title: Clinical, endocrinological and radiography features in a child with McCune-Albright syndrome and pituitary adenoma.
abstract: 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.
authors.affiliation: Department of Pediatrics, Martin-Luther-University Halle-Wittenberg, Halle, Germany. walter.zumkeller@medizin.uni-halle.de
authors.forename: W
authors.initials: W
authors.lastname: Zumkeller
authors.forename: A
authors.initials: A
authors.lastname: Jassoy
authors.forename: S
authors.initials: S
authors.lastname: Lebek
authors.forename: M
authors.initials: M
authors.lastname: Nagel
mesh_terms: D000236:Adenoma / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology*; D000970:Antineoplastic Agents / Q000627:therapeutic use; D018931:Antineoplastic Agents, Hormonal / Q000627:therapeutic use; D002648:Child; D004164:Diphosphonates / Q000494:pharmacology; D004359:Drug Therapy, Combination; D004702:Endocrine Glands / Q000503:physiopathology*; D005359:Fibrous Dysplasia, Polyostotic / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology*; D006801:Humans; D007091:Image Processing, Computer-Assisted; D008279:Magnetic Resonance Imaging; D008297:Male; D015282:Octreotide / Q000627:therapeutic use; D000077268:Pamidronate; D010911:Pituitary Neoplasms / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology*; D011859:Radiography; D011877:Radionuclide Imaging; D015899:Tomography, Emission-Computed, Single-Photon
chemical_list: D000970:Antineoplastic Agents; D018931:Antineoplastic Agents, Hormonal; D004164:Diphosphonates; D000077268:Pamidronate; D015282:Octreotide