CLASP Guidelines

CT Children’s CLASP Guideline

Early Onset Pubic Hair and/or Body Odor

INTRODUCTION Premature Pubarche (PP) refers to the early onset of pubic hair, body odor, and/or axillary hair in girls less than 8 years of age and boys less than 9 years. Premature adrenarche (PA) refers to the early onset of those symptoms in those age groups and is associated with increasing levels of adrenal androgens into the pubertal range. Idiopathic PA is more commonly observed in girls by a ratio of 9:1. Once thought of as a benign condition, new studies are linking PA to functional hyperandrogenism, polycystic ovarian syndrome, hyperinsulinism, Type II diabetes, and metabolic syndrome. PA needs to be distinguished from other conditions requiring treatment, including late onset congenital adrenal hyperplasia (CAH), virilizing adrenal or gonadal tumors, or exogenous exposure to androgens. A bone age (BA) x-ray is the single most important diagnostic test. Linear growth rate in PA and PP is within 2.5 SDs of normal with no evidence of a linear growth spurt.

INITIAL INITIAL EVALUATION: *Laboratory values . . EVALUATION History and physical exam consistent with PP and AND o Should include chronologic details of the onset of the PA: MANAGEMENT pubertal changes, including a family history of precocious . Boys and girls with puberty, congenital adrenal hyperplasia, hirsutism/acne, PP have a normal polycystic ovarian syndrome, or Type II diabetes and early DHEAS level and with cardiovascular disease PA typically have a . Laboratory evaluation DHEAS level of 40- o Indicated for all girls up to 7.99 years of age and all boys up 150 mcg/dL. to 8.99 years of age . In both PP and PA the testosterone o Laboratory evaluation to include BA, DHEAS/total level is < 20 ng/dL. testosterone, and 17-hydroxyprogesterone . We establish the . Careful review of the growth chart and calculation of the growth diagnosis of PP rate and/or PA if the . In addition for children ages 7 or older with BMI >85th percentile, DHEAS and see recommendations for screening for endocrine obesity testosterone levels comorbidities (obesity co-morbidity screening recommendations) fall within these ranges and if the BA INITIAL MANAGEMENT: < 2 SD for age and . Counseling on healthy lifestyle and obesity prevention; gender. consider Structured Weight Management Intervention

WHEN All children less than 4 years of age: TO REFER . Recommend workup above . Refer to Endocrinology Girls between 4-7.99 years of age/boys 4 – 8.99 years of age: . If laboratory results consistent with diagnosis of PP or PA, patient to be followed clinically through your office for tempo of pubertal progression . If laboratory results above specified ranges*, refer to Endocrinology Girls just over 8 years of age/boys just over 9 years of age: . These are the normal ages for children to develop adrenarche. Tempo of their adrenarchal and pubertal changes should be followed clinically through your office at 6 month intervals. . Laboratory evaluation and referral to Endocrinology is only recommended for those patients with a rapid progression of central puberty or virilization.

HOW Referral to Endocrinology via CT Children’s One Call Access Center TO REFER Phone: 833.733.7669 Fax: 833.226.2329

Information to be included with the referral: . Notes from the initial and follow-up visits with the PCP . Complete growth chart . Relevant laboratory and diagnostic studies . Bone age studies should be read by pediatric radiologist

WHAT TO What to expect from CT Children’s Visit: EXPECT . History, physical exam . Imaging studies if appropriate . Evaluation of prior laboratory . Initiation of treatment if appropriate testing and growth chart . Comprehensive patient education . Additional labs if appropriate