Results 31 to 40 of about 4,456 (153)

A Novel Nonsense Mutation in FGB (c.1421G>A; p.Trp474Ter) in the Beta Chain of Fibrinogen Causing Hypofibrinogenemia with Bleeding Phenotype

Biomedicines, 2020
Congenital hypofibrinogenemia is a rare bleeding disorder characterized by a proportional decrease of functional and antigenic fibrinogen levels. Hypofibrinogenemia can be considered the phenotypic expression of heterozygous loss of function mutations ...
Tomas Simurda, Rui Vilar, Jana Zolkova, Eliska Ceznerova, Zuzana Kolkova, Dusan Loderer, Marguerite Neerman-Arbez, Alessandro Casini, Monika Brunclikova, Ingrid Skornova, Miroslava Dobrotova, Marian Grendar, Jan Stasko, Peter Kubisz   +13 more
doaj   +1 more source

Kaposiform hemangioendothelioma: Diagnosis and treatment

Pediatric Investigation, EarlyView.
Vascular endothelial growth factor‐C (VEGF‐C)/vascular endothelial growth factor receptor‐3 (VEGFR‐3) and angiopoietin‐2 (Ang‐2)/tyrosine kinase with immunoglobulin‐like and EGF‐like domain 2 (TIE2) signaling pathways play an important role in lymphangiogenesis.
Yi Tian, Yuanyuan Tian, Li Wei, Zigang Xu, Li Li, Lin Ma   +5 more
wiley   +1 more source

Thromboelastography as an early prediction method for hypofibrinogenemia in emergency department patients with primary postpartum hemorrhage

Scandinavian Journal of Trauma, Resuscitation and Emergency Medicine
Background Timely and accurate assessment of coagulopathy is crucial for the management of primary postpartum hemorrhage (PPH). Thromboelastography (TEG) provides a comprehensive assessment of coagulation status and is useful for guiding the treatment of
Sang-Min Kim, Chang Hwan Sohn, Hyojeong Kwon, Seung Mok Ryoo, Shin Ahn, Dong Woo Seo, Won Young Kim   +6 more
doaj   +1 more source

Peste des Petits Ruminants: Aetiology, Pathology, Immunology, Disease Status in Africa, Diagnosis, Control, Prevention and Treatment: A Review

Notulae Scientia Biologicae, 2019
Peste des petits ruminants (PPR) is a disease of economic and veterinary importance leading to considerable economic losses. PPR affects small domestic and wild ruminants.
Iniobong C. I. UGOCHUKWU, Chukwunonso K. EZEASOR, Onyinyechukwu A. AGINA, Davinson C. ANYOGU, Ijeoma C. CHUKWUDI, Sunday I. IDOKO, Emmanuel I. UGOCHUKWU   +6 more
doaj   +1 more source

Central Nervous System Bleeding in Children With Haemophilia in Limited Resource

Haemophilia, EarlyView.
ABSTRACT Introduction Central nervous system (CNS) bleeding in children with haemophilia is a life‐threatening complication that may cause severe neurological sequelae or death. In resource‐limited settings, where prophylaxis is not universally accessible, its patterns and outcomes may differ from those in high‐income countries.
Patcharee Komvilaisak, Sudarat Sirichaipornsak, Paiporn Sripraya, Napat Laoaroon, Kunanya Suwannaying, Pichayen Duanthongphon   +5 more
wiley   +1 more source

Fatal Chronic Varicella‐Zoster Viral Infection in a Young Man With Chediak–Higashi Syndrome

Pediatric Dermatology, EarlyView.
ABSTRACT Chediak–Higashi syndrome (CHS) is a rare autosomal recessive primary immunodeficiency characterized by partial oculocutaneous albinism, neurologic involvement, and a predisposition to severe infections. Patients are particularly susceptible to developing hemophagocytic lymphohistiocytosis (HLH), which significantly worsens prognosis. We report
Albane Badet, Clément Pruvot, Zoé Manssens, Wadih Abou‐Chahla, Sébastien Buche   +4 more
wiley   +1 more source

Tocilizumab administration in cytokine release syndrome is associated with hypofibrinogenemia after chimeric antigen receptor T-cell therapy for hematologic malignancies

Haematologica
Chimeric antigen receptor (CAR-) T cell therapy causes serious side effects including cytokine release syndrome (CRS). CRS-related coagulopathy is associated with hypofibrinogenemia that is thus far considered the result of disseminated intravascular ...
Markus Perl, Konstantin Herfeld, Dennis C. Harrer, Matthias Höpting, Marina Schweiger, Ulrich Sterz, Leonard Knödler, Susanne Heimerl, Leo Hansmann, Wolfgang Herr, Hendrik Poeck, Daniel Wolff, Matthias Edinger, Christina Hart, Matthias A. Fante   +14 more
doaj   +1 more source

EBV‐associated hemophagocytic lymphohistiocytosis complicated by severe coagulation disorders and opportunistic infections: case report of a survivor

Clinical Case Reports, 2018
Key Clinical Message The possibility of hemophagocytic lymphohistiocytosis should always be kept in mind when examining/treating a patient with fever of unknown origin and sepsis‐like symptoms.
Kirsten Saevels, Dominique Robert, Sylvie Van den Broeck, Ronald Malfait, Alain Gadisseur, Philippe Jorens, Anke Verlinden   +6 more
doaj   +1 more source

HHV‐8/KSHV in Solid Organ Transplantation: Current Gaps of Knowledge and Future Directions

Transplant Infectious Disease, EarlyView.
Risk mitigation strategies, including donors/recipients screening, DNAemia monitoring in recipients at risk, CNI‐to‐mTOR inhibitors switch, antivirals, and rituximab for KICS, may mitigate the impact of HHV‐8/KSHV infection in SOT. This review provides an update on KICS, identifies research gaps, and summarizes advances in screening and management ...
Alessandra Mularoni, Andrea Cona, Malgorzata Mikulska, Francesca Pecoraro, Carlotta Piazza, Elda De Vita, Giada Pietrosi, Matteo Bulati, Tiziana Lazzarotto, Mario Luppi   +9 more
wiley   +1 more source

The impact of rapid infusers on the hemostatic potential of cryoprecipitate products

Transfusion, EarlyView.
Abstract Background In cases of massive transfusion, intravenous access can be limited. Additionally, delivering different products through separate lines and infusion methods adds unnecessary steps. RBC, plasma, and whole blood (WB) are delivered via rapid infusers; cryoprecipitate (CRYO) is infused through separate access and is not approved with ...
Connor D. Purvis, Samantha Redden, Andrew Jacobson, Yao‐Wei Wang, Julie Campbell, Jessica Cardenas, Jan‐Michael Van Gent, Bryan A. Cotton   +7 more
wiley   +1 more source