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Clinical Trial Details — Status: Completed

Administrative data

NCT number NCT00825617
Other study ID # 19963561
Secondary ID
Status Completed
Phase N/A
First received January 20, 2009
Last updated January 20, 2009
Start date October 1996
Est. completion date June 2000

Study information

Verified date January 2009
Source University of Aarhus
Contact n/a
Is FDA regulated No
Health authority Denmark: Danish Dataprotection AgencyDenmark: Danish Medicines AgencyDenmark: Ethics Committee
Study type Interventional

Clinical Trial Summary

Several studies have demonstrated that Turner Syndrome patients have elevated liver enzymes readily suppressible by a short course of HRT. We wanted to estimated quantitative liver functions in a young group of Turner syndrome patients compared to a healthy control group.


Description:

Turner syndrome is due to the absence of a part of or the entire X chromosome in females. Stature is short, and morbidity is increased due to risk of osteoporosis and fractures, type 2 diabetes, ischemic heart disease, hypertension, and stroke, but also the risk of cirrhosis is increased. Clinical studies have shown a frequent occurrence of elevated liver enzymes, primarily alanine aminotransferase, g-glutamyl-transferase, and alkaline phosphatase, while bilirubin is normal.

We and others have shown a normalizing effect of hormone replacement therapy (HRT), containing 17bestradiol and a gestagen, on liver enzymes, which may point towards a protective effect on hepatocyte integrity. Marked architectural changes, including nodular regenerative hyperplasia, multiple focal nodular hyperplasia and cirrhosis are observed in some patients and are associated with a risk of liver-related complications. These changes are frequently associated with vascular disorders such as obliterative portal venopathy, probably related to congenitally abnormal vessels. Steatosis, steatofibrosis, and steatohepatitis are seen and may be caused by metabolic disorders. In addition, bile duct alterations resembling small duct sclerosing cholangitis are observed in several patients. Presently, it is not known whether these perturbations in liver morphology and in liver-derived enzymes are related to functional defects in females with TS and whether this may change by HRT. To further explore quantitative liver function in TS, we examined adult women with TS on and off HRT and compared them with a control group of age matched normal women. We used the galactose elimination capacity to assess hepatocyte cytosol activity, the plasma clearance of indocyanine green to assess hepatic blood flow and excretory liver cell function independently of hepatic blood flow, the antipyrine plasma clearance to estimate hepatic microsomal system activity, and the functional hepatic nitrogen clearance to assess mitochondrial- cytosolic metabolic capacity for conversion of amino-nitrogen.We assumed that one or more these metabolic liver functions would be diminished in untreated TS and normalized by HRT. Our principal objective was to understand mechanistically how HRT improves liver function in TS.


Recruitment information / eligibility

Status Completed
Enrollment 16
Est. completion date June 2000
Est. primary completion date October 1999
Accepts healthy volunteers Accepts Healthy Volunteers
Gender Female
Age group N/A and older
Eligibility Inclusion Criteria:

- Turner syndrome by karyotype

Exclusion Criteria:

- Thyroid abnormality

- Glucocorticoid treatment

Study Design

Allocation: Randomized, Intervention Model: Crossover Assignment, Masking: Open Label, Primary Purpose: Treatment


Intervention

Drug:
Hormone replacement therapy
Women with TS were treated with oral hormone substitution consisting of 2 mg 17ß-estradiol/day for days 1-12, 2 mg 17ß-estradiol/day and 1 mg norethisterone acetate/day for days 13-22 and 1 mg 17ß-estradiol/day for days 23-28 (Trisekvens, Novo Nordisk A/S, Bagsværd, Denmark)

Locations

Country Name City State
Denmark Medical department M and Investigational Laboratories Aarhus C Jutland

Sponsors (1)

Lead Sponsor Collaborator
University of Aarhus

Country where clinical trial is conducted

Denmark, 

Outcome

Type Measure Description Time frame Safety issue
Primary quantitative liver function tests 1996-1999 No
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