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DOI: 10.1055/s-2006-933406
© Georg Thieme Verlag Stuttgart · New York
Sichelzellkrankheit in Deutschland - Krankheitsmanifestationen, Therapieoptionen und Möglichkeiten der Optimierung der Betreuung
Sickle Cell Disease in Germany - Disease Manifestations, Therapeutic Options and Possibilities for Improvement of CarePublikationsverlauf
Publikationsdatum:
10. Mai 2006 (online)
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Zusammenfassung
Die Zahl der Sichelzellpatienten in den europäischen Ländern steigt stetig an aufgrund der anhaltenden Zuwanderung aus Risikoländern für Hämoglobinkrankheiten und durch die verbesserte Lebenserwartung der Patienten. In Deutschland lebten im Jahr 2005 möglicherweise bis zu 1 000 Sichelzellpatienten, überwiegend in den alten Bundesländern. Wir verfügen über keine genauen Zahlen, da die seit 1987 angebotene Registrierung von Sichelzellpatienten in der deutschen Verlaufs-Beobachtungs-Studie auf freiwilliger Basis erfolgte. Im Dezember 2005 wurden von den seit 1987 registrierten 514 Patienten noch 273 (208 Kinder, 65 Erwachsene) in 92 Kliniken/Praxen behandelt, verstreut über das gesamte Gebiet der Bundesrepublik. Wir berichten über die Herkunft dieser Patienten, ihre Genotypen, Krankheitsmanifestationen, soziale Integration, Therapie und Todesursachen, zeigen die Schwierigkeiten bei der Betreuung von Sichelzellpatienten in Deutschland auf und schlagen Möglichkeiten der Optimierung der Betreuung vor.
Abstract
The number of sickle cell patients in Europe is steadily increasing due to continuing immigration and increase of average life expectancy. In 2005 probably up to 1 000 sickle cell patients were living in Germany, most of them in former West-Germany. Precise figures are not available as registration of sickle cell patients into a German surveillance study register that was initiated 1987 is on a voluntary basis. In December 2005 the register included a total of 514 patients, with 273 patients (208 children, 65 adults) still actively participating in the study. They were followed at 92 different institutions all over the country. We report on data regarding the origin of these patients, their genotypes, disease manifestations, social integration, therapy and causes of death. We discuss difficulties in the delivery of care and make suggestions for improvement.
Schlüsselwörter
Sichelzellkrankheit - Milzsequestration - akutes Thorax-Syndrom
Key words
sickle cell disease - splenic sequestration - acute chest syndrome
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R. Dickerhoff
Asklepios Kinderklinik
53754 St. Augustin
Telefon: 0 22 41/24 91
Fax: 0 22 41/24 92 72
eMail: roswitha.dickerhoff@uni-bonn.de