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At least 415 records · Page 23Linked to original sources
PubMed1989

Fibrous dysplasia and cherubism as an hereditary familial disease. Follow-up of four generations.

Five cases of dysplasia of the jaws in one family which has been under our observation since 1970 are reported. The disease appeared as a mixed display of jaw lesions, in some members as fibrous dysplasia and in others as cherubism. We were able to trace the disorder through an unbroken line of four generations, and thus to demonstrate autosomal dominant inheritance. Cytogenetic analysis performed on three members of this family revealed a significantly increased rate of chromosomal breakage.

Adult↗
PubMed1985

Gigantism associated with McCune-Albright's syndrome.

The case of a 16 year-old boy with McCune-Albright's syndrome which is rarely accompanied by gigantism was studied endocrinologically. The stimulation of growth hormone (GH) release by hypoglycemia, the decline of elevated GH by hyperglycemia and a little lower somatostatin like immunoreactivity (SLI) may support abnormalities of hypothalamic function, but the existence of pituitary microadenoma cannot be ruled out because of the paradoxical suppression of GH release by oral administration of bromocriptine (CB-154) and L-DOPA and the stimulation of GH release by intravenous administration of TRH.

Adolescent↗
PubMedDate not supplied

[Pubertas praecox in McCune-Albright syndrome--case report and review of the literature].

A patient is presented with the syndrome of polyostatic fibrous dysplasia and precocious puberty (PP). The endocrinopathy in McCune-Albright-syndrome (MAS) has formerly been ascribed to a central (hypothalamic) origin. In this patient the PP was caused by a luteinized follicular cyst, suggesting autonomous hyperfunction of this gland. High serum estradiol levels returned to normal after cystectomie. The review of the literature suggests the peripheral origin of PP to be more frequent in younger age groups (under 6 years). It appears possible that peripheral hypersecretion of sexual steroids may cause a rise of gonadotropins secondarily followed by true PP in older children provided such longstanding hypersecretion leads to a generalized maturation of the body including skeletal maturation. Treatment in pseudoprecocious puberty seems to be not effective with LH-RH-analogues. Cyproteroneacetate alone or in combination with a LH-RH analogue gave the impression of being more successful. Cystectomy can lead to a cure but a recurrence of ovarian cysts is possible. A combination of surgical and drug therapy may be beneficial under these circumstances. Until now there is no sufficient treatment for polyostotic fibrous dysplasia.

Child, Preschool↗
PubMed1979

Fibrous dysplasia and precocious puberty (McCune-Albright syndrome).

A patient with fibrous dysplasia and precocious puberty, with pneumogynographic demonstration of an enlarged uterus and ovaries at age 16 months has been followed up to age 14 years. The pneumogynographic findings and the chemistries implicate central nervous system stimulation as the cause of the precocious puberty.

Air↗
PubMed1989

Polyostotic fibrous dysplasia associated with extensive linear epidermal naevi.

Albright's syndrome is characterized by the combination of polyostotic fibrous dysplasia, precocious puberty and café-au-lait spots. We describe a patient with polyostotic fibrous dysplasia who also had extensive linear epidermal naevi, an association that has not been previously described in the English literature.

Adult↗
PubMed1986

Autonomous ovarian hyperfunction followed by gonadotrophin-dependent puberty in McCune-Albright syndrome.

A 5-year-old girl with the McCune-Albright syndrome presented with precocious puberty secondary to autonomously functioning ovarian cysts, followed by true central puberty. Progression from gonadotrophin-independent to gonadotrophin-dependent precocious puberty may occur from elevated sex steroid levels leading to the early maturation of the hypothalamic-pituitary axis.

Child, Preschool↗
PubMed1979

Hyperprolactinaemia in a patient with the McCune-Albright Syndrome.

A patient is presented with the syndrome of polyostotic fibrous dysplasia and precocious puberty (McCune-Albright Syndrome). In adult life she developed hyperprolactinaemia with galactorrhoea and amenorrhoea; there was also evidence of excessive secretion of growth hormone.

Adult↗