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Precocious puberty more prevalent in girls than boys
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Gonadotropin-independent precocious puberty in children without congenital adrenal hyperplasia appears to be about four times more common in girls than boys because of the two predominant etiologies underlying this condition — functional ovarian cyst and McCune-Albright syndrome — according to recent findings.
In the retrospective, multicenter, nationwide Web-based study, Abdullah Bereket, MD, professor of pediatrics at Marmara University Medical School in Istanbul, and colleagues collected demographic and clinical data on 129 patients with gonadotropin-independent precocious puberty not attributable to congenital adrenal hyperplasia (102 girls; mean age at final diagnosis, 5.3 years) from 42 pediatric endocrinology centers in Turkey from 2014 to 2015. Participants included those diagnosed with one of the following conditions: McCune-Albright syndrome, familial testotoxicosis, functional ovarian cyst, human chorionic gonadotropin–secreting tumors, sex steroid-producing tumors, aromatase excess syndrome and exogenous exposure to sex steroids.
The researchers estimated the overall prevalence of the condition at 14 in 1 million in the general pediatric population.
The most common etiology in the study population was functional ovarian cyst, which included 37% of all cases (46% of cases among girls). Of 47 patients with functional ovarian cysts, 36 had breast enlargement only, whereas 11 demonstrated vaginal bleeding as well. The second most prevalent etiology was McCune-Albright syndrome, which was the cause of 26% of the cases, all of which were girls. Of the 34 patients with McCune-Albright syndrome, 53% had the classic triad of café-au-lait spots, fibrous dysplasia and gonadotropin-independent precocious puberty; 32.3% had café-au-lait spots alone; and 11.7% had fibrous dysplasia alone. Vaginal bleeding was present in 59% of the patients with McCune-Albright syndrome, and two of these patients had no coexisting breast enlargement.
Seven patients had human chorionic gonadotropin-secreting tumors, and these included choriocarcinoma of the liver, hepatoblastoma and germ-cell tumors of the sellar-suprasellar region and mediastinum. Patients with adrenocortical tumor had elevated dehydroepiandrosterone and presented at an earlier age than patients with other etiologies (mean, 3.4 years); however, in a subgroup analysis by sex, this finding retained statistical significance only in girls. Although the etiologies had overlapping features, the researchers were able to discern distinct clinical and laboratory characteristics for each etiology.
The researchers noted that solitary ovarian cysts could potentially be a constituent of McCune-Albright syndrome and, therefore, these patients should be evaluated thoroughly before diagnosing functional ovarian cysts. – by Jennifer Byrne
Disclosure: The researchers report no relevant financial disclosures.
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