Puberte prekoks tanılı hastaların klinik özellikleri ve tedavi sonu bulgularının retrospektif olarak değerlendirilmesi
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Date
2012
Authors
Gül, Ülkü
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Publisher
Uludağ Üniversitesi
Abstract
Bu çalışmada Uludağ Üniversitesi Tıp Fakültesi Pediatrik Endokrinoloji Bilim Dalımız’da 10 yıllık izlem süresinde puberte prekoks tanılı hastaların etyolojisinin, prognozunun, kliniğinin ve tedavi yanıtının değerlendirilmesi amaçlandı. Ocak 1998 ile Aralık 2008 tarihleri arasında tanı almış hastaların dosyaları retrospektif olarak incelenmiştir. Toplam 97 hastanın 78’i (%79,8) SPP (%1 hipotalamik hamartom, %79,3 idiopatik ) ,19’u (%20,2) PPP (%2,1 over kisti ,%2,1 izole klasik KAH, %15,5 kombine puberte prekoks) tanısı almıştı. SPP grubunda PPP grubu ile karşılaştırıldığında kız hastaların daha fazla olduğu saptandı (p<0,0001). PPP grubunda erkek sayısı beklenildiği gibi daha fazlaydı ve yaş ortalaması SPP grubuna göre küçük bulundu (p=0,006). Kız hastalarda en sık geliş nedeninin meme büyümesi, erkek hastalarda pubik kıllanma olduğu görüldü. SPP‘lu kız hastaların en sık saptanan telarş evresi Evre 3, pubarş evresi Evre 2 idi. SPP‘lu erkek hastaların pubarş evrelemesinde Evre 2 ve 3’de eşit sayıda hasta vardı. PPP’lu kız hastaların telarş evrelemesinde en sık saptanan Evre 1 idi ve pubarş evrelemesinde Evre 2 ve Evre 3’de eşit sayıda hasta mevcuttu. PPP grubundaki erkek hastaların adenarş evrelerine bakıldığında en sık Evre 3’de başvurdukları görüldü. Kızlarda ve erkeklerde, hem SPP hem de PPP grubunda tanı anında ÖB ile tedavi sonunda ÖB arasında anlamlı fark bulundu (p<0,0001). Tedavi sonrasında ÖB’daki iyileşme sonucunda ÖB ile HB arasında anlamlı fark saptanmadı (p=0,51). Başvuru anında VKİ ortalama SDS değeri SPP ile PPP grubu arasında farklı değildi fakat tedavi sonunda PPP grubunda SPP grubuna göre yüksek bulundu (p=0,021). SPP grubunda tedavi öncesi ΔKY-TY farkının tedavi sonrası farka göre daha fazla olduğu saptandı (p<0,0001). PPP grubunda ise tedavi sonrası ΔKY-TY farkının tedavi öncesi farkla kıyaslandığında istatistiksel olarak anlamlı olmadığı görüldü (p=0,078). Erken tanı ve tedavi hem altta yatan asıl hastalığın kontrolü, hem de hastanın büyüme potansiyelinin korunabilmesi için önemlidir.
In this study we aimed to evaluate the etiology, prognosis, clinical properties, and response to treatment of precocious puberty patients followed up for ten years in our patients diagnosed between January 1998 and December 2008 were studied retrospectively. Of 97 patients, 78 (79,8%) patients’ diagnosis was CPP (1% hypothalamic hamartoma, 79,3% idiopathic), 19 (20,2%) patients’ diagnosis was PPP (2,1% ovarian cyst, 2,1% isolated classical CAH, 15,5% combined precocious puberty). The number of female patients were higher in PPP (p<0,0001) and lower in CPP (p=0,006). Enlargement of breasts was the most frequent complaint in girls, whereas appearance of pubic hair was the most common complaint among male patients. Thelarche stage 3 and pubarche stage 2 was the most common pubertal stage among girls with CPP. Among the male patients with CPP, the frequency of stage 2 and 3 pubarche was equal. Thelarche stage 1 was most common among female patients with PPP. In this group, the frequency of stage 2 and 3 pubarche was equal. Stage 3 adrenarche was the most common presentation ammong male patients with PPP. Significant difference between predicted height before and after treatment was fount in both CPP and PPP groups (p<0,0001). With the improvement of predicted height after treatment, the difference between predicted and target heights became nonsignificant (p=0,51). At presentation, there was no difference between mean SDS of BMI’s of PPP and CPP groups, whereas it was higher at PPP group after treatment (p=0,021). In CPP group, ΔBA-CA was lower after treatment than before (p<0,0001). However, at PPP group were there was no significant difference between before and after treatment ΔBA-CA (p=0,078). Early diagnosis is crucial both for controlling the underlying disease and for preservation of growth potential of the patient.
In this study we aimed to evaluate the etiology, prognosis, clinical properties, and response to treatment of precocious puberty patients followed up for ten years in our patients diagnosed between January 1998 and December 2008 were studied retrospectively. Of 97 patients, 78 (79,8%) patients’ diagnosis was CPP (1% hypothalamic hamartoma, 79,3% idiopathic), 19 (20,2%) patients’ diagnosis was PPP (2,1% ovarian cyst, 2,1% isolated classical CAH, 15,5% combined precocious puberty). The number of female patients were higher in PPP (p<0,0001) and lower in CPP (p=0,006). Enlargement of breasts was the most frequent complaint in girls, whereas appearance of pubic hair was the most common complaint among male patients. Thelarche stage 3 and pubarche stage 2 was the most common pubertal stage among girls with CPP. Among the male patients with CPP, the frequency of stage 2 and 3 pubarche was equal. Thelarche stage 1 was most common among female patients with PPP. In this group, the frequency of stage 2 and 3 pubarche was equal. Stage 3 adrenarche was the most common presentation ammong male patients with PPP. Significant difference between predicted height before and after treatment was fount in both CPP and PPP groups (p<0,0001). With the improvement of predicted height after treatment, the difference between predicted and target heights became nonsignificant (p=0,51). At presentation, there was no difference between mean SDS of BMI’s of PPP and CPP groups, whereas it was higher at PPP group after treatment (p=0,021). In CPP group, ΔBA-CA was lower after treatment than before (p<0,0001). However, at PPP group were there was no significant difference between before and after treatment ΔBA-CA (p=0,078). Early diagnosis is crucial both for controlling the underlying disease and for preservation of growth potential of the patient.
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Keywords
Puberte prekoks, Puberte prekoks tedavisi, Hedef boy, Öngörülen boy, Kemik yaşı, Vücut kitle indeksi, Precocious puberty, Precocious puberty treatment, Target height, Predicted height, Bone age, Body mass index
Citation
Gül, Ü. (2012). Puberte prekoks tanılı hastaların klinik özellikleri ve tedavi sonu bulgularının retrospektif olarak değerlendirilmesi. Yayınlanmamış uzmanlık tezi. Uludağ Üniversitesi Tıp Fakültesi.