Pubertas Terlambat pada Thalassemia Mayor

Diah Pramita, Jose RL. Batubara

Sari


Gangguan kelenjar endokrin akibat penimbunan besi pada pasien thalassemia mayor
semakin sering ditemukan seiring dengan meningkatnya harapan hidup. Kegagalan
pubertas merupakan komplikasi endokrin yang paling sering diikuti oleh amenore
sekunder. Angka kejadian pubertas terambat pada thalassemia mayor bervariasi tergantung
jenis kelamin, riwayat transfusi darah dan terapi kelasi besi yang pemah didapat, dan
usia saat memulai terapi kelasi.
Para peneliti mendapatkan kejadian pubertas terlambat lebih sering terjadi pada lakilaki.
Secara umum, penyebab pubertas terlambat dikiasifikasikan menjadi gangguan
temporer sekresi gonadotropin, dan steroid seks, kegagalan poros hipotalamus-hipofisis
dengan defisiensi sekresi gonadotropin dan kegagalan gonad primer. Gangguan tersebut
diakibatkan oleh proses peroksidasi lipid oleh penimbunan besi di dalam sel-sel kelenjar
hipofisis dan kerusakan di hipofisis mi menyebabkan sekresi gonadotropin menurun.
Secara klinis keterlambatan pubertas pada perempuan umumnya keterlambatan
pertumbuhan payudara dan rambut pubis, sedang pada lakilaki terjadi gangguan
perkembangan testis. Penting untuk melakukan pemeriksaan hormonal untuk mengetahui
fungsi poros hipotalamus-hipofisis-gonad. Tatalaksana thalassemia mayor dengan
pubertas terlambat selain terapi pengganti hormonal, perlu diperhatikan status nutrisi,
dan pemberian transfusi disertai terapi kelasi yang adekuat.


Kata Kunci


thalassemia; pubertas; gonadotropin; hipofisis; hipotalamus

Teks Lengkap:

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DOI: http://dx.doi.org/10.14238/sp5.1.2003.4-11

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