Late Cardiac Complications of Chronic, Severe, Refractory Anemia with Hemochromatosis.
نویسندگان
چکیده
A MONG patients wlho from infancy have had severe anemia, refractory to therapy and usually reqtiiring transfuisions, xve have observed the development of one or 1)0th of txxo cardiac complicationis uinuisuial for anicmia alone." One, acute pericarditis, appeared benign but often recutrred, xxhereas the seconid, congestive lheart failuire and arrhythmias, led to death xithin a few montlhs to a fecx years. These coomiplicationis coincided witlb signs of progressive massivxe overloacl of iron. WVe have observed the developruent of cardiac inivolvement an.d acquiiired hemochrmatosis in 41 patients, 39 xvith thalassemila major anid txxo xxith aregenerative aiteinia. Nineteen of the 41 patients had percarditis, 26 had congestive cardiac failuire, anid 10 patients suffered from both. Six of the 41 developed cardiac enlargement and an alnormal electrocardiogram; fou-ir of these died of sepsis before either pericarditis or congestive failulre xvas manifest. Only eight patients xho have passed the age of 8 years and have a sima-ilar anermia are thuis far freec fr-om. sigIIs of cardiac inivolvemenIt. There xvas
منابع مشابه
The role of hepcidin in iron metabolism.
Hepcidin is the central regulator of systemic iron homeostasis. Dysregulation of hepcidin production results in a variety of iron disorders. Hepcidin deficiency is the cause of iron overload in hereditary hemochromatosis, iron-loading anemias, and hepatitis C. Hepcidin excess is associated with anemia of inflammation, chronic kidney disease and iron-refractory iron deficiency anemia. Diagnostic...
متن کاملAssociation of HLA-linked hemochromatosis with idiopathic refractory sideroblastic anemia.
Five of seven patients with idiopathic refractory sideroblastic anemia carried an HLA-A3 alloantigen (relative risk, 7.3; P = 0.02). The significance of this association was strengthened by study of two pedigrees. An abnormality in iron metabolism was found in two siblings who had an HLA-A3,B14 haplotype in common with the first proband. A second proband with idiopathic refractory sideroblastic...
متن کاملThe hepcidin-ferroportin system as a therapeutic target in anemias and iron overload disorders.
The review summarizes the current understanding of the role of hepcidin and ferroportin in normal iron homeostasis and its disorders. The various approaches to therapeutic targeting of hepcidin and ferroportin in iron-overload disorders (mainly hereditary hemochromatosis and β-thalassemia) and iron-restrictive anemias (anemias associated with infections, inflammatory disorders, and certain mali...
متن کاملSevere Anemia and Helicobacter Pylori Infection in school age Children; A case reports
Abstract Background Iron-deficiency anemia is a widespread public health problem with major consequences for human health especially, children. However, in a fraction of patients an underlying cause is never found during routine investigation. Recent studies have suggested an association between Helicobacter pylori (H. Pylori) infection and iron-deficiency ...
متن کاملFour new mutations in the erythroid-specific 5-aminolevulinate synthase (ALAS2) gene causing X-linked sideroblastic anemia: increased pyridoxine responsiveness after removal of iron overload by phlebotomy and coinheritance of hereditary hemochromatosis.
X-linked sideroblastic anemia (XLSA) in four unrelated male probands was caused by missense mutations in the erythroid-specific 5-aminolevulinate synthase gene (ALAS2). All were new mutations: T647C, C1283T, G1395A, and C1406T predicting amino acid substitutions Y199H, R411C, R448Q, and R452C. All probands were clinically pyridoxine-responsive. The mutation Y199H was shown to be the first de no...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- Circulation
دوره 30 شماره
صفحات -
تاریخ انتشار 1964