Glanzmann's thrombasthenia, is one of the rarest congenital, genetically inherited platelet disorder. It has an incidence of about 1:1,000,000, but is more common in populations with increased consanguinity. Glanzmann's thrombasthenia is characterized by deficiency or dysfunction of glycoprotein (GP) lib and Ilia, which are the receptors of fibrinogen. Both sexes are equally affected. Typical mucocutaneous bleeding occurs at birth or early infancy. Obtaining appropriate dental history of excessive bleeding after dental extraction, unexplained spontaneous mucocutaneous bleeding, gingival bleeding during teething or shedding of deciduous teeth and petechiae, ecchymoses or purpura on mucous membranes can play an important part in diagnosis. Hence, the pediatric dentist plays a very crucial role for prompt diagnosis and management of Glanzmann's thrombasthenia. Presenting here is a known case of Glanzmann's thrombasthenia, of a 6-year-old girl who required to undergo dental extraction and its successful management using an "acrylic-splint" along with the placement of "Calgigraf-Ag Foam".
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http://dx.doi.org/10.5005/jp-journals-10005-1054 | DOI Listing |
Hamostaseologie
January 2025
Center for Thrombosis and Hemostasis, University Medical Center Mainz, Mainz, Germany.
Congenital platelet disorders are rare and targeted treatment is usually not possible. Inherited platelet function disorders (iPFDs) can affect surface receptors and multiple platelet responses such as defects of platelet granules, signal transduction, and procoagulant activity. If iPFDs are also associated with a reduced platelet count (thrombocytopenia), it is not uncommon to be misdiagnosed as immune thrombocytopenia.
View Article and Find Full Text PDFCureus
December 2024
Obstetrics and Gynecology, Latifa Hospital, Dubai, ARE.
Glanzmann thrombasthenia (GT) is an autosomal recessive platelet functional bleeding disorder caused by mutations in the ITGA2B or ITGB3 genes, often presenting as mucocutaneous bleeding. GT typically presents in infancy, but this study reports a rare case of neonatal presentation in a female infant born to consanguineous parents. The mother, a 27-year-old woman with a family history of GT, presented at 36 weeks gestation for an elective cesarean due to a breech presentation.
View Article and Find Full Text PDFJ Family Med Prim Care
November 2024
Department of Basic Science College of Medicine, Princess Nourah bint Abdulrahman University, Riyadh, Saudi Arabia.
Background: Glanzmann thrombasthenia (GT) is a rare disease with an autosomal recessive inheritance pattern. This disorder is not so uncommonly encountered in routine clinical practice and laboratory settings in Pakistan let alone in the rest of the world. To describe the bleeding phenotype of GT and treatment outcomes in over one hundred patients in north Pakistan.
View Article and Find Full Text PDFRes Pract Thromb Haemost
November 2024
University of Strasbourg, Faculty of Dental Surgery, Strasbourg, France.
Background: Glanzmann thrombasthenia (GT) is a very rare autosomal inherited bleeding disease affecting megakaryocyte lineage with impacts on oral health such as gingival bleeding, which requires specific management protocols. Very few clinical cases have been published in the dental and hematologic literature.
Objectives: This study focuses on a series of 21 patients affected specifically by GT and their hemorrhagic prophylaxis management with the use of recombinant activated factor VII (rFVIIa) for dental extractions and full-mouth debridement.
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