Sickle Cell Disease
Conditions
Keywords
Anemia, Sickle Cell, Sickle Cell disease
Brief summary
Sickle cell disease is one of the most common hereditary diseases. Most severe complications can be avoided if the disease is detected early and treated appropriately. The sickle cell disease registry of the Society for Paediatric Oncology/Haematology aims at describing the epidemiology of sickle cell disease in German-speaking central Europe. Patients with sickle cell disease will be characterized clinically and genetically and treatment will be documented with the aim to find predictors of the course of disease. In addition, the registry results should provide a solid evidence base to incorporate sickle cell disease into routine newborn screening and to update the national guidelines for the management of patients suffering from sickle cell disease in Germany. A consortium of five university hospitals (Berlin, Frankfurt, Hamburg, Heidelberg, Ulm) has been mandated by the Society for Paediatric Oncology/Haematology to implement this registry. The number of participating centers is constantly increasing and new centers that take care of either pediatric or adult patients with sickle cell disease are encouraged to support the registry. For further information please refer to: http://www.sichelzellkrankheit.info/
Interventions
None listed
Sponsors
GPOH Consortium Sickle Cell Disease
Johann Wolfgang Goethe University Hospital
Universitätsklinikum Hamburg-Eppendorf
University Hospital Ulm
Charite University, Berlin, Germany
German Cancer Research Center
Deutsche Kinderkrebsstiftung
Pfizer
University Hospital Heidelberg
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
0 Years to 100 Years
Healthy volunteers
No
Inclusion criteria
* signed informed consent * current residency in either Germany, Austria or Switzerland * sickle cell disease confirmed by hemoglobin analysis or molecular genetic analysis * Homozygous sickle cell disease (HbSS) * HbSC disease * Sickle cell disease HbS / bThal * Other, rare sickle cell syndromes such as HbS/OArab, HbS/HPFH, HbS/E, HbS/D Punjab, HbS/C Harlem, HbC/S Antilles, HbS/Quebec-CHORI, HbA/S Oman, HbA/Jamaica Plain
Exclusion criteria
\- isolated heterozygous trait for HbS
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Change in incidence of sickle-cell disease | Baseline and yearly, up to 10 years | The incidence of sickle-cell disease will be reported every year in comparison to the preceding Report. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Complications of sickle-cell disease | Baseline and yearly, up to 10 years | In addition to the incidence of the disease itself also possible complications will be reported in comparison to the preceding report (in case of the first report, only the prevalence will be reported as baseline). |
| Treatment of sickle-cell disease | Baseline and yearly, up to 10 years | In addition to the incidence of the disease itself also the treatment received will be reported in comparison to the preceding report (in case of the first report, only the prevalence will be reported as baseline). |
Countries
Germany
Contacts
Primary ContactJoachim Kunz, Dr.
Backup ContactLaura Tagliaferri, Dr.
Outcome results
None listed