The Exstrophy-epispadias complex

Exstrophy-epispadias complex (EEC) represents a spectrum of genitourinary malformations ranging in severity from epispadias (E) to classical bladder exstrophy (CEB) and exstrophy of the cloaca (EC). Depending on severity, EEC may involve the urinary system, musculoskeletal system, pelvis, pelvic floor, abdominal wall, genitalia, and sometimes the spine and anus. Prevalence at birth for the whole spectrum is reported at 1/10,000, ranging from 1/30,000 for CEB to 1/200,000 for EC, with an overall greater proportion of affected males. EEC is characterized by a visible defect of the lower abdominal wall, either with an evaginated bladder plate (CEB), or with an open urethral plate in males or a cleft in females (E). In CE, two exstrophied hemibladders, as well as omphalocele, an imperforate anus and spinal defects, can be seen after birth. EEC results from mechanical disruption or enlargement of the cloacal membrane; the timing of the rupture determines the severity of the malformation. The underlying cause remains unknown: both genetic and environmental factors are likely to play a role in the etiology of EEC. Diagnosis at birth is made on the basis of the clinical presentation but EEC may be detected prenatally by ultrasound from repeated non-visualization of a normally filled fetal bladder. Counseling should be provided to parents but, due to a favorable outcome, termination of the pregnancy is no longer recommended. Management is primarily surgical, with the main aims of obtaining secure abdominal wall closure, achieving urinary continence with preservation of renal function, and, finally, adequate cosmetic and functional genital reconstruction. Several methods for bladder reconstruction with creation of an outlet resistance during the newborn period are favored worldwide. Removal of the bladder template with complete urinary diversion to a rectal reservoir can be an alternative. After reconstructive surgery of the bladder, continence rates of about 80% are expected during childhood. Additional surgery might be needed to optimize bladder storage and emptying function. In cases of final reconstruction failure, urinary diversion should be undertaken. In puberty, genital and reproductive function are important issues. Psychosocial and psychosexual outcome depend on long-term multidisciplinary care to facilitate an adequate quality of life

Mechanisms and in vivo functions of contact inhibition of locomotion

Contact inhibition of locomotion (CIL) is a process whereby a cell ceases motility or changes its trajectory upon collision with another cell. CIL was initially characterized more than half a century ago and became a widely studied model system to understand how cells migrate and dynamically interact. Although CIL fell from interest for several decades, the scientific community has recently rediscovered this process. We are now beginning to understand the precise steps of this complex behaviour and to elucidate its regulatory components, including receptors, polarity proteins and cytoskeletal elements. Furthermore, this process is no longer just in vitro phenomenology; we now know from several different in vivo models that CIL is essential for embryogenesis and in governing behaviours such as cell dispersion, boundary formation and collective cell migration. In addition, changes in CIL responses have been associated with other physiological processes, such as cancer cell dissemination during metastasis

Developing the Questionnaire

AbstractThis chapter outlines the essential topics for developing and testing a questionnaire for a discrete choice experiment survey. It addresses issues such as the description of the environmental good, pretesting of the survey, incentive compatibility, consequentiality or mitigation of hypothetical bias. For the latter, cheap talk scripts, opt-out reminders or an oath script are discussed. Moreover, the use of instructional choice sets, the identification of protest responses and strategic bidders are considered. Finally, issues related to the payment vehicle and the cost vector design are the subject of this section

VARIABILITY OF MOTOR UNIT RECRUITMENT THRESHOLD IN MAN

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DIFFERENTIAL ACTIVATION OF MOTOR UNITS IN THE WRIST EXTENSOR MUSCLES DURING THE TONIC VIBRATION REFLEX IN MAN

International audience1. Single motor unit activity was recorded in the extensor carpi radialis longus and extensor carpi radialis brevis muscles of five healthy human subjects, using metal microelectrodes. 2. Motor units were characterized on the basis of their twitch contraction times and their force recruitment thresholds during voluntary imposed-ramp contractions. 3. The discharge patterns of forty-three motor units were studied during tonic vibration reflex elicited by prolonged (150 s) trains of vibration (30 Hz) applied to the distal tendons of the muscles. The temporal relationships between the individual small tendon taps of the vibratory stimulus and the motor unit impulses were analysed on dot raster displays and post-stimulus time histograms. 4. After tendon taps, the impulses of motor units with long twitch contraction times (mean +/- S.D., 47.2 +/- 10.7 ms) and low recruitment thresholds (0.88 +/- 0.6 N) formed a single narrow peak (P1) with a latency (22.7 +/- 1.4 ms) which was comparable to that of the tendon jerk in the extensor carpi radialis muscles. These motor units were named `P1 units'. On the other hand, the response of motor units with shorter twitch contraction times (31.1 +/- 3.3 ms) and higher recruitment thresholds (3.21 +/- 1.3 N) showed two peaks: a short latency (23.4 +/- 1.3 ms) P1 peak similar to the previous one and a P2 peak occurring 9.4 +/- 1.2 ms later. These motor units were named `P1-P2 units'. 5. When the reflex contraction increased slowly, the P1 peaks of `P1-P2 units' were clearly predominant at the beginning of the contraction, during the rising phase of the motor unit discharge frequency, while the P2 peaks became predominant when the units had reached their maximal discharge frequency. 6. Increasing the tendon vibration frequency (35, 55, 75, 95 Hz) did not modify the `P1 unit' discharge pattern. Due to interference between vibration period and peak latencies, increasing the vibration frequency caused the P1 and P2 peaks of `P1-P2 units' to overlap. 7. Superficial cutaneous stimulation of the dorsal side of the forearm during tendon vibration noticeably decreased the P1 peaks in both types of motor units. In the P2 peaks it could result in either a decrease or an increase but the average effect was a slight increase. 8. When applied 10 s before tendon vibration, cutaneous stimulation considerably suppressed the tonic vibration reflex. 9. The nature of the pathways mediating the P1 and P2 peaks and the effects of cutaneous stimulation are discussed in relation to data obtained from both animal and human experiments. The hypothesis is put forward that during the tonic vibration reflex, the slow and fast motor units are differentially activated on the basis of either a monosynaptic reflex process or mono- and polysynaptic processes, respectively. It is suggested that the monosynaptic pathway plays a major role in tonic vibration reflex initiation at the onset of the tendon vibration and plays a significant role during a maintained reflex contraction. This is contrary to what was previously thought to be the case

COMPARISON OF FLUCTUATIONS OF MOTOR UNIT RECRUITMENT AND DE-RECRUITMENT THRESHOLDS IN MAN

International audienceRecruitment and de-recruitment thresholds of motor units in the wrist extensor muscles can undergo important random fluctuations, even when they are measured during stereotyped contractions and relaxations. These fluctuations were statistically quantified and compared. The statistical analysis indicated that recruitment and de-recruitment thresholds display the same kind of fluctuations, and that the successive measurements are randomly distributed following a quasi-normal law. We suggest that the notion of force threshold for motor unit recruitment and de-recruitment might be oversimplified and that a motor unit seems to have a range of force in which it can be recruited or de-recruited. Comparison of the mean values of recruitment and de-recruitment thresholds of the motor units in the extensor carpi radialis muscles showed that de-recruitment thresholds were significantly lower than recruitment thresholds. This difference in the thresholds, together with the difference in the motor unit discharge frequency during a contraction and a relaxation, suggests a differential control of the motoneurone activity during contractions and relaxations

EFFECTS OF TONIC VIBRATION REFLEX ON MOTOR UNIT RECRUITMENT IN HUMAN WRIST EXTENSOR MUSCLES

International audienceTonic vibration reflex was used to investigate the effects of muscle spindle la afferent activation on motor unit (MU) recruitment in human wrist extensor muscles. The MU force recruitment threshold recorded in the extensor carpi radialis muscles were quantitatively compared under two experimental situations: (1) during tonic isometric reflex contractions induced by mechanical tendon vibration and during voluntary contractions performed at the same velocity; (2) during two voluntary imposed ramp contractions (0.25 N . s-1) performed the one immediately before, and the other immediately after a tonic vibration reflex. In the first situation, it was observed that the la afferents activated by tendon vibration exerted a strong homonymous facilitatory action on their bearing muscles (extensor carpi radialis longus and brevis), while their heteronymous action on the synergistic muscle (extensor carpi ulnaris) was very weak. The MU recruitment thresholds in the extensor carpi radialis muscles were therefore significantly lower during the tonic reflex contraction than during the voluntary contraction. In the second situation, the tonic vibration reflex induced a facilitatory after-effect which decreased the MU recruitment thresholds during the subsequent voluntary imposed ramp contraction. It is suggested that this post-vibratory effect may have been due either to a postsynaptic potentiation of the motoneurones or to a reflex sensitization of the muscle spindles increasing their response to voluntary isometric contraction and consequently, increasing their facilitatory reflex action on the motoneurone pool

FLUCTUATIONS IN MOTOR UNIT RECRUITMENT THRESHOLD DURING SLOW ISOMETRIC CONTRACTIONS OF WRIST EXTENSOR MUSCLES IN MAN

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