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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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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
JAMA, 1973A 66-year-old white man with a previously normal hematological history was studied for hemolysis during the recovery phase of acute tubular necrosis. He demonstrated a persistently worsening anemia in the absence of blood loss and had an increase of his serum-free hemoglobin. A hemolytic process consistent with paroxysmal nocturnal hemoglobinuria (PNH)
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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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Paroxysmal Nocturnal Hemoglobinuria
A.M.A. Archives of Internal Medicine, 1957Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hemolytic disease of insidious onset and chronic course, characterized by an intracorpuscular erythrocytic defect rendering the cells unusually susceptible to hemolysis in vitro and probably in vivo upon slight acidification of the plasma. The constant feature of the disease is hemosiderinuria, rather
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Managing Fatigue in Patients with Paroxysmal Nocturnal Hemoglobinuria: A Patient-Focused Perspective
Journal of Blood Medicine, 2022Bruno Fattizzo +2 more
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[Paroxysmal nocturnal hemoglobinuria].
La Revue du praticien, 2007Paroxysmal nocturnal haemoglobinuria is a rare clonal disease of the hematopoietic stem cell. Since the 80's considerable advances in the biology of this rare disease have been done. The pathophysiology, recent advances in the diagnosis and treatment of this disease are reviewed, pointing out, however, the yet unanswered question with regard to the ...
Régis, Peffault de Latour +1 more
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Safety of current treatments for paroxysmal nocturnal hemoglobinuria
Expert Opinion on Drug Safety, 2021Seung-Hwan Lee, Jong Wook Lee
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