Results 41 to 50 of about 6,740 (215)

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

Kasabach-Merritt phenomenon and prenatal counseling: a case series. [PDF]

, 2016
Kasabach-Merritt phenomenon can be encountered in the perinatal period. No consensus exists regarding prenatal management. We report one prenatal case leading to therapeutic abortion and one neonatal case, successfully treated by a multimodal therapy ...
Beissel, A., Blanc, S., Cassart, M., Claris, O., Fischer Fumeaux, C.J., Guibaud, L., Riou, S.   +6 more
core   +1 more source

Targeted high-throughput sequencing for genetic diagnostics of hemophagocytic lymphohistiocytosis [PDF]

, 2015
Background: Hemophagocytic lymphohistiocytosis (HLH) is a rapid-onset, potentially fatal hyperinflammatory syndrome. A prompt molecular diagnosis is crucial for appropriate clinical management.
Abboud, Miguel, Akar, Himmet Haluk, Belen, Burcu, Ben Bdira, Eya, Bryceson, Yenan T., Chiang, Samuel C. C., Devecioglu, Omer, Erichsen, Hans Christian, Fadoo, Zehra, Henter, Jan-Inge, Hästbacka, Johanna, Kaya, Zuhre, Lagerstedt-Robinson, Kristina, Meeths, Marie, Mottonen, Merja, Nordenskjold, Magnus, Nunes, Susana, Patiroglu, Turkan, Sabel, Magnus, Saribeyoglu, Ebru Tugrul, Tesi, Bianca, Tvedt, Tor Henrik, Unal, Ekrem, Unal, Sule, Unuvar, Aysegul, Yeoh, Allen E. J.   +25 more
core   +3 more sources

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

Plasma and whole blood exchange in meningococcal sepsis [PDF]

, 1992
Contains fulltext : 4755.pdf (publisher's version ) (Open ...
Dalen, R. van, Deuren, M. van, Meer, J.W.M. van der, Santman, F.W., Sauerwein, R.W., Span, L.F.R.   +5 more
core   +12 more sources

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

Rare Bleeding Disorders and Bleeding Disorder of Unknown Cause: Current Understanding and Recent Developments

Haemophilia, EarlyView.
ABSTRACT Rare bleeding disorders (RBDs) represent a diverse group of inherited conditions involving coagulation factors or platelets. These conditions, such as Glanzmann thrombasthenia (GT) or severe coagulation factor deficiencies, are uncommon. In contrast, bleeding disorder of unknown cause (BDUC) is a diagnosis of exclusion without an identifiable ...
Alessandro Casini, Robert Klamroth, Bauke Haisma, Dino Mehic, Saskia E. M. Schols   +4 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

A Rare Case of Hemophagocytic Lymphohistiocytosis of Unknown Etiology [PDF]

, 2017
Hemophagocytic lymphohistiocytosis (HLH) is an uncommon cytokine storm syndrome marked which can cause high mortality. In adults, acquired HLH usually has an underlying infectious, autoimmune or malignant process that triggers the syndrome.
Damsker, Jason A., Friedenheim, Richard, Gagnon, Marie Helene, Micaily, Ida   +3 more
core   +1 more source

The Swiss Haemophilia Registry–Report From the First 8 Years

Haemophilia, EarlyView.
ABSTRACT Introduction Patient registries capture disease related information and provide a valuable source for real‐world data on rare diseases and their management. The Swiss Haemophilia Registry (SHR) was established in 2015 on the basis of a new Swiss federal human research act.
Alessandra Bosch, Lorenzo Alberio, Pierre Fontana, Lukas Graf, Johanna A. Kremer Hovinga, Nicolas von der Weid, Mattia Rizzi, Manuela Albisetti, the Swiss Haemophilia Network   +8 more
wiley   +1 more source