{"id":5761,"date":"2022-12-27T16:39:30","date_gmt":"2022-12-27T13:39:30","guid":{"rendered":"https:\/\/zulfikarpolat.com\/?page_id=5761"},"modified":"2023-03-24T14:00:59","modified_gmt":"2023-03-24T11:00:59","slug":"primer-sklerozan-kolanjit","status":"publish","type":"page","link":"https:\/\/zulfikarpolat.com\/en\/primer-sklerozan-kolanjit\/","title":{"rendered":"Primer Sklerozan Kolanjit"},"content":{"rendered":"\t\t
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Primer Sklerozan Kolanjit Nedir? Tan\u0131s\u0131 Ve Tedavisi<\/h2>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/section>\n\t\t\t\t
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Primer sklerozan kolanjit (PSK) <\/b>karaci\u011fer i\u00e7i ve d\u0131\u015f\u0131 safra yollar\u0131n\u0131 tutan, v\u00fccudun kendi ba\u011f\u0131\u015f\u0131kl\u0131k sistemi (immun sistem) taraf\u0131ndan tetiklendi\u011fi d\u00fc\u015f\u00fcn\u00fclen, safra yollar\u0131nda olu\u015fan daralmalar nedeniyle safra ak\u0131m\u0131n\u0131n engellenmesine ba\u011fl\u0131 bulgularla kendini g\u00f6steren bir kronik safra yolu hastal\u0131\u011f\u0131d\u0131r. <\/span><\/p>\n

Safra kanallar\u0131nda ilerleyici harabiyet ve daralmayla karakterize olan bu hastal\u0131k genellikle bir inflamatuar barsak hastal\u0131l\u0131\u011f\u0131 birlikte bulunur (Bkz. \u0130nflamatuar barsak hastal\u0131klar\u0131, \u00fclseratif kolit ve Crohn hastal\u0131\u011f\u0131). Hastal\u0131\u011f\u0131n do\u011fal seyri s\u0131ras\u0131nda siroz ve safra yollar\u0131 kanseri (kolanjiokarsinom) geli\u015febilir.<\/span><\/p>\n

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Epidemiyoloji<\/span><\/h2>\n<\/div>\n<\/div>\n
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Primer sklerozan kolanjit<\/b>in bat\u0131 toplumlar\u0131ndaki prevalans\u0131n\u0131n 6-8\/ 100.000 oldu\u011fu tahmin edilmektedir. Olgular\u0131n %70-75 i bir inflamatuar barsak hastal\u0131l\u0131\u011f\u0131 (\u0130BH) ile birliktelik g\u00f6sterir. \u0130BH ile beraber olan primer sklerozan kolanjit hastalar\u0131n\u0131n %85 inde \u00fclseratif kolit, %15 inde de Crohn hastal\u0131\u011f\u0131 bulunur. Tersine \u0130BH hastal\u0131\u011f\u0131 bulunan olgular\u0131n \u00e7ok az bir k\u0131sm\u0131nda (%6-8) PSK bulunmaktad\u0131r.Hastalar\u0131n \u00be \u00fc erkektir ve ortalama tan\u0131 ya\u015f\u0131 40\u2019t\u0131r. Beraberinde \u0130BH olmayan olgular\u0131n \u00e7o\u011funlu\u011fu kad\u0131nd\u0131r.<\/span><\/p>\n<\/div>\n<\/div>\n<\/div>\n

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Etyoloji ve Patogenez<\/span><\/h2>

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PSK <\/b>in bir immun sistem hastal\u0131\u011f\u0131 oldu\u011fu d\u00fc\u015f\u00fcn\u00fclmekle birlikte hastal\u0131\u011f\u0131n etyolojisi ve patogenezi tam olarak bilinmemektedir.Toksinler, infeksiyonlar, iskemi (karaci\u011fer kanlanmas\u0131nda azalma), genetik yatk\u0131nl\u0131k ve otoimmun fakt\u00f6rler su\u00e7lanan nedenler aras\u0131ndad\u0131r. PSK li hastalar\u0131n %70 inde birlikte bir inflamatuar barsak hastal\u0131\u011f\u0131 (\u00f6zellikle \u00fclseratif kolit) bulunur. \u0130ki hastal\u0131k aras\u0131ndaki bu ili\u015fki, inflamasyonlu kal\u0131n barsak mukozas\u0131ndan kolayca ge\u00e7ebilen bakteriyel \u00fcr\u00fcnlerin veya toksik safra asitlerinin karaci\u011fere ve safra yollar\u0131na ula\u015fmas\u0131 sonucunda kronik kolanjit geli\u015febilece\u011fini d\u00fc\u015f\u00fcnd\u00fcrm\u00fc\u015ft\u00fcr.[\/caption]<\/span><\/p>\n

PSK de ailesel yatk\u0131nl\u0131k s\u00f6z konusudur. Di\u011fer otoimmun hastal\u0131klar\u0131n bulundu\u011fu ailelerde PSK, bulunmayanlara g\u00f6re daha s\u0131kt\u0131r. HLA ili\u015fkisi HLA B8,DR3 ve DR4 ile g\u00f6sterilmi\u015ftir.PSK li hastalarda kanda de\u011fi\u015fik otoantikorlar\u0131n bulunma s\u0131kl\u0131\u011f\u0131 artm\u0131\u015ft\u0131r. En s\u0131k g\u00f6r\u00fclen otoantikorlar, antin\u00fckleer stoplazmik antikor(ANCA)(%65-85), antikardiolipin antikor (ACA) (%65) ve antin\u00fckleer antikor (ANA)(%53) dur. Olgular\u0131n en az %80 inde serum kompleman d\u00fczeyleri y\u00fcksektir.<\/span>
\nBiliyer arteryel sistemdeki travmalar ve karaci\u011fer transplantasyonu sonras\u0131nda olu\u015fabilecek vask\u00fcler hasarlanma PSK\u2019ya benzer bir tabloya neden olabilmektedir.<\/span><\/p>\n<\/div>\n<\/div>\n<\/div>\n

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Primer Sklerozan Kolanjit Tan\u0131s\u0131<\/span><\/h3>

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PSK<\/b>\u2018de kullan\u0131labilecek karakteristik otoantikor testi yoktur. Kan tahlillerinde serum alkalen fosfataz (AF) ve gamma glutamil trasferaz (GGT) d\u00fczeylerinde belirgin art\u0131\u015f g\u00f6r\u00fcl\u00fcrken aminotransferaz d\u00fczeylerinde (ALT ve AST) hafif art\u0131\u015flar g\u00f6zlenir. Sar\u0131l\u0131\u011f\u0131 olan olgularda serum bilir\u00fcbin d\u00fczeyi y\u00fcksek bulunur (Bkz. Karaci\u011fer fonksyon testleri). Vakalar\u0131n 1\/3 inde hipergamaglobulinemi (\u00f6zellikle IgM) g\u00f6r\u00fcl\u00fcr. Olgular\u0131n %65-85 inde kanda antin\u00fckleer stoplazmik antikor (ANCA) pozitif bulunur.<\/span><\/p>\n

Tan\u0131, klinik ve biyokimyasal bulgular e\u015fli\u011finde yap\u0131lacak bir kolanjiografide tipik bulgular\u0131n g\u00f6r\u00fclmesiyle ve benzer bulgular olu\u015fturabilecek di\u011fer hastal\u0131klar\u0131n ay\u0131rdedilmesiyle koyulur. Kolanjiografi (safra yollar\u0131n\u0131n radyolojik olarak g\u00f6r\u00fcnt\u00fclenmesi) ERCP i\u015flemi ile ile yap\u0131l\u0131r ve PSK tan\u0131s\u0131nda kullan\u0131lan en de\u011ferli te\u015fhis y\u00f6ntemidir (Bkz. ERCP- Endoskopik Retrograd Kolanjio Pankreotografi). ERCP yap\u0131lamayan veya yap\u0131lmas\u0131 istenmeyen olgularda MRCP tan\u0131da yard\u0131mc\u0131d\u0131r (MRCP- Manyetik Rezonans Kolanjio Pankreotografi). <\/span><\/p>\n

Son y\u0131llardaki teknolojik ilerlemeler MRCP ile daha kaliteli g\u00f6r\u00fcnt\u00fcler al\u0131nmas\u0131n\u0131 sa\u011flad\u0131\u011f\u0131ndan MRCP bir\u00e7ok hastada te\u015fhiste ilk tercih edilecek y\u00f6ntem olmaya ba\u015flam\u0131\u015ft\u0131r. ERCP ve MRCP hastal\u0131\u011f\u0131n yayg\u0131nl\u0131\u011f\u0131 ve safra yollar\u0131ndaki daralmalar\u0131n yeri ve derecesi hakk\u0131nda bilgi verir. PSK de genellikle hem karaci\u011fer i\u00e7i (intrahepatik) hem de karaci\u011fer d\u0131\u015f\u0131 (ekstrahepatik) safra kanallar\u0131 yayg\u0131n olarak tutulmu\u015ftur. ERCP de ve MRCP de safra yollar\u0131nda daralmalar, tesbih taneleri \u015feklinde geni\u015flemeler ve kurumu\u015f -budanm\u0131\u015f a\u011fa\u00e7 g\u00f6r\u00fcn\u00fcm\u00fc PSK i\u00e7in karakteristik bulgulard\u0131r.<\/span><\/p>\n

Tan\u0131 i\u00e7in karaci\u011fer biyopsisine (karaci\u011ferden ince bir i\u011fne ile doku \u00f6rne\u011fi al\u0131nmas\u0131) nadiren ihtiya\u00e7 duyulur. Biopsi hastal\u0131\u011f\u0131n evrelendirilmesi ve prognozun belirlenmesinde yard\u0131mc\u0131d\u0131r. Karaci\u011fer biopsisinde en s\u0131k g\u00f6r\u00fclen karakteristik bulgu safra kanallar\u0131 \u00e7evresinde g\u00f6r\u00fclen \u2018so\u011fan zar\u0131\u2019 g\u00f6r\u00fcn\u00fcm\u00fcn\u00fc and\u0131ran (onion skin) fibrozisdir (ba\u011f dokusu geli\u015fmi). <\/span><\/p>\n

Kolanjiografide bir anormallik saptanmayan hastalar\u0131n %10 kadar\u0131nda karaci\u011fer biyopsisinde k\u00fc\u00e7\u00fck safra kanallar\u0131nda so\u011fan zar\u0131 g\u00f6r\u00fcn\u00fcm\u00fc saptanabilir. Bu durum k\u00fc\u00e7\u00fck kanal Primer sklerozan kolanjit olarak adland\u0131r\u0131l\u0131r (small-duct PSC). Hastal\u0131\u011f\u0131n bu t\u00fcr\u00fc klasik t\u00fcr\u00fcne g\u00f6re daha iyi bir prognoza sahiptir.<\/span> <\/span><\/p>\n<\/div>\n<\/div>\n<\/div>\n

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Ay\u0131r\u0131c\u0131 Tan\u0131<\/span><\/h3>\n<\/div>\n<\/div>\n
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Primer biliyer siroz, sekonder sklerozan kolanjit, yayg\u0131n karaci\u011fer metastaz\u0131, kolanjiokarsinom, yayg\u0131n karaci\u011fer apseleri, amiloidoz, do\u011fumsal safra yollar\u0131 hastal\u0131klar\u0131, greft versus hastal\u0131\u011f\u0131 (organ nakli sonras\u0131nda al\u0131c\u0131n\u0131n verilen ilik h\u00fccrelerini reddetmesi sonucunda ortaya \u00e7\u0131kan bir tablo), karaci\u011fer transplantasyonu sonras\u0131nda ve otoimmun pankreatitte karaci\u011ferde Primer sklerozan kolanjite benzer bulgular olu\u015fabilir. Uygun biyokimyasal, immunolojik ve radyolojik y\u00f6ntemlerin kullan\u0131lmas\u0131yla ay\u0131r\u0131c\u0131 tan\u0131 yap\u0131l\u0131r.<\/span><\/p>\n

Klinik<\/span><\/h3>

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Hastal\u0131k en s\u0131k 25-45 ya\u015f aras\u0131nda g\u00f6r\u00fcl\u00fcr. Olgular\u0131n %70 i \u0130BH ile beraberdir Hastalar\u0131n \u00e7o\u011funda hastal\u0131\u011fa ait bir belirti bulunmaz (Asemptomatik hasta). Tan\u0131 genellikle poliklinik ba\u015fvurusu s\u0131ras\u0131nda istenen karaci\u011fer fonksiyon testlerindeki bozukluklar\u0131n ara\u015ft\u0131r\u0131lmas\u0131 veya inflamatuar barsak hastal\u0131\u011f\u0131 olan bir hastan\u0131n rutin tetkilerinin incelenmesi s\u0131ras\u0131nda doktorun \u015f\u00fcphelenmesiyle koyulur. En s\u0131k g\u00f6r\u00fclen bulgular kar\u0131nda sa\u011f \u00fcst kadran a\u011fr\u0131s\u0131, yorgunluk, ka\u015f\u0131nt\u0131 ve kilo kayb\u0131d\u0131r. Olgular\u0131n baz\u0131lar\u0131 uzun y\u0131llar asemptomatik kalabilir. Asemptomatik olgular\u0131n baz\u0131lar\u0131nda histolojik ve radyolojik olarak hastal\u0131k g\u00f6r\u00fclebilir. Belirtiler ortaya \u00e7\u0131kt\u0131\u011f\u0131nda hastal\u0131k \u00e7o\u011funlukla ileri evrededir.<\/span><\/p>\n

Beden muayenesinde en s\u0131k g\u00f6r\u00fclen bulgular sar\u0131l\u0131k ve karaci\u011ferde b\u00fcy\u00fcmedir (hepatomegali). Hastalar\u0131n 1\/3 inde dalakta da b\u00fcy\u00fcme saptan\u0131r (splenomegali).<\/span><\/p>\n<\/div>\n<\/div>\n<\/div>\n

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Komplikasyonlar<\/span><\/h3>\n<\/div>\n<\/div>\n
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Barsa\u011fa safra ak\u0131m\u0131ndaki yetersizlik (kronik kolestaz) sonucunda ya\u011f sindirimi bozularak ya\u011fda \u00e7\u00f6z\u00fcnerek emilen vitaminlerin (A, D, E ve K vitaminleri) barsaklardan emilimi azal\u0131r. Hastalarda ya\u011fl\u0131 d\u0131\u015fk\u0131lama ve A, D, E ve K vitamini eksikli\u011fi olu\u015fur. Bu vitaminlerin eksiklikleri, g\u00f6rme bozukluklar\u0131na, kemik erimesine (osteoporoz) ve kanamalara neden olur. PSK in ciddi bir komplikasyonu safra yollar\u0131nda kanser geli\u015fmesidir (kolanjiokarsinom). PSK li bir hastada b\u00fcy\u00fck safra yollar\u0131ndaki belirgin daralmalarda kolanjiokarsinom akla gelmeli ve tan\u0131 i\u00e7in gerekli incelemeler yap\u0131lmal\u0131d\u0131r. Otopsi \u00e7al\u0131\u015fmalar\u0131 PSK li hastalar\u0131n 1\/3 e varan bir k\u0131sm\u0131nda karaci\u011fer i\u00e7indeki safra kanallar\u0131nda hen\u00fcz belirti vermemi\u015f kolanjiokarsinom geli\u015febilece\u011fini g\u00f6stermi\u015ftir.<\/span><\/p>\n<\/div>\n<\/div>\n<\/div>\n

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Primer Sklerozan Kolanjit Tedavisi<\/span><\/h3>

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PSK<\/b>\u2019n\u0131n etkin bir t\u0131bbi tedavisi yoktur. Primer biliyer siroz tedavisinde olduk\u00e7a etkili bir ila\u00e7 olan UDCA (Ursodeoksikolik asit) PSK tedavisinde genellikle etkisizdir. UDCA tedavisiyle biyokimyasal de\u011ferlerde bir miktar d\u00fczelme sa\u011flanabilirse de hastal\u0131\u011f\u0131n ilerleme h\u0131z\u0131, sa\u011f kal\u0131m s\u00fcresi ve karaci\u011fer transplantasyonunun geciktirilmesi \u00fczerinde olumlu etkisi yoktur. Tedavi i\u00e7in \u00f6nerilen UDCA dozu 10-15\/mg\/kg\/g\u00fcn\u2019d\u00fcr. \u0130lac\u0131n en s\u0131k g\u00f6r\u00fclen yan etkisi ishaldir. Yukar\u0131da belirtilen vitaminlerin eksikli\u011fi saptand\u0131\u011f\u0131nda bu vitaminler d\u0131\u015far\u0131dan verilerek a\u00e7\u0131\u011f\u0131n kapat\u0131lmas\u0131na ya \u00e7al\u0131\u015f\u0131l\u0131r.[\/caption]<\/span><\/p>\n

Safra yollar\u0131nda olu\u015fan darl\u0131klar ERCP yap\u0131larak balonla geni\u015fletilebilir ve dar b\u00f6lgeye stent yerle\u015ftirilebilir. \u0130leri evre PSK da ve safra yollar\u0131nda kolanjiokarsinom geli\u015fmi\u015f olan hastalarda etkili olabilecek tek tedavi y\u00f6ntemi karaci\u011fer transplantasyonudur. PSK de transplantasyon sonras\u0131nda \u00fc\u00e7 y\u0131ll\u0131k sa\u011f kal\u0131m s\u00fcresi %85-90 aras\u0131ndad\u0131r. Transplantasyon sonras\u0131nda safra yollar\u0131nda darl\u0131k geli\u015fmesi s\u0131k kar\u015f\u0131la\u015f\u0131lan bir problemdir. Olgular\u0131n %10-20\u2019sinde transplantasyon sonras\u0131nda tekrar PSK geli\u015fmektedir.<\/span><\/p>\n<\/div>\n<\/div>\n<\/div>\n<\/div>\n<\/div>\n<\/div>\n<\/div>\n<\/div>\n<\/div>\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/section>\n\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t","protected":false},"excerpt":{"rendered":"

Primer Sklerozan Kolanjit Nedir? Tan\u0131s\u0131 Ve Tedavisi Primer sklerozan kolanjit (PSK)\u00a0karaci\u011fer i\u00e7i ve d\u0131\u015f\u0131 safra yollar\u0131n\u0131 tutan, v\u00fccudun kendi ba\u011f\u0131\u015f\u0131kl\u0131k sistemi (immun sistem) taraf\u0131ndan tetiklendi\u011fi d\u00fc\u015f\u00fcn\u00fclen, safra yollar\u0131nda olu\u015fan daralmalar nedeniyle safra ak\u0131m\u0131n\u0131n engellenmesine ba\u011fl\u0131 bulgularla kendini g\u00f6steren bir kronik safra yolu hastal\u0131\u011f\u0131d\u0131r. Safra kanallar\u0131nda ilerleyici harabiyet ve daralmayla karakterize olan bu hastal\u0131k genellikle bir […]<\/p>\n","protected":false},"author":2,"featured_media":0,"parent":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":[],"yoast_head":"\nPrimer Sklerozan Kolanjit - Prof Dr Z\u00fclfikar Polat<\/title>\n<meta name=\"description\" content=\"Primer Sklerozan Kolanjit Nedir? Tan\u0131s\u0131 Ve Tedavisi Nelerdir ? 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