cystic fibrosis (cf) is an inherited disease that affects multiple organ systems. it is the most common cause of severe progressive lung disease and exocrine panceratic insufficiency. in our investigation 67 patients had cf. of these, 79% had panceratic insufficiency and 92% had lung disease under 2 years age. also 67% of patients were < 2 percentile and 28% between 3-10 percentile weight for a...

background: substitution of arterial with venous blood samples to estimate blood gas status is highly preferable due to practical and safety concerns. numerous studies support the substitution of arterial by venous blood samples, reporting strong correlations between arterial and venous values. this study further investigated the predictive ability of venous blood samples for arterial acid-base...

conclusions getting a complete knowledge of the condition and evaluating different treatment modalities to treat thoracic insufficiency syndrome. results a direct enlargement of the thorax that will thus provide sufficient space for lung growth; the correction of both the three-dimensional thoracic deformity and the progressive scoliosis. recent findings have revealed that veptr instrumentation...

The clearance of airway secretions from the lungs is normally supported by the mucociliary escalator and by cough. These protective mechanisms provide an effective means of pulmonary-hygiene maintenance in healthy individuals. Patients with neuromuscular disease that affects the respiratory pump (the muscles of breathing) can experience mild to profound limitation in both ventilation and cough....

OBJECTIVES Combined complex I+IV deficiency has rarely been reported to manifest with the involvement of the respiratory muscles. CASE REPORT A 45y male was admitted for hypercapnia due to muscular respiratory insufficiency. He required intubation and mechanical ventilation. He had a previous history of ophthalmoparesis since age 6y, ptosis since age 23y, and anterocollis since at least age 4...