Results 161 to 170 of about 8,082 (209)

Paroxysmal nocturnal hemoglobinuria

Hématologie, 2013
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disorder of hematopoietic stem cells. PNH is related to a somatic mutation in the phosphatidylinositol glycan class A (PIG-A), X-linked gene, responsible for a deficiency in glycosylphosphatidylinositol-anchored proteins (GPI-AP).
R, Peffault de Latour, Z, Amoura, G, Socié   +2 more
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Paroxysmal Nocturnal Hemoglobinuria

Journal of Computer Assisted Tomography, 1993
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic disorder, characterized by hemosiderin deposition in the proximal renal tubules.We examined six cases of PNH with MRI including gradient echo imaging.On T2- and T2*-weighted imaging a characteristic hypointense pattern was noted in five of six patients with PNH.
Y O, Tanaka, I, Anno, Y, Itai, T, Abe
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The syndrome of paroxysmal nocturnal hemoglobinuria

The American Journal of Medicine, 1964
Abstract The case histories and investigations are presented of four patients with paroxysmal nocturnal hemoglobinuria who satisfy the diagnostic criteria of abnormal erythrocyte acid hemolysis, positive intravascular hemolysis and low erythrocyte acetylcholinesterase. The clinical presentations of these patients ranged from a young man with a classic
R W, BEAL, H, KRONENBERG, B G, FIRKIN
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Paroxysmal nocturnal hemoglobinuria in pregnancy

Acta Obstetricia et Gynecologica Scandinavica, 2003
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.
Line, Bjørge, Peter, Ernst, Kjell O, Haram   +2 more
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Thrombosis in paroxysmal nocturnal hemoglobinuria

Blood, 2013
Abstract The most frequent and feared complication of paroxysmal nocturnal hemoglobinuria (PNH) is thrombosis. Recent research has demonstrated that the complement and coagulation systems are closely integrated with each influencing the activity of the other to the extent that thrombin itself has recently been shown to activate the ...
Anita, Hill, Richard J, Kelly, Peter, Hillmen   +2 more
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Paroxysmal Nocturnal Hemoglobinuria

Mayo Clinic Proceedings
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired clonal hematologic disorder characterized by intravascular destruction of red blood cells by the complement system. This intravascular hemolysis can lead to a constellation of symptoms, including anemia, fatigue, shortness of breath, smooth muscle dystonia, and dark urine that is classically
Richard C. Godby, Surbhi Shah
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Pregnancy and Paroxysmal Nocturnal Hemoglobinuria

Archives of Internal Medicine, 1988
Our study concerns eight pregnancies, six of which were successful, in four patients with paroxysmal nocturnal hemoglobinuria (PNH). Several complications of PNH during pregnancy were prevented: chronic anemia, folate and iron deficiency, and deep-vein thrombosis.
P, Solal-Céligny, G, Tertian, H, Fernandez, J C, Pons, T, Lambert, Y, Najean, J P, Clauvel, E, Papiernik, G, Tchernia   +8 more
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Paroxysmal Nocturnal Hemoglobinuria

New England Journal of Medicine, 1961
PAROXYSMAL 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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Paroxysmal Nocturnal Hemoglobinuria Revisited

New England Journal of Medicine, 1983
Immunologic 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

1992
Paroxysmal 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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