Organ-specific hemosiderosis and functional correlation in Chinese patients with thalassemia intermedia and hemoglobin H disease.
We performed MRI assessment in 37 adult Chinese patients with thalassemia intermedia and hemoglobin H disease. Despite abnormal ferritin and liver T2*, only 5% of patients had cardiac hemosiderosis. The two patients with reduced ejection fraction had normal cardiac T2*. Half of the cases showed pituitary and pancreatic iron loading. Subclinical endocrine abnormalities (HOMA, insulin growth factor) showed correlation with pancreatic, pituitary, and cardiac MRI values. Prospective data with serial functional and imaging monitoring is needed to verify the utility for chelation to improve cardiac and endocrine function in this group of patients.
Au
W Y
WY
Department of Medicine, Queen Mary Hospital, Hong Kong, Hong Kong. auwing@hotmail.com
Lam
W W M
WW
Chu
W W C
WW
Tam
S
S
Wong
W K
WK
Lau
J
J
Yeung
Y M
YM
Liu
H S Y
HS
Liang
R
R
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002681:China; D004703:Endocrine System / Q000503:physiopathology; D005260:Female; D006321:Heart / Q000502:physiology; D006486:Hemosiderosis / Q000209:etiology*; D006801:Humans; D008099:Liver / Q000473:pathology; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D009928:Organ Specificity; D010179:Pancreas / Q000473:pathology; D010902:Pituitary Gland / Q000473:pathology; D055815:Young Adult; D017085:alpha-Thalassemia / Q000150:complications*; D017086:beta-Thalassemia / Q000150:complications*