Hemoglobin Djelfa beta98 (FG 5) Val leads to Ala: isolation and functional properties of the heme saturated form.

Hemoglobin Djelfa beta98 (FG 5) Val leads to Ala is a neutrally substituted unstable hemoglobin, exhibiting the same gross features as hemoglobin Köln beta98 (FG 5) Val leads to Met. In addition to the presence of a deheminized fraction, a heme saturated abnormal hemoglobin was visualized and isolated by high resolution electrofocusing. By functional studies of the fully heminized form, a slightly increased oxygen affinity, an impairment of heme-heme interaction and a decreased response to organic phosphates were demonstrated. These functional perturbations point out the importance of the beta98 invariant valyl residue, in the quaternary contacts. They can account for the poor oxygen delivery of erythrocytes.

[Computer technology in otospongiosis. 2. Statistics on 15 years of stapedectomy]. / Apport de l'informatique dans l'otospongiose. Deuxième partie. Statistiques sur 15 ans de stapédectomies.

To complete their two previous data processing studies on systematization of parameters for otospongiosis and on statistics concerning the otospongiotic disease itself, the authors now develop the study of postoperative functional results obtained over 15 years stapectomies performed according to various techniques. The characteristic of this study is that the functional results are not considered only from the operative technique point of view, but that they also take into account all the factors having an influence upon the functional results, i.e. mechanical operative factors, otospongiotic cochlear factors and, finally the patient's general factor. This work is divided in three parts. The first part considers statistical data drawn from investigating elements taken over a period ranging from 1960 to 1975 from operative findings, surgical techniques and operative complications, particularly during reoperations, i.e., the mechanical part of hearing improvement in otospongiosis surgery. The second part deals with the problem of the medical postoperative care in stapedial surgery and with that of postoperative complications and the therapy used to combat them. It is the study of the cochlear support problem considering both the cochlear fragility facing the operative trauma, and the cochlear deterioration due to the enzymatic action of the otospongiotic microfoci of the lateral wall of the cochlear duct and of the vestibular side of the footplate. The third part is the statistical study made both by means of data processing and of manual investigation, on the long term functional results obtained, on one hand by the surgical solution of the stapedial fixation's mechanical problem, and on the other, by the medical solution of the cochlear problem considered both from the enzymatic and vascular point of view. This study of stapedectomies' long term functional results allows the authors to draw the conclusions which seem to result logically from this very elaborated statistical study concerning near 17000 stapedectomies performed from 1960 through 1975.

Modulation by alpha-2 macroglobulin of human lymphocyte proliferation in response to mitogens and antigen.

The effect was studied of purified alpha-2 macroglobulin (a potent and polyvalent protease inhibitor) on normal human lymphocytes stimulated by phytohaemagglutinin, pokeweed mitogen, or by purified protein derivative as an antigen. At physiological concentration alpha 2 M has been found to inhibit mitogenic as well as antigenic stimulation. However at lower dilutions alpha 2 M acts in synergy either with the mitogen or with the antigen. At these dilutions thymidine incorporation is enhanced. alpha 2 M has no toxic or mitogenic effect on normal human lymphocytes. These data might indicate that two proteases may play a role in the proliferation of normal human lymphocytes.

Mechanical explanation of the on-off effect (diphasic impedance change) in otospongiosis.

Impedancemetry gives important information in otospongiosis, both for relative compliance and impedance audiometry. The detection of the stapedius reflex is facilitated by recruitment in some audiometric types of otospongiosis and enables us to solve many of the masking problems which are particularly difficult in this disease due to the very important cross-hearing (or cross-over). Diphasic impedance changes (on-off effect) once detected must be systematically investigated since they allow us to detect otospongiosis early on. This sign is absolutely characteristic of the onset of a stapediovestibular involvement and is of primary interest in this disease because of its early appearance - before any classical audiometric change and any air-bone gap, i.e. before deafness. Diphasic impedance changes must thus be systematically investigated in case of so-called unilateral otospongiosis, in the audiometrically normal ear, and for the detection of otospongiosis in otospongiotic families. It is also helpful in confirming the otospongiotic origin of sensorineural losses before an air-bone gap appears. Our experience with routine stapedectomies and impedance audiometry measurements have led us to a purely mechanical explanation of the on-off effect, i.e. the response of an elastic system to a deformation with a different mechanism of the two inverse peaks, with a different mass and thus a different inertia. This mechanical explanation fits perfectly with anatomical findings. Everything occurs as if the evolutive stages of the onset of the stapediovestibular involvement were defined in impedancemetry, starting from the normal stapedius reflex, first by mixed forms, then by a pure on-off effect, finally by the disappearance of the stapedius reflex.

[Contribution of data processing in otospongiosis. Part I. Statistics on otospongiosis]. / Apport de l'informatique dans l'otospongiose. (Première partie). Statistiques sur la maladie otospongieuse

The use of data processing in their otology clinic allowed the authors a better approach to otospongiosis and to its treatment, thus completing the knowledge of the otospongiotic disease according to their enzymatic concept. An important material has been gathered since 1959 to the present (June 1, 1974), based on more than 16 000 stapedectomies and nearly 100 000 otology out-patients. It has been studied both through a conventional method and an informatic one on the computer of their clinic. Statistical data, apparently valuable, could be drawn from this mass of information for each patient, starting from his first consultation and following him through his secondary consultations, operation, immediate postoperative follow-up and various postoperative check-ups performed. It also takes into account the general state of the patient and all the accidents which may happen during a remote postoperative period. First of all, the authors give the data for these statistics established according to the systematization of the parameters for otospongiosis they have previously published in 1973. In the first part, they gather statistical data obtained about the otospongiotic disease itself, some of which differ from the classical notions of frequency or importance. These statistical conclusions confirm some percentages stated by other authors (predominance of the woman and of the white race, severity of the disease in the young, etc.), but differ in relation to the number of deafs, the percentage of unilateral otospongiosis in comparison, to the bilateral ones, the importance of pure cochlear otospongiosis, the separate valuation of audiometric stages and that of anatomic types, the relation between age and anatomic type, finally the respective frequency of pre-and postoperative vertigos. In the second part, which will be presented later, the authors will study the postoperative functional results obtained within a period of 15 years by stapedectomy according to three techniques. But the particularity of this study will be the fact that functional results will not be considered only from the operative point of view, but from the whole of the factors acting upon the functional result, i.e.: operative mechanical factor, otospongiotic cochlear component, finally general state of the patient. We will thus have a better knowledge of this very peculiar disease of the otic capsule, hat is otospongiosis, characterized by its two phases: a first lytic phase provoked by an enzymatic factor, then a second rebuilding phase according to a pseudo haversian process. During the long progression of the disease, these two phases intermingle and juxtapose much more than they follow each other in the course of time.