1.A case of acromegaly in McCune-Albright syndrome.
Hyeon KIM ; Jin Hyung JANG ; Dong Myung LIM ; Dae Cheol WEE ; Cheol Seung YOUN ; Sun Ho KIM ; Jung Kyu LEEM ; Jin Deug HEO
Journal of Korean Society of Endocrinology 1992;7(4):402-408
No abstract available.
Acromegaly*
;
Fibrous Dysplasia, Polyostotic*
2.A Suspected Case and Literature Review of McCune-Albright Syndrome.
Kyung Eun JUNG ; Ji Hae LEE ; Tae Yoon KIM
Annals of Dermatology 2014;26(5):639-640
No abstract available.
Fibrous Dysplasia, Polyostotic*
3.A Case Report of McCune Albright Syndrome.
Kei Hag SON ; Un Ki YOON ; Soon Ok BYUN ; Ji Sub OH
Journal of the Korean Pediatric Society 1986;29(12):82-87
No abstract available.
Fibrous Dysplasia, Polyostotic*
4.A Case Report of McCune Albright Syndrome.
Kei Hag SON ; Un Ki YOON ; Soon Ok BYUN ; Ji Sub OH
Journal of the Korean Pediatric Society 1986;29(12):82-87
No abstract available.
Fibrous Dysplasia, Polyostotic*
5.A Case of Fibrous Dysplasia.
Jung Soo KIM ; Young Mo SOHN ; Jae Song KIM ; Duk Hi KIM ; Kyoung Ja CHO
Journal of the Korean Pediatric Society 1979;22(3):234-238
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
6.McCune Albright syndrome - a unique mechanism of sexual development
Philippine Journal of Reproductive Endocrinology and Infertility 2005;2(1):26-31
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
7.McCune-Albright Syndrome with hypophosphatemic rickets
Manish Gutch ; Sukriti Kumar ; Keshav Kumar Gupta ; Abhinav Kumar Gupta ; Syed Mohd Razi
Journal of the ASEAN Federation of Endocrine Societies 2015;30(1):40-43
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
9.Fibrous Dysplasia Associated with Primary Hyperparathyroidism Absent of McCune-Albright Syndrome: Tc-99m MIBI and Tc-99m MDP Findings.
Seong Jang KIM ; Ju Won SEOK ; In Ju KIM ; Yong Ki KIM ; Dong Soo KIM
Korean Journal of Nuclear Medicine 2003;37(2):128-134
No abstract available.
Fibrous Dysplasia, Polyostotic*
;
Hyperparathyroidism
;
Hyperparathyroidism, Primary*
;
Technetium Tc 99m Medronate*
10.Malignant Transformation of Fibrous Dysplasia on Anterior Skull Base.
Journal of Korean Neurosurgical Society 2005;37(5):383-385
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*