The radiographic appearances following adequate transfusion in β-thalassaemia

Scuttellari, Pier Nuccio; Orzincolo, C; Franceschini, F; Bagni, B

doi:10.1007/bf02569398

Cited by 6 publications

(1 citation statement)

References 12 publications

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“…Many texts describe the appearance of "hair-on-end" seen on radiographs in extreme cases where the outer table has been resorbed and the diploe expanded with trabeculae radially oriented. However, this condition is not typical, as it is present in only 5-20% of clinical cases (Fernbach, 1984;Scutellari et al, 1989;Silverman, 1985). Generalized osteoporosis is much more common, although definitely less spectacular and much more difficult to differentiate from the normal appearance of the cranial vault on radiographs.…”

Section: Discussionmentioning

confidence: 99%

Anemia, genetic diseases, and malaria in prehistoric mainland southeast Asia

Tayles

1996

Am. J. Phys. Anthropol.

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The analysis of a sample of skeletons from the 4,000-year-old site of Khok Phanom Di on the coast of central Thailand has identified a number of individuals with skeletal evidence suggestive of severe anemia. The differential diagnosis of the lesions is discussed and the presence of one of the thalassemia syndromes is proposed. The implications of this for southeast Asian prehistory are discussed. The presence of these conditions has been suggested in previous analyses of prehistoric southeast Asian populations, but this is the first population in which the evidence, including postcranial responses, is presented in detail.

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Section: Discussionmentioning

confidence: 99%

Anemia, genetic diseases, and malaria in prehistoric mainland southeast Asia

Tayles

1996

Am. J. Phys. Anthropol.

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Growth plate injury of the long bones in treated ?-thalassemia

1992

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In 12 patients affected by thalassemia major who received an intensive transfusion regimen combined with continuous iron chelation therapy (desferrioxamine 50-80 mg/kg daily), radiologic abnormalities of the long bones were observed similar to those observed in rickets and scurvy. These abnormalities were associated with a growth retardation. The pathogenesis of these lesions is uncertain, but probably the toxic effect of desferrioxamine plays an important role in their development. A relative deficiency of vitamins D and/or C cannot be entirely excluded.

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Effects of deferoximine on chondrocyte alkaline phosphatase activity: Proxidant role of deferoximine in thalassemia

et al. 1995

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The homozygous form of beta-thalassemia, the most common single gene disorder, is treated by red cell transfusion therapy. Following transfusion, the chelator, deferoximine, is administered to patients to remove excess iron. However, when this drug is given to young children, metaphyseal dysplasia and abnormalities of linear growth are frequently observed. To explore the notion that deferoximine interferes with endochondral growth by chelating zinc, we examined the effect of the drug on chondrocytes maintained in long-term culture. We found that deferoximine caused a dose-dependent inhibition of a wide range of functions including cell proliferation, protein synthesis (and possibly under-hydroxylation of type X collagen), and mineral deposition. Directly relevant to the mineralization process was the observation that the drug dramatically lowered the activity of alkaline phosphatase, a zinc-requiring enzyme. To test the hypothesis that enzyme inhibition was due to chelation of zinc by deferoximine, the cell culture medium was supplemented with excess zinc. However, this treatment did not overcome the deferoximine-dependent change in enzyme activity. We next examined the possibility that deferoximine, in the presence of ascorbate, could form a free radical system that would serve to inactivate the enzyme. Using alkaline phosphatase extracted from chick cartilage, we noted that the activity of the phosphatase was markedly reduced in the presence of deferoximine and ascorbate. These effects were consistant with the notion that deferoximine and ascorbate can act as a prooxidant couple. This conclusion was confirmed when we measured the oxidative activities of the system using nitrobule tetrazolium and cytochrome c. Indeed, we noted that deferoximine markedly activates the autocatalytic oxidation of ascorbate.(ABSTRACT TRUNCATED AT 250 WORDS)

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The radiographic appearances following adequate transfusion in β-thalassaemia

Cited by 6 publications

References 12 publications

Anemia, genetic diseases, and malaria in prehistoric mainland southeast Asia

Anemia, genetic diseases, and malaria in prehistoric mainland southeast Asia

Growth plate injury of the long bones in treated ?-thalassemia

Effects of deferoximine on chondrocyte alkaline phosphatase activity: Proxidant role of deferoximine in thalassemia

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