Clinical Trial: Cardiovascular Risk Factors and LCH in Adults
Study Status: Completed
Recruit Status: Unknown status
Study Type: Observational
Official Title: CARDIOVASCULAR RISK FACTORS IN ADULT PATIENTS WITH MULTISYSTEM LANGERHANS-CELL HISTIOCYTOSIS: EVIDENCE OF GLUCOSE METABOLISM ABNORMALITIES
Brief Summary:
Langerhans-cell histiocytosis (LCH) is a rare disease with features of chronic inflammation and hypopituitarism, conditions associated with increased risk of cardiovascular diseases.
Objective: To investigate glucose and lipid metabolism, insulin resistance, structural arterial and functional endothelial properties in patients with multisystem LCH in a prospective, observational study.
Interventions:Cardiovascular risk factors: arterial blood pressure, lipid profile, mathematical indices of insulin resistance (IR), intima media thickness, brachial artery flow mediated dilatation, dynamic indices of IR, pituitary function and C-reactive protein will be estimated in patients with LCH and in a control group matched for gender, age, BMI and smoking habits.
Detailed Summary:
CARDIOVASCULAR RISK FACTORS IN ADULT PATIENTS WITH MULTISYSTEM LANGERHANS-CELL HISTIOCYTOSIS: EVIDENCE OF ABNORMALITIES OF CARBOHYDRATE METABOLISM
Langerhans cell histiocytosis (LCH) is a rare disease, usually affecting children although it has recently increasingly been recognized in adults with a prevalence of approximately 1/560.000 cases ( , ). LCH is characterized by the aberrant proliferation of dendritic cells of the monocyte-macrophage system that resemble normal epidermal Langerhans’ cells. These cells can infiltrate many sites of the body leading to either localized lesions or widespread systemic disease. Although LCH has been shown to be a clonal disorder ( ) it also exhibits features of an inflammatory disease, as altered expression of cytokines and cellular adhesion molecules important for the migration and homing of Langerhans cell has been documented ( , ). In addition, LCH shows a particular predilection for hypothalamo-pituitary axis (HPA) involvement leading to diabetes insipidus and/ or anterior pituitary dysfunction in 15–50% and 5–20%, of patients respectively (3, , , ). These percentages may be higher in adult patients with multisystem involvement, being 94% and 59% respectively ( ).
The ongoing inflammatory process and the presence of hypopituitarism are considered to be two independent risk factors for cardiovascular diseases probably through the induction of insulin resistance (IR) ( , , ). The various therapies used for the treatment of multisystem LCH, chemotherapy, radiotherapy and particularly glucocorticoids, may also adversely affect the cardiovascular system mostly through IR ( , ). It is therefore possible that patients with LCH represent a group at higher risk for cardiovascular diseases through the additive effect of a number of different contributing mechanisms known to
Sponsor: Laikon General District Hospital, Athens
Current Primary Outcome:
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Information By: Laikon General District Hospital, Athens
Dates:
Date Received: June 6, 2007
Date Started: September 2005
Date Completion:
Last Updated: June 7, 2007
Last Verified: June 2007
