1/7. McCune-Albright syndrome: clinical and molecular evidence of mosaicism in an unusual giant patient.Molecular genetics recently uncovered the mystery of the protean picture of McCune-Albright syndrome by identification of the somatic gain of function mutations in the GNAS1 gene. Here we present an adult patient with fibrous dysplasia and an endocrinopathy resulting in unusual giant height. The clinical diagnosis in the patient could be confirmed by molecular investigations in tissues involved in the process of fibrous dysplasia.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
2/7. Unususpected meningioma in a patient with pituitary gigantism. Case report with autopsy findings.A unique example of a clinically unsuspected large parasellar meningioma is described in a 36-yr-old pituitary giant who had been treated initially with conventional irradiation, subsequently by surgical excision of an acidophil adenoma, and ultimately with medroxyprogesterone acetate (MPA) prior to his demise. The patient died of increased intracranial pressure resulting from a combined mass effect of the meningioma and recurrent tumor. The relationship between radiation and the development of the meningioma is discussed, as well as the fine ultrastructure of a highly functioning acidophil adenoma.- - - - - - - - - - ranking = 0.2keywords = giant (Clic here for more details about this article) |
3/7. The 24-hour growth hormone secretion in a boy with giantism.The endocrinological and radiological findings in a 7.5-year-old boy with giantism are reported and compared with an age and sex matched normal tall boy. A 24-h GH profile demonstrated a persistently elevated GH concentration (mean GH concentration: giant 19.3 mU/l; tall boy 5.4 mU/l) with loss of the dominant GH periodicity of 3 h seen in the boy with tall stature and substitution with one of 8 h. These data support the view that giantism and acromegaly are similar diseases occurring prior to and following epiphyseal fusion, respectively.- - - - - - - - - - ranking = 1.4keywords = giant (Clic here for more details about this article) |
4/7. Anaesthesia for transsphenoidal surgery in a patient with extreme gigantism.The management of anaesthesia for transsphenoidal removal of a pituitary adenoma in a true pituitary giant with acromegaly is described. Problems which may be anticipated in such a patient and an approach to their management are discussed, with particular emphasis upon the need for thorough preoperative assessment of the upper airway and the provision of adequate pulmonary ventilation during anaesthesia.- - - - - - - - - - ranking = 0.2keywords = giant (Clic here for more details about this article) |
5/7. Mammosomatotroph adenoma of the pituitary associated with gigantism and hyperprolactinemia. A morphological study including immunoelectron microscopy.A 29-year old giantess with growth hormone excess and hyperprolactinemia underwent transsphenoidal surgery to remove her pituitary tumor. Electron microscopy revealed a mammosomatotroph adenoma composed of one cell type. Immunoelectron microscopy, using the immunogold technique, demonstrated predominantly growth hormone or prolactin or a varying mixture of both growth hormone and prolactin in the adenoma cells. The presence of growth hormone and prolactin was found not only in the cytoplasm of the same adenoma cells but also in the same secretory granules. In the nontumorous adenohypophysis, somatotrophs and lactotrophs showed ultrastructural signs of hyperactivity. This finding is in contrast with the presence of suppressed somatotrophs and lactotrophs seen in nontumorous portions of adult pituitaries harboring growth hormone or prolactin-secreting adenomas. Our morphological study reinforces the view that growth hormone-producing pituitary tumors, originating in childhood, are different from those of the adult gland.- - - - - - - - - - ranking = 0.2keywords = giant (Clic here for more details about this article) |
6/7. Buphthalmos in neurofibromatosis: is it an expression of regional giantism?A case of buphthalmos and regional giantism in neurofibromatosis with the characteristics of the Francois syndrome is reported. Difficulties of early diagnosis are emphasized. A discussion of the mechanisms which may produce buphthalmos in neurofibromatosis is presented. We propose that buphthalmos in neurofibromatosis may be primarily an expression of regional giantism rather than a consequence of uncontrolled intraocular pressure.- - - - - - - - - - ranking = 1.2keywords = giant (Clic here for more details about this article) |
7/7. The "Taiwanese giant": hormonal and genetic influences in fibrous dysplasia.A 14-year-old boy has been followed for 4 years with a rapidly growing, recurrent area of fibrous dysplasia of the left maxilla and zygoma following resection and bone grafting. Standing 190 cm tall, he was found to have elevated serum growth hormone levels and a pituitary adenoma. His case appears to represent a postzygotic gene mutation of McCune-Albright syndrome. It is possible that the elevated growth hormone levels are in part responsible for the rapid progression of the tumor.- - - - - - - - - - ranking = 0.8keywords = giant (Clic here for more details about this article) |