期刊论文详细信息
World Allergy Organization Journal
Allergic manifestations and cutaneous histamine responses in patients with McCune Albright syndrome
Scott A Sands1  Angela L Turpin1  Jill D Jacobson1 
[1] Section of Endocrinology, Children’s Mercy Hospital, University of Missouri-Kansas City, School of Medicine, 2401 Gillham Road, Kansas, MO 64108, USA
关键词: Anaphylaxis;    Atopy;    Wheal and flare;    Histamine responsiveness;    McCune Albright syndrome;   
Others  :  804077
DOI  :  10.1186/1939-4551-6-9
 received in 2012-09-20, accepted in 2013-04-16,  发布年份 2013
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【 摘 要 】

Background

McCune Albright syndrome (MAS) is a rare disorder characterized by precocious puberty, café-au-lait spots, and fibrous dysplasia. Its cause is an activating mutation in the GNAS gene, encoding a subunit of the stimulatory G protein, Gsalpha (Gsα). The action of any mediator that signals via Gsα and cyclic AMP can be up regulated in MAS. We had observed gastritis, gastroesophageal reflux, and anaphylaxis in McCune Albright patients.

Objective

As histamine is known to signal via histamine 1 (H1) and histamine 2 (H2) receptors, which couple with stimulatory G proteins, we attempted to mechanistically link histamine responsiveness to the activating GNAS mutation. We hypothesized that responsiveness to histamine skin testing would differ between MAS patients and healthy controls.

Patients and methods

After obtaining informed consent, we performed a systematic review of histamine responsiveness and allergic manifestations in 11 MAS patients and 11 sex-matched, Tanner-stage matched controls. We performed skin prick testing, quantifying the orthogonal diameters of wheals and erythema. We also quantitated G protein mRNA expression.

Results

The peak wheal and flare responses to histamine were significantly higher in MAS patients compared to controls.

Conclusions

This study suggests that MAS patients may be at risk for exaggerated histamine responsiveness compared to unaffected controls.

【 授权许可】

   
2013 Jacobson et al.; licensee BioMed Central Ltd.

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