RT Journal Article
SR Electronic
T1 Follow up of precocious pseudopuberty associated with isolated ovarian follicular cysts
JF Archives of Disease in Childhood
JO Arch Dis Child
FD BMJ Publishing Group Ltd and Royal College of Paediatrics and Child Health
SP 53
OP 56
DO 10.1136/adc.81.1.53
VO 81
IS 1
A1 K A Rodriguez-Macias
A1 E Thibaud
A1 M Houang
A1 C Duflos
A1 C Beldjord
A1 R Rappaport
YR 1999
UL http://adc.bmj.com/content/81/1/53.abstract
AB The clinical outcomes of seven girls presenting with pseudosexual precocity caused by isolated autonomous ovarian follicular cysts are presented. Six of the seven girls, aged 11 months to 6.9 years, had a unilateral ovarian cyst detected by ultrasound at the first acute episode. Plasma oestradiol was raised in only five of the cases, but all had a low response to luteinising hormone releasing hormone stimulation. Follow up lasted for up to eight years with recurrent episodes of variable frequency and severity in all seven patients. Evidence of McCune-Albright syndrome appeared later in only three patients. It could not be predicted from the initial symptoms or the clinical course. Mutations of the Gsα protein leading to activation were investigated in the lymphocytes and ovarian and bone tissues of four patients. Only one patient showed a mutation in bone tissue. Close follow up with repeated searches for skeletal lesions remains necessary since the distribution of somatic mutations cannot be assessed by molecular studies. Most patients with recurrent ovarian cysts require a conservative approach.