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

To examine the ability of plasma to prevent haemolysis caused by mechanical trauma and by hypotonicity, in-vitro experiments were carried out. Human red blood cells (RBC) were agitated with glass beads at 37 degrees C for 18 h in isotonic tris-buffered saline (TBS) or with additions of test substances to TBS. RBC were incubated for 18 h at 37 degrees C in a stationary water-bath to test effects of hypotonic solutions. Release of haemoglobin was quantitated by measuring optical density of supernates at 412 nm in a spectrophotometer. Agitation of RBC with glass beads in TBS resulted in release of about 30% of haemoglobin, and this haemolysis was inhibited by autologous plasma. Components of plasma that contributed to protection against mechanical haemolysis were albumin, a supernatant of plasma after heating to 100 degrees C that was albumin-free, and a heat-stable extract of fresh frozen plasma (FFP), whereas little or no protection was provided by gamma-globulin, haptoglobin, ceruloplasmin or alpha-2-macroglobulin. Relative viscosity of solutions containing plasma or dextran correlated poorly with protection against mechanical haemolysis. Hypotonic solutions of plasma and addition of the extract of FFP to TBS caused reductions in rates of haemolysis when compared to corresponding osmolalities of solutions of TBS. These results, which showed protective effects of plasma and plasma components against mechanical and hypotonic haemolysis, suggest that a deficiency of protective factors in plasma of patients could contribute to the development of haemolytic anaemia.
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PMID:Plasma components protect erythrocytes against experimental haemolysis caused by mechanical trauma and by hypotonicity. 157 75

In various anaemias the values of 8 acute phase factors were determined simultaneously before and at the end of treatment: seromucoid, sialic acid, acid alpha 1-glycoprotein, alpha 1-antitrypsin, haptoglobin, ceruloplasmin, transferrin and fibrinogen. In iron-deficiency anaemia without coexistent inflammatory changes in organs the levels of 4 proteins--seromucoid, alpha 1-antitrypsin, ceruloplasmin and transferrin, were consistently raised. In iron-deficiency anemia with concomitant infection 4 proteins also were increased, but in place of alpha 1-antitrypsin the haptoglobin level was raised. In megaloblastic anaemia the ceruloplasmin level was increased, and in haemolytic anaemia one factor--sialic acid--was decreased. At the end of treatment the concentrations of certain proteins were changed depending on their specific role in various forms of anaemia and on various additional factors. In iron-deficiency anaemia without coexistent infection the concentration of seromucoid was decreased, and in this anaemia with coexistent infection alpha 1-antitrypsin, haptoglobin, and fibrinogen levels were raised, in haemolytic anaemia only fibrinogen was increased, and megaloblastic anaemia was associated with raised seromucoid level. The therapeutic result was good in all these anaemias with the exception of iron-deficiency anaemia associated with infection in which it was less propitious.
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PMID:[Acute phase factors in anemia]. 172 69

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

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

Ninety-five cases of hepatolenticular degeneration have been studied, focusing particularly the clinical and laboratory characterization of the disease. On the clinical viewpoint the variability of the starting symptoms and the frequency of the Kayser-Fleischer rings were analyzed. As regards the laboratory findings the ceruloplasmin, blood and urinary copper, and aminoaciduria levels have been evaluated, as well as the radiological and scintillographic study of joints and bones, cranial computerized tomography and liver biopsy. In 54.4% of the cases the opening clinical picture was neurological, in 31.1% hepatic, in 14.4% psychiatric, in 7.8% osteoarticular, in 2.2% ophtalmologic (Kayser-Fleischer rings), in 1.1% hematologic (hemolytic anemia), and in 1.1% cardiac. Kayser-Fleischer rings were present in 84 of the 92 cases in which they have been searched for (91.3%). Concerning the laboratory findings, hypoceruloplasminemia was found in 98.8% of the cases in which it was investigated, hyperaminoaciduria in 94.7%, hypocupremia in 87.0%, increased cupruresis in 78.2%, osteoporosis in 79.4%, scintillographic changes of the joints in 67.6%; the CT-scan, performed in 11 cases, showed low attenuation areas in the basal ganglia of 2 patients. The significance of the mentioned laboratory findings and the presence of the Kayser-Fleischer rings for the diagnosis of Wilson's disease is discussed.
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PMID:[Hepatolenticular degeneration: critical evaluation of the diagnostic criteria in 95 cases]. 409 35

It has been demonstrated that the level of serum copper unbound to ceruloplasmin (loosely bound copper) is increased in Wilson's disease, although the total serum copper concentration is usually low, reflecting a low ceruloplasmin level. To assess the contribution of free radical reactions catalyzed by nonceruloplasmin copper to the development of complications in this disease, we investigated copper and antioxidant status in four untreated patients who had hepatic dysfunction with or without hemolytic anemia and made a comparison with five patients controlled on penicillamine therapy and 19 age-matched healthy children. We found that loosely bound copper in plasma measured by the phenanthroline assay was detectable in three of four untreated patients with Wilson's disease, but was not detectable in the patients during therapy or in the healthy controls. Among the various antioxidants, the ascorbate and urate levels were markedly reduced before treatment (mean +/- SD, 23 +/- 16 microM for ascorbate and 90 +/- 59 microM for urate) compared with the values in the patients during treatment with penicillamine (67 +/- 19 and 302 +/- 78 microM, p < 0.05) and in control children (60 +/- 8 and 254 +/- 48 microM, p < 0.05). We also demonstrated that the plasma concentration of allantoin, an oxidation product of uric acid and a possible marker of radical generation in vivo, was markedly elevated in the untreated patients (11.0 +/- 1.8 versus 4.3 +/- 0.5 microM in patients on therapy and 6.5 +/- 0.8 microM in controls, p < 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Plasma copper and antioxidant status in Wilson's disease. 773 61

We describe a patient who had severe neurologic symptoms, psychiatric abnormalities, and secondary amenorrhea superimposed on a history of hemolytic anemia and micronodular cirrhosis attributed to hemochromatosis. The correct diagnosis of Wilson's disease was delayed until the appearance of Kayser-Fleischer rings and a low serum ceruloplasmin level. Appropriate treatment ameliorated symptoms, and maintenance therapy has been effective in retarding progression. It is essential to consider Wilson's disease in patients with unexplained hepatic, neurologic, and psychiatric dysfunction, because appropriate early medical treatment can prevent further organ damage and reduce the risk of permanent damage to the liver and brain.
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PMID:Wilson's disease. 916 75

A 16-year-old girl was admitted for a detailed examination of hemolytic anemia in November 1995. Initial laboratory findings included a total bilirubin concentration of 1.46 mg/dl, hemoglobin of 9.1 g/dl, and a reticulocyte count of 89/1000 percent. The plasma haptoglobin concentration was below 10 mg/dl. A blood smear showed many dacryocytes and a few echinocytes and codocytes. GOT was 71 IU/l; GPT, 44 IU/l; and LDH, 812 IU/l; the results of a hepaplastin test were 45% of normal. On further investigation, the level of serum ceruloplasmin was found to be 4 mg/dl, and of serum copper, 43 micrograms/dl. Urinary copper excretion was markedly increased, at 345 micrograms per day. Slit-lamp examination of both corneas revealed obvious Kayser-Fleischer rings. A liver biopsy sample showed fibrosis histologically and an elevated copper concentration of 535 micrograms/g dry weight and 183 micrograms/g wet weight. In family studies, the patient's asymptomatic 5-year-old sister was observed to have metabolic abnormalities consistent with Wilson's disease. These findings suggested that the patient's hemolytic anemia with red cell deformities was due to abnormal copper metabolism associated with Wilson's disease.
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PMID:[Severe hemolytic anemia with tear drop red cells as initial manifestation of Wilson's disease]. 979

We report a 27-year-old woman who developed Coombs' negative hemolytic anemia and fulminant hepatic failure as the initial manifestation of Wilson's disease. Unmeasurably low level of serum alkaline phosphatase provided a clue to the diagnosis of Wilson's disease. The diagnosis was established with the presence of Kayser-Fleischer ring, decreased serum ceruloplasmin level, and elevated urine and serum copper levels. In spite of repeated plasmapheresis, she died of multiorgan failure on the fifth hospital day.
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PMID:Acute hemolytic crisis with fulminant hepatic failure as the first manifestation of Wilson's disease: a case report. 981 Nov 88


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