Results 31 to 40 of about 15,382 (226)

The kidneys in paroxysmal nocturnal hemoglobinuria [PDF]

Blood, 1981
Long-term study of 21 PNH patients revealed an unexpectedly high incidence of functional and anatomic renal abnormalities. Most patients demonstrated varying degrees of hematuria and proteinuria distinct from hemoglobinuria. Evaluation of renal function revealed hyposthenuria, abnormal tubular function, and declining creatinine clearance ...
D A, Clark, S A, Butler, V, Braren, R C, Hartmann, D E, Jenkins   +4 more
openaire   +3 more sources

The complement alternative pathway in paroxysmal nocturnal hemoglobinuria: From a pathogenic mechanism to a therapeutic target

Immunological Reviews, 2022
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare clonal, not malignant, hematological disease characterized by intravascular hemolysis, thrombophilia and bone marrow failure.
A. Risitano, C. Frieri, E. Urciuoli, L. Marano   +3 more
semanticscholar   +1 more source

Phase 2 study of danicopan in patients with paroxysmal nocturnal hemoglobinuria with an inadequate response to eculizumab

Blood, 2021
Some patients with paroxysmal nocturnal hemoglobinuria (PNH) remain persistently anemic despite treatment with eculizumab and may have significant extravascular hemolysis.
Austin Kulesekararaj, A. Risitano, J. Maciejewski, R. Notaro, P. Browett, J. W. Lee, Mingjun Huang, M. Geffner, R. Brodsky   +8 more
semanticscholar   +1 more source

Frequency of paroxysmal nocturnal hemoglobinuria in patients attended in Belém, Pará, Brazil

Revista Brasileira de Hematologia e Hemoterapia, 2011
BACKGROUND: Paroxysmal nocturnal hemoglobinuria is a hematological disease with complex physiopathology. It is genetically characterized by a somatic mutation in the PIG-A gene (phosphatidylinositol glycan anchor biosynthesis, class A), in which the best
Lacy Cardoso de Brito Junior, Maria do Socorro de Oliveira Cardoso, Euzamar Gaby Rocha, Herika Anijar, Mariana Cunha, João Carlos Pina Saraiva   +5 more
doaj   +1 more source

Phase II trials of zilucoplan in paroxysmal nocturnal hemoglobinuria

Haematologica, 2023
Not available.
Austin G. Kulasekararaj, Anna-Elina Lehtinen, C. Forsyth, Shreyans Gandhi, M. Griffin, S. Körper, G. Mikala, P. Muus, U. Overgaard, Christopher J. Patriquin, H. Pullon, Yu-min P Shen, R. Spearing, J. Szer, G. de la Borderie, P. Duda, R. Farzaneh-Far, Sharan Ragunathan, C. Sayegh, D. Vadysirisack, H. Schrezenmeier   +20 more
semanticscholar   +1 more source

Clinical and prognostic significance of small paroxysmal nocturnal hemoglobinuria clones in myelodysplastic syndrome and aplastic anemia

Leukemia, 2021
In this large single-centre study, we report high prevalence (25%) of, small (
B. Fattizzo, R. Ireland, A. Dunlop, D. Yallop, S. Kassam, Joanna Large, Shreyans Gandhi, P. Muus, Charles Manogaran, Katy Sanchez, D. Consonni, W. Barcellini, G. Mufti, J. Marsh, A. Kulasekararaj   +14 more
semanticscholar   +1 more source

Loss of expression of neutrophil proteinase-3: a factor contributing to thrombotic risk in paroxysmal nocturnal hemoglobinuria

Haematologica, 2011
Background A deficiency of specific glycosylphosphatidyl inositol-anchored proteins in paroxysmal nocturnal hemoglobinuria may be responsible for most of the clinical features of this disease, but some functional consequences may be indirect. For example,
Anna M. Jankowska, Hadrian Szpurka, Mark Calabro, Sanjay Mohan, Andrew E. Schade, Michael Clemente, Roy L. Silverstein, Jaroslaw P. Maciejewski   +7 more
doaj   +1 more source

Paroxysmal nocturnal hemoglobinuria: advances in the understanding of pathophysiology, diagnosis, and treatment.

Polish Archives of Internal Medicine, 2022
In recent years, "old" paroxysmal nocturnal hemoglobinuria (PNH) has achieved new advances in terms of the understanding of its pathophysiology, modern approach to diagnostics, optimization of therapy, and dynamic development of new therapeutic agents ...
U. Szlendak, B. Budziszewska, Justyna Spychalska, Joanna Drozd-Sokołowska, E. Patkowska, J. Nowak   +5 more
semanticscholar   +1 more source

Paroxysmal nocturnal hemoglobinuria clone in 103 Brazilian patients: diagnosis and classification

Revista Brasileira de Hematologia e Hemoterapia, 2015
Background: Paroxysmal nocturnal hemoglobinuria is an acquired chronic hemolytic ane- mia, which often manifests as peripheral blood cytopenias and thrombosis. Objective: The aim of this study is to describe a Brazilian population of paroxysmal nocturnal
Ana Paula de Azambuja, Mariester Malvezzi, Marco Antonio Bitencourt, Michel Michels Oliveira, Larissa Alessandra Medeiros, Ricardo Pasquini   +5 more
doaj   +1 more source

Inhibition of C3 with pegcetacoplan results in normalization of hemolysis markers in paroxysmal nocturnal hemoglobinuria

Annals of Hematology, 2022
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired hematologic disorder characterized by complement-mediated hemolysis. C5 inhibitors (eculizumab/ravulizumab) control intravascular hemolysis but do not prevent residual extravascular hemolysis.
R. Wong, H. Pullon, Ismail Amine, A. Bogdanović, Pascal Deschatelets, C. Francois, K. Ignatova, S. Issaragrisil, P. Niparuck, T. Numbenjapon, E. Roman, J. Sathar, Raymond Xu, M. Al-Adhami, L. Tan, E. Tse, F. Grossi   +16 more
semanticscholar   +1 more source