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Histamine Responsiveness in McCune-Albright Syndrome

This study is currently recruiting participants.
Verified by Children's Mercy Hospital Kansas City, November 2004

Sponsors and Collaborators: Children's Mercy Hospital Kansas City
KBR
Information provided by: Children's Mercy Hospital Kansas City
ClinicalTrials.gov Identifier: NCT00318097
  Purpose

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.


Condition
McCune-Albright Syndrome

Genetics Home Reference related topics:   Melnick-Needles syndrome   

ChemIDplus related topics:   Histamine    Histamine dihydrochloride    Histamine phosphate   

U.S. FDA Resources

Study Type:   Observational
Study Design:   Screening, Cross-Sectional, Case Control, Prospective Study
Official Title:   Histamine Responsiveness in Patients With McCune-Albright Syndrome

Further study details as provided by Children's Mercy Hospital Kansas City:

Estimated Enrollment:   30
Study Start Date:   November 2003

Show detailed description  Show Detailed Description

  Eligibility
Ages Eligible for Study:   up to 39 Years
Genders Eligible for Study:   Both
Accepts Healthy Volunteers:   Yes

Criteria

Inclusion Criteria:

  • 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 Criteria:

  • 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.
  Contacts and Locations

Please refer to this study by its ClinicalTrials.gov identifier: NCT00318097

Contacts
Contact: Angela L Turpin, MD     816-234-3804     aturpin@cmh.edu    
Contact: Jill D Jacobson, MD     816-234-3804     jjacobson@cmh.edu    

Locations
United States, Missouri
Children's Mercy Hospitals and Clinics     Recruiting
      Kansas City, Missouri, United States, 64108
      Contact: Angela L Turpin, MD     816-234-3804     aturpin@cmh.edu    
      Contact: Jill D Jacobson, MD     816-234-3804     jjacobson@cmh.edu    

Sponsors and Collaborators
Children's Mercy Hospital Kansas City
KBR

Investigators
Principal Investigator:     Angela L Turpin, MD     Children's Mercy Hospital Kansas City    
  More Information


Study ID Numbers:   03 11-116
First Received:   April 24, 2006
Last Updated:   May 1, 2006
ClinicalTrials.gov Identifier:   NCT00318097
Health Authority:   United States: Institutional Review Board

Keywords provided by Children's Mercy Hospital Kansas City:
McCune Albright Syndrome  
Histamine regulation  

Study placed in the following topic categories:
Fibrous dysplasia of bone
Musculoskeletal Diseases
McCune Albright syndrome
Bone Diseases, Developmental
Osteochondrodysplasias
Fibrous Dysplasia of Bone
Histamine phosphate
Fibrous Dysplasia, Polyostotic
Bone Diseases
Fibrous dysplasia
Histamine

Additional relevant MeSH terms:
Neurotransmitter Agents
Pathologic Processes
Disease
Molecular Mechanisms of Pharmacological Action
Histamine Agonists
Syndrome
Physiological Effects of Drugs
Histamine Agents
Pharmacologic Actions

ClinicalTrials.gov processed this record on October 06, 2008




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