Tunç LAÇİN, Nural BEKİROĞLU, Emel ERYÜKSEL, Berrin CEYHAN, Sait KARAKURT, Bedrettin YILDIZELİ, Turgay ÇELİKEL, Mustafa YÜKSEL

Pulmoner alveoler proteinozis için tüm akciğer lavajı: Hala en güncel tedavi

Amaç: Bu çalışmada, pulmoner alveoler proteinozlu hastalarda tüm akciğer lavajının lavaj öncesi ve sonrası kan gazı analizi değerlerindeki önemi değerlendirildi. Çalışma planı: Ocak 1998 - Mayıs 2010 tarihleri arasında Marmara Üniversitesi Hastanesi Göğüs Cerrahisi bölümünde girişimsel teknikler ile pulmoner alveoler proteinoz tanısı konulan dokuz hastanın (1 erkek, 8 kadın; ort. yaş 38.2 yıl; dağılım 29-60 yıl) verileri incelendi. Hastaların lavaj öncesi solunum fonksiyon testleri, lavaj öncesi ve sonrası kan gazı analizleri ve radyolojik görüntüleri değerlendirildi. Tüm hastalarda restriktif solunum paterni ve bozulmuş gaz değişimi tespit edildi ve tüm akciğer lavajı uygulandı. Bulgular: Tüm akciğer lavajı sırasında majör komplikasyon görülmedi. Her akciğer 15-30 L serum fizyolojik solüsyonu ile yıkandı. Hastalar ortalama 2.6 gün (dağılım, 1-16 gün) entübe olarak yoğun bakım ünitesinde takip edildi. Ortalama yoğun bakım yatış süresi 3.5 gün (dağılım, 1-16 gün) idi. Bir hasta beşinci lavajından 16 gün sonra perkütan trakeostomi açılırken solunum durması nedeniyle kaybedildi. Lavaj sonrası oksijen parsiyel basıncı ve oksijen satürasyonlarının yükselmesi istatistiksel olarak anlamlı idi. Bütün hastalarda subjektif düzelme görüldü. Sonuç: Deneyimimize göre, lavaj protokolüne tamamen uyulursa tüm akciğer lavajı güvenilir bir tekniktir. Lavaj sonrası semptomatik düzelme hızlıdır ve oksijenizasyon anlamlı düzeyde iyileşir.

Whole lung lavage for pulmonary alveolar proteinosis: still the most up-to-date treatment

Background: This study aims to evaluate the significance of whole lung lavage on prelavage and postlavage blood gas analysis values of patients with pulmonary alveolar proteinosis.Methods: Data of nine patients (1 male, 8 females; mean age 38.2 years; range 29 to 60 years) who were diagnosed to have pulmonary alveolar proteinosis with interventional techniques between January 1998 and May 2010 at Marmara University Hospital Department of Thoracic Surgery were reviewed. Patients’ prelavage pulmonary function tests, pre- and postlavage blood gas analyses, and radiologic images were evaluated. Restrictive ventilatory pattern and impaired gas exchange were detected and whole lung lavage was performed in all patients.Results: No major complications were observed during whole lung lavage. Each lung was washed with 15-30 L of physiological saline solution. Patients were followed-up in the intensive care unit intubated for a mean of 2.6 days (range, 1 to 16 days). Mean duration of stay at intensive care unit was 3.5 days (range, 1-16 days). One patient died 16 days after her fifth lavage due to respiratory arrest during an attempt for percutaneous tracheostomy. Increment of the postlavage partial pressures of oxygen and oxygen saturations was statistically significant. All patients showed subjective improvement.Conclusion: Our experience suggests that whole lung lavage is a safe technique when strict adherence to lavage protocol is maintained. Postlavage symptomatic relief is rapid and oxygenation improves significantly.

PDF

___

30. Aguinaga MA, Santos P, Renes E, Alvaro PF, Lorente JA, Maudes A, et al. Hemodynamic changes during whole bronchoalveolar lavage in two cases of pulmonary alveolar proteinosis. Intensive Care Med 1991;17:421-3.
29. Rogers RM, Szidon JP, Shelburne J, Neigh JL, Shuman JF, Tantum KR. Hemodynamic response of the pulmonary circulation to bronchopulmonary lavage in man. N Engl J Med 1972;286:1230-3.
28. Chesnutt MS, Nuckton TJ, Golden J, Folkesson HG, Matthay MA. Rapid alveolar epithelial fluid clearance following lung lavage in pulmonary alveolar proteinosis. Chest 2001;120:271-4.
27. Nagasaka Y, Takahashi M, Ueshima H, Tohda Y, Nakajima S. Bronchoalveolar lavage with trypsin in pulmonary alveolar proteinosis. Thorax 1996;51:769-70.
26. Dixit R, Chaudhari LS, Mahashur AA. Anaesthetic management of bilateral alveolar proteinosis for bronchopulmonary lavage. Chest 1998;113:1718-9.
25. Diaz JP, Manresa Presas F, Benasco C, Guardiola J, Munoz L, Clariana A. Response to surfactant activator (ambroxol) in alveolar proteinosis. Lancet 1984;1:1023.
24. Cheng SL, Chang HT, Lau HP, Lee LN, Yang PC. Pulmonary alveolar proteinosis: treatment by bronchofiberscopic lobar lavage. Chest 2002;122:1480-5.
23. Demirer E, Okutan O, Taş D, İncedayı M, Uysal A, Kartaloğlu Z, et al. Pulmonary alveolar proteinosis in Turkey: a review of twenty four cases. Turk Gogus Kalp Dama 2013;21:723-30.
22. Abdelmalak BB, Khanna AK, Culver DA, Popovich MJ. Therapeutic Whole-Lung Lavage for Pulmonary Alveolar Proteinosis: A Procedural Update. J Bronchology Interv Pulmonol 2015;22:251-8.
21. Honda Y, Kuroki Y, Matsuura E, Nagae H, Takahashi H, Akino T, et al. Pulmonary surfactant protein D in sera and bronchoalveolar lavage fluids. Am J Respir Crit Care Med 1995;152:1860-6.
20. Kuroki Y, Tsutahara S, Shijubo N, Takahashi H, Shiratori M, Hattori A, et al. Elevated levels of lung surfactant protein A in sera from patients with idiopathic pulmonary fibrosis and pulmonary alveolar proteinosis. Am Rev Respir Dis 1993;147:723-9.
19. Nakajima M, Manabe T, Niki Y, Matsushima T. Serum KL-6 level as a monitoring marker in a patient with pulmonary alveolar proteinosis. Thorax 1998;53:809-11.
18. Hirakata Y, Kobayashi J, Sugama Y, Kitamura S. Elevation of tumour markers in serum and bronchoalveolar lavage fluid in pulmonary alveolar proteinosis. Eur Respir J 1995;8:689-96.
17. Hoffman RM, Rogers RM. Serum and lavage lactate dehydrogenase isoenzymes in pulmonary alveolar proteinosis. Am Rev Respir Dis 1991;143:42-6.
16. Godwin JD, Müller NL, Takasugi JE. Pulmonary alveolar proteinosis: CT findings. Radiology 1988;169:609-13.
15. Venkateshiah SB, Thomassen MJ, Kavuru MS. Pulmonary alveolar proteinosis. Clinical manifestations and optimal treatment strategies. Treat Respir Med 2004;3:217-27.
14. Rosen SH, Castleman B, Liebow AA. Pulmonary alveolar proteinosis. N Engl J Med 1958;258:1123-42.
13. Claypool WD, Rogers RM, Matuschak GM. Update on the clinical diagnosis, management, and pathogenesis of pulmonary alveolar proteinosis (phospholipidosis). Chest 1984;85:550-8.
12. Paschen C, Reiter K, Stanzel F, Teschler H, Griese M. Therapeutic lung lavages in children and adults. Respir Res 2005;6:138.
11. Prakash UB, Barham SS, Carpenter HA, Dines DE, Marsh HM. Pulmonary alveolar phospholipoproteinosis: experience with 34 cases and a review. Mayo Clin Proc 1987;62:499-518.
10. Seymour JF, Presneill JJ. Pulmonary alveolar proteinosis: progress in the first 44 years. Am J Respir Crit Care Med 2002;166:215-35.
9. Shulenin S, Nogee LM, Annilo T, Wert SE, Whitsett JA, Dean M. ABCA3 gene mutations in newborns with fatal surfactant deficiency. N Engl J Med 2004;350:1296-303.
8. Dirksen U, Nishinakamura R, Groneck P, Hattenhorst U, Nogee L, Murray R, et al. Human pulmonary alveolar proteinosis associated with a defect in GM-CSF/IL-3/IL-5 receptor common beta chain expression. J Clin Invest 1997;100:2211-7.
7. Nogee LM, Dunbar AE, Wert S, Askin F, Hamvas A, Whitsett JA. Mutations in the surfactant protein C gene associated with interstitial lung disease. Chest 2002;121:20-1.
6. Nogee LM, Wert SE, Proffit SA, Hull WM, Whitsett JA. Allelic heterogeneity in hereditary surfactant protein B (SP-B) deficiency. Am J Respir Crit Care Med 2000;161:973-81.
5. Shah PL, Hansell D, Lawson PR, Reid KB, Morgan C. Pulmonary alveolar proteinosis: clinical aspects and current concepts on pathogenesis. Thorax 2000;55:67-77.
4. Cheng SL, Chang HT, Lau HP, Lee LN, Yang PC. Pulmonary alveolar proteinosis: treatment by bronchofiberscopic lobar lavage. Chest 2002;122:1480-5.
3. Schoch OD, Schanz U, Koller M, Nakata K, Seymour JF, Russi EW, et al. BAL findings in a patient with pulmonary alveolar proteinosis successfully treated with GM-CSF. Thorax 2002;57:277-80.
2. Trapnell BC, Whitsett JA, Nakata K. Pulmonary alveolar proteinosis. N Engl J Med 2003;349:2527-39.
1. Goldstein LS, Kavuru MS, Curtis-McCarthy P, Christie HA, Farver C, Stoller JK. Pulmonary alveolar proteinosis: clinical features and outcomes. Chest 1998;114:1357-62.