Faculty Bibliography

BACKGROUND: Although cystic fibrosis is the most common genetic disorder of children, its heterogeneous spectrum of severity lends itself to underdiagnosis in the older adult patient population where the index of suspicion is not high. METHODS: We report a 60-year-old Hispanic man with asthma who presented with progressive dyspnea and wheezing unresponsive to inhaled corticosteroid treatment. Additionally, he had clinical findings and a past history suggestive of cystic fibrosis. Skin testing, radiography and laboratory studies were completed to evaluate for allergic bronchopulmonary aspergillosis (ABPA) and cystic fibrosis. RESULTS: Test results revealed peripheral eosinophilia and hyper IgE. Skin testing to Aspergillus fumigatus (Af) was positive. IgG, IgM, and Af specific antibodies were present. High resolution CT scan showed central bronchiectasis. Sweat tests were positive on two separate occasions and gene analysis showed our patient to have a positive gene mutation at D127ON/D127ON. CONCLUSION: Cystic fibrosis should be suspected in the older adult patient with a compatible clinical presentation.

Continuous arteriovenous hemofiltration (CAVH) has been used infrequently in the treatment of infants and children with acute renal failure. In this report we describe the use of CAVH in a critically ill neonate who was unable to be managed with either peritoneal dialysis or hemodialysis. The procedure was safe and effective in the management of fluid overload and multiple electrolyte disturbances including life-threatening hyperkalemia. The technical aspects of this procedure are described, and its potential benefits and complications are assessed with reference to the ten previously reported cases