Results 11 to 20 of about 850 (150)

Congenital afibrinogenemia in a newborn

open access: yesSanamed, 2022
Introduction: Congenital afibrinogenemia is a rare coagulation disorder characterized by a deficiency in the fibrinogen molecule. Fibrinogen is a hexameric glycoprotein consisting of a polypeptide chain encoded by FGB, FGA, and FGG and is required for ...
Özay Mustafa   +2 more
doaj   +1 more source

Afibrinogenemia: A rare cause of refractory puberty menorrhagia

open access: yesIraqi Journal of Hematology, 2022
Excessive menstruation after attaining menarche during adolescent age group is known as puberty menorrhagia (PM). The most common presentation includes anemia due to excessive blood loss.
Vandana Kamatham   +2 more
doaj   +1 more source

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

open access: yesThe 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   +8 more
wiley   +1 more source

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

open access: yesBritish 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   +3 more
wiley   +1 more source

Women With Congenital Hypofibrinogenemia/Afibrinogenemia: From Birth to Death

open access: yesClinical 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   +2 more
doaj   +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

open access: yesHealth 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   +7 more
wiley   +1 more source

The role of new inflammatory markers in determining disease activation and severity in patients with hidradenitis suppurativa

open access: yesInternational Journal of Dermatology, Volume 62, Issue 8, Page 1076-1081, August 2023., 2023
Abstract Hidradenitis suppurativa (HS) is a chronic inflammatory disease. Recently published studies have suggested the use of markers of inflammation to monitor HS patients. These studies discuss the platelet/lymphocyte ratio (PLR), neutrophil/lymphocyte ratio (NLR), pan‐immune‐inflammation value (PIV), and systemic immune‐inflammation index (SIII ...
Eda Öksüm Solak   +4 more
wiley   +1 more source

Management of rare inherited bleeding disorders: Proposals of the French Reference Centre on Haemophilia and Rare Coagulation Disorders

open access: yesEuropean Journal of Haematology, Volume 110, Issue 6, Page 584-601, June 2023., 2023
Abstract Introduction The rare coagulation disorders may present significant difficulties in diagnosis and management. In addition, considerable inter‐individual variation in bleeding phenotype is observed amongst affected individuals, making the bleeding risk difficult to assess in affected individuals.
Marc Trossaert   +12 more
wiley   +1 more source

Physiological correction of hereditary mild hypofibrinogenemia during pregnancy

open access: yesHaemophilia, Volume 29, Issue 3, Page 836-843, May 2023., 2023
Abstract Introduction Hereditary hypofibrinogenemia is a rare fibrinogen disorder characterised by decreased levels of fibrinogen. Pregnant women with hypofibrinogenemia are at risk of adverse obstetrical outcomes, depending on the fibrinogen level.
Rita Marchi   +4 more
wiley   +1 more source

Cellulitis on face in a patient with congenital afibrinogenemia

open access: yesJournal of Indian Society of Pedodontics and Preventive Dentistry, 2011
Congenital afibrinogenemia is a rare coagulation disorder, with an estimated prevalence of 1 : 1,000,000, characterized by a complete absence to reduced level of circulating fibrinogen.
G D Chandan   +4 more
doaj   +1 more source

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