RESUMO
Cystic fibrosis [CF] is a genetically disease with different respiratory and gastrointestinal organ involvement. Because of gastrointestinal tract involvement, patients have mal-absorption for vitamins and mineral elements; for example vitamin A, D, E and trace elements such as Selenium [Se], Zinc [Zn] and Copper [Cu] have low serum level in CF patients in different studies. The goal of this study is to define relation between serum level of this elements and severity of respiratory and gastrointestinal tract involvement in these patients. This cross sectional study performed on 39 cystic fibrosis patients admitted in pediatric ward of Masih Daneshvari hospital, Tehran, Iran. Serum levels of Vitamin A, D and E, Selenium, Zinc and Copper deficiency were studied. Serum level of Vitamin A and D were lower than normal values. Also, 3 cases [7.68%] had serum level of Zinc lower than normal values. CF patients are susceptible to have deficiencies of fat soluble vitamins and trace elements such as Selenium and Zinc because of mal-absorption. It seems that a perfect diet and nutritional components are helpful in prevention of these problems
RESUMO
Pulmonary alveolar proteinosis [PAP] is a rare disease in children, characterized by intra-alveolar accumulation of large amounts of surfactant proteins, which severely reduce gas exchange. Whole lung lavage [WLL] is the preferred technique for the treatment of severe PAP. This report presents nine pediatric cases with advanced PAP who underwent WLL under general anesthesia during a 9 year period. One patient was treated with multiple unilateral WLL without employing cardiopulmonary bypass [CPB] and eight cases were treated by simultaneous lavage of both lungs using partial CPB. Our experience suggested that partial CPB was useful to support oxygenation during WLL in small children with severe PAP in whom lung separation and selective lavaging of each lung were impracticable