Sickle Cell Disease Ontology Project

Hemoglobin Barts Hydrops Fetalis

http://scdontology.h3abionet.org/ontology/SCDO_0000518

The most severe form of alpha-thalassemia and is almost always lethal, and characterized by fetal onset of generalized edema, pleural and pericardial effusions, and severe hypochromic anemia. It is caused by the presence of Hemoglobin Barts, a hemoglobin with four gamma chains, which has an extremely high affinity for oxygen, resulting in almost no oxygen delivery to the tissues

Term info

database cross reference
  • Orphanet_163596
curator note

Suggest update to description in ORDO as we have done.

database cross reference

Orphanet_163596

definition source

http://www.orpha.net/ORDO/Orphanet_163596

existence in other ontologies

Suggest update to description

Hb Barts Syndrome, Alpha-Thalassemia Major, Alpha-Thalassemia Hydrops Fetalis, Haemoglobin Barts Hydrops Fetalis, Homozygous Alpha 0-Thalassemia, Hemoglobin Barts Hydrops Fetalis Syndrome, Hemoglobin Bart's Hydrops Fetalis, Hb Barts