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Autor: B. Wonke, A.V. Hoffbrand

Publikováno v: Journal of Internal Medicine. 242:37-41

Desferrioxamine (DFX) remains the most effective and safe iron chelator for treatment of patients with transfusional iron overload. It is usually given by intermittent subcutaneous infusions for 8-12 h on 4-6 days weekly using a battery-driven pump.

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::2e2bd5992efc6c17e7705a65c05139cd
https://doi.org/10.1111/joim.1997.242.s740.37

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Interaction of hemoglobin E and pyrimidine 5' nucleotidase deficiency

Autor: David C. Rees, David J. Weatherall, John A. Duley, H.A. Simmonds, S. L. Thein, B. Wonke, John B. Clegg

Publikováno v: Blood. 88:2761-2767

A Bangladeshi family is described in which the genes for both hemoglobin E (Hb E) and pyrimidine 5′ nucleotidase deficiency are segregating. An individual homozygous for both these conditions has a severe hemolytic anemia, whereas family members wh

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::99e1ec661334187f1a0b9fe1c2e1a37d
https://doi.org/10.1182/blood.v88.7.2761.bloodjournal8872761

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Moderate reduction of beta-globin gene transcript by a novel mutation in the 5' untranslated region: a study of its interaction with other genotypes in two families

Autor: A Yardumian, Chris Fisher, B. Wonke, S. L. Thein, Jacques Rochette, P. J. Ho, MK Jarvis

Publikováno v: Blood. 87:1170-1178

We have identified two individuals of Greek Cypriot origin with thalassemia intermedia. Molecular analysis has shown that each individual is a compound heterozygote for a previously described beta zero thalassemia allele and a novel mutation, C-->G i

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::84113742b286239b27d857a42ed1aeaa
https://doi.org/10.1182/blood.v87.3.1170.bloodjournal8731170

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Efficacy and possible adverse effects of the oral iron chelator 1,2- dimethyl-3-hydroxypyrid-4-one (L1) in thalassemia major [see comments]

Autor: FN al-Refaie, B Wonke, AV Hoffbrand, DG Wickens, P Nortey, GJ Kontoghiorghes

Publikováno v: Blood. 80:593-599

Eleven patients with beta thalassemia major were entered into the trial of the oral chelator 1,2-dimethyl-3-hydroxypyrid-4-one (L1). Their ages ranged from 17 to 26 years (mean +/- SD, 22.3 +/- 2.7). Six were male and five were female. L1 was adminis

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::52e2885a2b5eef36abb84f36c942959d
https://doi.org/10.1182/blood.v80.3.593.bloodjournal803593

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Management of pregnancy in a patient with beta thalassaemia major

Autor: B. Wonke, Alexander J. Butwick, I. Findley

Publikováno v: International journal of obstetric anesthesia. 14(4)

beta thalassaemia is one of the world's most wide-spread monogenetic disorders. Advances in the management of beta thalassaemia major by extensive blood transfusions and chelation therapy have improved survival of patients into adult life. Due to the

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::28e20ab47891b8bb3baf660a826bf7a6
https://pubmed.ncbi.nlm.nih.gov/16140004

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Hepatic iron concentration combined with long-term monitoring of serum ferritin to predict complications of iron overload in thalassaemia major

Autor: P T, Telfer, E, Prestcott, S, Holden, M, Walker, A V, Hoffbrand, B, Wonke

Publikováno v: British journal of haematology. 110(4)

Clinical complications of transfusional iron overload are still common in patients with thalassaemia major (TM) and it is not clear how best to monitor body iron stores during long-term follow-up to anticipate tissue damage. In this study, we have re

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=pmid________::4dcd3a89e183b59a65d6e9b750077963
https://pubmed.ncbi.nlm.nih.gov/11054091

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Lifetime treatment costs of beta-thalassaemia major

Autor: J, Karnon, D, Zeuner, J, Brown, A E, Ades, B, Wonke, B, Modell

Publikováno v: Clinical and laboratory haematology. 21(6)

Beta-thalassaemia major is a serious genetic disorder, which results in a considerable increase in both acute and chronic morbidity, and mortality. Although beta-thalassaemia major is a rare disease affecting approximately 600 people in the UK, treat

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=pmid________::ad777cc7cf341d8f2e3abee752ad52de
https://pubmed.ncbi.nlm.nih.gov/10671989

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Pregnancy management and outcomes in women with thalassaemia major

Autor: S M, Tuck, C E, Jensen, B, Wonke, A, Yardumian

Publikováno v: Journal of pediatric endocrinologymetabolism : JPEM. 11

The health background, management and outcomes of 25 pregnancies in 18 women with transfusion dependent beta thalassaemia are described with particular consideration of appropriate preconceptual guidance for such women. This is an observation study o

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=pmid________::a5d3b01b63f67bcedfda5a2c306cc4ac
https://pubmed.ncbi.nlm.nih.gov/10091166

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