Results 21 to 30 of about 1,325 (125)

FREQUENCY OF INHERITED PLATELE FUNCTION DISORDERS–ARMED FORCES INSTITUTE OF PATHOLOGY EXPERIENCE

Pakistan Armed Forces Medical Journal, 2020
Objective: To determine the frequency and clinical features of inherited platelet function disorders diagnosed at Armed Forces Institute of Pathology. Study Design: Cross sectional study.
Saira Irum, Helen Mary Robert, Asad Mahmood, Rafia Mahmood, Ayesha Khurshid, Mansoor Ishaq   +5 more
doaj   +4 more sources

Cytoskeletal perturbation leads to platelet dysfunction and thrombocytopenia in variant forms of Glanzmann thrombasthenia

Haematologica, 2016
Several patients have been reported to have variant dominant forms of Glanzmann thrombasthenia, associated with macrothrombocytopenia and caused by gain-of-function mutations of ITGB3 or ITGA2B leading to reduced surface expression and constitutive ...
Loredana Bury, Emanuela Falcinelli, Davide Chiasserini, Timothy A. Springer, Joseph E. Italiano, Paolo Gresele   +5 more
doaj   +1 more source

THE CLINICAL PICTURE AND LABORATORY WORK-UP OF GLANZMANN THROMBASTHENIA

Hematology, Transfusion and Cell Therapy, 2021
Case report: We present the clinical picture and laboratory work-up of Glanzmann thrombasthenia, based on a group of 7 patients. Bleeding history was significant in all patients and included both mucosal and postsurgery bleeds.
Joanna Zdziarska, Teresa Iwaniec, Tomasz Sacha, Ewa Wypasek   +3 more
doaj   +1 more source

Hematological and surgical management in Glanzmann′s thrombasthenia: A case report

Journal of Indian Society of Pedodontics and Preventive Dentistry, 2014
Glanzmann′s thrombasthenia (GT) is a rare, congenital, and moderate to severe platelet disorder. The bleeding time is increased, due to lack of platelet aggregation, since the patients with GT have deficient or dysfunctional integrin membrane ...
Anjana Gopalakrishnan, Ravi Veeraraghavan, Prasanth Panicker   +2 more
doaj   +1 more source

Smartphone‐Based Teledentistry to Support Clinical Triage and Risk‐Informed Dental Care in Patients With Inherited Bleeding and Haemoglobin Disorders: A Cross‐Sectional Diagnostic Agreement Study

Haemophilia, EarlyView.
ABSTRACT Introduction Patients with inherited bleeding and haemoglobin disorders face barriers to accessing timely dental care, increasing the risk of untreated oral disease and complications related to invasive procedures. Aim To evaluate the agreement between smartphone‐based asynchronous teledentistry and face‐to‐face examination for oral conditions,
Victor Cordeiro da Silva, Carolina Mendes Frusca do Monte, Leandro Tavares da Silva, Gabriela Silva Almeida, Claudia Santos Lorenzato, José Vitor Nogara Borges de Menezes, Fabian Calixto Fraiz, Cassius Carvalho Torres‐Pereira   +7 more
wiley   +1 more source

Testing for Non‐Severe Heritable Platelet Function Disorders

International Journal of Laboratory Hematology, EarlyView.
ABSTRACT Heritable platelet function disorders (HPFD) are a diverse group of bleeding disorders characterised by a primary qualitative defect in platelet function rather than platelet number. HPFD may be broadly categorised according to the severity of bleeding, with Glanzmann thrombasthenia and Bernard Soulier syndrome classically considered severe ...
Kate Burley, Naomi Cornish, Maria Molina Carretero, Sarah K. Westbury   +3 more
wiley   +1 more source

Is It Time to Raise the Threshold for Critically Low Fibrinogen? Insights From a Retrospective, Consecutive‐Case Cohort Study of Low Fibrinogen due to Various Causes

International Journal of Laboratory Hematology, EarlyView.
ABSTRACT Introduction Fibrinogen deficiency is an important coagulation abnormality, with diverse causes. Methods A consecutive‐case cohort study of adult and pediatric patients with low Clauss fibrinogen at four acute care hospitals was undertaken to explore findings, causes, and predictors of outcomes, including the optimal fibrinogen critical value ...
Natalie Mathews, Subia Tasneem, Mana Al Dawsari, Keona Pang, Yasmeen Abulkhair, Guillame Paré, Siraj Mithoowani, Catherine P. M. Hayward   +7 more
wiley   +1 more source

Allogeneic hematopoietic cell transplantation in an adult patient with Glanzmann thrombasthenia

Clinical Case Reports, 2017
Key Clinical Message Glanzmann thrombasthenia is a rare bleeding disorder that can present life‐threatening bleeding. Our patients develop antiplatelet antibodies that become refractory to any pharmacological treatment. Allogeneic hematopoietic stem‐cell
Ana R. Cid, Pau Montesinos, Isabel Sánchez‐Guiu, Saturnino Haya, Jose I. Lorenzo, Jaime Sanz, Federico Moscardo, Nieves Puig, Dolores Planelles, Santiago Bonanad, Guillermo F. Sanz, Vicente Vicente, Consuelo González‐Manchón, María L. Lozano, José Rivera, Miguel A. Sanz   +15 more
doaj   +1 more source

Long-term treatment with thalidomide for severe recurrent hemorrhage from intestinal angiodysplasia in Glanzmann Thrombasthenia

Platelets, 2021
Gastrointestinal angiodysplasia (GIA) is the most common cause of occult gastrointestinal bleeding (GIB) requiring often hospitalization and transfusions, especially in patients with hemorrhagic disorders.
Francesco Paciullo, Tiziana Fierro, Filippo Calcinaro, Gianfranco Zucca Giucca, Paolo Gresele, Loredana Bury   +5 more
doaj   +1 more source

Functional validation of a modified platelet desialylation test for immune and hereditary thrombocytopenias

Transfusion Medicine, EarlyView.
Abstract Background Thrombocytopenia arises from heterogeneous inherited and acquired disorders, and identifying the underlying platelet clearance mechanisms remains challenging. Platelet desialylation, characterised by loss of sialic acid and consequent exposure of terminal β‐galactose residues recognised by the Ashwell–Morell receptor, represents an ...
Karen Nogueira Chinoca Ziza, Thamy Caroline Silva, Mateus Domentino, Marina Cavalcanti Albuquerque Veiga Conrado, João Vitor Bernardo Oliveira, Felipe Augusto Rós, Caroline Gabe, Elyse Moritz, Akemi Chiba, Paula Villaça, Vanderson Rocha, Alfredo Mendrone‐Junior, José Bordin, Dante Langhi, Carla Luana Dinardo   +14 more
wiley   +1 more source