No abstract

The goal of this study was to evaluate the safety and efficacy of recombinant human DNase (rhDNase) in hospitalized patients with cystic fibrosis (CF) experiencing acute pulmonary exacerbations. Eighty patients with documented CF were enrolled at 11 CF centers when admitted for antibiotic therapy. Patients were at least 5 yr old with a forced vital capacity (FVC) > or = 35% of predicted and an oxygen saturation > or = 90% on a fraction of inspired oxygen (FIO2) < 0.5. Patients were randomized to receive rhDNase 2.5 mg in 2.5 ml excipient twice a day (n = 43) or 2.5 ml excipient alone twice daily (n = 37) along with conventional treatment for exacerbations. Administration of rhDNase was not associated with acute adverse events or deaths, and no patients experienced allergic or anaphylactic reactions. Although forced expiratory volume in one second (FEV1) and FVC improved in both treatment groups during the double-blind period, there were no statistically significant differences in the mean change from baseline in FEV1 or FVC between the two groups. rhDNase therapy is safe and well tolerated in CF patients with acute exacerbations requiring hospitalization, but the study did not demonstrate a statistically significant therapeutic effect of rhDNase when added to a regimen of antibiotics and chest physical therapy.

No abstract

Persistent pleural effusions are not common in patients with primary systemic amyloidosis (AL). A recent review of this complication of the disease hypothesized that the pathophysiology of these effusions is pleural amyloid deposition, disrupting lymphatic drainage. We report the case of a 73-year-old woman with primary systemic AL and persistent bilateral pleural effusions, refractory to diuresis and repeated thoracenteses. The patient's cardiac and renal dysfunction was not severe enough to explain these persistent effusions. Thus, despite a lack of biopsy-proven amyloid deposition, we suggest that these effusions may be secondary to pleural amyloid deposition.