Histamine Responsiveness in McCune-Albright Syndrome

Completed

Conditions studied: McCune-Albright Syndrome

In brief

McCune-Albright syndrome (MAS) is a syndrome caused by a genetic mutation that causes a specific protein in the body called a G protein to be constantly active. Children with McCune-Albright syndrome classically have early puberty, areas of increased skin pigmentation, and bone lesions resulting from the constant activity of the specific protein involved. Histamines are known to play a role in allergies and related allergic problems. The effects of histamines are controlled by the same G protein that is overly active in McCune-Albright syndrome. Thus, one could predict that patients with McCune-Albright may be at high risk for allergic problems. To date, no studies have documented any form of histamine excess or allergic difficulties in patients with McCune-Albright syndrome. However, the investigators have made the observation that a high percentage of their patients with MAS exhibit a range of allergic symptoms, from mild symptoms, to severe, life-threatening symptoms. The purpose of this study is to demonstrate increased histamine response by using a histamine skin test in patients with MAS. If increased reactions to histamines can be documented in MAS patients when compared to controls, severe and potentially life threatening allergic reactions in children with MAS could be anticipated and avoided.

Key facts

Study ID

NCT00318097

Run by

Children's Mercy Hospital Kansas City

People needed

22

Starts

2004-08-01

Expected to finish

2007-08-01

Last updated by the study team

2018-02-06

Who can join

Age: any, up to 39. Sex: any. Healthy volunteers: accepted.

You may qualify if…

• Children with MAS ranging from toddlers to young adults.
• Diagnosis of MAS will be made on a clinical basis. Blood testing is not helpful in this condition, as bone marrow progenitor cells with the Gαs mutation display a survival disadvantage. All patients are mixed chimeras, as this mutation is lethal if it occurs in germline cells.
• Patients who exhibit two or more of the following clinical findings fit the diagnosis of MAS:
• GnRH independent precocious puberty
• Polyostotic fibrous dysplasia
• Café-au-lait spots with coast of Maine borders and respect for the midline.
• Non-autoimmune hyperthyroidism.
• Ten controls will also be recruited from family members of patients with MAS with no known allergies. An additional control group of ten unrelated subjects, also with no known allergies, will be recruited from the Endocrine Clinic for comparison.

You may not qualify if…

• Any MAS patient or control who has not, or cannot, discontinue(d) any home regimens of antihistamines or glucocorticoids (including inhaled steroids) at least seven days prior to skin testing.
• Any MAS patient or control on tricyclic antidepressants within two weeks prior to skin testing.

Where it is running

• Children's Mercy Hospitals and Clinics — Kansas City, Missouri, United States

Full record on ClinicalTrials.gov

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