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Biomedical subjects

B Freigang

Publications and source records attributed to B Freigang.

31 records · Page 2Linked to original sources

Dandy-Walker syndrome: analysis of 21 cases.

During the years 1950 to 1978, a total of 21 cases of Dandy-Walker syndrome were seen at the Vancouver General Hospital. Apart from hydrocephalus, the associated brain anomalies included agenesis of the corpus callosum in four cases, occipital meningocele in two and aqueductal stenosis in one patient. Systemic malformations were present in four patients and included two cases of cleft palate, one of polycystic kidneys and one of congenital rubella syndrome. The over-all mortality was 48 per cent, but has declined since 1965. Of the 12 cases treated surgically, only four have died. The number of shunt revisions was high (about two per patient). Of the 11 survivors, three have normal intelligence, four show mild mental retardation, and four are moderately to severely retarded. The differential diagnosis, clinical course and surgical therapy are discussed. It is recommended that double shunting of a lateral ventricle and of the enlarged fourth ventricle should be the primary procedure in cases associated with aqueductal stenosis or occlusion, and should be the secondary procedure in patients who exhibit recurrence of increased pressure in the posterior fossa after simple shunting of a lateral ventricle.

Cerebrospinal Fluid Shunts↗

Long-term follow-up of infants and children treated with beclomethasone aerosol by a special inhalation device.

A special inhalation device is described which enables young asthmatic children to inhale aerosols. Eighty children who were either too young or were otherwise handicapped to learn to inhale standard aerosols effectively took part in this study. The study period extended from three months to 41 months of treatment in patients ranging in age from 12 months to 15 years. Beclomethasone dipropionate aerosol (BDA) in doses up to 400 microgram/day were utilized. All children were observed to have an excellent response. There were no side effects such as moniliasis or growth failure. It was felt that age was no contraindication to the use of BDA and that better utilization of the aerosol was obtained by the use of the specal inhalation device.

Administration, Intranasal↗

[Problems of the electric response audiometry (ERA) during the natural and artificial sleep (author's transl)].

The investigators carried out threshold determinations on 16 children and 6 adults in wakefulness, under general anaesthesia (we used chloral hydrate anaesthesia) and in sleep (stage II-III and stage REM). Falling asleep (stage I and initial stage of anaesthesia respectively) the latencies of the individual components of the acoustically evoked potentials are prolonged in mean of 30 msec. Simultaneously the amplitude of N1 significantly decreases and N2 becomes a prominent point (Fig. 1). The generation mechanisms of wave N2 are obviously different from those of wave N1. Its input-output curve takes a very steep course (Fig. 5) and the shortening of latencies increases with growing intensity of stimulus too (Fig. 4). Amplitude histogrammes demonstrated the dependency of the form of the acoustically evoked potential on the degree of synchronisation of EEG activity. While in the case of desynchronisation N1 appears more markedly, N2 does in the case of synchronisation. The mean deviation of the ERA threshold totals plus 3.8 +/- 6.9 dB (n = 41) under chloral hydrate anaesthesia, plus 4.9 +/- 6.7 dB (n = 37) in natural sleep in contrast to the wakefulness. With a 99% confidence there occur confidence intervals ranging from + 1 to + 7 dB and from +2 to +8 dB respectively. In identifying the threshold potentials error I (existing potential not recognized) occurred in 15-20%, error II (random wave seen as potential) in 20% of these studies. All these experiments showed significant lower variances for the latencies compared with variancies of amplitudes. The variance of amplitudes is smallest in children (Table 1) under general anaesthesia as well as in adults in wakefulness (Table 2). For the practical performance of ERA chloral hydrate is recommended for studies on children. A uniform EEG-state as well as a uniform depth of sleep are basic conditions for ERA during sleep, sedation or under anaesthesia. These conditions must constantly be controlled by EEG, EOG and EMG.

Adult↗

[Postoperative haemorrhagia in a girl with congenital factor XI deficiency - successful treatment with desmopressin (DDAVP, Minirin(R))].

UNLABELLED: The rare factor XI deficiency is associated with different profuse bleeding without correlation to the severity of reduction of factor XI. Accordingly, traumata or surgical procedures may cause unexpected excessive bleeding in asymptomatic patients. After surgery of a nine-year-old girl with factor XI deficiency (8 per cent) profuse bleeding occurred which could only be stopped after infusion of desmopressin. After administration the factor XI activity was increased to 31 per cent, the factor VIII even to 290 per cent over the normal range. We suppose that the favorable clinical effectiveness is not only related to the increasing factor XI activity but also to the elevation of the factor VIII/von-Willebrand-complex. CONCLUSION: It is recommended to give desmopressin as firstline therapy of bleeding by factor XI deficiency since the only effective alternative such as substitution of factor XI by transfusion of fresh frozen plasma is associated with the risk of transmission of virus infections.

Child↗

Speech understanding with the CIS and the n-of-m strategy in the MED-EL COMBI 40+ system.

Speech tests have been performed on 6 subjects for comparing the standard 12-channel continuous interleaved sampling (CIS) strategy (CIS12), the 7-channel CIS strategy (CIS7) and the 7-of-12 strategy in the MED-EL COMBI 40+ system. An ABAB experimental design was used whereby each strategy was reversed and replicated. Speech tests were performed in quiet (vowels, consonants, monosyllables, sentences) and noise (sentences). Results showed that for vowels, CIS12 is significantly superior to CIS7, for consonants and sentences CIS12, CIS7 and 7-of-12 performed equally well, and that for monosyllables 7-of-12 is significantly superior to both CIS12 and CIS7. In addition, 7-of-12 is superior to CIS7 by almost the same amount as CIS12, but in this case the difference is not significant. Further, all strategies have been found to be equally robust in noise with respect to sentence understanding. The differences between CIS12 and 7-of-12 on the one hand and CIS7 on the other hand may be attributed to decreased spectral resolution of the latter. The fact that - in contrast to what has been reported for the SPEAK strategy - 7-of-12 is equally robust in noise as the CIS strategies is explained by the use of higher stimulation rates, wider frequency bands and a higher percentage of channels stimulated in each cycle.

Adult↗

Comparison of the TEMPO+ ear-level speech processor and the cis pro+ body-worn processor in adult MED-EL cochlear implant users.

A study was conducted to compare the new MED-EL TEMPO+ ear-level speech processor with the CIS PRO+ body-worn processor in the COMBI 40/COMBI 40+ implant system. Speech tests were performed in 46 experienced subjects in two test sessions approximately 4 weeks apart. Subjects were switched over from the CIS PRO+ to the TEMPO+ in the first session and used only the TEMPO+ in the time between the two sessions. Speech tests included monosyllabic word tests and sentence tests via the telephone. An adaptive noise method was used to adjust each subject's scores to approximately 50%. Additionally, subjects had to complete a questionnaire based on their 4 weeks of experience with the TEMPO+. The speech test results showed a statistically significant improvement in the monosyllabic word scores with the TEMPO+. In addition, in the second session, subjects showed a significant improvement when using the telephone with the TEMPO+, indicating some learning in this task. In the questionnaire, the vast majority of subjects found that the TEMPO+ allows equal or better speech understanding and rated the sound quality of the TEMPO+ higher. All these objective and subjective results indicate the superiority of the TEMPO+ and are mainly attributed to a new coding strategy called CIS+ and its implementation in the TEMPO+. In other words, based on the results of this study, it appears that after switching over from the CIS PRO+ to the TEMPO+, subjects are able to maintain or even improve their own speech understanding capability.

Adult↗