No abstract available.
Fibrous Dysplasia, Polyostotic*

No abstract available.
Acromegaly* ; Fibrous Dysplasia, Polyostotic*

No abstract available.
Fibrous Dysplasia, Polyostotic*

No abstract available.
Fibrous Dysplasia, Polyostotic*

Fibrous dysplasia of bone is a relatively rare condition characterized by fibrous tissue replacement of skeleton, usually not disabling, of slow progress, and showing a tendency to become arrested. It may be monostotic (confined to one bone) or Polyostotic (situated in many bones). The etiology of fibrous dysplasia is unkown but it is now believed to be a developmental error in which primitive fibrous tissue proliferates within the bony medulla and encroaches upon the cortex from within. This paper reports the one case of polyostotic fibrous dysplasia. The diagnosis was made by clinical, radiological and histological findings. The review of literatures was made briefly.
Diagnosis ; Fibrous Dysplasia of Bone ; Fibrous Dysplasia, Polyostotic ; Skeleton

McCune Albright Syndrome is a sporadic disease characterized by polyostotic fibrous dysplasia, cafe au lait spots, sexual precocity and hyperfunction of multiple endocrine glands. These manifestations may be explained by a somatic mutation in affected tissues that result in activation of the single transduction pathway generating cyclic AMP (cAMP). We report a case of a 16 year old female, who presented with the following endocrinologic features: menarche at 1 year 8 months old, with breast budding (Tanner Stage III) and appearance of pubic hair (Tanner Stage II) at 3 years of age. She has multiple skeletal deformities and cafe au lait spots on her cheek, nape, and on her flank. both seen on the left side. Management of this case involves a multidisciplinary approach, involving suppression of gonadal function, slowing of skeletal maturation and orthopedic care for possible injury to the skeletal abnormality.
Human ; Female ; Adolescent ; FIBROUS DYSPLASIA, POLYOSTOTIC

Humans ; Fibrous Dysplasia, Polyostotic/complications* ; Hemangioma

Fibrous dysplasia (FD) is sometimes accompanied by extraskeletal manifestations that can include any combination of café-au-lait macules, hyperfunctioning endocrinopathies, such as gonadotropin-independent precocious puberty, hyperthyroidism, growth hormone excess, FGF23-mediated renal phosphate wasting, and/or Cushing’s syndrome, as well as other less common features. The combination of any of these findings, with or without FD, is known as McCune-Albright syndrome (MAS). The broad spectrum of involved tissues and the unpredictable combination of findings is because of a molecular defect due to dominant activating mutations in the widely expressed signalling protein Gsα. These mutations arise sporadically, often early in development, prior to gastrulation and can distribute across many or few tissues.1,2 We present a case of a 3½ year-old-girl who presented simultaneously with precocious puberty and hypophosphatemic rickets, along with fibrous dysplasia and café au lait macules.
Fibrous Dysplasia, Polyostotic ; Puberty, Precocious ; Rickets, Hypophosphatemic

No abstract available.
Fibrous Dysplasia, Polyostotic* ; Hyperparathyroidism ; Hyperparathyroidism, Primary* ; Technetium Tc 99m Medronate*

Malignant transformation of fibrous dysplasia is a rare condition and the incidence is estimated at 0.4% for fibrous dysplasia and 4% for Albright's syndrome. The authors did not find a reported case of malignant change at skull base around the orbit in the literature. We experienced a case of fibrous dysplasia, in which neurologic symptoms were aggravated due to malignant change around the orbit, and report its favorable outcome obtained with total surgical removal.
Fibrous Dysplasia, Polyostotic ; Incidence ; Neurologic Manifestations ; Orbit ; Skull Base* ; Skull*