N, Gavriilaki E, Moka E, Vakalopoulou S.   +5 more
europepmc   +2 more sources

Correction to "A Case of Congenital Afibrinogenemia With Multiple Thrombotic and Hemorrhagic Disorders". [PDF]

Clin Case Rep
Clinical Case Reports, Volume 12, Issue 11, November 2024.
europepmc   +2 more sources

PB2675: QUALITY OF LIFE IN INDIAN ADULTS WITH INHERITED BLEEDING DISORDERS [PDF]

Hemasphere, 2023
HemaSphere, Volume 7, Issue S3, August 2023.
Satish S, Bhat V, Ravi D, Balakrishnan C, Alphonse T, Rao S, Anandram S, Ross C.   +7 more
europepmc   +2 more sources

Real‐World Experience with a Human Fibrinogen Concentrate: Clinical Data from Adult and Pediatric Patients Requiring Fibrinogen for Bleeding Control and Prevention

The Journal of Clinical Pharmacology, Volume 63, Issue 11, Page 1186-1196, November 2023., 2023
Abstract Human fibrinogen concentrate (Fibryga) received temporary approval for fibrinogen replacement therapy in France (2017), with subsequent full approval for congenital and acquired hypofibrinogenemia. We evaluated real‐world use for on‐demand treatment of bleeding and prophylaxis to enhance our knowledge on fibrinogen concentrate as an option for
Francois Stéphan, Loriane Gutermann, Stéphanie Bourget, Sarah Djabarouti, Johanna Berdugo, Yann Fardini, Pierre Clerson, Guillaume Hébert, Chafké Belmokhtar   +8 more
wiley   +1 more source

Women With Congenital Hypofibrinogenemia/Afibrinogenemia: From Birth to Death

Clinical and Applied Thrombosis/Hemostasis, 2020
Congenital fibrinogen disorders are a group of most frequent rare coagulation disorder, characterized by deficiency and/or defects in the fibrinogen molecule. Quantitative disorders include hypofibrinogenemia and afibrinogenemia.
Yan Zhang MD, Xiaohang Zuo MD, Yue Teng PhD, MD   +2 more
doaj   +1 more source

Congenital fibrinogen disorders: Strengthening genotype–phenotype correlations through novel genetic diagnostic tools

British Journal of Haematology, Volume 203, Issue 3, Page 355-368, November 2023., 2023
Summary Congenital fibrinogen disorders or CFDs are heterogenous, both in clinical manifestation and array of culprit molecular lesions. Correlations between phenotype and genotype remain poorly defined. This review examines the genetic landscape discovered to date for this rare condition.
Radha Ramanan, James D. McFadyen, Andrew C. Perkins, Huyen A. Tran   +3 more
wiley   +1 more source

Comparison of coagulation parameters associated with fibrinogen concentrate and cryoprecipitate for treatment of bleeding in patients undergoing cytoreductive surgery for pseudomyxoma peritonei: Subanalysis from a randomized, controlled phase 2 study

Health Science Reports, Volume 6, Issue 9, September 2023., 2023
Abstract Background and Aims The FORMA‐05 study compared the efficacy and safety of human fibrinogen concentrate (HFC) versus cryoprecipitate for hemostasis in bleeding patients undergoing cytoreductive surgery for pseudomyxoma peritonei (PMP). This subanalysis explores coagulation parameters in the FORMA‐05 patients, with a focus on the seven patients
Ashok Roy, Nigel Sargant, John Bell, Sophia Stanford, Cristina Solomon, Irina Kruzhkova, Sigurd Knaub, Faheez Mohamed   +7 more
wiley   +1 more source

Heterozygosity for Fibrinogen Results in Efficient Resolution of Kidney Ischemia Reperfusion Injury [PDF]

, 2012
Fibrinogen (Fg) has been recognized to play a central role in coagulation, inflammation and tissue regeneration. Several studies have used Fg deficient mice (Fg−/−) in comparison with heterozygous mice (Fg+/−) to point the proinflammatory role of Fg in ...
Ajay, Amrendra K, Bijol, Vanesa, Saikumar, Janani, Vaidya, Vishal S.   +3 more
core   +9 more sources