Biomedical subjects

C Braun

Publications and source records attributed to C Braun.

At least 73 records · Page 4Linked to original sources

PubMed1998

PROMM: the expanding phenotype. A family with proximal myopathy, myotonia and deafness.

We describe a family with a proximal myopathy, subclinical EMG myotonia, cataracts and deafness. Transmission through two generations and down the male line confirms autosomal dominant inheritance. There was no abnormal expansion of the CTG triplet repeat in the last exon of the dystrophia myotonica protein kinase (DMPK) gene associated with myotonic dystrophy. Heteroduplex analysis of all but the promoter region of the DMPK gene has excluded point mutations in this gene as an underlying cause for this myotonic disorder. The family was not sufficiently informative to exclude linkage to the sodium channel gene SCN4A or the chloride channel gene CLC1. This family clearly fulfils the recently established diagnostic criteria for PROMM (proximal myotonic myopathy) and in addition shows consistent severe deafness as a hitherto undescribed feature of PROMM. We discuss the diagnostic criteria of PROMM in relation to this family and other recent papers, all of which would now fulfil the aforementioned diagnostic criteria for PROMM.

Adult↗

PubMed1998

The cortical somatotopic map and phantom phenomena in subjects with congenital limb atrophy and traumatic amputees with phantom limb pain.

The extent of the cortical somatotopic map and its relationship to phantom phenomena was tested in five subjects with congenital absence of an upper limb, four traumatic amputees with phantom limb pain and five healthy controls. Cortical maps of the first and fifth digit of the intact hand, the lower lip and the first toe (bilaterally) were obtained using neuroelectric source imaging. The subjects with congenital upper limb atrophy showed symmetric positions of the left and right side of the lower lip and the first toe, whereas the traumatic amputees with pain showed a significant shift (about 2.4 cm) of the cortical representation of the lower lip towards the hand region contralateral to the amputation side but no shift for the toe representation. In healthy controls, no significant hemispheric differences between the cortical representation of the digits, lower lip or first toe were found. Phantom phenomena were absent in the congenital but extensive in the traumatic amputees. These data confirm the assumption that congenital absence of a limb does not lead to cortical reorganization or phantom limbs whereas traumatic amputations that are accompanied by phantom limb pain show shifts of the cortical areas adjacent to the amputation zone towards the representation of the deafferented body part.

Adult↗

PubMed1998

Influence of the renal endothelin A system on the autoregulation of renal hemodynamics in SHRs and WKY rats.

In this study, we investigated the influence of a short-term blockade of the renal endothelin A system on the autoregulation of total renal blood flow, cortical renal blood flow, and pressure-dependent plasma renin activity in spontaneously hypertensive rats (SHRs) and normotensive controls [Wistar-Kyoto (WKY) rats]. In anesthetized rats, renal blood flow was measured by a transit-time flow probe and cortical blood flow by a laser flow probe. Blood samples were taken for measurement of plasma renin activity. Renal perfusion pressure was reduced in 5-mm Hg steps by means of a servocontrolled electropneumatic device by an inflatable suprarenal cuff. During the experiments, the rats (n = 6, each group) received an intrarenal infusion of either the selective endothelin A-receptor antagonist BQ123 (3 mg/kg/h) or vehicle. We observed an improvement of total and cortical blood flow autoregulation as indicated by a shift of lower limits of autoregulation to lower threshold pressures [103 +/- 2 vs. 132 +/- 4 mm Hg compared with 98 +/- 3 vs. 120 +/- 4 mm Hg (mean +/- SEM); p < 0.01 resp. p < 0.05] in BQ123-treated SHRs, whereas BQ123 had no influence on breakpoints of autoregulation in WKY rats (p > 0.05). Pressure-dependent plasma renin activity in SHRs was not influenced by BQ123. Renal blood flow autoregulation is improved in SHRs after short-term blockade of the renal endothelin A system. This effect is independent of the renin-angiotensin system. The endothelin A system does not seem to play an important role in the autoregulation of renal blood flow in normotensive WKY rats.

Animals↗

PubMed1998

Improvement of postischemic acute renal failure with the novel orally active endothelin-A receptor antagonist LU 135252 in the rat.

The endothelin (ET) system may play an important role in the pathogenesis of acute renal failure (ARF). We hypothesize that the course of ARF in an ischemia-reperfusion model will be markedly attenuated by the orally active ET(A)-receptor antagonist LU 135252 (LU) because of an improvement of renal perfusion. ARF was induced in rats by clamping both renal arteries for 60 min. The study was divided into two parts. In part 1, Rats received LU orally (100 mg/kg/day) starting 1 h after induction of ARF for 14 days. Cr(s), Cl(cr) and FE(na) were measured on days 1, 6, 9, and 14 after ARF. Cr(s) was lower in the treatment group on days 1 [1.3 +/- 0.31 mg/dl (n = 9) vs. 2.7 +/- 0.46 mg/dl (n = 10); p < 0.05] and 6 [0.5 +/- 0.1 mg/dl (n = 9) vs. 1.0 +/- 0.2 mg/dl (n = 9); p < 0.05], and Cl(cr) was higher on day 1 [0.9 +/- 0.17 ml/min (n = 9) vs. 0.2 +/- 0.1 ml/min (n = 8); p < 0.05] and 6 [1.8 +/- 0.29 ml/min (n = 9) vs. 1.0 +/- 0.21 ml/min (n = 9); p < 0.05] compared with vehicle. Additionally, FE(na) was lower in treated rats on day 1 [1 +/- 0.4% (n = 9) vs. 8 +/- 3% (n = 8); p < 0.051 compared with vehicle. In part 2, ARF was induced as described. Treated animals received 10 mg/kg LU on days 0, 1, 3, 6, 9, and 14 after ARF as an i.v. bolus injection. RBF, cortex blood flow (CBF), and medulla blood flow (MBF) were measured after application of LU on the same days: LU induced an increase in RBF (day 1: 14 +/- 5.3%, n = 6, p = 0.04; day 3: 15 +/- 2.8%, n = 8; p = 0.0008; day 6: 21 +/- 5.8%, n = 6, p = 0.02; day 9: 13 +/- 4%, n = 6; p = 0.03) and CBF (day 1: 8 +/- 2.2%, n = 7, p = 0.03; day 3: 7 +/- 2.5%, n = 7; p = 0.05; day 6: 18 +/- 4.8%, n = 6, p = 0.04; day 9: 10 +/- 2.5%, n = 6; p = 0.008) up to the first 9 days. MBF did increase on days 1 (9 +/- 3.1%, n = 6; p = 0.04) and 6 (13 +/- 3.6%, n = 6; p = 0.03). Our data confirm the hypothesis that ET plays a major role in the genesis of ARF associated with ischemia-reperfusion.

Acute Kidney Injury↗

PubMed1998

Adaptive AR modeling of nonstationary time series by means of Kalman filtering.

An adaptive on-line procedure is presented for autoregressive (AR) modeling of nonstationary multivariate time series by means of Kalman filtering. The parameters of the estimated time-varying model can be used to calculate instantaneous measures of linear dependence. The usefulness of the procedures in the analysis of physiological signals is discussed in two examples: First, in the analysis of respiratory movement, heart rate fluctuation, and blood pressure, and second, in the analysis of multichannel electroencephalogram (EEG) signals. It was shown for the first time that in intact animals the transition from a normoxic to a hypoxic state requires tremendous short-term readjustment of the autonomic cardiac-respiratory control. An application with experimental EEG data supported observations that the development of coherences among cell assemblies of the brain is a basic element of associative learning or conditioning.

Adaptation, Physiological↗

PubMed1998

Comparative studies of diagnostic bacteriological methods for the recovery of Salmonella from faecal samples from flocks of layers.

Several conventional culture procedures were compared for the recovery of Salmonella from faecal samples of layer flocks. The cultural media employed consisted of Rappaport-Vassiliadis and tetrathionate broth for selective enrichment and three different plating media (Brilliant Green, Xylose-Lysine-Desoxycholate and modified semisolid Rappaport-Vassiliadis medium). The initial enrichment and plating procedures were repeated in a four-stage secondary enrichment. Selective enrichment in Rappaport-Vassiliadis or tetrathionate broth followed by plating on modified semisolid Rappaport-Vassiliadis medium (selective motility enrichment) resulted in a significantly higher isolation rate for Salmonella from faecal samples of layers compared to Brilliant Green and Xylose-Lysine-Desoxycholate medium. The selective enrichment yielded additional Salmonella isolates up to the second stage. Further enrichment did not increase the isolation rate.

Animals↗

PubMed1998

Preserved autonomic modulation of the sinus and atrioventricular nodes following posteroseptal ablation for treatment of atrioventricular nodal reentrant tachycardia.

INTRODUCTION: Following radiofrequency catheter ablation of AV nodal reentrant tachycardia (AVNRT), inappropriate sinus tachycardia may occur, possibly due to damage to autonomic cardiac nerve fibers. Furthermore, inducibility of AVNRT is often critically dependent on the autonomic balance. We investigated whether successful ablation of AVNRT is associated with an alteration of autonomic input to the sinus and AV nodes. METHODS AND RESULTS: To estimate changes in the autonomic modulation of the sinus and AV nodes, power spectra of beat-to-beat PP and PR intervals were analyzed from high-quality nighttime ECG recordings of 11 patients before and after radiofrequency application. Normalized HF power (nHF) of PP and PR intervals was used as an index of efferent vagal modulation and the LF/HF ratio as an index of sympathovagal balance of the sinus node (PP) and AV node (PR). Before ablation, LF/HF(PP) was 3.2 and nHF(PP) was 0.3 in the sinus node. For the AV node, LF/HF(PR) was 1.2 and nHF(PR) was 0.5. Following ablation, LF/HF(PP) (3.5) and nHF(PP) (0.3) of the PP intervals did not change. Similarly to the sinus node, there were no changes in the autonomic modulation of the AV node, as both LF/HF(PR) (1.2) and nHF(PR) (0.5) remained unchanged. CONCLUSION: Our results indicate that autonomic control of the sinus and AV nodes is preserved following successful radiofrequency ablation of AVNRT. The effects of posteroseptal radiofrequency current application are not necessarily mediated by changes in the autonomic input to the AV node.

Adult↗

PubMed1998

Demonstration of nonlinear components in heart rate variability of healthy persons.

We present a systematic approach for detecting nonlinear components in heart rate variability (HRV). The analysis is based on twenty-three 48-h Holter recordings in healthy persons during sinus rhythm. Although many segments of 1,024 R-R intervals are stationary, only few stationary segments of 8,192-32,768 R-R intervals can be found using a test of Isliker and Kurths (Int. J. Bifurcation Chaos 3:1573-1579, 1993.). By comparing the correlation integrals from these segments and corresponding surrogate data sets, we reject the null hypothesis that these time series are realization of linear processes. On the basis of a test statistic exploring the differences of consecutive R-R intervals, we reject the hypothesis that the R-R intervals represent a static transformation of a linear process using optimized surrogate data. Furthermore, time irreversibility of the heartbeat data is demonstrated. We interpret these results as a strong evidence for nonlinear components in HRV. Thus R-R intervals from healthy persons contain more information than can be extracted by linear analysis in the time and frequency domain.

Adult↗

PubMed1998

Automation, task difficulty, and aircrew performance.

The effects of an automated system on team processes and performance were assessed in a laboratory simulation. Two-person crews were required to fly a complex emergency-response scenario under conditions of low and high workload. These flights were completed with or without the aid of an autopilot. The results indicated that the autopilot was effective in reducing subjective workload. However, the automation was associated with improved performance on only 1 of 4 performance measures. Furthermore, it was observed that problem-solving performance was worse in the autopilot condition during the high-workload flights. Investigation of crew process data indicated that workload savings afforded by the autopilot might have been invested in more explicit coordination. The results are discussed in terms of their implications for military aviators' performance, system design, and team training.

Adult↗

PubMed1998

Renal endothelin system in polycystic kidney disease.

Polycystic kidney disease (PKD) is characterized by interstitial fibrosis and formation of renal cysts. Interestingly, interstitial fibrosis and renal cyst formation were also seen in human endothelin-1 (ET-1) transgenic mice. This study, therefore, analyzes the tissue distribution of ET-1, the tissue concentrations of ET-1, as well as the expression of ET receptor subtypes in the kidneys of a rat model of PKD: Han:SPRD rats. Six-week-old heterozygous (cy/+) and homozygous (cy/cy), as well as 6-mo-old heterozygous (cy/+) Han:SPRD rats and the corresponding age-matched Sprague Dawley littermates (SD) (+/+) were analyzed. Furthermore, the acute effects of the mixed (A/B) endothelin receptor antagonist bosentan on hemodynamic and renal function were investigated in 6-mo-old, conscious, chronically instrumented (cy/+) rats. The kidneys of affected rats showed significantly elevated tissue levels of ET-1 compared with age-matched controls (3.5 +/- 0.3-fold in young cy/cy rats, P < 0.01; 1.4 +/- 0.2-fold in young cy/+ rats, P < 0.01; 6.2 +/- 0.4-fold in old cy/+ rats, P < 0.001) due to a highly increased ET-1 synthesis within the epithelial cells of the cysts. Analyzing tissue sections from patients with typical autosomal dominant PKD demonstrated a high ET-1 expression within the epithelial cells of the cysts as well. Scatchard analysis revealed a markedly decreased ETA and ETB receptor density in all groups of affected rats. The acute blockade of both endothelin receptor subtypes using bosentan in 6-mo-old heterozygous PKD rats led to a significant decrease in mean arterial BP (MAP) (-19.7 +/- 2.1 mmHg, P < 0.05) and GFR (-41 +/- 5%, P < 0.005). Renal blood flow (RBF) was significantly increased (+2.1 +/- 0.5 ml/min, P < 0.05) after bosentan, whereas bosentan had no effect on MAP, GFR, and RBF in age-matched controls. These data show that the paracrine renal endothelin system is activated in PKD and participates in the regulation of MAP, GFR, RBF, and possibly contributes to renal cyst formation and fibrosis.

Analysis of Variance↗

PubMed1998

[Comparative stability studies with the modified Stellbrink external fixator].

We replaced original Stellbrink-fixator clamps with adjustable horizontal and vertical clamps. Thus, smaller bone fragments can be fixed and there is a possibility to correct their position after application of the fixator. This study compared the primary stability of different constructions by defined flexibility loadings in four-point bending. Statistical evaluation was done by the student-t-test. When used as compression osteosynthesis, the original Stellbrink fixator is the most stable (100%), whereas the modified fixator reaches only 90% of the stability of the original. The better stability of the original Stellbrink fixator only becomes significant when the flexion becomes greater than 13 degrees. Used for distance osteosynthesis, the original Stellbrink fixator is significantly more stable. Clinical examples are presented.

Biomechanical Phenomena↗

PubMed1997

Human lysosomal and jack bean alpha-mannosidases are retaining glycosidases.

The stereochemical course of the hydrolyses catalysed by two alpha-mannosidases has been determined directly by 1H NMR. Synthetic substrates were incubated with the enzymes and the anomeric configuration of the initially formed product was ascertained in each case by observation of the chemical shift of the anomeric proton at the hemiacetal centre. Both mannosidases were found to catalyse hydrolysis with retention of stereochemistry at the anomeric position. Human lysosomal alpha-mannosidase (a class II mannosidase) is a member of the glycosidase family 38 and thus has sequence similarity with several alpha-mannosidases responsible for glycoprotein biosynthesis. Jack bean alpha-mannosidase was shown to be mechanistically similar to the lysosomal enzyme and will provide a useful model system in mechanistic studies and inhibitor design.

Fabaceae↗

PubMed1997

Hepatitis G virus RNA and hepatitis G virus antibodies in renal transplant recipients: prevalence and risk factors.

BACKGROUND: Hepatitis G virus (HGV/GBV-C) RNA indicating current infection has been frequently isolated from the sera of transplant recipients and other multitransfused individuals. Lifetime exposure to the virus, however, is unknown. We carried out a study to determine the prevalence and risk factors of HGV antibodies and of HGV RNA among renal transplant recipients, and to investigate possible associations between HGV RNA and immunosuppressive treatment. METHODS: HGV RNA was detected by reverse transcriptase-polymerase chain reaction, and HGV antibodies (anti-E2) by a newly developed immunoassay. To assess risk factors for HGV exposure, univariate and multivariate analysis was performed. RESULTS: Of the 221 patients, 14% were HGV RNA positive and 40% had HGV antibodies. Both HGV RNA and anti-HGV were present in only two individuals. Thus, the overall HGV exposure prevalence was 53%. It increased significantly with the number of blood transfusions. In logistic regression, the adjusted HGV exposure prevalence odds ratio was 5.7 (95% confidence interval [CI]: 2.2-15) among patients with > or =10 transfusions (baseline: no transfusions). Other independent risk factors were a longer duration of hemodialysis and a longer time interval since transplantation. HGV viremia was not associated with the type of immunosuppressive treatment. Alanine aminotransferase levels were not significantly increased among HGV RNA-positive patients. CONCLUSIONS: Much higher proportions of renal transplant recipients were exposed to HGV than is suggested by HGV RNA detection alone. The majority of infected individuals apparently eliminate the virus over time. Contaminated blood transfusions have to be regarded as a main risk factor for HGV infection.

Adolescent↗

PubMed1997

Facilitation of membrane fusion during exocytosis and exocytosis-coupled endocytosis and acceleration of "ghost" detachment in Paramecium by extracellular calcium. A quenched-flow/freeze-fracture analysis.

We had previously shown that an influx of extracellular Ca2+ (Ca2+e), though it occurs, is not strictly required for aminoethyldextran (AED)-triggered exocytotic membrane fusion in Paramecium. We now analyze, by quenched-flow/freeze-fracture, to what extent Ca2+e contributes to exocytotic and exocytosis-coupled endocytotic membrane fusion, as well as to detachment of "ghosts"-a process difficult to analyze by any other method or in any other system. Maximal exocytotic membrane fusion (analyzed within 80 msec) occurs readily in the presence of [Ca2+]e > or = 5 x 10(-6) M, while normally a [Ca2+]e = 0.5 mM is in the medium. A new finding is that exocytosis and endocytosis is significantly stimulated by increasing [Ca2+]e even beyond levels usually available to cells. Quenching of [Ca2+]e by EGTA application to levels of resting [Ca2+]i or slightly below does reduce (by approximately 50%) but not block AED-triggered exocytosis (again tested with 80 msec AED application). This effect can be overridden either by increasing stimulation time or by readdition of an excess of Ca2+e. Our data are compatible with the assumption that normally exocytotic membrane fusion will include a step of rapid Ca(2+)-mobilization from subplasmalemmal pools ("alveolar sacs") and, as a superimposed step, a Ca(2+)-influx, since exocytotic membrane fusion can occur at [Ca2+]e even slightly below resting [Ca2+]i. The other important conclusion is that increasing [Ca2+]e facilitates exocytotic and endocytotic membrane fusion, i.e., membrane resealing. In addition, we show for the first time that increasing [Ca2+]e also drives detachment of "ghosts"-a novel aspect not analyzed so far in any other system. According to our pilot calculations, a flush of Ca2+, orders of magnitude larger than stationary values assumed to drive membrane dynamics, from internal and external sources, drives the different steps of the exo-endocytosis cycle.

Animals↗

PubMed1997

Extensive reorganization of primary somatosensory cortex in chronic back pain patients.

The hypothesis of reorganization of the primary somatosensory cortex in states of chronic pain was assessed in 10 low back pain patients and nine matched healthy controls. Intracutaneous electric stimuli were applied to the left back and index finger at a standard, a non-painful and a painful intensity. Magnetic fields were recorded by a 37-channel BTi biomagnetometer from the hemisphere contralateral to the site of stimulation. The power of the early evoked magnetic field (< 100 ms) elicited by painful stimulation of the painful back in very chronic patients was elevated relative to that elicited by painful back stimulation of healthy controls and showed a linear increase with chronicity (r = 0.74). The maximum activity elicited in primary somatosensory cortex was shifted more medially in the very chronic back pain subjects. These data suggest that chronic pain is accompanied by cortical reorganization and may serve an important function in the persistence of the pain experience.

Adult↗

PubMed1997

Microdomain Ca2+ activation during exocytosis in Paramecium cells. Superposition of local subplasmalemmal calcium store activation by local Ca2+ influx.

In Paramecium tetraurelia, polyamine-triggered exocytosis is accompanied by the activation of Ca2+-activated currents across the cell membrane (Erxleben. C., and H. Plattner. 1994. J. Cell Biol. 127:935-945). We now show by voltage clamp and extracellular recordings that the product of current x time (As) closely parallels the number of exocytotic events. We suggest that Ca2+ mobilization from subplasmalemmal storage compartments, covering almost the entire cell surface, is a key event. In fact, after local stimulation, Ca2+ imaging with high time resolution reveals rapid, transient, local signals even when extracellular Ca2+ is quenched to or below resting intracellular Ca2+ concentration ([Ca2+]e, < or = [Ca2+]i). Under these conditions, quenched-flow/freeze-fracture analysis shows that membrane fusion is only partially inhibited. Increasing [Ca2+], alone, i.e., without secretagogue, causes rapid, strong cortical increase of [Ca2+]i but no exocytosis. In various cells, the ratio of maximal vs. minimal currents registered during maximal stimulation or single exocytotic events, respectively, correlate nicely with the number of Ca stores available. Since no quantal current steps could be observed, this is again compatible with the combined occurrence of Ca2+ mobilization from stores (providing close to threshold Ca2+ levels) and Ca2+ influx from the medium (which per se does not cause exocytosis). This implies that only the combination of Ca2+ flushes, primarily from internal and secondarily from external sources, can produce a signal triggering rapid, local exocytotic responses, as requested for Paramecium defense.

Animals↗

PubMed1997

PMP-22 expression in the central nervous system of the embryonic mouse defines potential transverse segments and longitudinal columns.

PMP-22, a major constituent of peripheral nervous system (PNS) myelin, is also present in the central nervous system (CNS), in motoneurons of the cranial nerve motor nuclei and spinal cord (Parmantier et al. [1995] Eur. J. Neurosci. 7:1080-1088). The expression of PMP-22 in the CNS during embryonic and early postnatal development was investigated and showed a biphasic spatio-temporal pattern. The expression of PMP-22 started at embryonic day (E)11.5, in restricted longitudinal and transverse domains, in the ventricular zone of the spinal cord, rhombencephalon, mesencephalon and prosencephalon. In the mid- and forebrain, the PMP-22 signal was detectable in a longitudinal domain that followed ventrally the basal/alar boundary but could no longer be detected dorsally at some distance from the roof plate. Along the caudo-rostral axis, the territory in which PMP-22 was detected spanned the mesencephalon and the prosencephalon, extending caudally from the limit between the isthmus and the mesencephalon, and rostrally to the boundary between prosomeres 4 and 5 (p4/p5). In agreement with the prosomeric model of forebrain organization proposed by Puelles and Rubenstein ([1993] TINS 16:472-479), differences in the level of PMP-22 expression in p2, p3, and p4 clearly defined the p2/p3 and p3/p4 neuromeric boundaries. By E17.5, PMP-22 was no longer detected in the ventricular zone, but at E18.5 it began to be expressed in motoneurons of cranial nerve motor nuclei and, after birth, following a rostro-caudal gradient, in the ventral spinal cord.

Animals↗

PubMed1997

[Replantation of a leg after hip para-articular amputation].

Of all macroreplantations, reattachment of a leg amputated at the proximal thigh presents the biggest difficulty. A patient with no other injuries was treated in this way. A critical discussion of indications in general, the technique in the acute setting, and reconstructive procedures, including the ilizarov distraction technique, is presented. The outcome after 2.5 years is gratifying. The results obtainable in proximal thigh replantation can justify the risk of potentially lethal general complications.

Amputation, Traumatic↗