Document Detail


Pulmonary hypertension in a case of Hb-Mainz hemolytic anemia.
MedLine Citation:
PMID:  17356397     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The development of pulmonary arterial hypertension (PAH) is the leading cause of mortality in patients with thalassemia and sickle cell anemia and was reported to occur in hemolytic anemias such as hereditary stomatocytosis, and paroxysmal nocturnal hemoglobinuria. Here, we report for the first time on the development of PAH in a patient with Hb-Mainz hemolytic anemia. Hb-Mainz is an unstable hemoglobin variant resulting from mutations at codon 98 of the beta chain gene (Val>Glu) characterized by severe chronic hemolytic anemia. The development of PAH in this patient further supports the contention that there is a clinical syndrome of hemolysis-associated development of PAH.
Authors:
Holger N Lode; Gregor Krings; Ingram Schulze-Neick; Steffen Dähmlow; Ulrike Schroeder; Ragnhild Bonnet; Joao DaPalma; Werner Luck; Gabriele Strauss; Felix Berger; Gerhard Gaedicke
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of pediatric hematology/oncology     Volume:  29     ISSN:  1077-4114     ISO Abbreviation:  J. Pediatr. Hematol. Oncol.     Publication Date:  2007 Mar 
Date Detail:
Created Date:  2007-03-14     Completed Date:  2007-09-13     Revised Date:  2011-10-06    
Medline Journal Info:
Nlm Unique ID:  9505928     Medline TA:  J Pediatr Hematol Oncol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  173-7     Citation Subset:  IM    
Affiliation:
Department of Pediatrics, Charité Universitätsmedizin Berlin, Berlin, Germany. holger.lode@charite.de
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MeSH Terms
Descriptor/Qualifier:
Adult
Anemia, Hemolytic / complications*,  genetics,  therapy
Blood Transfusion / methods
Chronic Disease
Follow-Up Studies
Hemoglobins, Abnormal / analysis*,  genetics
Humans
Hydroxyurea / therapeutic use
Hypertension, Pulmonary / complications*,  diagnosis,  therapy
Male
Sensitivity and Specificity
Splenectomy
Tomography, X-Ray Computed / methods
Treatment Outcome
Chemical
Reg. No./Substance:
0/Hemoglobins, Abnormal; 127-07-1/Hydroxyurea

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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