Follow-Up on Puberty Development and Final Height in 41 Children with Simple Virilizing Form of 21-Hydroxylase Deficiency / 实用儿科临床杂志

ABSTRACT

Objective To explore the puberty and height development in childern with congenital adrenal hyperplasia(CAH) due to 21-hydroxylase deficiency(21-OHD) with the simple virilizing(SV) form.Methods Patients of SV 21-OHD,diagnosed and treated in Tianjin Children's Hospital from Jan.1970 to Jan.2008,were treated with cortisone acetate or hydrocortisone after diagnosed,and blood investigations were performed as part of monitoring,and dosages were adjusted accordingly to obtain normal growth velocity and slow bone age(BA) development.Gonadotropin-releasing hormone agonist(Gn-RHa) was given after the appearance of central precocious puberty(CPP).Forty-one patients(18 females,23 males) had achieved final height before Dec.31st 2008,and their puberty and height development were analyzed in this longitudinal study by contrast the ages and statures of obtaining final height(FH),chronological age(CA) and BA of G2/B2 stage,duration from G2/B2 stage to obtain FH and stature development of different gender during this period and the influences of compliancy on the ages and statures of obtaining FH were contrasted.Results With regarding to the age of testicular volume ≥4 mL,78.26% boys were before 9 years old.As far as the age of breast development was concerned,38.89% girls were before 8 year old and 66.67% menarche occurred before 10 year old.The differences between the duration and stature development of the period from the state of G2/B2 to skeletal maturation of different gender were significant (Pa