Prematüre Pubarş Olgularının Değerlendirilmesi: Tek Merkez Deneyimi

Öz

Amaç: Çalışmada prematüre pubarşlı (PP) olguların başlangıçtaki ve izlem süresindeki antropometrik, klinik ve laboratuvar verilerinin değerlendirilmesi amaçlandı.Gereç ve Yöntemler: Kızlarda 8, erkeklerde 9 yaşından önce pubik ve/veya aksiller kıllanma yakınmasıyla başvuran toplam 117 olgu (110 kız) (ortalama takip süreleri 16.2±13.5 ay) geriye dönük olarak değerlendirildi.Bulgular: Kızların ortalama yaşı 7.2±0.9 yıl (ort±standart sapma), semptomların görülmesi 6.4±1.3 yaş ve ortalama kemik yaşı 8.0±1.3 yıl, kemik yaşı/kronolojik yaş oranı 1.1, boy standart deviasyon skoru 0.6±1.2, hedef boy 159.1±5.3 cm ve tahmini erişkin boy 157.7±7.5 cm’di. Erkeklerde, kronolojik yaş 8.4±0.8 yıl, semptomların başlama yaşı 7.7±1.1’di. Kızların %53.7’si ve erkeklerin %56’sı fazla kiloluydu. Tüm hastaların %21’i düşük doğum ağırlıklıydı (DDA) ve %14’ü prematüre doğmuştu. Serum dehidroepiandrosteron sülfat seviyesi hastaların %76.5’inde 40 μg/dl üzerindeydi ve kemik yaşı ile pozitif ilişkiliydi. Başlangıçta iki hasta klasik konjenital adrenal hiperplazi (21 hidroksilaz eksikliği) ve 9 hasta konjenital adrenal hiperplazi taşıyıcısı tanısı aldı. İzlemde telarşa eşlik eden 12 kıza (%11) luteinizan hormon salgılatıcı hormon testi ile santral puberte prekoks tanısı konuldu. Klinik başlangıcı prematüre pubarş olan 117 olgunun son tanıları şu şekildeydi: 94 (%80) prematüre pubarş, 12 (%11) santral erken puberte, 9 (%7.3) konjenital adrenal hiperplazi taşıyıcısı ve 2 (%2.7) klasik konjenital adrenal hiperplazi.Sonuç: Prematüre pubarşlı olguların %20’sine başka bir klinik patoloji tanımlandığından, ayırıcı tanının sadece başlangıçta değil, takipte de dikkatle gözden geçirilmesi gerektiği vurgulanmak istendi. Ayrıca, ılımlı ilerlemiş kemik yaşının hedef boyu olumsuz etkilemeyeceği sonucuna varıldı.

Anahtar Kelimeler

• Konjenital adrenal hiperplazi, Over, Prematüre pubarş, Uterus

Evaluation of Premature Pubarche Cases: A Single Center Experience

Öz

Objective: We aimed to evaluate anthropometric, clinical and laboratory findings of cases with premature pubarche (PP) at baseline and during follow-up.Material and Methods: A total of 117 cases (110 girls), who presented with the complaint of pubic and/or axillary hair growth before 8 years of age for girls and 9 years for boys, were evaluated retrospectively (the mean fallow up period was 16.2±13.5 months).results: The mean age of the girls was 7.2±0.9 years, the mean age at symptom initiation was 6.4±1.3 years and the mean bone age (BA) was 8.0±1.3 years. The ratio of BA/chronological age (CA) was 1.1, height standard deviation score (SDS) was 0.6±1.2, target height was 159.1±5.3 cm and predicted height was 157.7±7.5 cm. The mean CA of boys was 8.4±0.8 years and the mean age at the initiation of symptoms among the patients was 7.7±1.1 years. 53.7% of girls and 56% of boys were overweight. 21% of all patients had low birth weight (LBW) and 14% were premature at birth. Serum dehydroepiandrosterone sulfate level was higher than 40 µg/dL in 76.5% of patients revealing positive correlation with BA. Two patients were diagnosed with classical congenital adrenal hyperplasia (CAH) (21 hydroxylase deficiency) and nine were found to be CAH carriers at the beginning. On follow-up, 12 girls (11%) with accompanying thelarche were diagnosed with central puberte precox (CPP) after the luteinizing hormone releasing hormone test. The final diagnosis of 117 cases that clinically had PP on the beginning was as follows: 94 (80%) PP, 12(11%) CPP, 9 (7.3%) CAH carrier and 2 (2.7%) classical CAH.conclusion: As another clinic pathology was determined in 20% of PP cases during the repeated evaluations, we would like to emphasize that the differential diagnosis should be performed very attentively not only at the beginning, but also on follow-up. It was found that mildly advanced bone age would not affect the final height unfavorably

Anahtar Kelimeler

• Congenital adrenal hyperplasia, Ovary, Premature pubarche, Uterus

Kaynakça

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