Evaluation of Iliac and Renal Artery for Mechanism of Intracranial Aneurysm in ADPKD

NCT ID: NCT03726463

Last Updated: 2019-03-11

Basic Information

• Recruitment Status: UNKNOWN
• Total Enrollment: 100 participants
• Study Classification: OBSERVATIONAL
• Study Start Date: 2018-12-20
• Study Completion Date: 2023-11-30

Brief Summary

ADPKD is the most common form of hereditary kidney disease and is known to occur in 1 of 400 to 1000 population in the U.S. ADPKD consists of 2.8% of patients receiving kidney transplantation in our center. It is known that ADPKD is associated with vascular anomalies, including abdominal aneurysms, valvular anomalies and especially intracranial aneurysms. Intracranial aneurysms occur in 9~12% of the ADPKD population which is higher than 2~3% in the general population and is known to be associated with PKD1 or PKD2 heritage.

Until now, most of the studies regarding intracranial aneurysms in ADPKD are conducted in animal models, and there are only few cellular studies conducted from human samples. While performing kidney transplantation to ESRD ADPKD patients, arterial tissues from nephrectomy specimens can be obtained. The objective of this study is to investigate the mechanism of intracranial aneurysm in ADPKD patients by analyzing iliac and renal artery characteristics.

Detailed Description

ADPKD is associated with PKD1 gene on chromosome 16 and PKD2 gene on chromosome 4 and these gene respectively code polycystin 1 and polycystin 2. Currently the hypotheses for increased intracranial aneurysm rate in ADPKD patients is that mutation of polycystin is not only confined to nephron tissues but also in endothelial cells and vascular smooth muscle cells and results in mutation of vascular phenotype. Also recent studies show polycystin complex causes cystic changes through mutation in primary cilia in renal epithelium. Wild type endothelial cells respond to fluid shear stress by regulating levels of intracellular calcium and nitric oxide, however, PKD1 or PKD2 mutation in fetal aortic endothelial cells revealed loss of these responses.

During kidney transplantation, bilateral nephrectomies are routinely performed to ADPKD patients. In this study, blood, urine, iliac artery and renal artery tissues will be collected from ADPKD patients receiving kidney transplantation to analyze the arterial characteristic and gene mutation of ADPKD patients. The aim of this study is to evaluate mechanisms associated with intracranial aneurysm occurence in ADPKD patients by analyzing the genetic mutation and vascular deformities of these patients.

Conditions

Kidney Transplant; Complications; Polycystic Kidney Diseases; Aneurysm, Brain

Study Design

• Observational Model Type: COHORT
• Study Time Perspective: PROSPECTIVE

Study Groups

polycystic kidney disease

patients with polycystic kidney disease who receive kidney transplantation at Asan Medical Center

Kidney transplantation

Intervention Type: PROCEDURE

Bilateral nephrectomy of polycystic kidneys are routinely performed during kidney transplantation in ADPKD patients

Interventions

Kidney transplantation

Bilateral nephrectomy of polycystic kidneys are routinely performed during kidney transplantation in ADPKD patients

Intervention Type: PROCEDURE

Eligibility Criteria

Inclusion Criteria

• ADPKD patients from ages 18 to 80 receiving kidney transplantation at Asan Medical Center

Exclusion Criteria

• those who refuse or are unable to provide consent form
• pregnancy

• Minimum Eligible Age: 18 Years
• Maximum Eligible Age: 80 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: No

Sponsors

Asan Medical Center

OTHER

Sponsor Role: lead

Responsible Party

Sung Shin

Associate Professor

Responsibility Role: PRINCIPAL_INVESTIGATOR

Locations

Asan Medical Center

Seoul, , South Korea

Site Status: RECRUITING

Countries

South Korea

Central Contacts

Sung Shin, MD, PhD

Role: CONTACT

sshin@amc.seoul.kr

82-2-3010-3964 ext. 3964

Facility Contacts

SUNG SHIN, Dr.

Role: primary

References

Iglesias CG, Torres VE, Offord KP, Holley KE, Beard CM, Kurland LT. Epidemiology of adult polycystic kidney disease, Olmsted County, Minnesota: 1935-1980. Am J Kidney Dis. 1983 May;2(6):630-9. doi: 10.1016/s0272-6386(83)80044-4.

Reference Type: BACKGROUND

PMID: 6846334 (View on PubMed)

Collins AJ, Foley RN, Chavers B, Gilbertson D, Herzog C, Johansen K, Kasiske B, Kutner N, Liu J, St Peter W, Guo H, Gustafson S, Heubner B, Lamb K, Li S, Li S, Peng Y, Qiu Y, Roberts T, Skeans M, Snyder J, Solid C, Thompson B, Wang C, Weinhandl E, Zaun D, Arko C, Chen SC, Daniels F, Ebben J, Frazier E, Hanzlik C, Johnson R, Sheets D, Wang X, Forrest B, Constantini E, Everson S, Eggers P, Agodoa L. 'United States Renal Data System 2011 Annual Data Report: Atlas of chronic kidney disease & end-stage renal disease in the United States. Am J Kidney Dis. 2012 Jan;59(1 Suppl 1):A7, e1-420. doi: 10.1053/j.ajkd.2011.11.015. No abstract available.

Reference Type: BACKGROUND

PMID: 22177944 (View on PubMed)

Torres VE, Harris PC, Pirson Y. Autosomal dominant polycystic kidney disease. Lancet. 2007 Apr 14;369(9569):1287-1301. doi: 10.1016/S0140-6736(07)60601-1.

Reference Type: BACKGROUND

PMID: 17434405 (View on PubMed)

Ecder T, Schrier RW. Cardiovascular abnormalities in autosomal-dominant polycystic kidney disease. Nat Rev Nephrol. 2009 Apr;5(4):221-8. doi: 10.1038/nrneph.2009.13.

Reference Type: BACKGROUND

PMID: 19322187 (View on PubMed)

Other Identifiers

2018-1210

Identifier Type: -

Identifier Source: org_study_id