病生-04.3酸碱平衡3.pptVIP

酸碱平衡和 酸碱平衡紊乱 A 14-year-old girl with cystic fibrosis has complained of an increased cough productive of green sputum over the last week. She also complained of being increasingly short of breath, and she is noticeably wheezing(喘息) on physical examination. Arterial blood was drawn and sampled, revealing the following values: 1. . Cystic fibrosis (CF), the most common inherited disease among Caucasians, is an autosomal recessive disorder that is marked by the production of excessively thick mucus in the respiratory tract, pancreatic ducts, and biliary tree. Individuals with CF produce a faulty version of a chloride channel protein called cystic fibrosis transmembrane conductance regulator (CFTR). The normal version of this protein helps transport chloride ions out of exocrine gland cells. In this process, water usually follows by osmosis, thus decreasing the viscosity of mucus secretions from these exocrine cells. However, in CF the faulty CFTR protein is not able to adequately pump chloride out through the cell membrane and thus the mucus secretions from exocrine cells are excessively viscous (i.e. thick). The thick mucus is difficult to clear from the respiratory tract, and it provides a nutrient-rich breeding ground for bacteria. Individuals with CF are therefore subjected to repeated bouts of lower respiratory tract infections. 2. How would you classify this girls acid-base status? This girl is in respiratory acidosis, as is evident from her decreased arterial blood pH, elevated arterial blood pCO2, and normal arterial HCO3- concentration. How does cystic fibrosis cause this acid-base imbalance? The pooling of excessively thick mucus obstructs the small and large airways. This reduces the patients minute ventilation, causing hypoventilation. As she hypoventilates, the pCO2 level rises and the excess CO2 reacts with H2O in the bloodstream to produce carbonic acid (H2CO3), which dissociates into H+ ions and HCO3- ions, lowering the pH of the blood. Wh