Sichelzellkrankheit

Zusammenfassung

Die Sichelzellkrankheit ist global gesehen eine der häufigsten Erbkrankheiten überhaupt. Betroffen sind überwiegend Menschen aus dem subsaharischen West- und Zentralafrika; aber auch in bestimmten Mittelmeeranrainerstaaten, im Nahen Osten und in Teilen Indiens leben viele Erkrankte. Durch Migration nehmen die Patientenzahlen auch in Mittel- und Nordeuropa kontinuierlich zu. Die Sichelzellkrankheit ist eine extrem komplexe Erkrankung. Für die meisten Komplikationen stehen nur symptomatische Therapien zur Verfügung. Die einzige kurative, aber nur sehr zurückhal-tend angewendete Behandlungsoption ist die Stammzelltransplantation.

Dieser Artikel fasst die wichtigsten Informationen über die Sichelzellkrankheit zusammen, die der niedergelassene Kinderarzt für seinen Praxisalltag benötigt.

Summary

Globally, sickle cell disease is one of the commonest genetic diseases. It is predominantly found in individuals from sub-Saharan West and Central Africa. However, several Mediterranean countries, the Middle East and parts of India are affected as well. Due to migration, the number of patients in Middle and Northern Europe increases continuously. Sickle cell disease is very complex. For most complications, only symptomatic therapies are available. The only curative treatment, though used very restrictively, is haematopoietic stem cell transplantation.

This article summarizes the most important information on sickle cell disease that paediatricians in private practise require.

Schlüsselwörter

Sichelzellkrankheit - Stammzelltransplantation - Neugeborenenscreening

Keywords

Sickle cell disease - stem cell transplantation - newborn screening

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