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Query: UMLS:C0002878 (hemolytic anemia)
 7,530 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In connection with 6 cases of Wilson's disease, the authors recall the main features of this hereditary metabolic disorder at late onset (usually the second decade), treatable with a chelating agent, when diagnosed at an early stage. Wilson's disease is first of all a liver disease and the authors emphasize the fact that cirrhosis is usually present when neurologic symptoms, revealing the disease in 5 cases, appear, even if there is no clinical or biological evidence for liver disease. In one instance hemolytic anemia and chronic active hepatitis were observed at clinical onset. Copper metabolism usually gives the key for diagnosis but its interpretation may be difficult, a normal serum ceruleoplasmin level being found in two patients and evaluated at 6% in the literature. This fact brings up the puzzling question of the pathogesis of the disease. Wilson's disease is not a simple ceruleoplasmin synthesis defect, but a lysosomal disease responsible for the lack of copper biliary excretion. This is pointed out by histochemical studies using a special rubeanic acid preparation (revealing copper deposit on the biliary side of the hepatic cell), and by electron microscopy showing lysosomal dystrophy.
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PMID:[Wilson's disease. A clinical and pathological study on 6 cases (author's transl)]. 22 95

Sheep were treated with large amounts of copper (20 mg of CuSO4,5H2O/kg body wt. per day) for 9 weeks to examine the effect of copper excess on iron metabolism. In addition to confirming that massive haemolysis and accumulation of copper occurs in the liver, kidney and plasma after 7 weeks of exposure to excess copper, it was observed that excess copper produced an increased plasma iron concentration and transferrin saturation within 1 week. Further, iron preferentially accumulated in the spleen between 4 and 6 weeks of copper treatment, producing 3-fold increases in the iron content of both the ferritin and non-ferritin fractions. A 3-4 fold increase was also observed in the amount of ferritin that could be isolated from the spleen. The copper treatment had little or no effect on the concentration of iron in the liver and bone marrow. The following properties of erythrocytes were also unaffected by copper treatment: size, haemoglobin content and pyruvate kinase activity, although the erythrocyte concentration of copper increased after 6 weeks. Copper accumulated in the spleen between 6 and 9 weeks, probably owing to the phagocytosis of erythrocytes containing high concentrations of copper. The data suggest that copper excess influences iron metabolism, initially by causing a compensated haemolytic anaemia, and later by interfering with re-utilization of iron from ferritin in the reticuloendothelial cells of the spleen.
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PMID:The effect of copper excess on iron metabolism in sheep. 62 72

Wilson's disease, an autosomal recessive disorder of copper metabolism, may defy diagnosis in children. The classical triad of Kayser-Fleischer rings, neurologic dysfunction, and hypoceruloplasminemia may be absent. Patients may be seen initially with acute or chronic hepatitis, hemolytic anemia, or neurologic dysfunction. Guidelines are presented for diagnosis of Wilson's disease based on a review of 25 pediatric and adolescent patients. A high index of suspicion in necessary so that therapy with penicillamine may be begun before irreversible liver or neurologic damage occurs. The prognosis is excellent when diagnosis and treatment are established early.
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PMID:Diagnostic dilemmas of Wilson's disease: diagnosis and treatment. 68 83

Although the development of hemolytic anemia as a complication of acute copper intoxication is well documented, the precise mechanism by which copper produces accelerated erythrocyte destruction is unknown. Normal erythrocyte survival depends in part on the ability of the cell to deform and pass through narrow areas of microcirculation in the liver and especially in the spleen. In the present study, it is demonstrated that toxic concentrations of copper rapidly and markedly reduce erythrocyte deformability. This reduction in cell deformability is associated with a marked increase in membrane permeability and osmotic fragility of copper-treated cells. Further, the decrease in deformability occurs despite normal levels of cell ATP and the apparent absence of oxidative damage to the cell. These observations indicate that copper-mediated changes in the erythrocyte membrane may be responsible for reducing the flexibility of the cell. The loss of deformability could act to reduce erythrocyte survival and thus explain the hemolysis associated with copper intoxication in vivo.
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PMID:The effect of copper on erythrocyte deformability: a possible mechanism of hemolysis in acute copper intoxication. 75 48

We report a case of cupric sulfate intoxication in a child who had a serum copper level of 1,650 mug/100 ml. His course was accompanied by hemolytic anemia and renal tubular damage. We review the pathophysiology of copper metabolism and intoxication. We also review modes of therapy, with specific reference to the initial approach, using dimercaprol (BAL) and edetic acid rather than penicillamine.
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PMID:Acute copper intoxication. Pathophysiology and therapy with a case report. 83 30

Severe anemia was found in a 4-month-old heifer, which was admitted with a 1-day history of anorexia, signs of depression, and recumbency. A diagnosis of immune-mediated hemolytic anemia (IHA) was made on the basis of a Coomb's titer of 1:128 and decreased resistance to osmotic stress, as determined by an RBC fragility test. Anaplasmosis and leptospirosis were ruled out as possible causes of the IHA. Other causes of hemolytic anemia, including intoxication by copper, water, Brassica spp, or drugs were ruled out. Therefore the IHA was considered idiopathic. Treatment consisted of supportive therapy, oxytetracycline, and dexamethasone. After 60 days of treatment, CBC, Coomb's test result, and RBC fragility were within normal limits.
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PMID:Idiopathic immune-mediated hemolytic anemia in a calf. 164 56

The pathophysiology, symptomatology, and treatment of Wilson's disease are reviewed, and new approaches to drug management are discussed. Wilson's disease is a rare, autosomal recessive disorder that occurs between the ages of 6 and 60 years. Disturbances in copper metabolism may result in the accumulation of excess copper in the liver, the basal ganglia of the brain (lenticular degeneration), the kidneys, the cornea (Kayser-Fleischer rings), and other tissues. The diagnosis of Wilson's disease is frequently overlooked; nonspecific symptoms and multisystem involvement may mimic other disease states, such as neurologic and psychiatric disorders, and hemolytic anemia. Screening tests for Wilson's disease include 24-hour urinary copper levels, serum ceruloplasmin and copper assays, radioactive uptake of 64Cu, and liver biopsy. Current methods of therapy include the use of a chelating agent--penicillamine or trientine--for initial rapid decoppering. Penicillamine therapy has been associated with many adverse reactions, including worsening of the neurologic symptoms of the patient. Zinc is a useful agent for maintenance therapy. Investigational studies exploring the use of ammonium tetrathiomolybdate for initial rapid decoppering have shown promising results. Unless it is recognized and treated, Wilson's disease can cause severe symptoms and, ultimately, death. Initial rapid decoppering with chelating agents, such as penicillamine and trientine, followed by lifelong maintenance therapy with zinc is the current method of treatment.
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PMID:Pathophysiology and treatment of Wilson's disease. 179 20

The study comprised 40 children of both sexes aged from 2 to 12 years, 22 suffering from bronchial asthma and 18 suffering from atopic dermatitis. Twenty healthy children of comparable age and sex to the patients were studied as controls. All the children were subjected to full history including dietetic questionnaire, thorough examination, and to estimation of serum IgE, serum ceruloplasmin, and zinc and copper levels in both serum and hairs. The mean concentrations of zinc in serum and hairs were respectively 70.3 +/- 13.2 micrograms/100 ml and 167.5 +/- 23.0 micrograms/gm in asthmatic cases and 65.9 +/- 11.7 micrograms/100 ml and 164.8 +/- 23.6 micrograms/gm in those with atopic dermatitis. These levels were significantly (p less than 0.001) decreased in comparison to the control values (88.4 +/- 11.0 micrograms/100 ml and 194.5 +/- 18.6 micrograms/gm). On the other hand, a significant (p less than 0.001) increase in serum and hairs copper was demonstrated in both allergic groups compared to the controls. Mean copper values were respectively 79.5 +/- 8.06 micrograms/100 ml and 18.7 +/- 1.9 micrograms/gm in the asthmatic cases and 81.4 +/- 8.4 micrograms/100 ml and 17.8 +/- 2.08 micrograms/gm in cases with atopic dermatitis. The control mean concentrations were 67.95 +/- 6.37 micrograms/100 ml and 14.5 +/- 2.53 micrograms/gm respectively. Significant (p less than 0.001) higher levels of serum ceruloplasmin were observed in the allergic patients compared to the controls and were correlated with the hypercupremia. The results were discussed and a good dietetic intake of high biological value protein and zinc supplement was recommended to these patients in order to correct their disturbances especially the hypozincemia which could lead to exaggeration of their allergic conditions. The field of trace elements metabolism has grown rapidly over the past few years, particularly after the development of novel techniques as the atomic absorption spectrophotometry which had allowed the reliable measurements of several trace elements in tissues and so had opened a new field for many researches (Henkin, 1976). Zinc and copper are involved in cell and tissue growth. Zinc plays an important role in DNA and protein synthesis and is intimately involved with copper as cofactors in several important enzyme systems. The effects of many pathological conditions as congestive heart failure, pneumonia, rheumatic heart diseases, bronchitis, recurrent infection, hemolytic anemia, psoriasis, and malnutrition on the levels of serum zinc, copper, and other trace elements have been of interest to investigators for a number of years (Sinha and Gabrieli, 1970; David et al., 1984).(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Zinc and copper status in children with bronchial asthma and atopic dermatitis. 213

Hepatolenticular degeneration (Wilson's disease) is a hereditary disease in which metabolic disorder of copper leads to its accumulation in the liver, brain, cornea and kidneys with consequent pathologic changes in those organs. Hereditary mechanism of the disease is autosomal recessive with prevalence of 30-100 per 1,000,000 inhabitants. Etiology of this disease is not yet explained. There are two hypotheses. The first one is that it is the disorder of ceruloplasmine metabolism caused by insufficient synthesis of normal ceruloplasmine, or synthesis of functionally abnormal ceruloplasmine. The second one is: the block of copper biliar excretion which is the consequence of the liver lysosomes functional defect. Pathogenetic mechanism of disease is firstly long-term accumulation of copper in the liver, and later, when the liver depo is full, its releasing in circulation and accumulation in the brain, cornea, kidneys and bones, which causes adequate pathologic changes. Toxic activity of copper is the consequence of its activity on enzymes, particularly on those with -SH group. There are two basic clinical forms of the disease: liver disease or neurologic disease. Before puberty the liver damage is more frequent, while in adolescents and young adults neurologic form of the disease is usual. The liver disease is nonspecific and characterized by symptoms of cirrhosis and chronic aggressive hepatitis. The only specificity is hemolytic anemia which, in combination with previous symptoms, is important for diagnosis of the disease. Neurologic symptoms are the most frequent consequence of pathologic changes in the basal ganglia. In our patients the most frequent symptoms were tremor (63%); dysarthria, choreoathetosis and rigor (38%); ataxia and mental disorders (31%); dysphagia and dystonia (12%), diplopia, hypersalivation, nystagmus and Babinski's sign (6%). Among pathologic changes in other tissues and organs the most important is the finding of Kayser-Fleischer ring in the cornea as a result of copper accumulation. Its importance for precise diagnosis is great. The diagnosis of the disease is based on anamnesis, clinical examination, specific and nonspecific laboratory tests. The therapy of choice is penicillamine. If we use it early, the result will be good remission in the majority of patients. Late diagnosis or delay in treatment cause death which is the result of bleeding from esophageal varices or basal ganglia disease. Immunologic damages caused by penicillamine demand interruption of therapy and substitution by three-ethyl-tetra-amine (TETA). We also use zinc salts and tetratiomolibdate in therapy of this disease. Pathogenesis, clinical picture and therapy of the disease are based on our own results.
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PMID:[Hepatolenticular degeneration]. 226 49

The concentrations of three trace elements (iron, copper and zinc) involved in hematopoiesis were studied in 200 patients with iron-deficit anemia, hemolytic anemia and Biermer's anemia, in comparison with a group of normal subjects. The method used was atomic absorption spectrophotometry. The results obtained showed that in iron-deficit anemia resistant to iron therapy the copper and ceruloplasmin serum concentrations are at the lowest limit of normal. This copper deficit has a negative effect on the disease by preventing hemoglobin synthesis. In iron-deficit anemias responding to iron therapy the relatively low (about 90 gamma %) zinc values have a negative effect by blocking the iron in the iron reserve. In hemolytic anemia the very high zinc serum concentrations (over 200 gamma %) may lead to a shortening of the erythrocytes life span due to its "entatic state". In Biermer's anemia zinc by its high concentration has a positive role owing to carbonic anhydrase which eliminates more rapidly carbon dioxide from the organism.
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PMID:Possible correlation between the zinc and copper concentrations involved in the pathogenesis of various forms of anemia. 228 64


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