Avtor/Urednik     Anderluh, Franc
Naslov     Lymphangioleiomyomatosis
Prevedeni naslov     Limfangioleiomiomatoza
Tip     članek
Vir     Radiol Oncol
Vol. in št.     Letnik 36, št. 1
Leto izdaje     2002
Obseg     str. 41-6
Jezik     eng
Abstrakt     Background. Lymphangioleiomyomatosis is a rare disease of unknown origin, which affects women in their reproductive period. It is characterised by non-neoplastic proliferation of atypical smooth muscle cells in the lung parenchyma, lymphatic vessels and mediastinal and abdominal lymph nodes. The most common presenting symptoms are spontaneous pneumothorax, dyspnea, hemoptysis and chylothorax. Conclusions. High-resolution computed tomography (HRCT) and open lung biopsy followed by the immunohistologic studies are two diagnostic procedures with which diagnosis can be confirmed. Various treatment modalities are applied, particularly hormonal therapy, though their efficacy remain unknown. The prognosis of patients is bad.
Izvleček     lzhodišča. Limfangioleiomiomatoza je redka bolezen neznanega vzroka, za katero zbolevajo ženske v rodnem obdobju. Za bolezen je značilna neneoplastična proliferacija atipičnih gladkomišičnih celic v pljučnem parenhimu, limfnih žilah ter mediastinalnih in abdominalnih bezgavkah. V klinični sliki se pojavljajo spontani pnevmotoraksi, dispneja, hemoptize in hilotoraks. Zaključlti. Računalniška tomografija visoke ločljivosti in imunohistološke metode v bioptičnih vzorcih pljučnega tkiva nam omogočajo pravilno postavitev diagnoze. Načini zdravljenja so različni, običajno hormonski, vendar je zaenkrat njihov uspeh še vprašljiv, prognoza bolnikov pa slaba.
Deskriptorji     LYMPHANGIOMYOMATOSIS
TOMOGRAPHY, X-RAY COMPUTED
BIOPSY