A model for the operationalization of empirical research of defense mechanisms is presented. Measures for operationalized defense diagnoses are discussed against the background of a cognitive model of different coding of information channels (linguistic and perceptual coding). Studies for the operationalized diagnoses in the clinical assessment of defense mechanism are discussed for the identification of the defense mechanism, reliability of rating procedures, and the prognostic aspects in psychotherapy and psychosomatics. The regulation of defense on different levels of a hierarchy of complex systems of variables is demonstrated with paradigmatic results.

An infant with congenital homozygous factor XIII deficiency demonstrated a severe retroperitoneal and intracerebral bleeding with development of a posthemorrhagic hydrocephalus in the first months of life. Factor XIII activity was not measurable by means of enzymatic method and the antiserum inhibition test. Quantitative immunoelectrophoresis according to Laurell presented absence of the subunit A, whereas the concentration of subunit S was reduced to 47% the normal value. After replacement therapy factor XIII activity was estimated at 23% and corresponded to the concentration of the subunit A, concentration of subunit S increased by 20%. The turnover rate of fibrin stabilizing factor could be observed over a period of 39 days. The half life was estimated at 4,7 days. The child developed normally after continous substitution with 250 units of factor XIII concentrate every 6 weeks.

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