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[Paroxysmal nocturnal hemoglobinuria].
Orvosi hetilap, 2002The current knowledge of the history, clinical course, diagnosis and treatment of Paroxysmal Nocturnal Hemoglobinuria (PNH) are summarized in this article.
Gyula, Reményi +3 more
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The pathophysiology of paroxysmal nocturnal hemoglobinuria
Experimental Hematology, 2007The molecular basis of PNH is known. Somatic mutation of the X-chromosome gene PIGA accounts for deficiency of glycosyl phosphatidylinositol-anchored proteins (GPI-AP) on affected hematopoietic stem cells and their progeny. However, neither mutant PIGA nor the consequent deficiency of GPI-AP provides a direct explanation for the clonal outgrowth of the
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Paroxysmal Nocturnal Hemoglobinuria
New England Journal of Medicine, 1952THE most accurate method of study of any disease is enlightened statistical analysis of a large number of cases. However, paroxysmal nocturnal hemoglobinuria is a rare blood dyscrasia; approximately 45 cases, not all of convincing authenticity, have appeared in the literature to this time.9 Consequently, no physician or group of physicians is likely to
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Paroxysmal Nocturnal Hemoglobinuria
1992Paroxysmal nocturnal hemoglobinuria (PNH) is a clonal disorder of the hematopoietic stem cell (Hartmann and Arnold 1977) resulting in the production of blood cells which are defective in that they lack or are markedly deficient in glycan-phosphatidylinositol (GPI)-linked surface proteins (Rosse 1990a).
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Paroxysmal Nocturnal Hemoglobinuria Revisited
New England Journal of Medicine, 1983Immunologic systems are now known to have a major role in the pathophysiology of many human disease processes. One such system is serum complement.
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Paroxysmal Nocturnal Hemoglobinuria
New England Journal of Medicine, 1961PAROXYSMAL nocturnal hemoglobinuria is a disease with a fascinating diversity of challenges and implications. These have prompted numerous clinical and basic investigations. The clinical features are often bizarre and may be misleading, even with the most typical presentation of abdominal pain, fatigue, weakness, anemia and dark urine and with venous ...
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Eculizumab in Paroxysmal Nocturnal Hemoglobinuria
New England Journal of Medicine, 2006To the Editor: The study of eculizumab by Hillmen et al. (Sept. 21 issue)1 showed stabilization of hemoglobin levels and a reduction in transfusion requirements in patients with paroxysmal nocturnal hemoglobinuria (PNH). However, there were more patients with a history of aplastic anemia in the placebo group than in the eculizumab group (27% vs.
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Paroxysmal Nocturnal Hemoglobinuria
2007Paroxysmal nocturnal hemoglobinuria (PNH) is a complex hematological disorder probably first described three centuries ago, and regarded as a mystery until the 1980s, when most of its pathophysiology was elucidated, followed by the 1990s, when the underlying molecular defect was finally unraveled.
Bruno Rotoli +2 more
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[Paroxysmal nocturnal hemoglobinuria].
Klinische Wochenschrift, 1990Paroxysmal nocturnal hemoglobinuria, first described in the late 19th century, is an acquired disorder characterized by hemoglobinemia and hemoglobinuria. The major clinical manifestation of PNH is chronic intravascular hemolysis of various severity. Patients-mostly young adults - may also present with episodes of abdominal or back pain.
P, Blaas +3 more
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Managing Fatigue in Patients with Paroxysmal Nocturnal Hemoglobinuria: A Patient-Focused Perspective
Journal of Blood Medicine, 2022Esther Natalie Oliva +2 more
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