Cardiac hemochromatosis: Beneficial effects of iron removal therapy

Candell‐Riera, Jaume; LiZhi, Lu; Serés, Luis; Gonzalez, Joaquin B.; Batlle, Jaime Feliú; Permanyer-Miralda, Gaietà; Garcia-Del-Castillo, H.; Soler‐Soler, Jordi

doi:10.1016/0002-9149(83)90422-8

Cited by 65 publications

(36 citation statements)

References 23 publications

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“…34 Survival and symptomatology have been improved dramatically by iron removal with phlebotomy. 34,36,37 Direct confirmation of the degree of iron overload in hemochromatosis patients is difficult. Endomyocardial biopsies can be obtained to confirm iron deposition.…”

Section: Hemochromatosismentioning

confidence: 99%

“…Candell-Riera et al showed reductions in left ventricular dimensions and improvement in fractional shortening, and ejection fraction in 11 primary hemochromatosis patients who underwent phlebotomy. 36 Dabestani et al, using echocardiography and equilibrium blood pool imaging, reported that a large percentage of patients with baseline diastolic or systolic dysfunction improve after phlebotomy. 37 Not all cases of hemochromatosis respond to the removal of excess iron by phlebotomy.…”

Section: Hemochromatosismentioning

confidence: 99%

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Iron-mediated cardiovascular injury

Horwitz

Rosenthal

1999

Vasc Med

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Abstract:Iron is an essential element for normal cellular function and general health. However, iron may play a pathologic role in certain cardiac conditions including reperfusion injury, hemochromatosis, ␤-thalassemia and coronary atherosclerosis. It also may play a role in injury due to anthracycline cardiotoxicity. Removal of iron via phlebotomy for hemochromatosis and chelation therapy for ␤-thalassemia are proven treatments. Cell culture, and isolated organ and animal studies suggest that depleting iron stores may prevent reperfusion injury, restenosis and even atherogenesis. This article will review mechanisms by which iron overload states and normal iron stores contribute to cardiovascular pathophysiology and the accumulating evidence that iron chelation may prevent restenosis and atherogenesis.

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Section: Hemochromatosismentioning

confidence: 99%

Section: Hemochromatosismentioning

confidence: 99%

Iron-mediated cardiovascular injury

Horwitz

Rosenthal

1999

Vasc Med

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“…6,7 Left ventricular dysfunction, which occurs in 35-60% of patients usually appears later. 8,9 It is said that liver, which is the first organ damaged in idiopathic hemochromatosis, is almost always firm and enlarged, having an average weight of 2,500 g. However, the present patient did not have clinical liver cirrhosis; asparate aminotransferase, alanine aminotransferase and prothrombin time were all normal as well as other pertinent laboratory data. As shown by abdominal ultrasonography, hepatomegaly was present, but the liver surface was smooth, which suggested that there was no clinical liver cirrhosis on admission.…”

Section: Discussionmentioning

confidence: 52%

Cardiac Dysfunction Because of Secondary Hemochromatosis Caused by Congenital Non-Spherocytic Hemolytic Anemia

et al. 2001

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“…[1][2][3][4][5][6][7] There is, however, no emphasis in the literature on the potential of even the most severe HC to respond as well to venesections, and on the advantage of combining ironchelation therapy with small-volume venesections in the initial treatment of such patients who are usually unable to tolerate large-volume venesections.We report two cases of severe hereditary HC that responded remarkably well, and are currently leading a normal life off all cardiac medications, following smallvolume phlebotomies for a combination of two years, in the first three and six months of treatment, with subcutaneous deferoxamine. The first case presented with a cardioembolic stroke, a unique presentation of HC that has not been previously reported.…”

mentioning

confidence: 99%

mentioning

confidence: 99%

Severe Hereditary Hemochromatotic Cardiomyopathy Responsive to Small-Volume Venesections Combined with Deferoxamine

Madani

Bormanis

1996

Ann Saudi Med

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Several reports of patients with mildly to moderately severe hereditary hemochromatotic cardiomyopathy (HC) that responded well to venesections have been published. [1][2][3][4][5][6][7] There is, however, no emphasis in the literature on the potential of even the most severe HC to respond as well to venesections, and on the advantage of combining ironchelation therapy with small-volume venesections in the initial treatment of such patients who are usually unable to tolerate large-volume venesections.We report two cases of severe hereditary HC that responded remarkably well, and are currently leading a normal life off all cardiac medications, following smallvolume phlebotomies for a combination of two years, in the first three and six months of treatment, with subcutaneous deferoxamine. The first case presented with a cardioembolic stroke, a unique presentation of HC that has not been previously reported. Case 1A 36-year-old male engineer presented to the Ottawa Civic Hospital with a three-hour history of confusion, expressive aphasia and right hemiparesis. He had been on erythromycin for a flu-like illness in the preceding five days. He had noticed mild pedal edema during the preceding two months, with no other symptoms of heart failure. The patient had never smoked or drunk alcohol in excess. Three years earlier he had been diagnosed as having isolated hypogonadotrophic hypogonadism, for which he had been receiving monthly injections of testosterone. Four months prior to presentation the patient was diagnosed as having insulin-dependent diabetes mellitus. There was no family history of hemochromatosis.Physical examination revealed a conscious but inattentive patient with aphasia, a tanned bronze clammy skin, a pulse rate of 102 beats/minute with frequent ectopics, a blood pressure of 120/80 mmHg, a respiratory rate of 22 breaths/minute, and a temperature of 36.7°C. He had no stigmata and a jugular venous pressure of 4 cm above the sternal angle. A gallop rhythm with a loud third heart sound was audible on cardiac auscultation, but no heart murmurs. Chest examination revealed bilateral stony dullness with no crepitations. The abdomen was soft with no organomegaly or ascites. The patient had expressive aphasia, right facial droop and right hemiparesis of 4/5 power and an extensor right plantar reflex. Fundal examination was normal.Investigations included hemoglobin 149 g/L, white blood count 9.8x10 9 /L, platelets 230x10 9 /L, serum iron 26 µmol/L (N:5-25), ferritin 5213 µg/L (N:22-447), TIBC 31 µmol/L (N:42-71), transferrin saturation 84% (N:20-45), moderately elevated liver enzymes, normal total proteins, albumin, and prothrombin (PT) and activated thromboplastin (PTT) times, normal T 4 and TSH, negative rheumatoid factor, antinuclear, anti-Sm, anti-RNP, antiLa and anti-Ro antibodies. ECG showed sinus tachycardia, left axis deviation of -30, left atrial enlargement, poor Rwave progression, nonspecific T-waves changes and no Q-waves. Chest x-ray showed bilateral pleural effusion, mild cardiomegaly and no signs ...

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Cardiac hemochromatosis: Beneficial effects of iron removal therapy

Cited by 65 publications

References 23 publications

Iron-mediated cardiovascular injury

Iron-mediated cardiovascular injury

Cardiac Dysfunction Because of Secondary Hemochromatosis Caused by Congenital Non-Spherocytic Hemolytic Anemia

Severe Hereditary Hemochromatotic Cardiomyopathy Responsive to Small-Volume Venesections Combined with Deferoxamine

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