ESPE Abstracts (2015) 84 P-1-112

Aetiological Spectrum and Clinical Characteristics of 129 Children with Gonadotropin Independent Precocious Puberty: A Nationwide Cohort Study

Zeynep Ataya, Ediz Yeşilkayab, Senay Savas Erdevec, Leyla Akind, Erdal Erene, Esra Dögerf, Zehra Aycanc, Zehra Yavaş Abalig, Ayşehan Akincih, Zeynep Şiklari, Samim Özenj, Cengiz Karak, Meltem Tayfunl, Filiz Tutunculerm, Gülcan Seymen Karabulutn, Gülay Karagüzelo, Halil Sağlame, Aysun Bidecif, Selim Kurtoğlud, Abdullah Bereketa, Serap Turana, Erkan Sarib, Semra Çetinkayab & Tülay Güranb

aDepartment of Pediatric Endocrinology, Medical School, Marmara University, Istanbul, Turkey; bDepartmet of Pediatric Endocrinology, Gulhane Military Medical Academy, Ankara, Turkey; cDr Sami Ulus Research Hospitali Department of Pediatric Endocrinology, Ankara, Turkey; dDepartment of Pediatric Endocrinology, Medical School, Erciyes University, Kayseri, Turkey; eDepartment of Pediatric Endocrinology, Medical School, Uludag University, Bursa, Turkey; fDepartment of Pediatric Endocrinology, Medical School, Gazi University, Ankara, Turkey; gDepartment of Pediatric Endocrinology, Medical School, Istanbul University, Istanbul, Turkey; hDepartment of Pediatric Endocrinology, Medical School, Inonu University, Malatya, Turkey; iDepartment of Pediatric Endocrinology, Medical School, Ankara University, Ankara, Turkey; jDepartment of Pediatric Endocrinology, Medical School, Ege University, Izmir, Turkey; kDepartment of Pediatric Endocrinology, Medical School, Ondokuz Mayis University, Samsun, Turkey; lDiskapi Research Hospital, Ankara, Turkey; mMedical School, Trakya University, Edirne, Turkey; nMedical School, Kocaeli University, Kocaeli, Turkey; oDepartment of Pediatric Endocrinology, Medical School, Karadeniz Technical University, Trabzon, Turkey

Background: Gonadotropin independent precocious puberty (GIPP) is caused by a heterogenous group of disorders. With the exception of congenital adrenal hyperplasia (CAH), disorders causing GIPP are uncommon, and there are no studies evaluating the etiologic distribution of GIPP in a large cohort.

Objective and hypotheses: To find out the relative frequencies of each etiological group in patients with non-CAH GIPP and also to evaluate the clinical and laboratory features of these patients.

Method: In this multicenter, nationwide, web-based study; data regarding the patients presenting with GIPP (excluding those with CAH) were gathered.

Results: 129 patients (104 females, 25 males) from 28 centres were registered. Functional ovarian cysts were the most common aetiology and constituted 37% of all cases. 55% of the cysts were located in the right ovary, average cyst size was 37 mm (10–88 mm). McCune Albright syndrome (MAS) was the second most common aetiology. Cafe au lait and fibrous dysplasia were seen in 32 and 44% respectively in MAS patients, 18% had both. HCG secreting tumours were choriocarcinoma of the liver, hepatoblastoma and germ cell tumors of sellar-suprasellar region. Patients with adrenocortical tumours presented at an earlier age than other aetiologies. Ten were carcinomas and both adrenals were affected equally. Ovarian tumours were mature cystic teratoma, dysgerminoma, juvenile granulosa tumour and steroid cell tumour.

Table 1 Aetiological spectrum, clinical and laboratory features of each diagnostic group. (for abstract P1.112)
DiagnosisOvarian cystMcCune Albright SyndromeTestotoxicosisHypothyroidismAdrenocortical tumorHCG secreting tumorLeydig cell tumorOvarian tumorSertoli call tumorUndetermined
Patient Number (%)47 (37%)34 (26%)5 (4%)7 (5.5%)12 (9%)7 (5.5%)5 (4%)5 (4%)3 (2%)4 (3%)
Sex F/M47/034/00/55/27/50/50/55/00/34/0
Mean Age at diagnosis (year)5.4 (0.4–10.1)5.2 (0.8–9.6)4.1 (1.4–8.3)6.8 (2.8–9.6)3.4 (0.8–7.7)7 (0.3–10.6)6.1 (4.6–8.8)7.3 (4–11.3)6.1 (4.6–7.2)4.3 (3.3–6.5)
Height SDS0.55 (–1.72/3.11)0.76 (–4.01/5.97)3.06 (0.9/6.26)−0.76 (−4.47/2.88)0.91 (−1.54/3.66)1.1 (−0.18/4.68)1.69 (−0.41/3.96)−0.11 (−0.66/1.02)0.9 (0.03/1.4)1.18 (0.5/1.73)
BA-CA (year)−1.722.
Basal FSH (mIU/ml)0.6 (0.01–3.6)0.7 (0.01–3.1)0.4 (0.05–1.03)4 (0.3–11.7)0.3 (0.09–0.7)0.2 (0.05–0.6)0.5 (0.1–0.8)1.5 (0.1–4.8)0.12 (0.1–0.2)0.2 (0–0.7)
Basal LH (mIU/ml)0.1 (0–0.72)0.1 (0–0.65)0.1 (0.07–0.2)0.1 (0–0.23)0.1 (0–0.6)0.1 (0.01–0.2)0.2 (0.1–0.2)1 (0.1–4.8)0.08 (0.05–0.1)0.06 (0.01–0.2)
E2 (pg/ml)211 (6.2–879)193.5 (5–2792)80.5 (40.7–163.7)25 (10–73.9)20 (28–48)29 (9–48)74.6 (30–170.4)
Peak LH (mIU/ml)0.6 (0.07–4.9)0.9 (0–5.1)1.83 (0.4–2.7)
Peak FSH (mIU/ml)2.1 (0.2–8.7)3.8 (0.1–16.6)4.4 (1–6.7)
Testosterone (ng/ml)15.6 (2.6–32)0.5 (0.2–0.84)4.3 (1.08–8.7910.9 (0.1–23.9)4.7 (0.9–1.6)1.7 (0.1–4.4)0.1 (0.03–0.2)
DHEAS (ng/ml)827 (30–1543)77.9 (21.8–152)
*Values are given as mean (range).

Conclusion: This largest cohort of non-CAH GIPP demonstrates that ovarian cysts and MAS predominates over the other aetiologies. Detailed clinical features of each diagnostic group will be discussed at the meeting.

Funding: Turkish Pediatric Endocrinology Society Favor Group.

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