Academic Journal
PF739 SICKLE CELL DISEASE IN GERMANY IS HETEROGENEOUS AND RESULTS IN SEVERE MORBIDITY: ANALYSES FROM A NATIONAL REGISTRY
| العنوان: | PF739 SICKLE CELL DISEASE IN GERMANY IS HETEROGENEOUS AND RESULTS IN SEVERE MORBIDITY: ANALYSES FROM A NATIONAL REGISTRY |
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| المؤلفون: | Kunz, J., Lobitz, S., Andrea, J., Regine, G., Cario, H., Oevermann, L., Hakimeh, D., Le Cornet, L., Beck, D., Karapanagiotou‐Schenkel, I., Tagliaferri, L., Kulozik, A. |
| المصدر: | HemaSphere ; volume 3, issue S1, page 323-324 ; ISSN 2572-9241 2572-9241 |
| بيانات النشر: | Wiley |
| سنة النشر: | 2019 |
| المجموعة: | Wiley Online Library (Open Access Articles via Crossref) |
| الوصف: | Background: Sickle Cell Disease (SCD) in Germany exclusively affects immigrants and their descendants. Its increasing prevalence results in challenges for medical care for these patients. Aims: The registry aims at describing the epidemiology, clinical course and treatment of patients with SCD in Germany. Methods: To date, 367 patients have been included in the registry by 36 mainly pediatric institutions. At initial registration, basic data including date of birth, date and circumstances of diagnosis, genotype, country of origin, blood type and complications in the patient's history are reported. Annual follow‐ups document details on complications, laboratory parameters and treatment. Data of 327 patients were analyzed for this abstract. Results: Genotypes of registry patients were homozygous SCD in 75% of patients, HbSC disease in 12%, HbS/β 0 thal in 6% and HbS/β + thal in 7%. The main regions of origin were sub‐Saharan Africa (in total 69% of patients, including Ghana 19%, Nigeria 19%, Togo 9%) and the Middle East (in total 21%, including Lebanon 6%, Turkey 5%, Syria 4%). Although pilot projects for newborn screening for SCD were in place in some of the centers, only 35% of patients were diagnosed during their first year of life, 85% before the age of six. SCD was diagnosed on the occasion of presenting symptoms in 60% of patients. The mean age at registration was 12 years (median 10), with 10% of patients being older than 21 years at registration. The most frequent complications in the patients’ prior history were pain crises with hospitalization (67% of patients), acute chest syndrome (32%), splenic sequestration (15%), aplastic crisis (10%), stroke (4%) and sepsis (4%). During the first year of observation, 47% of the registry patients were hospitalized, 11% required treatment on an intensive care unit. Red blood cell transfusions were given to 27% of patients. 96% of patients with homozygous SCD or with HbS/β 0 thal at an age of 2 years or older were prescribed hydroxycarbamide, 76% of these patients ... |
| نوع الوثيقة: | article in journal/newspaper |
| اللغة: | English |
| DOI: | 10.1097/01.hs9.0000561240.27342.da |
| DOI: | 10.1097/01.HS9.0000561240.27342.da |
| الاتاحة: | http://dx.doi.org/10.1097/01.hs9.0000561240.27342.da https://onlinelibrary.wiley.com/doi/pdf/10.1097/01.HS9.0000561240.27342.da |
| Rights: | http://onlinelibrary.wiley.com/termsAndConditions#vor |
| رقم الانضمام: | edsbas.7F64C531 |
| قاعدة البيانات: | BASE |
| DOI: | 10.1097/01.hs9.0000561240.27342.da |
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