Kunz, Joachim B., Lobitz, Stephan ORCID: 0000-0001-5398-0610, Grosse, Regine, Oevermann, Lena, Hakimeh, Dani, Jarisch, Andrea, Cario, Holger ORCID: 0000-0002-6923-488X, Beier, Rita, Schenk, Daniela, Schneider, Dominik, Gross-Wieltsch, Ute, Prokop, Aram, Heine, Sabine, Khurana, Claudia, Erlacher, Miriam, Duerken, Matthias, Linke, Christina, Fruehwald, Michael, Corbacioglu, Selim, Claviez, Alexander, Metzler, Markus, Ebinger, Martin ORCID: 0000-0002-4229-8058, Full, Hermann, Wiesel, Thomas, Eberl, Wolfgang, Reinhard, Harald, Tagliaferri, Laura, Allard, Pierre, Karapanagiotou-Schenkel, Irini, Rother, Lisa-Marie, Beck, Dorothea, Le Cornet, Lucian and Kulozik, Andreas E. (2020). Sickle cell disease in Germany: Results from a national registry. Pediatr. Blood Cancer, 67 (4). HOBOKEN: WILEY. ISSN 1545-5017

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Abstract

Background Limited data on the prevalence and medical care of sickle cell disease (SCD) in Germany are available. Here, we make use of a patient registry to characterize the burden of disease and the treatment modalities for patients with SCD in Germany. Procedure A nationwide German registry for patients with SCD documents basic data on diagnosis and patient history retrospectively at the time of registration. A prospective annual documentation provides more details on complications and treatment of SCD. For the current analyses, data of 439 patients were available. Results Most patients had homozygous SCD (HbSS 75.1%, HbS/beta-thalassemia 13.2%, and HbSC 11.3%). The median age at diagnosis was 1.9 years (interquartile range, 0.6-4.4 years), most patients were diagnosed when characteristic symptoms occurred. Sepsis and stroke had affected 3.2% and 4.2% of patients, respectively. During the first year of observation, 48.3% of patients were admitted to a hospital and 10.1% required intensive care. Prophylactic penicillin was prescribed to 95.6% of patients with homozygous SCD or HbS/beta thalassemia below the age of six and hydroxycarbamide to 90.4% of patients above the age of two years. At least one annual transcranial Doppler ultrasound was documented for 74.8% of patients between 2 and 18 years. Conclusion With an estimated number of at least 2000, the prevalence of SCD in Germany remains low. Prospectively, we expect that the quality of care for children with SCD will be further improved by an earlier diagnosis after the anticipated introduction of a newborn screening program for SCD.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Kunz, Joachim B.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lobitz, StephanUNSPECIFIEDorcid.org/0000-0001-5398-0610UNSPECIFIED
Grosse, RegineUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Oevermann, LenaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hakimeh, DaniUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Jarisch, AndreaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Cario, HolgerUNSPECIFIEDorcid.org/0000-0002-6923-488XUNSPECIFIED
Beier, RitaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schenk, DanielaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schneider, DominikUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gross-Wieltsch, UteUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Prokop, AramUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Heine, SabineUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Khurana, ClaudiaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Erlacher, MiriamUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Duerken, MatthiasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Linke, ChristinaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Fruehwald, MichaelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Corbacioglu, SelimUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Claviez, AlexanderUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Metzler, MarkusUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Ebinger, MartinUNSPECIFIEDorcid.org/0000-0002-4229-8058UNSPECIFIED
Full, HermannUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Wiesel, ThomasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Eberl, WolfgangUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Reinhard, HaraldUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Tagliaferri, LauraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Allard, PierreUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Karapanagiotou-Schenkel, IriniUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Rother, Lisa-MarieUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Beck, DorotheaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Le Cornet, LucianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kulozik, Andreas E.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-124057
DOI: 10.1002/pbc.28130
Journal or Publication Title: Pediatr. Blood Cancer
Volume: 67
Number: 4
Date: 2020
Publisher: WILEY
Place of Publication: HOBOKEN
ISSN: 1545-5017
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
CHILDREN; HYDROXYUREA; PREVENTION; ANEMIA; MANAGEMENT; MORTALITY; SURVIVAL; STROKE; COHORTMultiple languages
Oncology; Hematology; PediatricsMultiple languages
Refereed: Yes
URI: http://kups.ub.uni-koeln.de/id/eprint/12405

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