beta-thalassaemia major hos børn og unge i Danmark

Translated title of the contribution: Beta-thalassemia major in children and adolescents in Denmark

Anne Jung, Katharina Maria Main, Elma Scheibel, Birgit Peitersen, Niels Clausen, Gunna Erichsen, Kjeld Schmiegelow, Niels Illum

Research output: Contribution to journalJournal articleResearchpeer-review

Abstract

INTRODUCTION: Beta-thalassemia major occurs with increasing frequency among Danish children as a result of immigration. The aim of the study was to estimate the occurrence of beta-thalassemia major in Denmark, analyse the treatment and organ functions, and identify areas for an improved treatment strategy.

MATERIAL AND METHODS: During 1998-99 all Danish pediatric departments were contacted for identification of children aged 0-18 years with beta-thalassemia major. Blood transfusions and chelation therapy were registered, and for Eastern Denmark clinical, endocrine, cardiac, and serologic parameters were performed.

RESULTS: Twenty-six children had beta-thalassemia major. Out of these, 20 received blood transfusions, and 17 patients were chelated. Eight patients were not chelated owing to previous bone marrow transplantation, treatment with hydroxyurea or ferritin < 2000 micrograms/l and young age. One patient had died. The body height was between 1.5 and -5.4 SDS (median -1.7) and the sitting height was -0.6 to -5.6 SDS (median -2.3). The bone age was delayed 1-5 years (median -2.5) in six out of ten examined patients, and puberty delayed in four out of five. A dilated left ventricle was documented in one out of eight patients examined. All patients were HIV and hepatitis C negative. For 75% of the children, the parents were related.

DISCUSSION: Children and adolescents with beta-thalassemia major in Denmark experience major heterogenicity with regard to treatment and late effects. An earlier and more effective iron chelation therapy together with improved patient support may reduce growth disturbances and endocrine and cardiac late effects.

Original languageDanish
JournalUgeskrift for Laeger
Volume164
Issue number49
Pages (from-to)5803-6
Number of pages4
ISSN0041-5782
Publication statusPublished - 2. Dec 2002

Fingerprint

beta-Thalassemia
Denmark
Chelation Therapy
Delayed Puberty
Hydroxyurea
Emigration and Immigration
Hepatitis C
Bone Marrow Transplantation
Heart Ventricles
Iron
Parents
HIV
Pediatrics

Cite this

Jung, A., Main, K. M., Scheibel, E., Peitersen, B., Clausen, N., Erichsen, G., ... Illum, N. (2002). beta-thalassaemia major hos børn og unge i Danmark. Ugeskrift for Laeger, 164(49), 5803-6.
Jung, Anne ; Main, Katharina Maria ; Scheibel, Elma ; Peitersen, Birgit ; Clausen, Niels ; Erichsen, Gunna ; Schmiegelow, Kjeld ; Illum, Niels. / beta-thalassaemia major hos børn og unge i Danmark. In: Ugeskrift for Laeger. 2002 ; Vol. 164, No. 49. pp. 5803-6.
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Jung, A, Main, KM, Scheibel, E, Peitersen, B, Clausen, N, Erichsen, G, Schmiegelow, K & Illum, N 2002, 'beta-thalassaemia major hos børn og unge i Danmark', Ugeskrift for Laeger, vol. 164, no. 49, pp. 5803-6.

beta-thalassaemia major hos børn og unge i Danmark. / Jung, Anne; Main, Katharina Maria; Scheibel, Elma; Peitersen, Birgit; Clausen, Niels; Erichsen, Gunna; Schmiegelow, Kjeld; Illum, Niels.

In: Ugeskrift for Laeger, Vol. 164, No. 49, 02.12.2002, p. 5803-6.

Research output: Contribution to journalJournal articleResearchpeer-review

TY - JOUR

T1 - beta-thalassaemia major hos børn og unge i Danmark

AU - Jung, Anne

AU - Main, Katharina Maria

AU - Scheibel, Elma

AU - Peitersen, Birgit

AU - Clausen, Niels

AU - Erichsen, Gunna

AU - Schmiegelow, Kjeld

AU - Illum, Niels

PY - 2002/12/2

Y1 - 2002/12/2

N2 - INTRODUCTION: Beta-thalassemia major occurs with increasing frequency among Danish children as a result of immigration. The aim of the study was to estimate the occurrence of beta-thalassemia major in Denmark, analyse the treatment and organ functions, and identify areas for an improved treatment strategy.MATERIAL AND METHODS: During 1998-99 all Danish pediatric departments were contacted for identification of children aged 0-18 years with beta-thalassemia major. Blood transfusions and chelation therapy were registered, and for Eastern Denmark clinical, endocrine, cardiac, and serologic parameters were performed.RESULTS: Twenty-six children had beta-thalassemia major. Out of these, 20 received blood transfusions, and 17 patients were chelated. Eight patients were not chelated owing to previous bone marrow transplantation, treatment with hydroxyurea or ferritin < 2000 micrograms/l and young age. One patient had died. The body height was between 1.5 and -5.4 SDS (median -1.7) and the sitting height was -0.6 to -5.6 SDS (median -2.3). The bone age was delayed 1-5 years (median -2.5) in six out of ten examined patients, and puberty delayed in four out of five. A dilated left ventricle was documented in one out of eight patients examined. All patients were HIV and hepatitis C negative. For 75% of the children, the parents were related.DISCUSSION: Children and adolescents with beta-thalassemia major in Denmark experience major heterogenicity with regard to treatment and late effects. An earlier and more effective iron chelation therapy together with improved patient support may reduce growth disturbances and endocrine and cardiac late effects.

AB - INTRODUCTION: Beta-thalassemia major occurs with increasing frequency among Danish children as a result of immigration. The aim of the study was to estimate the occurrence of beta-thalassemia major in Denmark, analyse the treatment and organ functions, and identify areas for an improved treatment strategy.MATERIAL AND METHODS: During 1998-99 all Danish pediatric departments were contacted for identification of children aged 0-18 years with beta-thalassemia major. Blood transfusions and chelation therapy were registered, and for Eastern Denmark clinical, endocrine, cardiac, and serologic parameters were performed.RESULTS: Twenty-six children had beta-thalassemia major. Out of these, 20 received blood transfusions, and 17 patients were chelated. Eight patients were not chelated owing to previous bone marrow transplantation, treatment with hydroxyurea or ferritin < 2000 micrograms/l and young age. One patient had died. The body height was between 1.5 and -5.4 SDS (median -1.7) and the sitting height was -0.6 to -5.6 SDS (median -2.3). The bone age was delayed 1-5 years (median -2.5) in six out of ten examined patients, and puberty delayed in four out of five. A dilated left ventricle was documented in one out of eight patients examined. All patients were HIV and hepatitis C negative. For 75% of the children, the parents were related.DISCUSSION: Children and adolescents with beta-thalassemia major in Denmark experience major heterogenicity with regard to treatment and late effects. An earlier and more effective iron chelation therapy together with improved patient support may reduce growth disturbances and endocrine and cardiac late effects.

KW - Adolescent

KW - Blood Transfusion

KW - Child

KW - Child, Preschool

KW - Consanguinity

KW - Denmark

KW - Emigration and Immigration

KW - Female

KW - Humans

KW - Infant

KW - Iron Chelating Agents

KW - Male

KW - beta-Thalassemia

M3 - Tidsskriftartikel

C2 - 12523224

VL - 164

SP - 5803

EP - 5806

JO - Ugeskrift for Laeger

JF - Ugeskrift for Laeger

SN - 0041-5782

IS - 49

ER -

Jung A, Main KM, Scheibel E, Peitersen B, Clausen N, Erichsen G et al. beta-thalassaemia major hos børn og unge i Danmark. Ugeskrift for Laeger. 2002 Dec 2;164(49):5803-6.