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At least 1,747 records · Page 97Linked to original sources
PubMed2000

Pulmonary haemosiderosis in Saudi children: report of two cases and review of the literature.

Two children with idiopathic pulmonary haemosiderosis are reported. These are the first cases of this disease from Saudi Arabia and highlight the diagnostic problem caused by their presentation as cases of iron deficiency anaemia. The aetiopathogenesis, clinical presentation, diagnosis and management are discussed. The attention of paediatricians is drawn to this relatively rare cause of iron deficiency anaemia.

Anemia↗
PubMed2006

Low serum ferritin levels are misleading for detecting cardiac iron overload and increase the risk of cardiomyopathy in thalassemia patients. The importance of cardiac iron overload monitoring using magnetic resonance imaging T2 and T2*.

The incidence of cardiomyopathy was monitored in a 6-year follow-up study involving 56 transfused thalassemia patients treated with deferoxamine (DFO), deferiprone (L1) or their combination. During this period, five female patients on regular subcutaneous or intravenous DFO presented with cardiac complications. Three patients suffered congestive heart failure and the other two arrhythmias. Four of the five patients maintained serum ferritin levels of about 1 mg/L or below and the fifth about 1.5 mg/L for several years prior to the cardiomyopathy. Cardiac magnetic resonance imaging (MRI) T2* and T2 was performed in four patients after the cardiomyopathy, identifying the presence of moderate-to-heavy siderosis. The treatment of the five patients has since changed, involving mainly the use of L1. Low serum ferritin levels appear to be misleading for detecting cardiac iron overload and this may increase the risk of cardiomyopathy. The MRI T2 and T2* relaxation time measurements are a more accurate method of detecting cardiac iron overload. Chelation therapy using L1 or appropriate L1/DFO combinations can reduce cardiac iron overload and the mortality rate in thalassemia patients.

Administration, Oral↗
PubMed2006

Regression of myocardial dysfunction after switching from desferrioxamine to deferiprone therapy in beta-thalassemia major patients.

This study tries to compare the cardioprotective effects of the desferrioxamine (DFO) and deferiprone (L1) therapies on thalassemia major patients. We enrolled nine patients with thalassemia major complicated by some degree of myocardial dysfunction. These patients, recipients of >10 years of DFO injection therapy, were switched from DFO to L1. Echocardiographic measures of left ventricular systolic, diastolic and global functions, were assessed regularly every 6 months. Mean values of each parameter for the DFO and L1 treatment periods were compared using paired t-test and Wilcoxon signed-rank test. Global cardiac function improved significantly. Myocardial dysfunction in patients with thalassemia major can be reversed by regular use of the oral iron chelator L1.

Adolescent↗
PubMed2006

Effective combination therapy of deferiprone and deferoxamine for the rapid clearance of excess cardiac IRON and the prevention of heart disease in thalassemia. The Protocol of the International Committee on Oral Chelators.

The International Committee on Oral Chelators (ICOC) combination therapy protocol involving the administration of deferiprone (L1) during the day (80-110 mg/kg/day) and deferoxamine (DFO) (40-60 mg/kg at least 3 days/week) during the night for 8-12 hours using a pump, or the whole 24 hours using an elastomeric pump infuser, has been tested in 11 thalassemia patients (seven males, four females) over a period of 9-28 months. The patients had variable serum ferritin levels (0.54-4.6 mg/L) and cardiac iron load ranging from normal to severe siderosis levels (MRI T2*: 4.7-45 ms). There was a substantial overall reduction in serum ferritin levels (0.17-2.16 mg/L) and normalization of cardiac iron (MRI T2* >20 ms) in all patients. In two patients with severe and moderate cardiac iron load range levels, cardiac iron normalization was achieved within 9-10 months. Two patients on L1 monotherapy (80-120 mg/kg/day) maintained normal range MRI T2* cardiac iron levels over the same period. The ICOC combination therapy protocol appears to be the most effective and least cumbersome form of chelation treatment for the rapid clearance of excess iron from the heart.

Administration, Oral↗
PubMed1995

Dietary ferric vs. ferrous iron in copper-deficient rats fed fructose-based diets.

OBJECTIVE: This preliminary study was undertaken to determine whether the valence state of dietary iron affects signs associated with copper deficiency in rats fed fructose. METHODS: Rats were fed either copper-deficient or adequate diets containing 62% fructose as the sole dietary carbohydrate for 5 weeks. The mineral mixture contained equal concentration of either ferric or ferrous iron. RESULTS: Copper deficiency resulted in growth retardation, anemia, heart hypertrophy but pancreatic atrophy. The consumption of ferrous iron resulted in increased hematocrit and pancreas size. The combination of ferrous iron with copper deficiency reduced heart size. CONCLUSIONS: Copper deficiency had a major impact on each parameter measured. Although the valence state of iron did not protect the rats against the pathological consequence of copper deficiency it did have some positive effects. It may be that ferrous iron is a more available form than ferric iron.

Anemia↗
PubMed1980

Transfusion therapy: its role in the anemias.

Potentially hazardous and at best supportive, transfusion therapy obligates the clinician to make careful clinical judgments regarding anticipated benefits versus possible complications. Ways to maximize benefit and minimize risk are outlined.

Anemia↗
PubMed1970

Hemoglobin uptake by rat hepatocytes and its breakdown within lysosomes.

The peroxidatic activity of hemoglobin permitted visualization of its uptake by rat hepatocytes by means of the Graham-Karnovsky 3,3'-diaminobenzidine (DAB) procedure. Lysosomes were visualized by their acid phosphatase, beta-glucuronidase, and glucosaminidase activities. When large doses of rat, cow, or human hemoglobin are intravenously injected, or when hemoglobinemia is induced by injection of distilled water, DAB-positive hemoglobin is engulfed by pinocytosis. Pinocytotic vacuoles become digestive vacuoles ("phagolysosomes") by fusion with lysosomes of the dense body type that have moved from their pericanalicular position. By 16-24 hr after even massive amounts of hemoglobin (400 mg/100 g), the protein is barely demonstrable in hepatocytes. At the lowest doses of injected hemoglobin (15 mg/100 g body weight), DAB-positive vacuoles are demonstrable only in the Kupffer cells.

Acid Phosphatase↗
PubMed1975

The detection of hepatitis B antigen in hepatic parenchyma by the fluorescent antibody technic.

Tissue sections from 42 specimens of liver were examined by indirect immunofluorescence microscopy for the presence of hepatitis B antigen (HB Ag). In all cases the serologic status of HB Ag was known. Fourteen of the specimens were also examined by electron microscopy. In four biopsies from three patients positive cytoplasmic fluorescence was detected using antisera prepared in animals and 20-nm. nuclear particles were found by electron microscopy. These patients were all seropositive for HB Ag, all had chronic aggressive hepatitis or active cirrhosis, and all were receiving immunosuppressive therapy at the time of examination. Nuclear fluorescent staining was demonstrated when one of these biopsies was re-examined using a human antiserum.

Cell Nucleus↗
PubMed1978

Microcytic normochromic anemia associated with iron storage by hypernephroma.

A renal-cell carcinoma was discovered and resected in a 38-year-old female patient who had microcytic normochromic anemia. During treatment with ferrous gluconate, the anemia regressed temporarily but reappeared with the onset of metastases to the abdominal lymph nodes. Heavy deposits of hemosiderin were observed in tumor cells in the resected kidney and lymph nodal metastases. It is postulated that the anemia resulted from metabolic diversion and storage of iron by the tumor cells.

Adenocarcinoma↗
PubMed1979

Comparison of stainable iron in aspirated and needle-biopsy specimens of bone marrow.

A comparison of stainable iron in simultaneously obtained aspirated smears and needle-biopsy specimens from 1,000 patients was undertaken. Significant differences occurred when iron was assessed as absent in the aspirated smear. In only 35% of the corresponding needle-biopsy specimens was iron absent. When only the aspirated smear was used, there was a significant overdiagnosis of iron deficiency. In general, iron tended to be less in the aspirated smear; however, correlations were better when iron stores were assessed as being present or increased in the aspirated smears, for stainable iron in the needle-biopsy specimen was always present in equal or greater amounts. Hemosiderotic smears (increased stainable iron) and needle-biopsy specimens (3+ -4+) correlated well. The aspirated smear and needle-biopsy are complementary procedures, and each has advantages. In the authors' experience, the needle-biopsy was preferable to the aspirated smear for evaluation of iron stores, particularly when iron stores were low or absent.

Biopsy, Needle↗
PubMed1987

Special report: transfusion risks.

The benefits of blood transfusion must be considered and evaluated in terms of risk factors relating to the adverse effects of transfusion. Transfusions may result in either serious or troublesome complications. Although the risk of transfusion-associated acquired immune deficiency syndrome (AIDS) is of paramount concern in the patient population, it has been virtually eliminated because of testing of donor units for antibody to human immunodeficiency virus. Serious and troublesome adverse effects of blood transfusion are listed and ranked in order of approximate frequency. About 20% of all transfusions result in some type of adverse effect. The major serious risk of blood transfusion today continues to be transfusion-associated viral hepatitis. This entity is usually subclinical but frequently results in serious chronic liver disease. Transfusions should be avoided unless patient care would be compromised if withheld.

Acquired Immunodeficiency Syndrome↗