Peripheral Precocious Puberty Due to Autonomous Gonadal Activation: A Multicenter Experience

TL;DR

This study examines children with peripheral precocious puberty in India, highlighting the importance of early diagnosis and treatment to prevent growth issues.

Contribution

The paper provides a multicenter Indian clinical experience on managing peripheral precocious puberty due to gonadal activation.

Findings

Most children presented with menarche as the primary symptom, with a mean age of 4.6 years.

Letrozole was the primary treatment, with leuprolide acetate added for those progressing to central precocious puberty.

Thirty percent of children with peripheral PP progressed to central precocious puberty.

Abstract

Introduction: Peripheral precocious puberty (PP) is far less commonly encountered compared to central precocious puberty (CPP) in pediatric endocrine practice. Long-standing non-diagnosis may cause rapidly advancing bone age, leading to CPP and resultant short stature. We aimed to report the clinical profile and current Indian experience in the management of children with peripheral PP due to autonomous gonadal activation.

Methods: This multicentric retrospective study reports data on 23 children (20 girls) presenting with peripheral PP as a result of autonomous gonadal activation from eight pediatric endocrine centers across India. Their clinicodemographic, anthropometric, and laboratory measurements were reviewed.

Results: The mean ± SD chronological age at the time of presentation was 4.9 ± 2.0 years, and the mean bone age was 7.6 ± 2.6 years. Nine (39%) children were tall for mid-parental height. Thirteen (57%) children were diagnosed with McCune Albright syndrome (MAS), one boy (4%) with familial male-limited PP, and nine (39%) with functional ovarian cysts. Ninety-five percent of girls had menarche as their presenting complaint, with mean age at menarche being 4.6 ± 2.3 years. Ovarian cysts were present in 16 girls, of whom seven (43.8%) had MAS. PP in seven (30%) children progressed to CPP, with mean age of CPP being 6.6 ± 2.1 years. Letrozole was the primary drug of choice, while leuprolide acetate was added in children who progressed to CPP.

Conclusion: We report the clinical profile of children with peripheral PP due to autonomous gonadal activation. Clinical diagnosis and timely intervention to halt the progression of puberty and prevent early epiphyseal fusion, thereby improving final height, along with close follow-up, are vital in the management of these disorders.

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