Document Detail


McCune Albright Syndrome (MCAS): a case series.
MedLine Citation:
PMID:  12554915     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
McCune Albright Syndrome (MCAS) is an association of, Café-au-lait macules, polyostotic fibrous dysplasia and autonomous hyperfunctioning endocrinopathy. This is a rare disorder seen more commonly in females. We evaluated 7 (6F & 1M) cases under six years of age (4 months to 5.5 yrs) presenting with Café-au-lait spots, polyostotic fibrous dysplasia and/or sexual precocity. All the 7 cases had large Café-au-lait spots, radiologic features of polyostotic fibrous dysplasia were seen in 5 cases. Six girls had precocious puberty with large ovarian follicles and elevated S. Estradiol levels (14-65 pg/dl) with prepubertal gonadotropin levels in 5 of them. Medroxy-progrestrone acetate was used to treat the sexual precocity. Five girls on follow up for 6 months (6mo-16mo) showed cessation of menstrual episodes and regression of ovarian follicles in three, regression in breast size in one, and three girls continued to grow at a height velocity >95th centile for age. Skeletal lesions and skin features did not show any change. No other endocrinopathy was noted. Gonadotropin independent precocious puberty was the only endocrine affection seen in this series.
Authors:
Sudha Rao; M P Colaco; M P Desai
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Indian pediatrics     Volume:  40     ISSN:  0019-6061     ISO Abbreviation:  Indian Pediatr     Publication Date:  2003 Jan 
Date Detail:
Created Date:  2003-01-29     Completed Date:  2003-03-12     Revised Date:  2009-11-19    
Medline Journal Info:
Nlm Unique ID:  2985062R     Medline TA:  Indian Pediatr     Country:  India    
Other Details:
Languages:  eng     Pagination:  29-35     Citation Subset:  IM    
Affiliation:
Bai Jerbai Wadia Hospital for Children Institute of Child Health and Research. Acharya Dhonde Marg, Parel, Mumbai, India. c_sudha@hotmail.com
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MeSH Terms
Descriptor/Qualifier:
Cafe-au-Lait Spots / etiology
Child, Preschool
Estradiol / blood
Female
Fibrous Dysplasia, Polyostotic / complications,  diagnosis*,  drug therapy
Gonadotropins / blood
Humans
Infant
Male
Medroxyprogesterone Acetate / therapeutic use
Progesterone Congeners / therapeutic use
Puberty, Precocious / diagnosis*,  drug therapy,  etiology
Treatment Outcome
Chemical
Reg. No./Substance:
0/Gonadotropins; 0/Progesterone Congeners; 50-28-2/Estradiol; 71-58-9/Medroxyprogesterone Acetate

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