ESPE Abstracts (2015) 84 P-3-1155

Familial Precocious Puberty: Clinical Characteristics and GnRH Agonist Response

Hwalrim Jeonga, Eun Byul Kwonb, Hae Sang Leeb, Jung Sub Limc & Jin Soon Hwangb

aSchool of Medicine, Gyengsang National University, Jinju, Republic of Korea; bSchool of Medicine, Ajou University, Suwon, Republic of Korea; cKorea Cancer Center Hospital, Seoul, Republic of Korea

Background: Familial precocious puberty is defined by the existence of more than one affected member either in the proband generation or in the pedigree. Recently, several gene mutation cause familial CPP is elucidated, gain of function mutations in KISS1 and KISS1R, loss of function mutations in the MKRN3, the feature of familial precocious puberty is not fully understood.

Objective: To investigate the clinical characteristics of familial precocious puberty (FPP) and the response of GnRH agonist treatment compared to sporadic precocious puberty (SPP).

Method: This study was conducted retrospectively. 62 sibling pateints with familial precocious puberty and 60 patients with sporadic precocious puberty was included. We reviewed their auxological data, family history and laboratory finding and also analyzed the response of GnRH agonist treatment and change of predicted adult height.

Results: The onset of precocious puberty was not available. Baseline characteristics including age, bone age, height SDS, the bone age advancement, BMI, Tanner stage, LH peak on GnRH stimulation test and PAH revealed no significant difference between familial CPP and sporadic CPP. Target height, paternal height and maternal age at menarche were lower in FPP group than SPP group (158.58±3.33 and 160.12±3.44 (cm), 170.88±4.50 and 173.10±5.32 (cm), 12.60±1.329 and 13.19±1.02 (year) respectively, P<0.05). PAH after GnRH agonist was greater in familial CPP group than sporadic group (165.21±5.29 and 162.28±4.80 (cm), P<0.05).

Conclusion: Familial precocious puberty was characterised by significantly lower target height, paternal height and maternal age at menarche than sporadic precocious puberty. GnRH agonist treatment can improve the growth outcome of FPP. Detailed family history, close follow up of growth and pubertal changes in the younger siblings is necessary.

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