Pulmoner alveoler proteinozis
Yükleniyor...
Tarih
2011
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Dicle Üniversitesi Tıp Fakültesi
Erişim Hakkı
Attribution-NonCommercial 3.0 United States
info:eu-repo/semantics/openAccess
info:eu-repo/semantics/openAccess
Özet
Pulmoner alveoler proteinozis alveollerde lipoproteinoz materyalin birikimi ile karakterize nadir görülen bir hastalıktır. Otuzaltı yaşında erkek hasta, yaklaşık 5 ay önce başlayan nefes darlığı şikayeti ile hastanemize başvurdu. Akciğer grafisinde bilateral yaygın infiltrasyon görüldü. Arter kan gazında (AKG) : pH:7.44, pCO2 : 36 mmHg, pO2 : 49 mmHg, SaO2 : %85, HCO3 : 24 mmol/L olarak saptandı. Yüksek rezolüsyonlu bilgisayarlı tomografisinde (YRBT) bilateral yaygın buzlu cam alanları ve bu alanlarda interlobuler septal kalınlaşmalar raporlandı. Üç defa yapılan balgam mikroskobisinde, post bronkoskopik balgamda ve bronkoalveoler lavajda aside dirençli bakteri saptanmadı. Hastaya video destekli torakoskopik cerrahi ile wedge rezeksiyon yapıldı ve patoloji sonucu “Pulmoner Alveoler Proteinozis (PAP)” olarak raporlandı. Hastaya genel anestezi altında masif akciğer lavajı planlandı. Yatışından
itibaren klinik düzelme görülen hastanın takiplerde AKG değerleri de düzelme eğilimine girdi. Oksijensiz AKG’da parsiyel oksijen basıncı 65 mmHg’ye yükselen hasta lavaj yapılmadan takibe alındı. Kontrol PA akciğer grafisi ve YRBT’de belirgin radyolojik düzelme saptandı. Üç ay sonra yapılan kontrol YRBT’sinde lezyonlar radyolojik olarak tamamen gerilemişti. Sonuç olarak dispne şikayeti ile başvuran hastalarda, radyolojik bilateral infiltrasyon varlığında, ayırıcı tanıda nadir bir hastalık olan PAP da düşünülmelidir
Pulmonary alveolar proteinosis is a rare disease characterized by the accumulation of lipoproteinosis material within the alveoli. A 36 age male patient applied to our hospital with dyspnea that began 5 months ago. Bilateral diffuse infiltration on postero-anterior chest graphy was seen. Arterial blood gas measurements (ABG) was: pH:7.44, pCO2 : 36 mmHg, pO2 : 49 mmHg, SaO2 : 85%, HCO3 : 24 mmol/L. High resolution computed tomography (HRCT) reported as bilateral diffuse ground glass areas and interlobuler septal thickening on this areas. Asido resistant bacillus was negative in sputum at three times and was also negative in post bronchoscopic sputum and bronchoalveoler lavage material. Wedge resection was made by video-assisted thoracoscopic surgery. Histopathology report of biopsy material revealed “Pulmonary alveolar proteinosis (PAP)”. Massive lung lavage under general anesthesia was planned for patient. Clinical improvement was seen in patient beginning from admission and ABG measurements entered the recovery trend in follow-up. Partial pressure of oxygen was increased to 65 mmHg and patient was followed without lung lavage. Control chest radiography and HRCT showed significant radiological improvement. After three months, radiological lesions had completely regressed at control HRCT. As a result, in patients with symptoms and radiologically bilateral diffuse infiltration physicians should consider PAP as a rare disease in the differential diagnosis.
Pulmonary alveolar proteinosis is a rare disease characterized by the accumulation of lipoproteinosis material within the alveoli. A 36 age male patient applied to our hospital with dyspnea that began 5 months ago. Bilateral diffuse infiltration on postero-anterior chest graphy was seen. Arterial blood gas measurements (ABG) was: pH:7.44, pCO2 : 36 mmHg, pO2 : 49 mmHg, SaO2 : 85%, HCO3 : 24 mmol/L. High resolution computed tomography (HRCT) reported as bilateral diffuse ground glass areas and interlobuler septal thickening on this areas. Asido resistant bacillus was negative in sputum at three times and was also negative in post bronchoscopic sputum and bronchoalveoler lavage material. Wedge resection was made by video-assisted thoracoscopic surgery. Histopathology report of biopsy material revealed “Pulmonary alveolar proteinosis (PAP)”. Massive lung lavage under general anesthesia was planned for patient. Clinical improvement was seen in patient beginning from admission and ABG measurements entered the recovery trend in follow-up. Partial pressure of oxygen was increased to 65 mmHg and patient was followed without lung lavage. Control chest radiography and HRCT showed significant radiological improvement. After three months, radiological lesions had completely regressed at control HRCT. As a result, in patients with symptoms and radiologically bilateral diffuse infiltration physicians should consider PAP as a rare disease in the differential diagnosis.
Açıklama
Anahtar Kelimeler
Pulmoner alveoler proteinozis, Nefes darlığı, Masif akciğer lavajı, Pulmonary alveolar proteinosis, Dyspnea, Massive lung lavage
Kaynak
Dicle Tıp Dergisi
WoS Q Değeri
Scopus Q Değeri
Cilt
38
Sayı
2
Künye
Şen, H.S., Dallı, A., Önder, Ö.F., Abakay, A., Fırat, U., Eren, T.Ş. (2011). Pulmoner alveoler proteinozis. Dicle Tıp Dergisi, 38(2), 234-238.
