Results 81 to 90 of about 79,367 (221)

Púrpura trombocitopênica imunológica como manifestação inicial de lúpus eritematoso sistêmico juvenil Idiopathic thrombocytopenic purpura as initial manifestation of juvenile systemic lupus erythematosus

open access: yesRevista Brasileira de Reumatologia, 2003
Os pacientes com púrpura trombocitopênica imunológica apresentam risco aumentado para desenvolver lúpus eritematoso sistêmico, principalmente quando a doença evolui de forma crônica. Alguns autores observaram que o sexo feminino, a idade mais avançada, a
Josefina Aparecida Pellegrini Braga   +3 more
doaj   +1 more source

A Novel Homozygous GFI1B Mutation in Siblings With Thrombocytopenia and Bleeding Tendency

open access: yesClinical Case Reports, Volume 13, Issue 10, October 2025.
Pedigree chart showing affected siblings. ABSTRACT Platelet type bleeding disorder 17 (OMIM #187900) is a type of “gray platelet syndrome” and occurs due to a mutation in the GFI1B gene on chromosome 9q34.13. Patients usually present with a history of easy bleeding tendencies, recurrent epistaxis or gum bleeding, and rarely with severe hemorrhage, and ...
Shova Aryal   +3 more
wiley   +1 more source

Living under the sword of Damocles”: a psychological support project for patients with immune thrombocytopenia and their caregivers

open access: yesBleeding, Thrombosis and Vascular Biology
Immune thrombocytopenia (ITP) is an autoimmune disease that causes a drop in platelet count
Giuseppe Auteri   +3 more
doaj   +1 more source

Residues Arg568 and Phe592 contribute to an antigenic surface for anti-ADAMTS13 antibodies in the spacer domain

open access: yesHaematologica, 2011
Background The majority of patients diagnosed with thrombotic thrombocytopenic purpura have autoantibodies directed towards the spacer domain of ADAMTS13.Design and Methods In this study we explored the epitope specificity and immunoglobulin class and ...
Wouter Pos   +6 more
doaj   +1 more source

Management of Gaucher Disease Type 1 in a Resource‐Limited Setting: A Pediatric Case Study

open access: yesClinical Case Reports, Volume 13, Issue 10, October 2025.
ABSTRACT This case report depicts the management of an 8‐year‐old male with Gaucher Disease Type 1, manifesting as massive splenomegaly, anemia, and skeletal involvement in a resource‐constrained environment. Treated with splenectomy due to the absence of enzyme replacement therapy, it underscores the necessity for enhanced therapeutic access and ...
Bipesh Kumar Shah   +4 more
wiley   +1 more source

Use of PopPK and E‐R Analyses toward Explaining Causal Link Between ADAMTS13 in Recombinant vs. Plasma‐Based Therapies and Clinical Effects in cTTP

open access: yesClinical Pharmacology &Therapeutics, Volume 118, Issue 4, Page 813-822, October 2025.
Congenital thrombotic thrombocytopenic purpura (cTTP) is an ultra‐rare, potentially life‐threatening condition caused by a deficiency of the blood enzyme ADAMTS13. Until now, ADAMTS13 replacement has been achieved with infusions of plasma or plasma‐based therapies (PBT).
Munjal Patel   +11 more
wiley   +1 more source

Diagnosis, Treatment Patterns and Eradication Success for Helicobacter pylori Infections in China: A Retrospective Observational Real‐World Study

open access: yesJGH Open, Volume 9, Issue 10, October 2025.
ABSTRACT Background and Aim In Mainland China, Helicobacter pylori infection is prevalent in about 50% of adults. However, there is limited real‐world evidence on its diagnosis and treatment patterns. This study investigated diagnosis, treatment patterns, and effectiveness of eradication treatment in patients with Helicobacter pylori infections in ...
Changqin Xu   +6 more
wiley   +1 more source

Quantitative Systems Pharmacology Modeling of Platelet Responses to Recombinant ADAMTS13 in Patients With Congenital Thrombotic Thrombocytopenic Purpura

open access: yesCPT: Pharmacometrics &Systems Pharmacology, Volume 14, Issue 10, Page 1575-1586, October 2025.
ABSTRACT Congenital thrombotic thrombocytopenic purpura (cTTP) is an ultra‐rare, life‐threatening thrombotic microangiopathy caused by a severe inherited deficiency of ADAMTS13, a von Willebrand factor (VWF) cleaving enzyme. Inadequate clinical endpoint data often make it challenging to statistically power clinical trials in ultra‐rare diseases ...
Cameron McBride   +8 more
wiley   +1 more source

Diffuse Alveolar Haemorrhage Complicated by Pulmonary Metastasis of Cardiac Angiosarcoma and Kasabach–Merritt Syndrome: A Case Report

open access: yesRespirology Case Reports, Volume 13, Issue 10, October 2025.
We report an exceptional case of cardiac angiosarcoma in which diffuse alveolar haemorrhage was caused by both pulmonary metastases and Kasabach–Merritt syndrome. Strategies to achieve transfusion‐independent haemostasis such as high PEEP may create an opportunity for prompt biopsy and facilitate timely initiation of definitive treatment.
Takuma Ikeda, Satoru Terada, Kazuo Endo
wiley   +1 more source

Improvement of thrombocytopenia after treatment for Helicobacter pylori in a patient with immunologic thrombocytopenic purpura

open access: yesRevista Brasileira de Hematologia e Hemoterapia, 2014
Immune thrombocytopenic purpura is the most common autoimmune hematologic disease, affecting individuals of different ages. Recently, the bacterium Helicobacter pylori entered the list of causes of immune thrombocytopenic purpura.
Aline Maia Rocha   +2 more
doaj   +1 more source

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