[Isolation frequency of Burkholderia cepacia from cystic fibrosis patients]. / Kistik fibrozisli hastalarda Burkholderia cepacia izolasyon sikligi.

Microorganisms such as Pseudomonas aeruginosa, Staphylococcus aureus and Haemophilus influenzae frequently cause colonization and infection in airways of patients with cystic fibrosis. Burkholderia cepacia has also been isolated in patients with cystic fibrosis since 1980. In this study, we aimed to determine the colonization rate of B. cepacia in 286 sputum samples obtained from 129 cystic fibrosis patients. Selective media for B. cepacia were used besides the routine microbiological media in order to increase the isolation rate. The colonies were identified by biochemical tests and the antibiotic susceptibilities of the strains were determined by disc diffusion method. Pathogenic bacteria (S. aureus, P. aeruginosa, Enterobacteriaceae, Streptococcus pneumoniae, H. influenzae) were isolated in 52 of 129 patients (40%) and 66 of 286 sputum samples (23%). In addition 2 B. cepacia strains were isolated from two different patients (1.55%). B. cepacia is now being considered as a pathogen isolated from sputum samples of patients with cystic fibrosis with an increasing frequency and causing severe clinical features. According to these results it can be concluded that, the use of selective media for B. cepacia isolation, should be taken into consideration especially by the clinical microbiology laboratories collaborating with the cystic fibrosis centers.

Analysis of the modifying effects of TAP 1/2 genes on cystic fibrosis phenotype.

Phenotypic variability has been reported in cystic fibrosis (CF) patients. TAP1 and TAP2 genes are encoding "the transporter associated with antigen processing" proteins. The aim of the present study was to analyze the frequency of TAP 1/2 variants in the Turkish population and to investigate a possible modifying role of these variants in CF phenotype. Sixty-three CF patients of known genotypes and 100 healthy control subjects were analyzed. There was a significant difference in the frequencies at positions 333 and 637 of TAP 1 gene and at position 665 of TAP 2 gene between patients and controls. Comparison of TAP gene polymorphisms in 36 CF patients homozygous for AF508 mutation with control subjects revealed a significant difference at position 665 of TAP 2 gene. These findings may be useful to assess the predisposition and to predict severity of the disease. We demonstrated that TAP genes might have modifying effects on the CF phenotype.

Hodgkin's disease and ataxia telangiectasia with pulmonary cavities.

Ataxia telangiectasia (AT) homozygotes have an increased risk for development of Hodgkin's disease (HD). Parenchymal lung involvement is not uncommon in HD; however, cavitary pulmonary lesions are quite unusual. We report on 3 cases of AT with HD who had mediastinal disease and parenchymal pulmonary involvement with cavitation. Of 6 AT patients in our HD series, 3 developed pulmonary cavities. The patients displayed pulmonary infiltration, cavitation in the lung parenchyma, and mediastinal enlarged lymph nodes on both plain chest X-rays and thoracic computed tomographies. No infectious etiologies were established for the pulmonary findings. Histopathological examination of open lung and mediastinal biopsies revealed HD, and all patients received multiagent chemotherapies. The outcome was fatal in all 3 patients. Respiratory infections are the principle cause for morbidity and mortality in AT patients. Reports on cavitating pulmonary lesions in HD are quite rare. Furthermore, data regarding the patterns of pulmonary involvement in AT patients with or without HD are lacking. The increased incidence of malignancies in AT patients may relate to immunodeficiency and to the chromosomal alterations identified.

Treatment and follow-up of pulmonary alveolar microlithiasis with disodium editronate: radiological demonstration.

A 9-year-old girl with pulmonary alveolar microlithiasis is presented. She was asymptomatic except for failure to thrive. Plain chest radiographs on admission showed sand-like opacities with calcific densities throughout both lung fields, predominantly in the lower zones. A black pleural line was also seen between the ribs and lung parenchyma. High-resolution CT (HRCT) revealed diffuse ground-glass opacities throughout both lungs, micronodular densities, interlobular septal thickening, linear calcifications along the pleura and a few scattered subpleural cysts. Disodium editronate, which is known to inhibit the microcrystal growth of hydroxyapatite, was started at 10 mg/kg per day orally. After 1 year of therapy, considerable regression of the calcific densities was detected on chest radiographs and HRCT scans.