Status:
COMPLETED
Histamine Responsiveness in McCune-Albright Syndrome
Lead Sponsor:
Children's Mercy Hospital Kansas City
Collaborating Sponsors:
KBR
Conditions:
McCune-Albright Syndrome
Eligibility:
All Genders
Up to 39 years
Brief Summary
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 ...
Detailed Description
Objectives of this study are to determine if: 1. Patients with McCune-Albright syndrome differ from unaffected controls in wheal and flare response to histamine and codeine. 2. Affected skin will dif...
Eligibility Criteria
Inclusion
- 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.
Exclusion
- 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.
Key Trial Info
Start Date :
August 1 2004
Trial Type :
OBSERVATIONAL
Allocation :
ACTUAL
End Date :
August 1 2007
Estimated Enrollment :
22 Patients enrolled
Trial Details
Trial ID
NCT00318097
Start Date
August 1 2004
End Date
August 1 2007
Last Update
February 6 2018
Active Locations (1)
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1
Children's Mercy Hospitals and Clinics
Kansas City, Missouri, United States, 64108