Bousquet JR, Granado Á, Bousquet JA, Mata R, Rodríguez B, Caballero R, Casado Y

Language: Spanish
References: 19
Page: 48-54
PDF size: 616.20 Kb.

ABSTRACT

The lymphangioleiomyomatosis is a suffering of unknown etiology, little frequents that it affects almost exclusively to women in reproductive age. It is generally of very slow and lethal progression due to the destruction of the pulmonary weave that entails to chronic respiratory insufficiency. Objective: To inform the case of a 38 year old feminine patient of age, carrier of a pulmonary lymphangioleiomyomatosis. Method: Description of the clinical case and review of the literature. Environment: Department of Surgery. University Hospital Dr. Luis Razetti. Service of Medicine Hospitalizes - Service of Surgery. Hospital Dr. Domingo Guzmán Lander IVSS. Barcelona. State Anzoátegui. Result: One proceeded to realize bulectomy left side, pleurodesis apical, biopsy to opened sky, for bullas apical base them bilateral with severe affectation of the pulmonary architecture. Conclusions: The pulmonary lymphangioleiomyomatosis is a disease systemic caused by the proliferation of smooth muscle in pulmonary parenchyma, lymphatic spaces, lymphatic ganglions, mediastinals and retroperitoneals and in the lymphatic glasses. Nowadays his relation is investigated by the sexual feminine hormones but the investigation of recipients of estrogens and progesterone they are contradictory. The clinical usual presentation is a progressive difficulty in breathing, pneumothorax spontaneous recidivated, hemopthysis and chylothorax. There has not been described an effective treatment that alters the natural evolution of the disease. Reports of survivals exist to 8.5 years of 38% and 78%.

REFERENCES

1. Baldi, S., Papotti, M., Valente, M.L., et al. Pulmonary lymphangioleiomyomatosis in postmenopausal women: report of two cases and review of the literature. Eur Respir J 1994;7:1013.

2. Berger, J.L., & Shaff, M.I. Pulmonary lymphangioleiomyomatosis. J Comput Assist Tomogr 1981;5:565.

3. Bernstein, S.M., Newell, J.D., & Adanczyk, D. .How common are renal angiomyolipomas in patients with pulmonary lymphangioleiomyomatosis? Am J Respir Crit Care Med 1995;152:2138

4. Berkman, N., Bloom, A., & Cohen, P. Bilateral spontaneous pneumothorax as the presenting feature in lymphangioleiomyomatosis. Respir Med 1995;89:381.

5. Carrington, C.B., Cugell, D.W., & Gaensler, E.A. Lymhangioleiomyomatosis. Physiologic-pathologic-radiologic correaltions. Am Rev Respir Dis 1977.

6. Crausman, R.S., Jennings, C.A., Mortenson, R.L., et al. Lymphangioleiomyomatosis: the pathophysiology of diminished excercise capacity. Am J Respir Crit Care Med 1996;153:1368.

7. Crausman, R.S., Lynch, D.A., & Mortenson, R.L. Quatitative CT predicts the severity of physiologic dysfunction in patients with lymphangioleiomyomatosis. Chest 1996;109:131.

8. Corrin, B., Liebow, A.A., & Friedman, P.J. Pulmonary lymphangioleiomyomatosis. A review. Am J Pathol 1975;79:348.

9. Johnson, S. Lymphangioleiomyomatosis. Clinical features, management and basic mechanisms. Thorax 1999;54:254.

10. Johnson, S.R., & Tattersfield, A.E. Clinical experience of lymphangioleiomyomatosis in the UK. Thorax 2000;55:1052.

11. Kalassin, K.G., Doyle, R., & Kao, P. Lymphangioleiomyomatosis. New insights. Am J Respir Crit Care Med 1997;155:1183.

12. Kitachi, M., Nishimura, K., Itoh, H., & Izumi, T. Pulmonary lynphangioleiomyomatosis. A report of 46 patients including a clinicopathologic study of prognostic factors. Am J Resp Crit Care Med 1995;151:527.

13. NHLBI Workshop Summary. Report on lymphangioleiomyomatosis. National Heart Lung and Blood Institute. Am J Respir Crit Care Med 1999;159:679.

14. Silverstein, E.F., Ellis, K., Wolff, M., Jaretzki, A., Pulmonary lymphangioleiomyomatosis. Am J Roentgenol Radium Ther Nucl Med 1974;120:832.

15. Sinclair, W., Wright, J.L., Churg, A. Lymphangioleiomyomatosis presenting in a postmenopausal woman. Thorax 1985;40:475.

16. Sullivan, E.J. Lymphangioleiomyomatosis. A review. Chest 1998;114:1689.

17. Taylor, J.R., Ryu, J., Colby, T.V., & Raffin, T.A. Lymphangioleiomyomatosis. Clinical course in 32 patients. N Engl J Med 1990;323:1254.

18. Urban, T., Lazor, R., Lacronigue, J. Pulmonary lymphangioleiomyomatosis. A study of 69 patients. Groupe d'Estudea et de Recheche sur les Maladies & quot; Orpheline & quot; Pulmonaires (GERM & quot; O & quot; P). Medicine (Baltimore) 1999;78:321.

19. Valenti. V.G., Raffin, T.A. The management of chylothorax. Chest 1992;102:586.