Severe pulmonary artery hypertension

1988 ◽  
Vol 14 (5) ◽  
pp. 510-518 ◽  
Author(s):  
A. Roglan ◽  
A. Artigas ◽  
J. Solé
Keyword(s):  
Pulmonary Artery ◽  
Pulmonary Artery Hypertension

scholarly journals Pulmonary artery hypertension prior to the relapse of adult‐onset Still's disease

2021 ◽  
Vol 9 (5) ◽  
Author(s):  
Yuki Hara ◽  
Takayoshi Morita ◽  
Katsunao Tanaka ◽  
Fusako Sera ◽  
Yasushi Sakata ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Pulmonary Artery Hypertension ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Adult Onset Still's Disease

scholarly journals Clinical improvement by farnesyltransferase inhibition in NK large granular lymphocyte leukemia associated with imbalanced NK receptor signaling

Blood ◽  
2008 ◽  
Vol 112 (12) ◽  
pp. 4694-4698 ◽  
Author(s):  
P. K. Epling-Burnette ◽  
Lubomir Sokol ◽  
Xianhong Chen ◽  
Fanqi Bai ◽  
Junmin Zhou ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Clinical Manifestations ◽  
Erythroid Differentiation ◽  
Pulmonary Artery Hypertension ◽  
Large Granular Lymphocyte ◽  
Receptor Signaling ◽  
Pulmonary Endothelial Cells ◽  
First Case ◽  
Nk Receptor ◽  
Lgl Leukemia

Abstract Large granular lymphocyte (LGL) leukemia is commonly associated with poor hematopoiesis. The first case of pulmonary artery hypertension (PAH) was observed in a 57-year-old woman with natural killer (NK)–LGL leukemia and transfusion-dependent anemia. Using a genetic approach, we demonstrated that killing of pulmonary endothelial cells by patient NK cells was mediated by dysregulated balance in activating and inhibitory NK-receptor signaling. Elevated pulmonary artery pressure and erythroid differentiation improved after disrupting the NK-receptor signaling pathway with 4 courses of a farnesyltransferase inhibitor, tipifarnib. Coincidental association between PAH and LGL leukemia suggest a causal relationship between the expanded lymphocyte population and these clinical manifestations. This trial is registered at www.ClinicalTrials.gov as NCI 6823.

Rapidly progressive pulmonary artery hypertension and end-stage liver disease

1996 ◽  
Vol 40 (1) ◽  
pp. 126-129 ◽  
Author(s):  
E. Mortier ◽  
M. Ongenae ◽  
J. Poelaert ◽  
N. den Blauwen ◽  
J. Decruyenaere ◽  
...  
Keyword(s):  
Liver Disease ◽  
Pulmonary Artery ◽  
Pulmonary Artery Hypertension ◽  
End Stage Liver Disease ◽  
End Stage
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