Pulmoner lenfanjioleiyomiyomatozis: İki olgu nedeniyle
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Dosyalar
Tarih
2015
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Cilt Başlığı
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Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
Pulmoner lenfanjioleiyomiyomatozis (LAM) daha çok doğurganlık çağındaki kadınları etkileyen akciğerin nadir kistik hastalığıdır. Progresif dispne, tekrarlayan spontan pnömotoraks, şilotoraks ve bazen tekrarla- yan hemoptizi görülebilen semptomlardır. Hastalığın tanısı tipik klinik bulguları olan hastalarda akciğer biyopsisi veya yüksek rezolusyonlu bilgisayarlı tomog- rafisi (YRBT) bulguları ile konabilir. OLGU 1: Otuz iki yaşında bayan hasta tekrarlayan spontan pnömoto- raks ve sağ böbrekte anjiyomiyolipom öyküsü olan hastaya videotorakoskopi eşliğinde akciğer biyopsisi yapıldı. Sonucu LAM ile uyumlu idi. Hasta takibe alındı. OLGU 2: Kırk üç yaşında bayan hasta hemop- tizi şikâyeti ile kliniğimize başvurdu. Hasta beş yıldır aralıklı olan nefes darlığı ve göğüs ağrısından şikâyetçiydi. YRBT' de bilateral ince duvarlı hava kistleri izlendi. Torakotomi eşliğinde wedge rezeksiyon uygulandı. Histopatolojik bulguları LAM ile uyumlu olarak raporlandı. Hasta takibe alındı. Sporadik LAM tanısı alan iki farklı klinik özellikteki olgu literatür eşliğinde sunuldu.
Lymphangioleiomyomatosis (LAM) is an uncommon cystic lung disease, primarily affecting women of childbearing age. The clinical presentation is general- ly progressive dyspnea, recurrent spontaneous pneu- mothorax, chylous effusion, and occasional hemopty- sis. The diagnosis of the disease can be established by lung biopsy or high-resolution computed tomog- raphy (HRCT) combined with typical clinical features. CASE 1: A 32-year-old women had recurrent sponta- neous pneumothorax and right renal angiomyolipo- ma. A lung biopsy was performed via videothoraco- scopy. The histopathological results were reported as LAM. The patient was followed up. CASE 2: A 43- year-old woman was admitted to our clinic due to hemoptysis after each cough. She complained about chest pain and stridorous breathing for five years. HRCT revealed bilateral thin-walled air cysts. A wedge resection was performed via thoracotomy. The patient was diagnosed with LAM based on the histo- pathological examination. The patient was moni- tored. The two rare cases with two different clinical presentations were reported with a literature review.
Lymphangioleiomyomatosis (LAM) is an uncommon cystic lung disease, primarily affecting women of childbearing age. The clinical presentation is general- ly progressive dyspnea, recurrent spontaneous pneu- mothorax, chylous effusion, and occasional hemopty- sis. The diagnosis of the disease can be established by lung biopsy or high-resolution computed tomog- raphy (HRCT) combined with typical clinical features. CASE 1: A 32-year-old women had recurrent sponta- neous pneumothorax and right renal angiomyolipo- ma. A lung biopsy was performed via videothoraco- scopy. The histopathological results were reported as LAM. The patient was followed up. CASE 2: A 43- year-old woman was admitted to our clinic due to hemoptysis after each cough. She complained about chest pain and stridorous breathing for five years. HRCT revealed bilateral thin-walled air cysts. A wedge resection was performed via thoracotomy. The patient was diagnosed with LAM based on the histo- pathological examination. The patient was moni- tored. The two rare cases with two different clinical presentations were reported with a literature review.
Açıklama
Anahtar Kelimeler
Lenfanjioleiyomiyomatozis, Ayırıcı Tanı, Tedavi, Sirolimus, Lymphangioleiomyomatosis, Differential Diagnosis, Treatment, Sirolimus
Kaynak
Respiratory Case Reports
WoS Q Değeri
Scopus Q Değeri
Cilt
4
Sayı
2
