Cruz MJJ, Alcántar MJL, Enríquez MV, Luna PR, Casimiro PB, Magaña OJR

Language: Spanish
References: 10
Page: 399-403
PDF size: 206.97 Kb.

ABSTRACT

Introduction: Cystic fibrosis (CF) is a generalized autosomal recessive disorder characterized by alterations in hydroelectrolytic transport across epithelial cells, with liberation of neutrophil DNA and secretion of an abnormal mucus that obstruct glands and ducts in various organs. In the respiratory tract, inadequate hydration of tracheobronchial epithelium impairs mucocilliary function. High concentrations of DNA in airway secretions also render the sputum thick and viscous. Pulmonary manifestations include acute and chronic bronchitis, bronchiectasis, pneumonia, and atelectasis; cor pulmonale occurs in advanced cases and signifies poor prognosis. Treatment involves several disciplines and must be permanent and aggressive, including ventilatory support. Objective: To report a case of a patient with CF; Cor pulmonale and acute respiratory failure, treated with continuous positive airway pressure (CPAP) with excellent response. Site: Inhalotherapy Unit, General Hospital 1, IMSS, Morelia, Michoacán, Mexico. Case: Female, 4 years of age, three brothers previously dead by respiratory diseases, with history of chronic lung disease, sweat chlorid concentration above 110 Meq/L, and cor pulmonale with acute heart failure. This patient was treated with continuous positive airway pressure (CPAP) including expiratory positive airway pressure (EPAP) and inspiratory positive airway pressure (IPAP), instead of the conventional systems of mechanical ventilation with orotracheal intubation . Discussion: The gasometric and radiologic parameters improved and the patient showed clinical relief; therefore, we considered CPAP a real alternative in FQ treatment.

REFERENCES

1. Wilson DC, Ellis L, Zielenski J, Corey M, Wan FI, Tsui LCH, et al. Uncertainty the diagnosis of cystic fibrosis: Possible role of in vivo nasal potential difference measurements. J Pediatrics 1998;132(4): 596-599.

2. Rosenstein BJ, Cutting GR. The diagnosis of cystic fibrosis: A consensus statement. J Pediatrics 1998; 132;(4):589-595.

3. Diez-Jarilla JL. Acropaquias y osteoartropatía hipertrófica. En: Diez J. Problemas clínicos en neumología. México: McGraw-Hill-Interamericana; 1998. p. 47-55.

4. McKoy J. Pediatric parenchymal diseases. En: Beck KP, Eitzman D, Neu J. Neonatal and pediatric respiratory care. Second edition. St. Louis, USA: Mosby; 1993. p. 92-101.

5. Díaz M, Rendón A, Cano ME. Corrección aguda de la hipoxemia nocturna y del patrón del sueño mediante la aplicación continua de presión positiva nasal en enfermos con el síndrome de la apnea obstructiva del sueño. Gac Med Mex 1998;135(4): 669-675.

6. Czervinke MP. Continuous positive airway pressure. En: Beck KP, Eitzman D, Neu J. Neonatal and pe-diatric respiratory care. Second edition. St. Louis, USA: Mosby; 1993. p. 265-284.

7. Scott AA, Koff PB. Airway care and chest physio-therapy. En: Beck KP, Eitzman D, Neu J. Neonatal and pediatric respiratory care. Second edition. St. Louis, USA: Mosby; 1993. p. 285-302.

8. Casas JP, Robles AM, Pereyra MA, Abona HL, Lopez AL. Domiciliary noninvasive positive pressure ventilation in chronic alveolar hipoven-tilation. Spanish Medicine 2000;60 (5 pt 1):545-550.

9. Tobias JD. Noninvasive ventilation using bi-level positive airway pressure to treat impending res-piratory failure in the insufficiency and the post-anesthesia care unit. J Clin Anesth 2000;12(5):409-412.

10. Caronia CG, Silver P, Nimkoff L, Gorvoy J, Quinn C, Sagy M. Use of bi-level positive airway pressure (BIPAP) in end-stage patients with cystic fibrosis awaiting lung transplantation. Clin Pediatr 1998; 37(9):555-559.