title: Severe arterial hypertension: a possible complication of McCune-Albright syndrome.
abstract: McCune-Albright syndrome is characterized by café-au-lait spot, multiple endocrine hyperfunction, and polyostotic fibrous dysplasia. A somatic point mutation of Gsalpha protein leads to an increase in the Gsalpha-associated hormone activity in McCune-Albright syndrome. Because cyclic adenosine 3',5'-monophosphate stimulates the dopamine beta hydroxylase gene, an activating mutation of the Gsalpha protein may cause the hyperproduction of norepinephrine via dopamine. We report on a 9-year-old girl with McCune-Albright syndrome complicated by severe arterial hypertension. The urinary excretion of norepinephrine was 5- to 10-fold higher than in age-matched controls. Meta-iodobenzylguanidine scintigraphy and positron emission tomography/computed tomography (PET/CT) revealed no hot spots. These findings suggest that severe hypertension might be due to an activating mutation of Gsalpha protein in sympathetic ganglia. Because of the reported association of GNAS1 gene polymorphism with hypertension, our patient provides further evidence for a role of Gsalpha protein in hypertension.
authors.affiliation: Department of Pediatrics, Minoh City Hospital, Minoh City, Osaka, Japan.
authors.forename: Yasuhisa
authors.initials: Y
authors.lastname: Ohata
authors.forename: Takehisa
authors.initials: T
authors.lastname: Yamamoto
authors.forename: Ikuko
authors.initials: I
authors.lastname: Mori
authors.forename: Toru
authors.initials: T
authors.lastname: Kikuchi
authors.forename: Toshimi
authors.initials: T
authors.lastname: Michigami
authors.forename: Yasuo
authors.initials: Y
authors.lastname: Imanishi
authors.forename: Kenichi
authors.initials: K
authors.lastname: Satomura
authors.forename: Shinobu
authors.initials: S
authors.lastname: Ida
authors.forename: Keiichi
authors.initials: K
authors.lastname: Ozono
mesh_terms: D001794:Blood Pressure; D002648:Child; D005260:Female; D005359:Fibrous Dysplasia, Polyostotic / Q000150:complications* / Q000000981:diagnostic imaging / Q000652:urine; D006801:Humans; D006973:Hypertension / Q000209:etiology* / Q000503:physiopathology; D009638:Norepinephrine / Q000652:urine*; D049268:Positron-Emission Tomography; D011877:Radionuclide Imaging
chemical_list: D009638:Norepinephrine