[Effectiveness of secondary preventive programs for patients with asthma and rhinitis in the baking industry]. / Effektivität eines Sekundär-Präventionsprogramms für asthmatisch und rhinitisch Erkrankte im Bäckerhandwerk.

Since 1992 the German industrial professional association for the food industry and gastronomy (Berufsgenossenschaft Nahrungsmittel und Gaststätten), which is responsible for statutory accident insurance has been offering a specific patient education programme to bakers suffering from occupationally induced obstructive pulmonary disease or allergic rhino-conjunctivitis, who do not quit their jobs and wish to continue working as bakers. The programme aims at preventing aggravation of the disease 74 male bakers with a mean duration of illness of 10 years on entering the programme were examined between 1 and 5 years (mean: 2.9) after participating in the patient education seminar. For a subpopulation of 55 of these patients spirometric measurements were available for the time before programme entry and at follow-up. Data on time spent at the bakehouse, prevalence of symptoms, frequency of symptoms at the site of working and during leisure hours, sleep disturbances and emergency situations were collected at the follow-up examination both for the time before programme entry and at follow-up, respectively. Direct ratings of the amount of changes in distress caused by the disease and of the quality of life were also obtained. The prevalence of 8 of the 9 most frequently reported symptoms decreased significantly, e.g. dysponoea from 70% to 42% and fear of suffocation from 30% to 3%. Participants reported on highly significant reductions in the frequency of disease-specific complaints during work and outside the working place, sleep disturbances and situations requiring immediate medical intervention. Between 64% and 85% of the patients experienced improvement of distress, physical and work performance, private and family life, and ability for self-management. Lung function did not deteriorate: mean FEV1 was 88% (+/- 23) at first and 90% (+/- 19) at second measurement. These results could not be explained by changes in medication. It is concluded that the programme is effective in reducing symptoms and distress and in improving quality of life in bakers with occupational respiratory disease who voluntarily continue to work as bakers.

Defective lysosomal release of glycoprotein-derived sialic acid in fibroblasts from patients with sialic acid storage disease.

Fibroblasts from patients with sialic acid storage disease (SASD), sialidosis, mucolipidosis II, and from normal controls, were incubated in the presence of the glycoprotein fetuin that was tritium-labelled in its sialic acid residues by the periodate/[3H]borohydride reduction method, and the fate of the intracellular radioactive sialic acid (C7-sialic acid) followed in pulse-chase experiments. The model glycoprotein was readily endocytosed and degraded, more than 90% of the radioactivity being trichloroacetic acid (TCA)-soluble after 4 days of incubation. In all of the patients' fibroblasts, there was an increased accumulation of TCA-soluble radioactivity and, upon chase, a much lower rate of elimination than in normal controls. Gel chromatography of the material from the chase experiment showed that, in normal cells, most of the radioactivity at zero time behaved as free C7-sialic acid. This, as well as material of larger size (sialyloligosaccharides), was very much diminished by 48 h. In cells from two patients with SASD, there were large peaks both in the sialic acid and oligosaccharide positions; whereas the oligosaccharides were somewhat decreased by the end of the chase period, the sialic acid was essentially unchanged. In sialidosis fibroblasts, the radioactive material consisted of oligosaccharides, but very little C7-sialic acid; the elimination of the oligosaccharides was retarded. In normal cells, about 80% of the radioactivity released into the medium after 48 h chase behaved as free C7-sialic acid upon gel chromatography and t.l.c. Subcellular fractionation in Percoll gradients showed that the radioactive C7-sialic acid remaining in normal cells after 48 h of chase was mainly localized in the cytosol. In SASD cells, on the other hand, it was associated with lysosomal fractions which, unexpectedly, exhibited an abnormally low density. Our findings demonstrate that SASD fibroblasts degrade the sialoglycoprotein but, unlike normal cells, accumulate the liberated C7-sialic acid along with sialyloligosaccharides in their lysosomes. The results therefore support the concept of a defective transport system for sialic acid in the lysosomal membrane of patients with SASD.