Pyelolithotomy in a patient with Glanzmann thrombasthenia and antiglycoprotein IIb/IIIa antibodies: the shortest possible duration of treatment with recombinant activated factor VII and platelet transfusions [PDF]
Transfusion of platelet concentrates remains the first-line therapy for Glanzmann thrombasthenia in case of bleeding or preparation for surgery. However, development of antibodies to platelet glycoprotein (Gp) IIb/IIIa complex or human leukocyte ...
Omer Devecioğlu +5 more
doaj +1 more source
International Practices in Managing Preconception, Pregnancy and Childbirth in Women With Glanzmann Thrombasthenia: A Survey From the European Association of Haemophilia and Allied Disorders (EAHAD). [PDF]
ABSTRACT Background Glanzmann thrombasthenia (GT) is an inherited platelet disorder resulting in severely reduced platelet aggregation and increased bleeding tendency. Pregnancy and childbirth in women with GT present significant challenges for both mother and child.
Rutten KHG +11 more
europepmc +2 more sources
Clinical and Genetic Characterization of 269 Patients With Suspected Inherited Platelet Disorders: The Padua Monocentric Experience. [PDF]
ABSTRACT Background Inherited platelet disorders (IPDs) are rare hematologic conditions encompassing a heterogeneous spectrum of quantitative and qualitative platelet defects, frequently associated with variable clinical phenotypes and comorbidities. Accurate diagnosis necessitates comprehensive genetic characterization, detailed clinical and bleeding ...
Ferrari S +6 more
europepmc +2 more sources
Inherited thrombocytopenia with thrombasthenia [PDF]
A family with congenital thrombocytopenia is described through four generations where the mode of inheritance appears to be an autosomal dominant. Spontaneous bruising of varying severity, menorrhagia, and profuse bleeding at operation necessitating transfusion were predominant in the history. Platelet function tests were performed on
N K, Sheth, T A, Prankerd
openaire +2 more sources
Glanzmann’s thrombasthenia: A rare case [PDF]
Glanzmann’s thrombasthenia is an extremely rare autosomal recessive inherited bleeding disorder characterized by the impaired function of platelets leading to prolonged bleeding time.
Sunder, Ashok, Mohanty, Bijaya
core +1 more source
The Rationale: Glanzmann thrombasthenia is a rare platelet disorder affecting 0.0001% of the population. Dentists may often be unaware of this condition, and manipulation of soft tissue can lead to grave consequences, which may even result in fatality ...
Lalitha S Jairam, Sunil Babu Kotha
doaj +1 more source
Optimizing Emergency Department Care for People With Bleeding Disorders: A Scoping Review of Barriers and Interventions for Improvement. [PDF]
ABSTRACT Background Emergency department (ED) care is critical for managing acute bleeding events in people with bleeding disorders. Despite international guidelines recommending haemostatic treatment within 30–60 min, delays and deviations from best practices are common and associated with poorer outcomes.
Guo LY +7 more
europepmc +2 more sources
Introduction: Glanzmann’s thrombasthenia (GT) is a rare, genetically inherited, functional disorder of platelets. The pathology is deficient or dysfunctional platelet glycoprotein IIb/IIIa (GP IIb/IIIa) complex resulting in bleeding diathesis.
Ali Abbas +3 more
doaj +1 more source
Standard treatment for Glanzmann thrombasthenia, a severe inherited bleeding disorder, is platelet transfusion. Recombinant factor VIIa is reported to be effective in Glanzmann thrombasthenia with platelet antibodies and/or refractoriness to platelet ...
Man-Chiu Poon +5 more
doaj +1 more source
Glanzmann's thrombasthenia: updated
Glanzmann's thrombasthenia is an autosomal recessive disorder, rare in a global context, but a relatively more common platelet function defect in communities where consanguineous marriages are more frequent. On clinical grounds alone, it cannot be distinguished from other congenital platelet function defects.
Sona, Nair +4 more
openaire +2 more sources

