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排序方式: 共有286条查询结果,搜索用时 31 毫秒
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Clémence Jacquin Emilie Landais Céline Poirsier Alexandra Afenjar Ahmad Akhavi Nathalie Bednarek Caroline Bénech Adeline Bonnard Damien Bosquet Lydie Burglen Patrick Callier Sandra Chantot-Bastaraud Christine Coubes Charles Coutton Bruno Delobel Margaux Descharmes Jean-Michel Dupont Vincent Gatinois Nicolas Gruchy Sarah Guterman Abdelkader Heddar Lucas Herissant Delphine Heron Bertrand Isidor Pauline Jaeger Guillaume Jouret Boris Keren Paul Kuentz Cedric Le Caignec Jonathan Levy Nathalie Lopez Zoe Manssens Dominique Martin-Coignard Isabelle Marey Cyril Mignot Chantal Missirian Céline Pebrel-Richard Lucile Pinson Jacques Puechberty Sylvia Redon Damien Sanlaville Marta Spodenkiewicz Anne-Claude Tabet Alain Verloes Gaelle Vieville Catherine Yardin François Vialard Martine Doco-Fenzy 《American journal of medical genetics. Part A》2023,191(2):445-458
Chromosome 1p36 deletion syndrome (1p36DS) is one of the most common terminal deletion syndromes (incidence between 1/5000 and 1/10,000 live births in the American population), due to a heterozygous deletion of part of the short arm of chromosome 1. The 1p36DS is characterized by typical craniofacial features, developmental delay/intellectual disability, hypotonia, epilepsy, cardiomyopathy/congenital heart defect, brain abnormalities, hearing loss, eyes/vision problem, and short stature. The aim of our study was to (1) evaluate the incidence of the 1p36DS in the French population compared to 22q11.2 deletion syndrome and trisomy 21; (2) review the postnatal phenotype related to microarray data, compared to previously publish prenatal data. Thanks to a collaboration with the ACLF (Association des Cytogénéticiens de Langue Française), we have collected data of 86 patients constituting, to the best of our knowledge, the second-largest cohort of 1p36DS patients in the literature. We estimated an average of at least 10 cases per year in France. 1p36DS seems to be much less frequent than 22q11.2 deletion syndrome and trisomy 21. Patients presented mainly dysmorphism, microcephaly, developmental delay/intellectual disability, hypotonia, epilepsy, brain malformations, behavioral disorders, cardiomyopathy, or cardiovascular malformations and, pre and/or postnatal growth retardation. Cardiac abnormalities, brain malformations, and epilepsy were more frequent in distal deletions, whereas microcephaly was more common in proximal deletions. Mapping and genotype–phenotype correlation allowed us to identify four critical regions responsible for intellectual disability. This study highlights some phenotypic variability, according to the deletion position, and helps to refine the phenotype of 1p36DS, allowing improved management and follow-up of patients. 相似文献
3.
Bonnard C Anastakis DJ van Melle G Narakas AO 《The Journal of bone and joint surgery. British volume》1999,81(2):212-217
We have assessed the final strength of the deltoid in 121 patients who had repair of isolated or combined lesions of the axillary (circumflex) nerve and were available for statistical analysis. Successful or useful results were achieved in 85% after grafting of isolated lesions. The strength was statistically better when patients had grafting of the axillary nerve within 5.3 months from the time of injury. The dramatic decrease in the rate of success seen with longer delays suggests that surgery should be undertaken within three months of injury. A statistically significant downward trend of the rate of success was noted with increasing age. The force and level of injury to the shoulder play an important role in the type, combination and level of nerve damage and the incidence of associated rotator-cuff, vascular and other injuries to the upper limb. Management of isolated and combined lesions of the axillary nerve after injury to the shoulder needs to be thorough and systematic. 相似文献
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Montalva Louise Carricaburu Elisabeth Levy Michael Bonnard Arnaud 《European Surgery》2022,54(4):207-211
European Surgery - Although gastro-esophageal reflux disease (GERD) is the most frequent long-term morbidity in congenital diaphragmatic hernia (CDH) survivors, no consensual guidelines exist... 相似文献
6.
Vincent Guinard-Samuel Arnaud Bonnard Michel Peuchmaur Dominique Berrebi 《Pediatric surgery international》2014,30(8):803-808
Background/purpose
Calretinin immunohistochemistry is now widely used to diagnose Hirschsprung’s disease (HD), since loss of calretinin expression within the mucosa and muscularis mucosae of rectal suction-biopsy is pathognomonic of HD. However, a stippled staining may be observed within hypertrophic nerves in the submucosae in some HD patients. The aim of the study was to test the hypothesis that such findings may announce the beginning of the transitional zone.Methods
We retrieved 44 consecutive patients (10 girls and 34 boys; median age 6.5 days), diagnosed with aganglionosis on rectal suction biopsies, followed by surgery. According to calretinin immunohistochemistry performed on all paraffin-embedded rectal biopsies, we defined two HD groups: P? showing an absence of any staining within mucosa, muscularis mucosae and submucosa et P+ showing an absence of staining within the mucosa and muscularis mucosae, but a positivity of some submucosal hypertrophic nerves. These data were correlated to the length of total pathological segment (aganglionic and transitional zones) obtained from the original surgery reports.Results
18/44 patients (40.9 %) belonged to the P+ group and 26/44 (59 %) patients were within the P? group. In the P+ group, the maximal length of the aganglionic zone was 9 cm [mean 4 (1–9)] and the total pathological zone never exceeded 14 cm [mean 8 (3.8–14)]. In the P? group, the maximal length of aganglionic zone was 55.5 cm [mean 11.3 (2.5; 55.5)] and the total pathological zone extended to 59.5 cm [mean 17.75 (4.5; 59.5)]. Aganglionic segment was significantly shorter in the P+ group (p < 0.0001).Conclusion
Staining of some hypertrophic nerves in the submucosa in suction rectalbiopsy of HD patients using calretinin immunohistochemistry is only encountered in short-segment aganglionosis with a pathological zone always restricted to rectal and sigmoid colon. This information could be crucial for the surgeons in the decision to choose a transanal procedure. 相似文献7.
Bonnard C. Wirth T. Gebus O. Fahrer P. Montaut S. Robelin L. Tuzin N. Tranchant C. Anheim Mathieu 《Journal of neurology》2020,267(3):855-859
Journal of Neurology - Despite the consensus criteria for multiple system atrophy (MSA), the diagnosis of MSA of cerebellar type (MSA-C) may be difficult in the early stage of the disease. There... 相似文献
8.
Y Lenne A Gaillard J Bonnard J Billet M Fiche A Gordeeff G Ayoun 《Revue de stomatologie et de chirurgie maxillo-faciale》1985,86(6):378-381
A rare affection of unknown etiology that is benign but frequently recurrent, Kimura's disease involves infiltration of the dermis and hypodermis usually of the face. Documented data exists describing clinical findings and results of histopathology that are analogous but are grouped under other names: angiolymphoid hyperplasia with eosinophilia, pyogenic pseudogranuloma, atypical pyogenic granuloma. A case followed up for 13 years is reported. 相似文献
9.
Varraine E Bonnard M Pailhous J 《Experimental brain research. Experimentelle Hirnforschung. Expérimentation cérébrale》2000,130(2):248-257
The intentional control of stride length is a fundamental basis for the adaptation of the stride to environmental constraints (obstacle avoidance, for example). Controlling the propulsive forces during the stance and/or controlling the pendular movement of the oscillating leg constitute the two potential and non-exclusive mechanisms underlying intentional stride length modulation. The present experiment was conducted in order to determine if these two mechanisms contribute to voluntary length modulation and, if so, how they cooperate according to whether the subject has to lengthen or shorten a stride and how these mechanisms are implemented at the neuromuscular level. Subjects had to produce a temporarily modulated stride of the same length, but originating from two different initial steady-states: one from shorter stride length and one from longer stride length. We found that the shortening was essentially realized by a swing-duration decrease (an increased activity in the hip extensor--biceps femoris--during the swing of the ipsilaterally shortened stride stopped the pendular leg movement earlier). The lengthening was realized by two mechanisms: (1) an increase in the propulsive forces (via an increased activity of the ankle extensor muscles--soleus--and the hip extensors--biceps femoris--from the stance of the ipsilaterally modulated stride, which was prolonged during the following stance of the contralateral leg), and (2) an increase in swing duration on the ipsilateral leg (an increased activity in hip and ankle flexors--rectus femoris and tibialis anterior--maintained the ipsilateral leg in flexion during the lengthened swing so that the foot landed later). In this experiment, the subjects were faced with a spatial constraint of the same magnitude in the direction of stride lengthening and stride shortening. However, under these conditions, subjects used a different balance between swing control (that directly modifies the foot trajectory without affecting the trajectory of the head-arm-trunk system) and/or the control of propulsive forces (that indirectly influences foot trajectory by modifying the trajectory of the head-arm-trunk system). In the first case, this concerns a voluntary control of gesture produced by the legs and usually implicated in the locomotor pointing; in the second case, this concerns a voluntary control of propulsive forces. 相似文献
10.
Hasmim M Vassalli G Alghisi GC Bamat J Ponsonnet L Bieler G Bonnard C Paroz C Oguey D Rüegg C 《Thrombosis and haemostasis》2005,94(5):1060-1070
Expression of isolated beta integrin cytoplasmic domains in cultured endothelial cells was reported to induce cell detachment and death. To test whether cell death was the cause or the consequence of cell detachment, we expressed isolated integrin beta1 cytoplasmic and transmembrane domains (CH1) in cultured human umbilical vein endothelial cells (HUVEC), and monitored detachment, viability, caspase activation and signaling. CH1 expression induced dose-dependent cell detachment. At 24 h over 90% of CH1-expressing HUVEC were detached but largely viable (>85%). No evidence of pro-caspase-8,-3, and PARP cleavage or suppression of phosphorylation of ERK, PKB and Ikappa-B was observed. The caspase inhibitor z-VAD did not prevent cell detachment. At 48 h, however, CH1-expressing cells were over 50% dead. As a comparison trypsin-mediated detachment resulted in a time-dependent cell death, paralleled by caspase-3 activation and suppression of ERK, PKB and Ikappa-B phosphoyrylation at 24 h or later after detachment. HUVEC stimulation with agents that strengthen integrin-mediated adhesion (i.e. PMA, the Src inhibitor PP2 and COMP-Ang1) did not prevent CH1-induced detachment. Expression of CH1 in rat carotid artery endothelial cells in vivo caused endothelial cell detachment and increased nuclear DNA fragmentation among detached cells. A construct lacking the integrin cytoplasmic domain (CH2) had no effect on adhesion and cell viability in vitro and in vivo. These results demonstrate that isolated beta1 cytoplasmic domain expression induces caspase-independent detachment of viable endothelial cells and that death is secondary to detachment (i.e. anoikis). They also reveal an essential role for integrins in the adhesion and survival of quiescent endothelial cells in vivo. 相似文献