Clinical Trials Logo

Clinical Trial Details — Status: Completed

Administrative data

NCT number NCT02176603
Other study ID # PKU-EnDys
Secondary ID
Status Completed
Phase N/A
First received June 26, 2014
Last updated February 22, 2017
Start date May 2014
Est. completion date January 1, 2017

Study information

Verified date February 2017
Source Charite University, Berlin, Germany
Contact n/a
Is FDA regulated No
Health authority
Study type Observational

Clinical Trial Summary

The purpose of this study is to determine whether adolescent and adult patients with phenylketonuria who are on long-term phenylalanine-restricted diet suffer from endothelial dysfunction.


Description:

The aim of the study is the examination of endothelial dysfunction and oxidative stress markers in adolescent and adult patients with phenylketonuria (PKU), in correlation to phenylalanine values und phenylalanine-restricted diet. 25-50 patients with PKU aged ≥16 years of age and a control group of 25 age- and sex-matched healthy volunteers will be included in the study.

Endothelial dysfunction will be measured by venous occlusion plethysmography on the lower legs in the PKU patients and in the control group. Wave velocity, aortic stiffness, and intima media thickness of the common carotid artery will be measured by vicorder and duplex ultrasound scanner in the PKU patients and in the control group. Analyses of phenylalanine metabolites and renal function will be performed only in the PKU patients, analyses of oxidative stress markers in the PKU patients and in the control group. Dietary protocols of the patients with PKU will be calculated for their daily phenylalanine, total protein, and calorie intake. Results of the study will be kept in the medical records and paper case report forms. Data will be analyzed only in pseudonymized form.


Recruitment information / eligibility

Status Completed
Enrollment 51
Est. completion date January 1, 2017
Est. primary completion date July 22, 2015
Accepts healthy volunteers Accepts Healthy Volunteers
Gender All
Age group 16 Years and older
Eligibility Inclusion Criteria:

- phenylketonuria due to phenylalanine hydroxylase deficiency

- age =16 years

Exclusion Criteria:

- age <16 years

- pregnancy or maternity

- late-treated phenylketonuria

- phenylketonuria due a defect in BH4 synthesis or BH4 regeneration

Study Design


Related Conditions & MeSH terms


Locations

Country Name City State
n/a

Sponsors (2)

Lead Sponsor Collaborator
Charite University, Berlin, Germany Johannes Gutenberg University Mainz

References & Publications (5)

Briese S, Claus M, Querfeld U. Arterial stiffness in children after renal transplantation. Pediatr Nephrol. 2008 Dec;23(12):2241-5. doi: 10.1007/s00467-008-0894-y. — View Citation

Hennermann JB, Querfeld U. Unknown pathomechanisms of renal impairment in PKU. J Inherit Metab Dis. 2013 Nov;36(6):1087-8. doi: 10.1007/s10545-013-9652-9. — View Citation

Hennermann JB, Roloff S, Gellermann J, Vollmer I, Windt E, Vetter B, Plöckinger U, Mönch E, Querfeld U. Chronic kidney disease in adolescent and adult patients with phenylketonuria. J Inherit Metab Dis. 2013 Sep;36(5):747-56. doi: 10.1007/s10545-012-9548-0. — View Citation

Kracht D, Shroff R, Baig S, Doyon A, Jacobi C, Zeller R, Querfeld U, Schaefer F, Wühl E, Schmidt BM, Melk A; 4C Study Consortium.. Validating a new oscillometric device for aortic pulse wave velocity measurements in children and adolescents. Am J Hypertens. 2011 Dec;24(12):1294-9. doi: 10.1038/ajh.2011.147. — View Citation

Ribas GS, Sitta A, Wajner M, Vargas CR. Oxidative stress in phenylketonuria: what is the evidence? Cell Mol Neurobiol. 2011 Jul;31(5):653-62. doi: 10.1007/s10571-011-9693-2. Review. — View Citation

Outcome

Type Measure Description Time frame Safety issue
Primary Post-ischaemic venous flow (measured in ml/min) in patients with PKU compared to healthy volunteers 18 months
Secondary Aortic pulse wave velocity in patients with PKU compared to healthy volunteers. Intima media thickness in patients with PKU compared to healthy volunteers. 18 months
See also
  Status Clinical Trial Phase
Completed NCT02122198 - Vascular Mechanisms for the Effects of Loss of Ovarian Hormone Function on Cognition in Women N/A
Completed NCT04156711 - Remote Ischemic Preconditioning in Patients Undergoing Acute Minor Abdominal Surgery N/A
Recruiting NCT06133634 - Fisetin to Improve Vascular Function in Older Adults Phase 1/Phase 2
Completed NCT05872139 - Role of Mitochondrial-derived Oxidative Stress to Promote Vascular Endothelial Dysfunction in Non-exercisers With Aging N/A
Recruiting NCT04558450 - Covid-19 Effects on Arterial Stiffness and Vascular Aging (CARTESIAN) N/A
Terminated NCT03325933 - Resistance Training and Cardiometabolic Health N/A
Not yet recruiting NCT05939934 - Impact of the Mandibular Advancement Device on Sleep Apnea During CPAP Withdrawal N/A
Completed NCT02652975 - Anticancer Treatment of Breast Cancer Related to Cardiotoxicity and Dysfunctional Endothelium N/A
Recruiting NCT02334839 - The Association Between Severity of Hypertensive Disorder During Pregnancy and Endothelial Dysfunction N/A
Recruiting NCT02020044 - Outcome After Descemet Membrane Endothelial Keratoplasty (DMEK) and Ultra-thin Descemet Stripping Automated Endothelial Keratoplasty (DSAEK) N/A
Completed NCT01691404 - Study on the Effects of Epicatechin and Quercetin Supplementation on Vascular Function and Blood Pressure (FLAVO) N/A
Completed NCT01775865 - Targeting Inflammation to Treat Cardiovascular Aging Phase 2
Terminated NCT01412216 - The Effects of Sedentarism on Vascular Function, Inflammation, and Insulin Resistance Phase 2
Completed NCT01319344 - Effect of Eplerenone on Endothelial Function in Metabolic Syndrome Phase 3
Completed NCT00990730 - Atherosclerosis in Rheumatoid Arthritis N/A
Completed NCT00987974 - Short Term Statin Treatment and Endothelial Dysfunction Due to Ischemia and Reperfusion Injury Phase 4
Completed NCT00848302 - Endothelial Function in Human Arteries Early Phase 1
Completed NCT00532844 - A Phase 2, Pharmacokinetic (PK) Study of 6R-BH4 Alone or 6R-BH4 With Vitamin C in Subjects With Endothelial Dysfunction Phase 2
Completed NCT00376246 - Effect of Ezetimibe on Flow-mediated Brachial Artery Reactivity in Healthy Subjects Phase 4
Completed NCT00775099 - Combustion Derived Air Pollution and Vascular Function N/A