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

Area of Science:

• Pediatric Endocrinology
• Reproductive Medicine
• Genetics

Background:

• Peripheral precocious puberty (PP) is less common than central PP (CPP).
• Delayed diagnosis of PP can lead to advanced bone age, potentially resulting in CPP and short stature.
• Understanding the clinical profile of PP due to autonomous gonadal activation is crucial for effective management.

Purpose of the Study:

• To describe the clinical characteristics of children with peripheral PP caused by autonomous gonadal activation.
• To present the Indian experience in managing these cases.
• To highlight the importance of early diagnosis and intervention.

Main Methods:

• A multicentric retrospective study involving 23 children (20 girls) from eight Indian pediatric endocrine centers.
• Review of clinicodemographic, anthropometric, and laboratory data.
• Analysis of presenting complaints, diagnoses, and treatment outcomes.

Main Results:

• The average age at presentation was 4.9 years, with a bone age of 7.6 years.
• McCune Albright syndrome (MAS) was diagnosed in 57% of cases; 39% had functional ovarian cysts.
• Menarche at a mean age of 4.6 years was the primary complaint in 95% of girls; 30% of children progressed to CPP.

Conclusions:

• Peripheral PP due to autonomous gonadal activation presents unique challenges in pediatric endocrine practice.
• Early diagnosis and intervention are critical to halt puberty progression, prevent early epiphyseal fusion, and optimize final height.
• Close follow-up is essential for managing these complex cases.