McCune Albright Syndrome (MCAS): a case series.
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.
Rao
Sudha
S
Bai Jerbai Wadia Hospital for Children Institute of Child Health and Research. Acharya Dhonde Marg, Parel, Mumbai, India. c_sudha@hotmail.com
Colaco
M P
MP
Desai
M P
MP
D019080:Cafe-au-Lait Spots / Q000209:etiology; D002675:Child, Preschool; D004958:Estradiol / Q000097:blood; D005260:Female; D005359:Fibrous Dysplasia, Polyostotic / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D006062:Gonadotropins / Q000097:blood; D006801:Humans; D007223:Infant; D008297:Male; D017258:Medroxyprogesterone Acetate / Q000627:therapeutic use; D045167:Progesterone Congeners / Q000627:therapeutic use; D011629:Puberty, Precocious / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology; D016896:Treatment Outcome
D006062:Gonadotropins; D045167:Progesterone Congeners; D004958:Estradiol; D017258:Medroxyprogesterone Acetate