نتایج جستجو برای: microthrombocytopenia

تعداد نتایج: 24  

2015
Paula Danielle Santa Maria De Albuquerque Juliana Letícia Poli Maria Eduarda Pontes Cunha De Castro Persio Roxo Jr

Background Wiskott–Aldrich syndrome (WAS) is a rare X-linked recessive disorder characterized by early microthrombocytopenia, eczematous skin disease and recurrent infections. The syndrome is caused by mutations in gene WAS which codes WASP protein, that is expressed selectively in hematopoietic cells and it is involved in cell signaling and cytoskeleton reorganization. Microthrombocytopenia is...

2014
Markus Bender Simon Stritt Paquita Nurden Judith M.M. van Eeuwijk Barbara Zieger Karim Kentouche Harald Schulze Henner Morbach David Stegner Katrin G. Heinze Sebastian Dütting Shuchi Gupta Walter Witke Hervé Falet Alain Fischer John H. Hartwig Bernhard Nieswandt

Wiskott-Aldrich syndrome (WAS) is caused by mutations in the WAS gene and is characterized by immunodeficiency, eczema and microthrombocytopenia. The molecular link between WAS mutations and microthrombocytopenia is unknown. Profilin1 (Pfn1) is a key actin-regulating protein that, besides actin, interacts with phosphoinositides and multiple proline-rich proteins, including the WAS protein (WASp...

Journal: :Rare diseases 2014
Christian Joerg Braun Maximilian Witzel Anna Paruzynski Kaan Boztug Christof von Kalle Manfred Schmidt Christoph Klein

Wiskott-Aldrich-Syndrome (WAS) is a rare X-linked recessive disease caused by mutations of the WAS gene. It is characterized by immunodeficiency, autoimmunity, low numbers of small platelets (microthrombocytopenia) and a high risk of cancer, especially B cell lymphoma and leukemia.

Journal: :international journal of pediatrics 0
kamleshun ramphul department of pediatrics, shanghai xin hua hospital affiliated to the shanghai jiao tong university school of medicine, china. sunjaye ramjuttun department of pediatrics, sir seewoosagur ramgoolam national hospital, mauritius, china. vinita poorun department of pediatrics, sir seewoosagur ramgoolam national hospital, mauritius, china.

wiskott-aldrich is an x-lined recessive disorder typically characterized by thrombocytopenia, eczema and recurrent infections. we report the four year treatment progress of a six year old boy who initially presented with vesicular lesions over the trunk, upper and lower extremities and face and blood tinged stools at the age of 2 weeks. from the family pedigree, there were two suspected cases t...

Journal: :Blood 2015
Anja J Gerrits Emily A Leven Andrew L Frelinger Sophie L Brigstocke Michelle A Berny-Lang W Beau Mitchell Shoshana Revel-Vilk Hannah Tamary Sabrina L Carmichael Marc R Barnard Alan D Michelson James B Bussel

UNLABELLED Because Wiskott-Aldrich syndrome (WAS) and X-linked thrombocytopenia (XLT) patients have microthrombocytopenia, hemorrhage is a major problem. We asked whether eltrombopag, a thrombopoietic agent, would increase platelet counts, improve platelet activation, and/or reduce bleeding in WAS/XLT patients. In 9 WAS/XLT patients and 8 age-matched healthy controls, platelet activation was as...

Journal: :Thrombosis and haemostasis 2015
Praewphan Ingrungruanglert Pramuk Amarinthnukrowh Ruttachuk Rungsiwiwut Supang Maneesri-le Grand Darintr Sosothikul Kanya Suphapeetiporn Nipan Israsena Vorasuk Shotelersuk

Wiskott-Aldrich syndrome (WAS) is an X-linked recessive disorder characterised by microthrombocytopenia, complex immunodeficiency, autoimmunity, and haematologic malignancies. It is caused by mutations in the gene encoding WAS protein (WASP), a regulator of actin cytoskeleton and chromatin structure in various blood cell lineages. The molecular mechanisms underlying microthrombocytopenia caused...

2016
Mohd Farid Baharin Jasbir Singh Dhaliwal Smrdhi V. V. Sarachandran Siti Zaharah Idris Seoh Leng Yeoh

BACKGROUND Wiskott-Aldrich syndrome is a rare X-linked disorder characterized by microthrombocytopenia, eczema, and recurrent infections. It is caused by mutations of the WAS gene. Microthrombocytopenia has been regarded as the key criteria in diagnosing this rare condition. However, in this case report, we describe a case of Wiskott-Aldrich syndrome with normal platelet size. CASE PRESENTATI...

Journal: :The Malaysian journal of pathology 2015
Mohd Farid Baharin Sabeera Begum Kader Ibrahim Song Hong Yap Aina Mariana Abdul Manaf Adiratna Mat Ripen Jasbir Singh Dhaliwal

The Wiskott-Aldrich Syndrome (WAS) is an X-linked immunodeficiency condition characterized by microthrombocytopenia, eczema and recurrent infections. It is caused by mutations in the Wiskott-Aldrich Syndrome protein (WASP) gene. We investigated two Malay boys who presented with congenital thrombocytopenia, eczema and recurrent infections. Here we report two cases of WASP mutation in Malaysia fr...

Journal: :Indian pediatrics 2014
Dejan Skoric Aleksandar Dimitrijevic Goran Cuturilo Petar Ivanovski

BACKGROUND Wiskott-Aldrich syndrome is a rare X-linked immunodeficiency disorder with a variable phenotype. CASE CHARACTERISTICS 3.5-year-old boy diagnosed with Wiskott-Aldrich syndrome. OBSERVATION Unusual and persistent thrombocytopenia with increased platelet volume (>10fL). He did not exhibit characteristic clinical and laboratory finding for the syndrome. OUTCOME Maternally inherited...

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