Disease Information for Hemoglobin Bart hydrops fetalis syndr.

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Clinical Manifestations
Signs & Symptoms
Neonatal edema/anasarca
Demographics & Risk Factors
Population Group
Child
Population/Pediatrics population
Family History
Family history/Anemia
Sex & Age Groups
Population/Child
Population/Child-Infant Only
Population/Children/all
Laboratory Tests
Abnormal Lab Findings (Non Measured)
Fast-migrating hemoglobin/electrophoresis (Lab)
Abnormal Lab Findings - Increased
Bilirubin, serum (Lab)
Indirect bilirubin (Lab)
MCV/RBC Ratio (Mentzer Index)
Diagnostic Test Results
Other Tests & Procedures
Amniocentesis/Abnormality
Associated Diseases & Rule outs
Associated Disease & Complications
Bart hemoglobin/fetal incompatibility
Bilirubinemia
Intrauterine death/fetus
Neonatal hyperbilirubinemia
Disease Mechanism & Classification
Class
CLASS/Pediatric disorders (ex)
CLASS/Fetal/conceptual products (category)
CLASS/Fetus disorder (ex)
CLASS/Hematologic (category)
Pathophysiology
Pathophysiology/Isoantibody/preformed alloantibodies/incr
Process
PROCESS/Allergy/collagen/autoimmune (category)
PROCESS/Hemoglobinopathy disorder (ex)
Synonyms
Synonym
Haemoglobin Barts present, Hemoglobin Barts present, Synonym/Alpha thalassemia hydrops/Bart (homozygous)
Definition

The loss of all four alpha genes produces a condition that is incompatible with life. The gamma chains produced during fetal life (see Hemoglobin Overview) associate in groups of four to form an abnormal hemoglobin called "hemoglobin Barts"; Most people with four-gene deletion alpha thalassemia die in utero or shortly after birth; Rarely, four gene deletion alpha thalassemia has been detected in utero, usually in a family where the disorder occured in an earlier child; In utero blood transfusions have saved some of these children; These patients require life-long transfusions and other medical support

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External Links Related to Hemoglobin Bart hydrops fetalis syndr.
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Wikipedia
Merck
Images
PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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