Biomedical subjects
J Hein
Publications and source records attributed to J Hein.
[Long-term treatment of mucoviscidosis. Results with a microencapsulated pancreatic enzyme preparation].
METHODS: In 30 children suffering from cystic fibrosis, the long-term effect (2 years) of treatment with an acid-protected micro-encapsulated pancreatin preparation was investigated in comparison with prior enzyme replacement with a conventional pancreatin preparation given over a period of years. Assessment criteria was the development of body length and body weight, lung function (vital capacity, FEF1) and the Shwachman-Kulczycki score. RESULTS: The therapeutic efficacy of the acid-protected micro-encapsulated preparation is considered to be higher than that of conventional preparations, since the same effect was achieved with only one-quarter to one-third of the pancreatin dose of the conventional preparation (average: 6.5 +/- 2.9 g pancreatin/d, as compared with 1.6 +/- 0.6 g pancreatin in the case of the acid-protected micro-encapsulated preparation). However, none of the clinical parameters investigated revealed any significant changes during the course of the observation period. The results indicate that, if the general state of health and nutritional status of patients with cystic fibrosis is to be improved further, in addition to a special diet, attempts must be made to achieve further optimization of the intraluminal digestion.
Prevalence of cystic fibrosis mutations in the East German population.
A representative multicenter cystic fibrosis (CF) mutation analysis on about half of all known cystic fibrosis patients of the 5 East German Länder is reported. Analyses for 17 mutations, among them Delta F508, R553X, G542X, S549R,N,I, G551D, S1255X, R347P,H, and Y122X, were performed. As expected, the delta F508 mutation in exon 10 of the CFTR gene is the major gene alteration causing CF in our patients. However, in comparison to studies from Western Germany, a significantly lower percentage of just over 60% is found in our patients, resembling data obtained from slavonic populations. The severe phenotype of cystic fibrosis is most frequently associated with homozygosity for the delta F508 mutation. No particular allele association could be found with the intermediate and mild phenotypes of this disease. The next most frequent of the investigated mutations is R553X (13.3% of non-delta F chromosomes) followed by R347P (9.2%) and G542X (4.4%).
Effect of inhaled methanol on pituitary and testicular hormones in chamber acclimated and non-acclimated rats.
Two experiments were conducted in which the acute effects of inhaled methanol on serum hormones associated with reproductive function in the male rat were evaluated. In the first experiment, rats exposed to methanol (0, 200, 5000 and 10,000 ppm) for 6 h were killed at the end of the exposure period (6 h) or the following morning (24 h). Also, because the process of exposure itself could modify neuroendocrine function, the effect of the handling associated with placing the rat in the exposure chamber was evaluated further by dividing the exposed animals into acclimated (2 weeks of prior handling) and non-acclimated groups. At 6 h, an effect of prior handling was noted in the sham-exposed rats, with serum luteinizing hormone (LH) of the non-acclimated group being greater than that of the acclimated group. Serum LH concentrations were altered by methanol exposure, but the direction of change and the exposure level at which an effect was noted differed between the acclimated and non-acclimated rats. Methanol (5000 ppm) reduced serum LH in the non-acclimated animals, while 10,000 ppm increased LH in the acclimated rats. Follicle stimulating hormone (FSH) and testosterone were unchanged by methanol in rats killed at 6 h. Thus, this experiment did not confirm earlier reports that exposure to 200 ppm for 6 h reduced serum testosterone. At 24 h, an effect of prior handling was still present in the hormonal measures, with serum and interstitial fluid testosterone concentrations being greater in the non-acclimated rats. Also, there was a dose x handling interaction with methanol exposure inducing an increase in serum testosterone in the non-acclimated rats (up to 5000 ppm) and a decrease in the acclimated rats (up to 10,000 ppm). In the second experiment, groups of acclimated and non-acclimated rats were exposed to 0 or 5000 ppm methanol for 1, 2 and 6 h and killed immediately after removal from the chamber. Serum LH, testosterone and FSH values were not different in sham- vs methanol-exposed rats at any time point. As in experiment 1, an effect of prior handling was noted. In general, the concentrations of these hormones and serum prolactin in the non-acclimated rats were greater than those observed for acclimated rats. Methanol exposure resulted in increased prolactin concentrations under both handling conditions.(ABSTRACT TRUNCATED AT 400 WORDS)
[Bronchoalveolar lavage--a diagnostic method in chronic nonspecific bronchopulmonary diseases in childhood? 2. Studies of cellular and humoral parameters in BAL irrigation fluid].
With a view of the pathogenesis of chronic bronchopulmonary diseases the interrelations between infections and evolving defense system are of interest, they are perhaps detectable by means of diagnostic bronchoalveolar lavage. We carried out cytodifferentiation, investigated adenosine deaminase activities and interleukin 1 formation of macrophages, determined immunoglobulin concentrations (secretory IgA), lysozyme, alpha 2-macroglobulin, alpha 1-antitrypsin, albumin. Because the cytodifferentiation yields insight into topical inflammatory reactions, shows diagnostic useful informations in single cases and because it is simple to carry out we can recommend it for each bronchological examination. There were no results specific for any disease group for parameters mentioned above.
[Thiocyanate--a pathogenetic factor in cystic fibrosis (mucoviscidosis)?].
Thiocyanate (SCN-) concentrations in serum samples of 32 cystic fibrosis (CF) patients, 30 controls as well as 23 heterozygotes (parents of CF patients) were investigated, because SCN- influenced viscosity of secretions, membrane functions as well as nonspecific and specific defense processes. CF patients showed little smaller SCN- serum levels, but the differences were not statistically significant. There were remarkable smaller values for CF patients with most serious clinical conditions due to severe pulmonary changes.
First analysis of the F508 deletion in cystic fibrosis patients from the GDR.
Cystic fibrosis (CF) patients (n = 157) from the GDR were analysed for the occurrence of the recently discovered 3bp deletion causing CF. About 50% of all investigated patients were homozygotes and about 30% heterozygotes for this deletion. Of the analysed CF chromosomes from these patients, 62% carry the deletion, which is in strong linkage disequilibrium with the KM19 restriction fragment length polymorphism allele 2 and the 1/2 XV2c/KM19 haplotype.
Reconstructing evolution of sequences subject to recombination using parsimony.
The parsimony principle states that a history of a set of sequences that minimizes the amount of evolution is a good approximation to the real evolutionary history of the sequences. This principle is applied to the reconstruction of the evolution of homologous sequences where recombinations or horizontal transfer can occur. First it is demonstrated that the appropriate structure to represent the evolution of sequences with recombinations is a family of trees each describing the evolution of a segment of the sequence. Two trees for neighboring segments will differ by exactly the transfer of a subtree within the whole tree. This leads to a metric between trees based on the smallest number of such operations needed to convert one tree into the other. An algorithm is presented that calculates this metric. This metric is used to formulate a dynamic programming algorithm that finds the most parsimonious history that fits a given set of sequences. The algorithm is potentially very practical, since many groups of sequences defy analysis by methods that ignore recombinations. These methods give ambiguous or contradictory results because the sequence history cannot be described by one phylogeny, but only a family of phylogenies that each describe the history of a segment of the sequences. The generalization of the algorithm to reconstruct gene conversions and the possibility for heuristic versions of the algorithm for larger data sets are discussed.
Unified approach to alignment and phylogenies.
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Secretory immunoglobulin A in serum of CF patients.
Increased sIgA values were found in CF patients in about 70 per cent (3 studies with 23, 43, 45 CF patients). With higher severity of CF disease (Shwachman score) we could observe higher sIgA levels. Postmortem histological findings and sIgA concentrations correlated well in 13 cases. Determinations of sIgA in serum could serve as parameter for hepatobiliary involvement in CF. Secretory immunoglobulin A (sIgA) is the predominant immunoglobulin of exocrine secretions. In serum large and frequent elevations of sIgA could be found in patients with chronic liver disease characterized by biliary obstruction (3). Slight and irregular elevations were observed in patients with disorders of gastrointestinal tract and of respiratory tract as well as in lactating women (3). Manifestations of the hepatobiliary system in CF will become more important with advancing age and progressively higher life expectancy of CF patients. A simple reliable test for detection of hepatobiliary involvement in CF would be useful.
Bacterial spectrum of the germs in the respiratory tract of patients with CF at smaller centres.
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Diagnosis of cystic fibrosis by means of particle electrophoresis test.
Incubation of polystyrene latex particles with CF serum leads to reduced electrophoretic mobility (EPM) measured by a particle electrophoresis device. About 85% of 50 CF patients, 60 CF heterozygotes and 190 controls (healthy subjects and patients with different disorders) could be identified correctly.
[Some new treatment strategies for lung involvement in patients with mucoviscidosis].
Autogenic drainage, PEP mask breathing as well as physical exercise are now well established in the treatment of pulmonary disease in cystic fibrosis, but there are different opinions about oxygen therapy over a long period as well as corticosteroid treatment. First reports on amiloride inhalations seem to be hopeful, but there is no experience with cystic fibrosis children till now, therefore clinical use can not be recommended. Heart lung transplants are not to consider as an alternative treatment for all cystic fibrosis patients, in special cases it may be successful.
A new method that simultaneously aligns and reconstructs ancestral sequences for any number of homologous sequences, when the phylogeny is given.
Among the fundamental problems in molecular evolution and in the analysis of homologous sequences are alignment, phylogeny reconstruction, and the reconstruction of ancestral sequences. This paper presents a fast, combined solution to these problems. The new algorithm gives an approximation to the minimal history in terms of a distance function on sequences. The distance function on sequences is a minimal weighted path length constructed from substitutions and insertions-deletions of segments of any length. Substitutions are weighted with an arbitrary metric on the set of nucleotides or amino acids, and indels are weighted with a gap penalty function of the form gk = a + (bxk), where k is the length of the indel and a and b are two positive numbers. A novel feature is the introduction of the concept of sequence graphs and a generalization of the traditional dynamic sequence comparison algorithm to the comparison of sequence graphs. Sequence graphs ease several computational problems. They are used to represent large sets of sequences that can then be compared simultaneously. Furthermore, they allow the handling of multiple, equally good, alignments, where previous methods were forced to make arbitrary choices. A program written in C implemented this method; it was tested first on 22 5S RNA sequences.
A tree reconstruction method that is economical in the number of pairwise comparisons used.
A fast method for reconstructing phylogenies from distance data is presented. The method is economical in the number of pairwise comparisons needed. It can be combined with a new phylogenetic alignment procedure to yield an algorithm that gives a complete history of a set of homologous sequences. The method is applicable to very large distance matrices. An auxiliary program was developed that simplifies large phylogenies without ignoring biologically essential features. A set of 213 globins from vertebrates, plants, and Vitreoscilla (a prokaryote) were analyzed using this method.
[The effect of bronchologic studies on blood gases and hemodynamics in children].
During 78 bronchological examinations in children of all age groups out of 6 centers of children bronchology of the GDR PaO2, PaCO2 and pH were registered, in a part of them additionally pulse, systolic and diastolic blood pressure. Following to the intubation--under ventilation with 100% oxygen or a halothane-NO2-oxygen mixture--there was a marked rise of PaO2, which normalized after extubation quickly. After extubation a slight hypoxemia occurred for a short period, a quick rise of PaCO2 and a slight acidosis, too. Besides, the hearth rate, systolic and diastolic blood pressure elevated following to the intubation and diminished after extubation.
[Secretory immunoglobulin A (sIgA) in parotid saliva of patients with cystic fibrosis].
Investigations of immunoglobulin concentrations--especially of secretory IgA (sIgA)--were performed in isolated parotid saliva samples by means of a modified Mancini technique considering the structural specialties of sIgA. The measurement of flow rates under continuous stimulation of parotid gland secretion by citric acid allowed calculations of glandular output. Cystic fibrosis patients showed decreased secretion rates of sIgA during gland secretion stimulation. Because parotid glands and bronchial glands are parts of BALT, we can conclude, that a functional sIgA deficiency is a pathogenetic factor under others in chronic bronchopulmonary infections of cystic fibrosis patients.
Secretory immunoglobulin A (sIgA) in the serum of cystic fibrosis patients.
Clearly increased sIgA values were found in cystic fibrosis patients in more than 70%. The highest concentrations were seen in patients with obstructive liver disease. There were no correlations between sIgA and liver-associated enzymes. Postmortem histology and sIgA correlated well. These data suggest a pertinent role of sIgA assessment in evaluation of hepatic involvement in cystic fibrosis.