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Notes: Summary The “hair-standing-on-end” appearance or the “brushlike” symptom on the skull occurs in a number of severe blood diseases and is due to the action of the hyperplastic and hypertrophic red marrow filling up the bones in these disorders. No special metabolic or hereditary features of the osseous tissue can be made responsible for the alterations: the changes of the bones are brought forth by the direct effect of the erythroid cells on the bony substance without the interference of osteoclasts and osteoblasts. It is the pressure exerted by the growing and multiplying red cells, whose immature precursors require a largely increased space, that constitutes the main factor causing the alterations. The characteristic changes observed in the X-ray pictures as well as in the autoptic preparations are resulting from the adjustment of the bones and the osseous substance to the forces of pressure and originating from the overgrowing marrow. They cause a widening of the bones and arrange the structure of the spongious substance according to their direction and distribution. That can be recognized by the characteristic pattern of the cancellous substance. An other important factor for the development of the “brush-like” alterations are the shape and the anatomical conditions of the cranial bones. As the marrow constitutes a kind of semiliquid matter and is surrounded from all sides by a solid osseous layer, the forces of pressure arising in the marrow must be directed, according to physical laws, perpendicularly to the enveloping bone. Also the forces of stress evolved in the marrow will act in the same direction. Thus the direction and the distribution of the forces of pressure and stress depend largely on the morphological feature of the bones. Thus, due to the peculiar shape and anatomical conditions of the cranial bones, these forces are distributed in a special manner and owing to the strong bending of the bones, they are acting in a radial direction. They are diverging outwards and converging inwards respectively. That is demonstrated by the structure of the spongious substance showing a vertical striation and a radial arrangement of the spiculae composing the “brush” on the skull. The “brush-like” symptom, therefore, represents the arrangement of the spongious substance in the cranial bones according to the particular distribution of the forces of pressure and stress in the bones caused by the overgrowing marrow and, as the anatomical structure of the cranial bones is very different from all other bones, the “brush-like” symptom is only to be found on the skull. In consequence of the divergency or convergency respectively of the forces developed in the bones secondary effects are produced resulting in a tension in the outer parts of the cranial bones, moreover, and in a compression in the inner parts. Due to it the tabula externa and the neighbouring layers of the osseous substance are loosened and rarefied and the tabula interna and the adjacent strata are condensed and thickened. As a result of it, a different resistance in the interior of the bones is developed facilitating the expansion of the marrow and the enlargement of the spongious substance into the exterior direction. Against it, the growth of the marrow and the enlargement of the bony tissue into the interior direction are rendered difficult. By diverting the growth and expansion in an unilateral way the provision of new space for the marrow on the exterior surface of the cranial bones is strongly favoured, whereas the invasion in the cerebral cavity is prevented. The “brush-like” symptom, therefore, is the result of the close association between function, structure and anatomical conditions. The organism, by making use of their connection, is meeting with the increased demands put on him in severe blood disorders and provides new and organized space for the badly needed blood forming tissue without doing harm to the body and its functions. Thus the organism sets an impressive example of an autonomous way of self-help in a difficult situation by functional and structural adaptation.

Notes: Schlußsätze 1. Es wurde dasVerhalten desCaatleschen Fermentes im Magen bei derfunikulären Spinalerkrankung untersucht und dabei festgestellt, daßkeine sichere Beziehung zwischen demVerhalten dieses Fermentes —Vorhandensein oderFehlen — und derfunikulären Spinalerkrankung besteht. TrotzProgredienz derNervenerscheinungen kann das Castlesche Ferment im Magenvorhanden sein; esfehlt nur in solchen Fällen, bei denengleichzeitig eineperniziöse Anämie, sei es im Stadium der Dekompensation oder in Remission, vorliegt. 2. Auch das klinische Verhalten im Verein mit diesem Untersuchungsergebnis spricht dafür, daß es sich bei der funikulären Spinalerkrankung und der perniziösen Anämie trotz des häufigen gleichzeitigen Vorkommens urn zweikoordinierte, imwesentlichen und auchgenetisch prinzipiell verschiedene Krankheitsprozesse handelt. 3. DasCastlesche Ferment steht allem Anschein nachnur mit derBlutbildung in direkter Beziehung. SeinFehlen istnur für die Genese deranämischen Blutveränderung als solcher verantwortlich zu machen. 4. Es wird auf die Vermutung R.Schmidts hingewiesen, daß möglicherweise die Entstehung der funikulären Spinalerkrankung durch einenMangel an einembesonderen vielleicht lipoidartigenBaustoff verursacht wird, der zumAufbau derNervensubstanz, vor allem des spinalen Nervensystems unbedingterforderlich ist und der infolge desFehlens eineseigenen Fermentes, das von dem Castleschen Ferment verschieden ist, das ihm vielleicht aber nahesteht, im Magen aus der zugeführten Nahrungnicht gebildet werden kann.

Notes: Summary The effect of bezafibrate on HMG-CoA reductase, the key enzyme of cholesterol synthesis, and LDL metabolism was studied in human mononuclear cells. Bezafibrate at concentrations achieved during administration in patients did not suppress preformed reductase in mononuclear cells. Similarly, the drug was ineffective in regulating reductase when added to the medium of cultured cells. Also, the fibrate did not modulate the enzyme suppression mediated by LDL. At very high concentrations bezafibrate enhanced LDL binding, but both total cell association and degradation were unchanged. Thus, the previously observed decrease of HMG-CoA reductase activity in mononuclear cells of patients treated with fibrates is likely to be indirect and probably due to changes in LDL structure.

Notes: Zusammenfassung Es wird über das Vorkommen, das Röntgenbild und die Differentialdiagnose des “Bürstensymptoms” am Schädel berichtet. Berichtet wird ferner über die pathologisch-anatomischen Grundlagen des Symptoms, wobei ein Vergleich zwischen den Alterationen an den Knochen und den röntgenologischen Veränderungen vorgenommen wird. Es wird dabei hervorgehoben, daß die Röntgenaufnahme die anatomischen Verhältnisse meist richtig wiedergibt, daß aber die charakteristische Streifung im Röntgenbild nicht dem anatomischen Substrat entspricht, sondern daß sie durch einen röntgenologischen Überlagerungseffekt erzeugt wird. In Wirklichkeit bestehen die Knochenveränderungen aus zahlreichen, parallel verlaufenden dünnen Röhrchen, in denen das Knochenmark enthalten ist. Es wird auch gezeigt, daß die Alterationen an den Schädelknochen der Spongiosaschicht angehören und daß dabei periostale Auflagerungen keine wesentliche Rolle spielen. Beginn, Entwicklung und Verlauf des “Bürstensymptoms” werden endlich besprochen und auf die Bedeutung der feinen Röhrchen aus Knochensubstanz, die die Grundlage des “Bürstensymptoms” bilden, für die vermehrte Aufnahme und Stützung des blutbildenden Gewebes in den Schädelknochen wird hingewiesen.