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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.

The determination of the ADA activity was only of importance in special affections, as for instance typhus, epidemic jaundice, active liver cirrhosis till now, where high enzyme activities are measured. Nevertheless this enzyme is also useful for the pathogenetic classification of hyperuricemia, the elucidation of immunoaffections of obscure etiology, and, above all, of the genetic ADA defect. Our results show that the determination of the ADA activity enables some declaration for the therapy and the control of progress of the acute and the chronic leukemia. Investigations of the distribution in the organs and age-dependence in animals emphasize the great biological importance of this enzyme in the metabolism, where much questions are open till now.

Our investigation of the occurrence of the enzymes phosphoglucomutase (PGM), glutamate-pyruvate-transaminase (GPT), adenylatkinase (AK), adenosine-desaminase (ADA), and 6-phophogluconate-dehydrogenase (6-PGD) produced the following results: The phosphoglucomutase type was demonstrated in the most sperm samples and seminal stains in accordance with the corresponding blood type. This enzyme is rather stable and could still be demonstrated well in 1-month old stains. The glutamate-pyruvate-transaminase can only seldom be determined in semen and seminal stains. We only found the GPT 1 type, which is known to have usually the strongest activity. The adenylatekinase was demonstrable in the most fresh ejaculates (not older than 24 h) and in about half the seminal stains (not older than 7 days)--The AK--2-band gets weak with increasing lay days, which may lead to incorrect determinations. The adenosine-desaminase could not be determined in sperm. On the contrary, 6-phosphogluconate-dehydrogenase could be demonstrated in fresh semen samples and also partly in seminal stains up to 7 days. The demonstration of the enzymes did not depend in any system on the secretor type.

The enzymatic deamination of adenosine to inosine produces ammonia, which causes a pH-increase of the reaction mixture. The pH-stat method is based on the continuous addition of protons to keep the pH at a constant value. The theoretical principles are discussed. The quantitative limits of the assay and the effect of changing the pH were investigated. A correction factor was derived and calculated for the pH range 6.4 to 8.5. This sensitive method allows the continuous recording of the adenosine deaminase activity in strongly coloured or turbid biological samples.

The occurrence of severe combined immunodeficiency (SCID) with adenosine deaminase (ADA) deficiency in erythrocytes has been reported in 14 patients. Enzyme deficiency may result in early depression of the lymphatic system. ADA is detectable in different tissues by photometric and electrophoretic methods. The gene locus for ADA has been localised on chromosome 20. Studies on the enzyme defect in different forms of primary immunodeficiencies led to the description of a well defined nosological entity. New aspects can be expected in the fields of pathogenesis, prenatal diagnosis, genetic councelling, and possibly therapeutic trials.