Sindrom Kartagener PRESENTASI
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Sindrom Kartagener
Pembimbing: Dr. Erwin Azmar, Sp.PD
oleh: Sekhar Methis GS, S.Ked
Departemen Penyakit DalamRSMH Palembang/FK Unsri
2010
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Daftar Isi
DEFINISIEPIDEMIOLOGIETIOPATOGENESISMANIFESTASI KLINIKPEMERIKSAAN PENUNJANGPENATALAKSANAANKOMPLIKASIPROGNOSISPENCEGAHAN
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DEFINISI
suatu kelainan resesif autosomal yang terdiri dari trias bronkiektasis, sinusitis kronik, dan situs inversus dengan dekstrokardia.
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EPIDEMIOLOGI
1:32000 kelahiran hidupTidak ada predileksi pada jenis
kelamin, etnis, atau daerah geografis tertentu
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ETIOPATOGENESIS
Genetik resesif autosomalGen DNAH5, DNAH11 dan DNA11
pada pita kromosom 5p5.1 dan 9p13.3Lengan dynein
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Axonem silia 2 mikrotubulus sentral dikelilingi oleh 9 doublet mikrotubulus. Setiap doublet mempunyai subunit A dan subunit B yang melekat sebagai setengah lingkaran. Selubung sentral membungkus 2 mikrotubulus sentral, yang mana melekat di doublet mikrotubulus paling luar melalui jari-jari radial. Doublet diinterkoneksi lewat rantai nexin, dan setiap subunit A dilekatkan pada 2 lengan dynein yang berisi ATP; 1 lengan dalam dan 1 lengan luar.Lengan dyneinpergerakan silia
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gerakan silia normal Gerakan silia tidak beraturan
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MANIFESTASI KLINIK
SITUS INVERSUS DENGAN DEKSTROKARDIA
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manifestasi klinik con’t
BRONKIEKTASIS
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manifestasi klinik con’t
SINUSITIS KRONIK
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manifestasi klinik con’t
HIDUNG POLIP NASI
TELINGA OTITIS MEDIA
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PEMERIKSAAN PENUNJANG
Laboratorik : analisa semenPencitraan : radiograf, CT ScanTes skrining : tes sakarin, pengukuran nitric
oxideTes audiologi Uji fungsi paruHistopatologik : biopsi mukosaEndoskopikUji Genetik
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PENATALAKSANAAN
AntibiotikBronkodilatorDrainase posturalTerapi bedah : tuba timpanostomi, operasi sinus
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KOMPLIKASI
Situs inversus penyakit jantung kongenitalSinusitis kronik komplikasi orbita,
komplikasi intrakranialOtitis media kronik komplikasi intrakranial,
komplikasi ekstrakranial
Infertilitas
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PROGNOSIS
Semakin mendekati dekade kedua, keluhan semakin berkurang
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PENCEGAHAN
KONSELING GENETIK EDUKASI GENETIKKONSELING PSIKOSOSIAL