Role of chronic hepatitis in development of thalassaemic liver disease.

Masera, Giuseppe; Jean, G.; Gazzola, Gian Biattista; Nováková, M

doi:10.1136/adc.51.9.680

Cited by 36 publications

(20 citation statements)

References 17 publications

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“…Transfusion therapy plays a marginal role, because the hypergammaglobulinemia is also present in not yet transfused patients. The hemosiderosis, usually associated with this anemia, leads to liver damage, which is worsened by chronic hypoxemia and some times by viral hepatitis [7,8]. It has been suggested that hepatopathy may be respon sible for hypergammaglobulinemia [1,2,4], but our results show that younger patients, with low iron overload and normal hepatic enzyme values, also have a significant Ig in crease.…”

Section: Discussioncontrasting

confidence: 49%

Serum Immunoglobulins in Homozygous β-Thalassemia

et al. 1981

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IgG, IgA and IgM levels were studied in 187 homozygous β-thalassemic patients and compared with age-matched normal control subjects. The not yet transfused and the polytransfused nonsplenectomized patients showed a significant increase of all Ig classes. The polytransfused splenectomized patients showed a significant increase only of IgG and IgA. The splenectomized patients, when compared to the nonsplenectomized ones, showed a significant increase of IgG, of IgA in the elder ones and a significant reduction of IgM. High ASLO and ASFLO titers were found especially in the splenectomized patients. The high Ig levels in younger not yet transfused patients, with little iron storage and normal hepatic enzyme values, demonstrate that transfusion therapy and liver damage do not play a main role in hypergammaglobulinemia. The absence of antimitochondrial, anticonnective tissue and homogeneous antinuclear antibodies seems to exclude a deficient suppressor mechanism. The hemocatheteric ‘overworking’ of RES may reduce the antigen clearance with a consequent increase of antibody response. Splenectomy, by removing a consistent part of RES, enhances the phenomenon.

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Section: Discussioncontrasting

confidence: 49%

Serum Immunoglobulins in Homozygous β-Thalassemia

et al. 1981

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“…Nearly all ,-thalassaemic patients develop chronic liver disease (Masera et al, 1976). In this hepatic impairment, circulating immune complexes involving HBs antigen have been demonstrated (Theofilopoulos et al, 1976); in our cases HBs antigen could have been acquired as a result of repeated blood transfusion.…”

Section: Methodssupporting

confidence: 47%

“…Many patients with /3-thalassaemia major develop glomerulonephritis, pericarditis, or arthritis (Wasi, 1971), and nearly all develop chronic liver disease (Masera et al, 1976). In this last condition, circulating immune complexes are often present and they are thought to play a significant part in the genesis of these immunopathological disorders.…”

mentioning

confidence: 99%

Occurrence of circulating immune complexes in beta-thalassaemia major.

Casali¹,

Borzini²,

Vergani³

et al. 1978

Archives of Disease in Childhood

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SUMMARY The presence of circulating soluble immune complexes and the level of complement were investigated in sera from 21 patients with L3-thalassaemia major, including both splenectomised and nonsplenectomised patients. A high level of immune complexes was found in half of these cases. Reduced complement levels were seen less frequently. There was no correlation between the presence of circulating immune complexes, decreased complement levels, and the presence or absence of the spleen. The level of immune complexes increased with the age of the individual, i.e. with the duration of the disease.

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“…Chronic hepatitis is a very frequent com plication of transfusion-dependent thalasse mia (thal) major in medium and underde veloped countries [1], The main causes of this complication are hepatitis B or non-A, non-B and transfusional iron overload [2,3]. In a previous study by our group [4] these two factors were found to be related, as higher liver iron overload was seen in thal major patients with chronic hepatitis than in patients with no hepatitis [4].…”

Section: Introductionmentioning

confidence: 98%

Iron Chelation in Transfusion-Dependent Thalassemia with Chronic Hepatitis

Virgiliis¹,

Cossu²,

Sanna³

et al. 1982

Acta Haematol

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In this study maximum urinary iron elimination with continuous desferrioxamine subcutaneous infusion was obtained in thalassemia major patients with chronic persistent or active hepatitis with lower doses (60 mg/kg) than those necessary in patients without hepatitis (80 mg/kg). Since dose-response curves were highly variable the treatment schedule should be tailored to the individual needs of each patient. Both groups may achieve iron balance but chronic hepatitis patients have more frequently a net urinary iron excretion. In patients with chronic hepatitis no correlation was found between serum ferritin levels or serum ferritin/aspartate aminotransferase ratios and transfusional iron overload while serum ferritin/aspartate aminotransferase ratios were seen to be correlated with liver iron stores.

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Role of chronic hepatitis in development of thalassaemic liver disease.

Cited by 36 publications

References 17 publications

Serum Immunoglobulins in Homozygous β-Thalassemia

Serum Immunoglobulins in Homozygous β-Thalassemia

Occurrence of circulating immune complexes in beta-thalassaemia major.

Iron Chelation in Transfusion-Dependent Thalassemia with Chronic Hepatitis

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