Imaging of McCune-Albright syndrome using bone single photon emission computed tomography

Kairemo, Kalevi; Verho, S; Dunkel, Leo

doi:10.1007/s004310051031

Cited by 21 publications

(3 citation statements)

References 15 publications

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“…Sonography revealed struma multinodosa which has also previously been described in other patients with MAS 20 . Bone SPECT data from a patient with MAS have only been reported once before 25 . MRI of the skull was particularly useful in demonstrating the extensive lesions involving diverse cranial bones.…”

Section: Discussionmentioning

confidence: 99%

Clinical, Endocrinological and Radiological Features in a Child with McCune-Albright Syndrome and Pituitary Adenoma

Zumkeller¹,

Jassoy²,

Lebek³

et al. 2001

Journal of Pediatric Endocrinology and Metabolism

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McCune-Albright syndrome is a rare syndrome presenting with polyostotic dysplasia, cafe-au-lait spots and multiple endocrinopathies that is very often combined with precocious puberty. We examined the clinical, endocrinological and radiological features in a boy with McCune-Albright syndrome and pituitary adenoma. X-rays, magnetic resonance (MRI) scan, whole body scintigraphy, single photon emission computer tomography (SPECT) and 3D-reconstruction from bone SPECT was performed to evaluate clinical improvement after treatment with sandostatin and pamidronic acid. After a six-month period of treatment with sandostatin and pamidronate, bone scintigraphy revealed significantly reduced activity. Treatment with bromocriptine and methimazole led to normalization of prolactin and thyroid hormone levels. Mobility of the patient improved. A significant improvement as a result of treatment with sandostatin and pamidronic acid was found in this patient with generalized fibrous dysplasia. So far, this condition has been treated with pamidronate only in adults, but severely affected children also benefit from this treatment regimen.

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Section: Discussionmentioning

confidence: 99%

Clinical, Endocrinological and Radiological Features in a Child with McCune-Albright Syndrome and Pituitary Adenoma

Zumkeller¹,

Jassoy²,

Lebek³

et al. 2001

Journal of Pediatric Endocrinology and Metabolism

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“…Lebensjahr beobachtet (70% innerhalb der ersten 3 Lebensdekaden). Eine Kombination der polyostotischen fibrösen Dysplasie mit Pigmentflecken der Haut (Café-au-lait-Flecken) und hormonellen Störungen in Form einer Pubertas praecox sowie Skelettdeformationen wird als McCune-Albright-Syndrom bezeichnet [6,11,22]. Das McCune-Albright-Syndrom tritt überwiegend beim weiblichen Geschlecht auf.…”

Section: Fibröse Dysplasieunclassified

“…Die Mutation führt zu einem Aktivitätsverlust der GTPase und damit zu einer Aktivitätserhöhung der Adenylatzyklase mit nachfolgender cAMP-Überproduktion. Nach diesen Erkenntnissen ist die cAMP-Stimulation für die Genese der fibrösen Dysplasie zumindest mitverantwortlich [6,11,16,17]. [22,23].…”

Section: Fibröse Dysplasieunclassified

Klinische Symptomatik, differenzialdiagnostische Schwierigkeiten und Therapieoptionen bei tumorartigen Läsionen der Orbita

Smolarz-Wojnowska¹,

Klein²

2009

Klin Monatsbl Augenheilkd

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Tumour-like lesions are very rare entity among orbital diseases. Between 1996 and 2007, 83 patients with orbital disorders were treated and three of them complained about different tumour-like lesions: giant cell granuloma, eosinophilic granuloma and fibrous dysplasia. Because of the considerable increase of functional disorders and tumour growth, all of the patients underwent surgical treatment. In this paper we describe the clinical symptoms, diagnostics and the methods of therapy for each of the lesions. The results shows that the preoperative neuro-ophthalmological examination as well as radiological imaging are necessary to assess the motility, visual field, vision, eye ball dislocation and to plan the surgical strategy. In terms of this descriptive character of the paper, we hope to submit some relevant information in order to improve the diagnostics and therapeutic procedures for tumour-like lesions of the orbit.

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McCune-Albright Syndrome

2005

Abnormal Skeletal Phenotypes

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A ten-year-old female presented to our dermatology clinic with her mother, our patient, at which time we incidentally noted a large light brown patch on the daughter's cheek (shown above). The girl was a fifth grader in elementary school. On physical exam, a large brown smooth patch with jagged borders consistent with a caféau-lait patch was noted on the right cheek. The lesion was present since birth, was asymptomatic, and had grown in proportion with her growth overtime. The girl noted a history of early menses beginning at the age of two for which she follows with endocrinology. She noticed breast enlargement at the age of seven years old. She also noted a history of multiple bone fractures, most recently of the femur after falling in the yard. Musculoskeletal exam revealed asymmetric limb length with the left leg longer than the right. She was ambulating on crutches after fracturing her femur in a fall. Breast exam revealed large breasts relative to height and age. The remainder of the physical exam was non-contributory. DISCUSSION Given the clinical manifestations, the patient was diagnosed with McCune Albright Syndrome (MAS), a genetic syndrome characterized by the clinical triad of polyostotic fibrous dysplasia, endocrine

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Imaging of McCune-Albright syndrome using bone single photon emission computed tomography

Abstract: McCune-Albright syndrome should be considered in the differential diagnosis when interpreting extensive unilateral predominance in paediatric bone scans.

Cited by 21 publications

References 15 publications

Clinical, Endocrinological and Radiological Features in a Child with McCune-Albright Syndrome and Pituitary Adenoma

Clinical, Endocrinological and Radiological Features in a Child with McCune-Albright Syndrome and Pituitary Adenoma

Klinische Symptomatik, differenzialdiagnostische Schwierigkeiten und Therapieoptionen bei tumorartigen Läsionen der Orbita

McCune-Albright Syndrome

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