European Calciphylaxis Registry Network

NCT ID: NCT02635373

Last Updated: 2016-10-14

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

UNKNOWN

Total Enrollment

1000 participants

Study Classification

OBSERVATIONAL

Study Start Date

2015-12-31

Study Completion Date

2020-12-31

Brief Summary

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Observational, registry, prospective, non-interventional collection of CUA patient data. Patient treatment is carried out within clinical routine, at the discretion of the physicians and according to existing treatment guidelines. Participating physicians will not be subject to any instructions with regard to the diagnosis and therapy of their patients.

Detailed Description

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Calcific uremic arteriolopathy (CUA, calciphylaxis) is a rare disease (ORPHA280062) with dramatically high mortality characterized clinically by the occurrence of painful debilitating cutaneous lesions and ulcerations. Median survival time is about 1.5 years. Many uncertainties still exist about risk factors and optimal therapy. CUA mainly occurs in patients with severe renal insufficiency or ESRD.

The aim of the present initiative is to establish an international (European) observational registry for CUA patients. This registry will help to reach a critical mass of CUA patients, will allow novel insights into pathophysiology, and will help establishing systematically an overview upon risk factors and long-term outcome of CUA patients. The design of the international registry is supposed to adopt the structure of the presently running nation-wide German calciphylaxis registry.

The investigators plan to initiate a registry in which treating physicians can provide data upon demographics, clinical picture, comorbidities, medical treatments and laboratory data at the time of diagnosis and serial follow-up time points. In addition investigators will build-up a biobank for full blood, serum and plasma samples as well as tissue samples.

The registry will allow investigations regarding potential subgroups of patients (proximal versus distal forms of CUA), geno-phenotype correlations, description of international state-of-the-art treatment, and identification of preventive tools, risk factors and biomarkers for estimation of prognosis.

Furthermore, the registry will be the nidus for further scientific exchange between experts and clinicians on the field of uremic vascular calcification.

Conditions

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Calcific Uremic Arteriolopathy (CUA) Calciphylaxis

Study Design

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Study Time Perspective

PROSPECTIVE

Eligibility Criteria

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Inclusion Criteria

* diagnosed CUA according to the judgement of locally treating physicians, who have the option to ask for external diagnostic advice from the country-specific sub-investigators
* Male or female aged \> 18 years
* Written informed consent prior to study participation
* The subject is not mentally or legally incapacitated

Exclusion Criteria

• The subject is not able to give informed consent
Minimum Eligible Age

18 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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RWTH Aachen University

OTHER

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Locations

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Department of Medicine, Division of Cardiology, Pulmonary Diseases and Vascular Medicine at the University Hospital, RWTH Aachen

Aachen, North Rhine-Westphalia, Germany

Site Status RECRUITING

Countries

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Germany

Central Contacts

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Vincent Brandenburg, MD, Prof.

Role: CONTACT

+49 (0)241/80 ext. 36072

Facility Contacts

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Verena Deserno, M.Sc.

Role: primary

+49 241 80 ext. 80092

References

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Weenig RH. Pathogenesis of calciphylaxis: Hans Selye to nuclear factor kappa-B. J Am Acad Dermatol. 2008 Mar;58(3):458-71. doi: 10.1016/j.jaad.2007.12.006. Epub 2008 Jan 18.

Reference Type BACKGROUND
PMID: 18206262 (View on PubMed)

Brandenburg VM, Cozzolino M, Ketteler M. Calciphylaxis: a still unmet challenge. J Nephrol. 2011 Mar-Apr;24(2):142-8. doi: 10.5301/jn.2011.6366.

Reference Type BACKGROUND
PMID: 21337312 (View on PubMed)

Meissner M, Gille J, Kaufmann R. Calciphylaxis: no therapeutic concepts for a poorly understood syndrome? J Dtsch Dermatol Ges. 2006 Dec;4(12):1037-44. doi: 10.1111/j.1610-0387.2006.06127.x. English, German.

Reference Type BACKGROUND
PMID: 17176411 (View on PubMed)

Hafner J, Keusch G, Wahl C, Sauter B, Hurlimann A, von Weizsacker F, Krayenbuhl M, Biedermann K, Brunner U, Helfenstein U. Uremic small-artery disease with medial calcification and intimal hyperplasia (so-called calciphylaxis): a complication of chronic renal failure and benefit from parathyroidectomy. J Am Acad Dermatol. 1995 Dec;33(6):954-62. doi: 10.1016/0190-9622(95)90286-4.

Reference Type BACKGROUND
PMID: 7490365 (View on PubMed)

Mazhar AR, Johnson RJ, Gillen D, Stivelman JC, Ryan MJ, Davis CL, Stehman-Breen CO. Risk factors and mortality associated with calciphylaxis in end-stage renal disease. Kidney Int. 2001 Jul;60(1):324-32. doi: 10.1046/j.1523-1755.2001.00803.x.

Reference Type BACKGROUND
PMID: 11422768 (View on PubMed)

Janigan DT, Hirsch DJ, Klassen GA, MacDonald AS. Calcified subcutaneous arterioles with infarcts of the subcutis and skin ("calciphylaxis") in chronic renal failure. Am J Kidney Dis. 2000 Apr;35(4):588-97. doi: 10.1016/s0272-6386(00)70003-5.

Reference Type BACKGROUND
PMID: 10739777 (View on PubMed)

Kramann R, Brandenburg VM, Schurgers LJ, Ketteler M, Westphal S, Leisten I, Bovi M, Jahnen-Dechent W, Knuchel R, Floege J, Schneider RK. Novel insights into osteogenesis and matrix remodelling associated with calcific uraemic arteriolopathy. Nephrol Dial Transplant. 2013 Apr;28(4):856-68. doi: 10.1093/ndt/gfs466. Epub 2012 Dec 6.

Reference Type BACKGROUND
PMID: 23223222 (View on PubMed)

Fine A, Zacharias J. Calciphylaxis is usually non-ulcerating: risk factors, outcome and therapy. Kidney Int. 2002 Jun;61(6):2210-7. doi: 10.1046/j.1523-1755.2002.00375.x.

Reference Type BACKGROUND
PMID: 12028462 (View on PubMed)

Weenig RH, Sewell LD, Davis MD, McCarthy JT, Pittelkow MR. Calciphylaxis: natural history, risk factor analysis, and outcome. J Am Acad Dermatol. 2007 Apr;56(4):569-79. doi: 10.1016/j.jaad.2006.08.065. Epub 2006 Dec 1.

Reference Type BACKGROUND
PMID: 17141359 (View on PubMed)

Brandenburg VM, Floege J, Ketteler M. Kalzifizierende urämische Arteriolopathie. Der Nephrologe 2009; 4(1):65-66

Reference Type BACKGROUND

Krueger T, Westenfeld R, Schurgers L, Brandenburg V. Coagulation meets calcification: the vitamin K system. Int J Artif Organs. 2009 Feb;32(2):67-74. doi: 10.1177/039139880903200202.

Reference Type BACKGROUND
PMID: 19363777 (View on PubMed)

Jahnen-Dechent W, Schafer C, Ketteler M, McKee MD. Mineral chaperones: a role for fetuin-A and osteopontin in the inhibition and regression of pathologic calcification. J Mol Med (Berl). 2008 Apr;86(4):379-89. doi: 10.1007/s00109-007-0294-y. Epub 2007 Dec 15.

Reference Type BACKGROUND
PMID: 18080808 (View on PubMed)

Cranenburg EC, Vermeer C, Koos R, Boumans ML, Hackeng TM, Bouwman FG, Kwaijtaal M, Brandenburg VM, Ketteler M, Schurgers LJ. The circulating inactive form of matrix Gla Protein (ucMGP) as a biomarker for cardiovascular calcification. J Vasc Res. 2008;45(5):427-36. doi: 10.1159/000124863. Epub 2008 Apr 10.

Reference Type BACKGROUND
PMID: 18401181 (View on PubMed)

Schafer C, Heiss A, Schwarz A, Westenfeld R, Ketteler M, Floege J, Muller-Esterl W, Schinke T, Jahnen-Dechent W. The serum protein alpha 2-Heremans-Schmid glycoprotein/fetuin-A is a systemically acting inhibitor of ectopic calcification. J Clin Invest. 2003 Aug;112(3):357-66. doi: 10.1172/JCI17202.

Reference Type BACKGROUND
PMID: 12897203 (View on PubMed)

Other Identifiers

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13-093

Identifier Type: -

Identifier Source: org_study_id

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