No abstract available.
Thrombasthenia*

No abstract available.
Thrombasthenia*

No abstract available.
Humans ; Siblings* ; Thrombasthenia*

No abstract available.
Blood Platelets* ; Diagnosis* ; Flow Cytometry* ; Integrin beta3* ; Thrombasthenia*

No abstract available.
Blood Platelets* ; Electrophoresis, Polyacrylamide Gel* ; Membrane Glycoproteins* ; Membranes* ; Thrombasthenia*

Glanzmann's thrombasthenia is an autosomal recessive bleeding disorder caused by qualitative or quantitative abnormalities of the platelet glycoprotein IIb/IIIa (GP IIb/IIIa), which can lead to excessive bleeding. Glanzmann thrombasthenia is associated with clinical variability, with some patients only having minimal bruising and others having frequent, severe and potentially fatal hemorrhages. Platelet transfusions, which used to be the standard treatment, may lead to the development of antibodies to HLA and/or GPIIb/IIIa, thereby rendering future transfusions ineffective. Glanzmann's thrombasthenia can be a severe hemorrhagic disease; however, the prognosis is excellent with careful supportive care. In this case, administering allogenic plateletpheresis to patients with Glanzmann's thrombasthenia who were refractory to platelet transfusions was found to be successful during bone surgeries.
Anesthesia, General ; Antibodies ; Blood Platelets ; Glycoproteins ; Hemorrhage ; Humans ; Orthopedics ; Platelet Transfusion ; Plateletpheresis ; Prognosis ; Thrombasthenia

Glanzmann's thrombasthenia is a rare autosomal recessive disorder characterized by bleeding tendency due to decreased coagulation function of platelets. Perioperative adequate blood transfusion including preoperative platelet transfusion seems to be the most important component affecting anesthetic management. Also, minimally invasive techniques should be used at the time of induction of anesthesia and the use of recombinant activated coagulation factor VII may be considered. We successfully conducted an anesthetic management for dental procedure in a four-year-old child with Glanzmann's thrombasthenia using preoperative platelet transfusion. Thus we present this anesthetic experience and share informations on anesthetic management of patients with Glanzmann's thrombasthenia.
Anesthesia ; Blood Transfusion ; Child ; Factor VII ; Hemorrhage ; Humans ; Platelet Transfusion ; Thrombasthenia*

No abstract available.
Blood Platelets* ; Electrophoresis, Polyacrylamide Gel* ; Flow Cytometry* ; Membrane Glycoproteins* ; Membranes* ; Thrombasthenia*

Glanzmann's thrombasthenia (GT) is a rare inherited platelet disorder, which is characterized by a complete lack of platelet aggregation due to a deficiency or abnormality of the membrane glycoprotein IIb/IIIa complex. Anti-GPIIb/IIIa antibodies have also been identified to cause platelet dysfunction in patients with a normal platelet count, but this has only been rarely encountered. The condition is also known as acquired GT. Herein, we describe a patient with acquired GT and a history of Evans' syndrome, who presented with severe bleeding and platelet dysfunction, but with a normal platelet count and GP IIb/IIIa expression.
Antibodies ; Blood Platelets ; Hemorrhage ; Humans ; Membrane Glycoproteins ; Platelet Aggregation ; Platelet Count ; Thrombasthenia*

Child ; Child, Preschool ; Female ; Flow Cytometry ; methods ; Humans ; Infant ; Male ; Thrombasthenia ; classification ; diagnosis