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Unsustained or Slowly Progressive Puberty in Young Girls: Initial Presentation and Long-Term Follow-Up of 20 Untreated Patients¹

Division of Endocrinology, Department of Medicine, Children’s Hospital (M.R.P.), and the Clinical Investigator Training Program, Beth Israel Deaconess Medical Center-Harvard/Massachusetts Institute of Technology Division of Health Sciences and Technology, in collaboration with Pfizer, Inc. (M.R.P.), Boston, Massachusetts 02115; the Reproductive (H.V.M., P.A.B.) and Pediatric Endocrine ( P.A.B.) Units, Massachusetts General Hospital, Boston, Massachusetts 02114

Address all correspondence and requests for reprints to: Paul A. Boepple, M.D., Reproductive Endocrine Unit, Bartlett Hall Extension 5, Massachusetts General Hospital, Fruit Street, Boston, Massachusetts 02114.

A small number of young girls with unsustained or slowly progressive puberty have been described, but few data regarding their final heights and adult reproductive function have been reported.

We have conducted a study that delineates the initial presentation and 12-yr follow-up of 20 patients who initially presented with unsustained or slowly progressive puberty as young girls. The patients were first seen between 1984–1987. They all underwent extensive clinical and hormonal studies, including frequent blood sampling and pelvic ultrasound to characterize pituitary-gonadal function. Twelve years later, we were able to locate 17 of the patients, and 16 of these agreed to participate in a questionnaire-based follow-up study. Follow-up data about the other patients were gleaned from available medical records as were corroborative data regarding the 16 study participants.

Our results indicate that this form of early puberty is a benign entity. Seventy percent of our patients experienced cessation of their early pubertal development, whereas the remainder reported a slowly progressive course. Those with a slowly progressive course were older than those with an unsustained course [mean age of thelarche, 6.1 vs. 3.4 yr (P < 0.01); age of pubarche, 6.0 vs. 4.0 yr (P = 0.02); age at our evaluation, 7.1 vs. 5.2 yr (P = 0.02)]. They also had more advanced skeletal maturation (bone age, 10.2 vs. 7.3 yr; P = 0.04) at the time of our evaluation. Both groups, however, had similar outcomes with respect to linear growth and young adult reproductive function. On the average, the study patients reached their genetic targets for final height (mean final height, 165.5 ± 2.2 cm; mean genetic target height, 164.0 ± 1.1 cm; P = NS). The average age of menarche was 11.0 ± 0.4 yr. Twenty-three percent of our patients have evidence of anovulatory menstrual cycles, which is comparable to the 28% found in normative studies of similarly aged women. Two of the patients have become pregnant to date. Unsustained or slowly progressive puberty in young girls does not warrant therapy with GnRH agonists. Thus, when evaluating patients with early pubertal development, one should ensure that sexual maturation is continually progressive before initiating potentially unnecessary therapy.

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