Pseudomonas aeruginosa and Burkholderia cepacia infection in cystic fibrosis patients treated in Toronto and Copenhagen.

Differences in the course of pulmonary disease in cystic fibrosis (CF) may be altered by different treatment strategies in different CF centers. The Copenhagen clinic uses scheduled, regular and very aggressive treatment of lung infection. The Toronto clinic treats pulmonary infection with oral, inhaled, or intravenous antibiotics, and has emphasized aggressive nutritional therapy. This study compared the clinical status of CF patients treated in the two centers (Toronto, Canada, n=302, and Copenhagen, Denmark, n=214) using a cross-sectional design in terms of Pseudomonas aeruginosa (PA) and Burkholderia cepacia (BC) lung infections, pulmonary function, and levels of PA and BC precipitating antibodies (precipitins). Median ages were similar, but the age distribution was significantly different, with a higher proportion of patients under 10 and > or = 25 years in Toronto, and higher proportion of patients 11-24 years of age in Copenhagen. A higher number of female patients was observed in Copenhagen than in Toronto. Seventy-nine percent of Copenhagen patients, and 52% of Toronto patients were deltaF508 homozygous. Of all the patients, 20.1% of Copenhagen patients and 38% of Toronto patients were deltaF508 heterozygous. Ten percent of Toronto patients had two uncommon mutations. Pulmonary function and nutritional status in both groups were similar despite varying treatment strategies. The prevalence of PA was lower in Danish children and higher in Danish adults than in Canada. These differences are probably due to cohort isolation, which was introduced in Copenhagen in 1981. The prevalence of BC was higher in Toronto than in Copenhagen patients at all ages. In both centers, the number of PA and BC precipitins increased with age in patients chronically infected with PA and BC, respectively, and the number of both PA and BC precipitins rose with declining lung function. This study suggests that the clinic populations had similar pulmonary and nutritional statuses despite differing clinic antibiotic treatment strategies. Microbial colonization seemed to differ, at least in part, because of differences in cohort isolation strategies. Early, aggressive anti-pseudomonal chemotherapy may have reduced pseudomonal colonization among younger patients in Copenhagen. Future studies will be required to assess the impact of this on this cohort's outcome.

Changes in lactate dehydrogenase isoenzymes associated with relapse of childhood acute lymphocytic leukemia.

Twenty-eight children with high-risk acute lymphocytic leukemia underwent monthly serum lactate dehydrogenase (LDH) and LDH isoenzyme fraction determinations to examine whether LDH isoenzyme fractions change with an increase in the body burden of tumor cells. The 9 patients who relapsed and 5 patients who presented with leukemia during the study period had a slightly lower mean LDH-1 isoenzyme fraction. When the period from 3 months before to 3 months after relapse was examined, significant increases in the LDH-3 isoenzyme fraction and decreases in the LDH-1 and LDH-2 isoenzyme fractions were seen at the time of relapse. These results were highly significant when patients with non-T-cell and T-cell leukemia were combined and when bone marrow and central nervous system relapse was included. The changes at relapse appeared to revert with intensification of chemotherapy. The changes at relapse were not different in magnitude from random variation occurring in patients who remained in remission throughout the study. Although changes in LDH isoenzymes appeared to occur at the time of relapse compared with the periods immediately before and after relapse, these changes were not specific for relapse. LDH isoenzymes do not appear to be useful in predicting relapse in children with leukemia.

Plasma carnitine levels in cystic fibrosis.

Plasma carnitine concentrations were measured in 43 children and adults with cystic fibrosis (CF), and values were compared with those from normal controls. Clinically significant abnormalities of plasma carnitine concentration were not found in CF patients. The concentration of free carnitine was slightly but significantly elevated in CF patients, and the acylcarnitine concentration and acylcarnitine/free-carnitine ratio were slightly but significantly lower. Total carnitine concentrations were similar to those of controls. The CF patients did not have abnormal urinary acylcarnitines. Altered concentrations of free and esterified carnitine were not associated with nutritional status or with liver or pulmonary function.