نتایج جستجو برای: pnh

تعداد نتایج: 752  

Journal: :The Plant cell 2002
Karyn Lynn Newman Anita G Fernandez M Kathryn Barton

Plants produce proximal-distal growth axes with two types of growth potential: they can be indeterminate, in which case growth continues indefinitely, or they can be determinate, in which case growth is limited to the production of a single organ or a discrete set of organs. The indeterminate shoot axes of Arabidopsis pinhead/zwille mutants frequently are transformed to a determinate state. PIN...

Journal: :Blood 1991
K M Josten J A Tooze C Borthwick-Clarke E C Gordon-Smith T R Rutherford

We used X-chromosome methylation patterns to study clonality in aplastic anemia (AA) and paroxysmal nocturnal hemoglobinuria (PNH). AA is usually not considered to be a clonal stem cell disorder, although this has not been directly investigated. PNH is generally assumed to be a clonal disorder, although there is contradictory evidence. Methylation analysis was performed on DNA from separated gr...

Journal: :Acta obstetricia et gynecologica Scandinavica 2003
Line Bjørge Peter Ernst Kjell O Haram

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic anemia in which a defect of glycophosphatidylinositol (GPI)-anchored proteins in the cell membrane of bone marrow stem cells leads to increased sensitivity of the red cells to complement, causing intravascular hemolysis and hemoglobinuria. Other clinical features of this disease are cytopenia and an increased frequency of thromb...

Journal: :The New England journal of medicine 2004
Peter Hillmen Claire Hall Judith C W Marsh Modupe Elebute Michael P Bombara Beth E Petro Matthew J Cullen Stephen J Richards Scott A Rollins Christopher F Mojcik Russell P Rother

BACKGROUND Paroxysmal nocturnal hemoglobinuria (PNH) arises from a somatic mutation of the PIG-A gene in a hematopoietic stem cell and the subsequent production of blood cells with a deficiency of surface proteins that protect the cells against attack by the complement system. We tested the clinical efficacy of eculizumab, a humanized antibody that inhibits the activation of terminal complement...

Journal: :Blood 2003
Yousef Mortazavi Bruno Merk Jenny McIntosh Judith C W Marsh Hubert Schrezenmeier Tim R Rutherford

Paroxysmal nocturnal hemoglobinuria (PNH) may arise during long-term follow- up of aplastic anemia (AA), and many AA patients have minor glycosylphosphatidylinositol (GPI) anchor-deficient clones, even at presentation. PIG-A gene mutations in AA/PNH and hemolytic PNH are thought to be similar, but studies on AA/PNH have been limited to individual cases and a few small series. We have studied a ...

Journal: :Medicine 2004
Jun-Ichi Nishimura Yuzuru Kanakura Russell E Ware Tsutomu Shichishima Hideki Nakakuma Haruhiko Ninomiya Carlos M Decastro Sharon Hall Akihisa Kanamaru Keith M Sullivan Hideaki Mizoguchi Mitsuhiro Omine Taroh Kinoshita Wendell F Rosse

: To determine and directly compare the clinical course of white and Asian patients with paroxysmal nocturnal hemoglobinuria (PNH), data were collected for epidemiologic analysis on 176 patients from Duke University and 209 patients from Japan. White patients were younger with significantly more classical symptoms of PNH including thrombosis, hemoglobinuria, and infection, while Asian patients ...

2012
C Sugimori E Padron G Caceres K Shain L Sokol L Zhang R Tiu C L O'Keefe M Afable M Clemente J M Lee J P Maciejewski A F List P K Epling-Burnette D J Araten

Paroxysmal nocturnal hemoglobinuria (PNH) is a hemolytic and pro-thrombotic disorder associated with the clonal expansion of hematopoietic stem cells harboring somatic mutations in the PIG-A gene. Mutations in PIG-A result in a lack of surface expression of all glycosylphosphatidylinositol (GPI)-anchored proteins, including the complement inhibitors CD55 and CD59, which is responsible for the h...

Journal: :Tidsskrift for den Norske laegeforening : tidsskrift for praktisk medicin, ny raekke 2015
Lise Sofie H Nissen-Meyer Geir E Tjønnfjord Elzbieta Golebiowska Jens Kjeldsen-Kragh Çiğdem Akalın Akkök

BACKGROUND Paroxysmal nocturnal haemoglobinuria (PNH) is a rare haematological disease characterised by chronic haemolysis, pancytopenia and venous thrombosis. The condition is attributable to a lack of control of complement attack on erythrocytes, thrombocytes and leukocytes, and can be diagnosed by means of flow cytometry. In this quality assurance study, we have reviewed information from the...

Journal: :The Journal of clinical investigation 1997
V Rosti G Tremml V Soares P P Pandolfi L Luzzatto M Bessler

Paroxysmal nocturnal hemoglobinuria (PNH) develops in patients who have had a somatic mutation in the X-linked PIG-A gene in a hematopoietic stem cell; as a result, a proportion of blood cells are deficient in all glycosyl phosphatidylinositol (GPI)-anchored proteins. Although the PIG-A mutation explains the phenotype of PNH cells, the mechanism enabling the PNH stem cell to expand is not clear...

Journal: :Blood 2002
Kentaro Horikawa Tatsuya Kawaguchi Sonoko Ishihara Shoichi Nagakura Michihiro Hidaka Tadashi Kagimoto Hiroaki Mitsuya Hideki Nakakuma

Acquired mutations of the PIG-A gene result in the hemolysis characteristic of paroxysmal nocturnal hemoglobinuria (PNH). Although the etiology of the mutation(s) is unclear, mutable conditions have been suggested by the coexistence of multiple clones with different mutations of PIG-A and by the appearance of leukemic clones in patients with PNH. This study sought to test this hypothesis by exa...

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