Sickle-cell Disease Registry of the GPOH

Sickle Cell Disease Clinical Trial

— SichReg  

Official title:

Register Sichelzellkrankheit Der GPOH

Clinical Trial Details — Status: Recruiting

Administrative data

• NCT number: NCT03327428
• Other study ID #: Register Sichelzellkrankheit
• Status: Recruiting
• Start date: December 15, 2016
• Est. completion date: December 31, 2040

Study information

• Verified date: November 2023
• Source: University Hospital Heidelberg
• Contact: Joachim Kunz, Dr.
• Phone: 06221 56 4555
• Email: Joachim.Kunz[@]med.uni-heidelberg.de
• Is FDA regulated: No
• Study type: Observational [Patient Registry]

Clinical Trial 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/

Recruitment information / eligibility

• Status: Recruiting
• Enrollment: 1000
• Est. completion date: December 31, 2040
• Est. primary completion date: December 31, 2026
• Accepts healthy volunteers: No
• Gender: All
• Age group: 0 Years to 100 Years

Eligibility

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

Related Conditions & MeSH terms

• Anemia, Sickle Cell
• Sickle Cell Disease

Locations

Country	Name	City	State
Germany	Center for Child and Adolescent Medicine, University Medical Center Heidelberg	Heidelberg	BW

Sponsors (9)

Lead Sponsor	Collaborator
University Hospital Heidelberg	Charite University, Berlin, Germany, Deutsche Kinderkrebsstiftung, German Cancer Research Center, GPOH Consortium Sickle Cell Disease, Johann Wolfgang Goethe University Hospital, Pfizer, Universitätsklinikum Hamburg-Eppendorf, University Hospital Ulm

Country where clinical trial is conducted

Germany, 

References & Publications (9)

Allard P, Alhaj N, Lobitz S, Cario H, Jarisch A, Grosse R, Oevermann L, Hakimeh D, Tagliaferri L, Kohne E, Kopp-Schneider A, Kulozik AE, Kunz JB. Genetic modifiers of fetal hemoglobin affect the course of sickle cell disease in patients treated with hydro — View Citation

Frommel C, Brose A, Klein J, Blankenstein O, Lobitz S. Newborn screening for sickle cell disease: technical and legal aspects of a German pilot study with 38,220 participants. Biomed Res Int. 2014;2014:695828. doi: 10.1155/2014/695828. Epub 2014 Jul 23. — View Citation

Grosse R, Lukacs Z, Cobos PN, Oyen F, Ehmen C, Muntau B, Timmann C, Noack B. The Prevalence of Sickle Cell Disease and Its Implication for Newborn Screening in Germany (Hamburg Metropolitan Area). Pediatr Blood Cancer. 2016 Jan;63(1):168-70. doi: 10.1002/pbc.25706. Epub 2015 Aug 14. — View Citation

Kunz JB, Awad S, Happich M, Muckenthaler L, Lindner M, Gramer G, Okun JG, Hoffmann GF, Bruckner T, Muckenthaler MU, Kulozik AE. Significant prevalence of sickle cell disease in Southwest Germany: results from a birth cohort study indicate the necessity for newborn screening. Ann Hematol. 2016 Feb;95(3):397-402. doi: 10.1007/s00277-015-2573-y. Epub 2015 Dec 12. — View Citation

Kunz JB, Cario H, Grosse R, Jarisch A, Lobitz S, Kulozik AE. The epidemiology of sickle cell disease in Germany following recent large-scale immigration. Pediatr Blood Cancer. 2017 Jul;64(7). doi: 10.1002/pbc.26550. Epub 2017 Apr 6. — View Citation

Kunz JB, Lobitz S, Grosse R, Oevermann L, Hakimeh D, Jarisch A, Cario H, Beier R, Schenk D, Schneider D, Gross-Wieltsch U, Prokop A, Heine S, Khurana C, Erlacher M, Durken M, Linke C, Fruhwald M, Corbacioglu S, Claviez A, Metzler M, Ebinger M, Full H, Wie — View Citation

Kunz JB, Schlotmann A, Daubenbuchel A, Lobitz S, Jarisch A, Grosse R, Cario H, Oevermann L, Hakimeh D, Tagliaferri L, Kulozik AE. Benefits of a Disease Management Program for Sickle Cell Disease in Germany 2011-2019: The Increased Use of Hydroxyurea Corre — View Citation

Lobitz S, Frommel C, Brose A, Klein J, Blankenstein O. Incidence of sickle cell disease in an unselected cohort of neonates born in Berlin, Germany. Eur J Hum Genet. 2014 Aug;22(8):1051-3. doi: 10.1038/ejhg.2013.286. Epub 2014 Jan 8. — View Citation

Lobitz S. Neugeborenenscreening auf Sichelzellkrankheiten in Deutschland. Kinder- und Jugendmedizin 2017;17(2):82-86 [German]

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Change in incidence of sickle-cell disease	The incidence of sickle-cell disease will be reported every year in comparison to the preceding Report.	Baseline and yearly, up to 10 years
Secondary	Complications of sickle-cell disease	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).	Baseline and yearly, up to 10 years
Secondary	Treatment of sickle-cell disease	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).	Baseline and yearly, up to 10 years

External Links

•   Official website of the Registry [German]
•   Sickle Cell Disease Guidelines, German language