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Biomedical subjects

H Kollberg

Publications and source records attributed to H Kollberg.

At least 55 records · Page 3Linked to original sources

Meconium screening for cystic fibrosis. An eight-year follow-up study.

The results of more than eight years of screening for cystic fibrosis (CF), utilizing the albumin concentration in meconium, are reported. Over 28 000 meconium samples were tested, over 24 000 of them by the Boehringer-Mannheim strip test, and all samples considered as showing a trace or positive result were followed up by an immunological technique. Eight-three meconium samples contained more than 20 g albumin/kg dry weight meconium and five of these were from children who were found to have CF. One child with CF was missed in the screening. From these figures the sensitivity of the method for detecting CF children at birth was calculated to be 75%, the specificity 99.7%, the negative predictive value 100%, the positive predictive value 4.0% and the incidence of CF in Sweden about 1:4,500. The incidence of pathologically elevated meconium albumin concentrations was found to be 25 times higher in premature than in full-term infants. Since the number of false positive results creates a problem, we suggest that premature children (birth weight less than 2500 g) and newborns with melaena should be excluded from further follow-up studies.

Albumins↗

Acid hydrolases in sera and plasma from patients with cystic fibrosis.

The enzyme activities of alpha-fucosidase (pH 4.0 and pH 5.5), alpha-galactosidase, beta-galactosidase, alpha-glucosidase (pH 4.5 and pH 6.0), beta-glucosidase, beta-glucuronidase, beta-hexosaminidase, and alpha-mannosidase (pH 4.5 and pH 5.5) were investigated in sera from cystic fibrosis (CF) patients. Several of these activities were significantly increased in sera from patients compared to age-matched control children. CF-patients in a more advanced stage of the disease had a tendency to higher values of some of these hydrolases than those in better condition. No new isoenzymes of these hydrolases were found. Only minor differences could be detected in the pH-profiles of alpha-mannosidase and acid phosphatase from age-matched normal controls, heterozygotes and homozygotes for CF. With our technique, alpha-mannosidase and acid phosphatase showed the same thermostability in CF-patients. CF-heterozygotes and age-matched controls, except at 56 degrees C, when the activity of acid-phosphatase in the plasma from adult CF-heterozygotes decreased more than that from adult controls

Acid Phosphatase↗

Effects of cystic fibrosis serum and cell culture medium on the mucociliary activity of the respiratory tract. An in vitro study in rabbits.

As previously reported a cystic fibrosis factor (CFF) is associated with the dyskinetic ciliary motion induced by serum and cell culture medium from patients with cystic fibrosis (CF). In this study a sensitive, standardization method, for the photoelectric recording of mucociliary activity was used to examine the effect of sera and media from cell cultures taken from patients with CF and healthy controls, on the mucociliary activity of rabbit trachea in vitro. No signs of decreased mucociliary activity were observed and electron microscopy showed normal ultrastructure and orientation of cilia.

Animals↗

Ultrastructural studies on cultured fibroblasts from patients with cystic fibrosis.

The ultrastructure of cultured fibroblasts of patients with cystic fibrosis (CF) was investigated and compared with control cultures. CF-fibroblasts showed a significantly increased number of pinocytotic vesicles along the plasma membrane. Other differences in ultrastructure between CF-fibroblasts and controls could not be observed. Cytochemical localisation of calcium was studied after fixation in the presence of oxalate. In CF-fibroblasts, oxalate precipitates (presumably indicating the presence of calcium) were found in the cytoplasm, often associated with membranes of the endoplasmic reticulum and Golgi vesicles, and in association with nuclear membrane and plasma membrane. In control fibroblasts, oxalate precipitates were scarce. The results are in accordance with earlier findings of elevated calcium in fibroblasts from CF-patients, and point to a localisation of at least part of the excess calcium in the cytoplasm and microsomal fraction.

Adult↗

Autoantibodies to Tamm-Horsfall protein in patients with cystic fibrosis.

Sera from 35 patients, 17 boys and 18 girls, with cystic fibrosis were analysed for autoantibodies to the Tamm-Horsfall protein. Significantly (p less than 0.001) elevated levels of specific IgG and IgA, but not IgM antibodies to Tamm-Horsfall protein were found. There was a considerable overlap between the values in the disease group and the control group. The highest values were found among the patients with liver involvement. The patients with marked lung abnormalities as well as those with positive bacterial culture of sputum had normal antibody levels.

Adolescent↗

Electrolytes in nails analysed by X-ray microanalysis in electron microscopy. Considerations on a new method for the diagnosis of cystic fibrosis.

Patients with cystic fibrosis (CF) have an increased concentration of sodium in their nails. Hitherto, only neutron activation analysis has been considered for the diagnosis of cystic fibrosis by analysis of electrolytes in nails. It has been thoroughly tested methodologically and clinically. However, the intrinsic advantages of X-ray microanalysis and the results obtained in this study suggest that this method, after further testing, may be a useful diagnostic acid for cystic fibrosis. In comparison with neutron activation analysis, X-ray microanalysis has the advantage of simultaneously giving the concentrations of several elements and may be accessible at any hospital with an electron microscope fitted with the necessary equipment. Nails of CF-patients are here shown to have increased concentrations of Na, K and Cl, which will make the diagnosis of cystic fibrosis more reliable. The possibility of using sulphur as a reference element may eliminate the weighing procedure necessary in neutron activation analysis.

Child, Preschool↗

Reliable diagnosis of the major type of cystic fibrosis with fibroblast cultures. A double blind study.

A double blind study has been carried out to demonstrate that the most common type of cystic fibrosis (CF) can be reliably diagnosed with skin-derived fibroblast cultures. Alkaline phosphatase (AIP) activity has been measured in 6 normal controls, 12 CF-heterozygotes and 6 CF-homozygotes before and after stimulation with Tamm-Horsfall-glycoprotein (THP), isoproterenol and theophyline ("THP-induction test"). The mean AIP-activities after THP-induction were 8.8, 12.7 and 34.6 for the three different genotypes respectively. There was no overlap between the values of CF-homozygotes on the one hand, and the values of CF-heterozygotes and normal controls on the other hand. All 24 specimens were correctly diagnosed in the present double blind study, indicating the very high degree of reliability of the THP-induction test in the detection of the predominant type of cystic fibrosis with fibroblast cultures. Normal controls and CF-heterozygotes could not be discriminated on an individual basis, but as a group the CF-heterozygotes displayed higher AIP-values.

Adolescent↗

Cystic fibrosis compared with the immotile-cilia syndrome. A study of mucociliary clearance, ciliary ultrastructure, clinical picture and ventilatory function.

Patients with cystic fibrosis (CF) were investigated for mucociliary clearance (with and without stimulation by terbutaline), clinical picture, ventilatory function and ultrastructure of cilia. The results were compared with those of patients with congenitally immotile cilia (immotile-cilia syndrome). Mucociliary clearance could be demonstrated in all the seven CF patients who succeeded in inhaling the test aerosol. Ciliary ultrastructure from a deceased CF patient was normal. Patients with the immotile cilia syndrome had no substantial clearance and defective cilia. The CF patients coughed more during the clearance measurements than any other group studied earlier, and their coughing was effective. One patient succeeded in avoiding coughing in both measurements and had faster clearance when he got terbutaline than when he got the vehicle. Although younger, the CF patients tended to be more obstructed in their lungs and more handicapped than the patients suffering from the immotile-cilia syndrome. The latter patients had more discomfort from rhinitis, sinusitis and otitis than had the CF patients. An impairment of the mucociliary transport rate is hence unlikely to be a primary pathogenic factor for the respiratory tract disease in CF patients.

Adult↗