Nasledna hemoragijska teleangiektazija sa obostranim plućnim vaskularnim malformacijama

  • Olivera LJ Lončarević Clinic for Lung Diseases, Military Medical Academy, Belgrade, Serbia
  • Siniša Rusović Institute for Radiology, Military Medical Academy, Belgrade, Serbia
  • Marko Stojisavljević Clinic for Lung Diseases, Military Medical Academy, Belgrade, Serbia
  • Jelena Vuković Clinic for Lung Diseases, Military Medical Academy, Belgrade, Serbia
  • Goran Plavec Clinic for Lung Diseases, Military Medical Academy, Belgrade, Serbia; Faculty of Medicine of the Military Medical Academy, University of Defence, Belgrade, Serbia
  • Slobodan Aćimović Clinic for Lung Diseases, Military Medical Academy, Belgrade, Serbia
  • Gordana Cvetković Clinic for Lung Diseases, Military Medical Academy, Belgrade, Serbia
  • Marina Petrović Department of Pulmology, Clinical Centre Kragujevac, Faculty of Medicine Sciences, University of Kragujevac, Kragujevac, Serbia
DOI: https://doi.org/10.2298/VSP150515094L
Ključne reči: telangiectasia, hereditary hemorrhagic||, ||teleangiektazija, nasledna, hemoragijska, arteriovenous malformations||, ||arteriovenske malformacije, lung diseases||, ||pluća, bolesti, hemoptysis||, ||hemoptizije, diagnosis||, ||dijagnoza, embolization, therapeutic||, ||embolizacija, terapijska, treatment outcome||, ||lečenje, ishod,

Sažetak



Uvod. Hereditarna hemoragijska teleangiektazija (HHT) ili Osler-Weber-Rendu sindrom  je  autozomno dominantno oboljenje nastalo usled vaskularne displazije povezane sa poremećajem u signalnom putu transformišućeg faktora rasta β (TGF β). Klinička posledica jeste poremećaj krvnih sudova u više organa, sa  postojanjem teleangiektazija  koje uzrokuju dilataciju kapilara i vena. Promene su prisutne od samog rođenja i lokalizovane su po koži i mukozi usne duplje, respiratornog, gastrointestinalnog i urinarnog  trakta; mogu napraviti rupture sa posledičnim ozbiljnim krvarenjem koje se može završiti i smrtnim ishodom. Kako postoji poremećaj na krvnim sudovima više organskih sistema, postavljanje dijagnoze je veoma kompleksno i zahteva multidisciplinarni pristup. Prikaz bolesnika. Prikazali smo 40-godišnju bolesnicu sa dugogodišnjom evolucijom tegoba, dijagnostikovanu i lečenu u Klinici za pulmologiju Vojnomedicinske akademije u Beogradu, zbog bilateralnih plućnih arteriovenskih malformacija udruženih sa HHT. Urađena je embolizacija u dva akta, nakon čega je došlo do normalizacije kliničkog, radiološkog i funkcijskog nalaza, uz prestanak hemoptizija, intolerancije na napor i uz značajno poboljšanje kvaliteta života. Zaključak. HHT je retka, dominantno nasledna multisistemska bolest, koja zahteva multidisciplinarni pristup u dijagnostici i lečenju. Embolizacija je metoda izbora u lečenju arteriovenskih malformacija u plućima, sa neznatnim neželjenim efektima i veoma zadovoljavajućim terapijskim ishodom.

Biografija autora

Olivera LJ Lončarević, Clinic for Lung Diseases, Military Medical Academy, Belgrade, Serbia
Specijalista za plućne bolesti- Klinički lekar

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Objavljeno
2017/03/08
Broj časopisa
Vol. 73 Br. 10 (2016)
Rubrika
Prikaz bolesnika