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Biomedical subjects

D Claus

Publications and source records attributed to D Claus.

222 records · Page 13Linked to original sources

[Initial diagnosis of anorectal malformations].

An essential prerequisite for the correct surgical approach of the congenital anorectal anomalies is a precise diagnostic work-up demonstrating the level of the atresia and the nature of possibly associated anomalies. To be acquainted with basic embryology allows a better understanding of this pathology. In this study the value of clinical and radiological parameters has been analysed in sixty cases. An accurate diagnosis was established in one half of the patients only by clinical examination. In the other half, the X-ray with the child inverted according to Wangensteen and Rice proved to be frequently unreliable in contrast with opacification technics. In boys, retrograde urethrography (personal modification) demonstrated the fistula and its location in two-thirds of the cases with a doubtful clinical examination; similarly vaginal and rectal opacification led to a correct diagnosis in one half of the girls. During these investigations with a contrast medium, attention should be focused on the puborectalis sling in contraction and relaxation and its relationship with the terminal part of the bowel. In the remaining cases a colostomy or dilatation of the fistula during the neonatal period allowed the deferral of the definitive diagnosis.

Anus, Imperforate↗

[Anomalies of the urogenital system associated with anorectal malformations].

Urinary anomalies associated with anorectal malformations were found in more than one half of 61 consecutive cases treated during the last ten years. This well known high frequency stresses the necessity of systematic investigations in order to diagnose these associated anomalies early in the life. The high incidence of associated anomalies (40%) found in the group of translevator deformities makes careful clinical examination and complete radiological investigation mandatory in all cases. Vesico-ureteral reflux (14 cases), renal agenesis (6 cases), multicystic kidneys (2 cases), urogenital sinus (3 cases) and hypospadias (6 cases) were the most significant findings. A normal aspect of renal ultrasonography at birth should allow to postpone intravenous pyelography which can be tricky in the neonatal period.

Anus, Imperforate↗

[Diaphragmatic eventration and paralysis in children].

Diaphragmatic relaxation is either congenital, due to muscular dysplasia ("eventration"), or acquired and related to phrenic nerve lesion ("paralysis"); phrenic nerve paralysis can be a complication of a difficult delivery (breech presentation or forceps) or of a surgical--mostly cardiac--procedure. The authors review their own experience (12 eventrations and 9 paralysis) and recall the pathophysiology, the symptoms and treatment of this condition. They recommend the surgical plication, as most experienced authors do, when the symptoms are not completely alleviated by medical treatment which should be of short duration and must include intubation and assisted ventilation in case of acute respiratory distress.

Diaphragm↗

Fibrillations in regenerating muscle in dystrophic myopathies.

In the presented study we attempted to demonstrate a correlation between muscle regeneration and fibrillations in electromyography in dystrophic myopathies. Especially in Emery-Dreifuss muscular dystrophy there is much abnormal spontaneous activity, and NCAM (neural cell adhesion molecule)and cytoskeletal protein vimentin expressing myocytes are predominantly seen. Therefore, definitely regenerating fibers are identified apart from only a few remnants of previous necrosis. Moreover, in the other biopsies of dystrophic myopathies there are also scattered and clustered NCAM and vimentin expressing regenerating myofibers. Here, regressive fiber changes, like necrosis, are more prominent. Furthermore, most regenerating fibers show pseudo-cholinesterase activity indicating innervation. Interestingly, motor end-plate changes in regeneration and in disuse atrophy are very similar. They predominantly consist of terminal sprouting and pseudo-cholinesterase spread. However, in disuse atrophy there is no abnormal spontaneous spread in electromyography. Therefore, in regenerating muscle not innervation, but regeneration itself is likely to be the cause of fibrillations. In conclusion, a correlation is evident between regenerating muscle and fibrillations in electromyography.

Adolescent↗

Cardiac autonomic involvement and peripheral nerve function in patients with diabetic neuropathy.

The aim of our study was to investigate the relationship between cardiac autonomic neuropathy and dysfunction of myelinated and unmyelinated nerve fibres in the peripheral nerve. We measured nerve conduction velocities, warmth/cold perception thresholds at the foot dorsum, sympathetic skin response (SSR), and performed the quantitative sudomotor axon reflex test (QSART). Forty-three diabetic patients with distal-symmetric polyneuropathy were included. According to the results of heart rate variation, 20 patients had cardiac autonomic neuropathy (CAN+). Apart from motor nerve conduction velocities, all tests were more often abnormal in CAN+ patients. Warmth thresholds (afferent C-fibres) and reduced compound muscle action potentials (CMAPs) of the tibial and peroneal nerve, indicating axonal damage, were more often abnormal in CAN+. Cold threshold and sural nerve conduction velocity were indicators of involvement of myelinated small and large nerve fibres, but not of the cardiac autonomic system. Ninety-four percent (94%) of patients with absent SSR and 78% of patients with abnormal QSART had CAN+. SSR and QSART may be useful for assessment of autonomic neuropathy in diabetic patients with cardiac arrhythmia where direct measurement of heart rate variability is not possible. In the majority of our patients with CAN+, the vagal-cardiac and the sudomotor-sympathetic systems were involved simultaneously, although two entirely different systems were tested. This may reflect a C-fibre directed selectivity of the pathological process in autonomic diabetic neuropathy. In conclusion our results show that diabetics with and without cardiac autonomic neuropathy have a different profile of involvement of peripheral nerve fibres.

Adult↗