Quantitative Liver Functions in Turner Syndrome With and Without Hormone Replacement Therapy
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Purpose
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.
| Condition | Intervention |
|---|---|
|
Turner Syndrome |
Drug: Hormone replacement therapy |
| Study Type: | Interventional |
| Study Design: | Allocation: Randomized Intervention Model: Crossover Assignment Masking: Open Label Primary Purpose: Treatment |
| Official Title: | Quantitative Liver Functions in Turner Syndrome With and Without Hormone Replacement Therapy |
- quantitative liver function tests [ Time Frame: 1996-1999 ] [ Designated as safety issue: No ]
| Enrollment: | 16 |
| Study Start Date: | October 1996 |
| Study Completion Date: | June 2000 |
| Primary Completion Date: | October 1999 (Final data collection date for primary outcome measure) |
| Arms | Assigned Interventions |
|---|---|
|
Experimental: HRT
Women with TS were treated with oral hormone substitution consisting of 2 mg 17β-estradiol/day for days 1-12, 2 mg 17β-estradiol/day and 1 mg norethisterone acetate/day for days 13-22 and 1 mg 17β-estradiol/day for days 23-28 (Trisekvens, Novo Nordisk A/S, Bagsværd, Denmark)
|
Drug: Hormone replacement therapy
Women with TS were treated with oral hormone substitution consisting of 2 mg 17β-estradiol/day for days 1-12, 2 mg 17β-estradiol/day and 1 mg norethisterone acetate/day for days 13-22 and 1 mg 17β-estradiol/day for days 23-28 (Trisekvens, Novo Nordisk A/S, Bagsværd, Denmark)
Other Name: Trisekvens, Novo Nordisk A/S, Bagsværd, Denmark
|
Detailed 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.
Eligibility| Genders Eligible for Study: | Female |
| Accepts Healthy Volunteers: | Yes |
Inclusion Criteria:
- Turner syndrome by karyotype
Exclusion Criteria:
- Thyroid abnormality
- Glucocorticoid treatment
Contacts and Locations| Denmark | |
| Medical department M and Investigational Laboratories | |
| Aarhus C, Jutland, Denmark, 8000 | |
| Principal Investigator: | J S Christíansen, Professor | Medical departmnet M, Aarhus University Hospital, NBG, Denamrk, |
More Information
No publications provided
| Responsible Party: | MD, PhD, DMSc Claus H. Gravholt, Medical department M, Aarhus University Hospital, Denmark |
| ClinicalTrials.gov Identifier: | NCT00825617 History of Changes |
| Other Study ID Numbers: | 19963561 |
| Study First Received: | January 20, 2009 |
| Last Updated: | January 20, 2009 |
| Health Authority: | Denmark: Danish Dataprotection Agency Denmark: Danish Medicines Agency Denmark: Ethics Committee |
Keywords provided by University of Aarhus:
|
TS HRT Liver function tests |
Additional relevant MeSH terms:
|
Turner Syndrome Gonadal Dysgenesis Primary Ovarian Insufficiency Disorders of Sex Development Urogenital Abnormalities Sex Chromosome Disorders of Sex Development Heart Defects, Congenital Cardiovascular Abnormalities Cardiovascular Diseases Heart Diseases Congenital Abnormalities Sex Chromosome Disorders Chromosome Disorders Genetic Diseases, Inborn Gonadal Disorders |
Endocrine System Diseases Ovarian Diseases Adnexal Diseases Genital Diseases, Female Hormones Norethindrone Norethindrone acetate Trisequens Hormones, Hormone Substitutes, and Hormone Antagonists Physiological Effects of Drugs Pharmacologic Actions Contraceptives, Oral, Synthetic Contraceptives, Oral Contraceptive Agents, Female Contraceptive Agents |
ClinicalTrials.gov processed this record on May 21, 2013