[Abnormal consequences of non-faulty medical accidents: Jurisprudence of the Conseil d'État]. / Conséquences anormales des accidents médicaux non fautifs : jurisprudence du Conseil d'État.

Compensation for no-fault medical accidents to professionals or establishments is provided for by article L. 1142-1 of the public health code, created by the law of March 4, 2002 relating to the rights of patients and the quality of the health system. It indicates the conditions of accountability, clinical criteria and severity. Among the clinical criteria "a medical accident (…) gives the right to compensation for damages (…) in the name of national solidarity, when (…) they have had abnormal consequences for the patient in view of his condition as well as the foreseeable evolution of it (…)". The jurisdictional orders have had to characterize this "abnormality of the consequences". According to the jurisprudence of the Conseil d'État of December 12, 2014 the condition of abnormality is always "satisfied when the medical act entailed consequences significantly more serious than those to which the patient was exposed in a sufficiently probable way in the absence of treatment". And if this is not the case, "they cannot be regarded as abnormal unless, under the conditions in which the act was performed, the occurrence of damage presented a low probability; that thus, they cannot be regarded as abnormal with regard to the state of the patient when the gravity of this state led to practicing an act involving high risks whose realization is at the origin of the damage". The Conseil d'État specified in 2019 that a probability of occurrence of 3% was a low probability.

Successful early surgical treatment in neonatal compartment syndrome: case report.

Neonatal compartment syndrome is rare, and the diagnosis is often missed or delayed because other ischemic diseases can mimic clinical signs observed on the skin. A premature newborn infant presented with skin lesions during the first hours of life that were recognized as the sentinel finding in compartment syndrome of the newborn. We restored normal function by emergency surgery. The authors highlight the importance of effective collaboration between pediatricians and surgeons to improve the management of this neonatal condition.

Human leptospira isolates circulating in Mayotte (Indian Ocean) have unique serological and molecular features.

Leptospirosis is one of the most widespread zoonoses in the world. However, there is a lack of information on circulating Leptospira strains in remote parts of the world. We describe the serological and molecular features of leptospires isolated from 94 leptospirosis patients in Mayotte, a French department located in the Comoros archipelago, between 2007 and 2010. Multilocus sequence typing identified these isolates as Leptospira interrogans, L. kirschneri, L. borgpetersenii, and members of a previously undefined phylogenetic group. This group, consisting of 15 strains, could represent a novel species. Serological typing revealed that 70% of the isolates belonged to the serogroup complex Mini/Sejroe/Hebdomadis, followed by the serogroups Pyrogenes, Grippotyphosa, and Pomona. However, unambiguous typing at the serovar level was not possible for most of the strains because the isolate could belong to more than one serovar or because serovar and species did not match the original classification. Our results indicate that the serovar and genotype distribution in Mayotte differs from what is observed in other regions, thus suggesting a high degree of diversity of circulating isolates worldwide. These results are essential for the improvement of current diagnostic tools and provide a starting point for a better understanding of the epidemiology of leptospirosis in this area of endemicity.

Use of an iliac crest growth plate for the development of a neo-articulation for congenital transverse deficiencies at the wrist.

PURPOSE: Terminal congenital transverse deficiencies of the wrist and hand are rare malformations. They are usually treated with prosthetic devices. We describe here a surgical technique to create a neo-articulation that could provide mobility distal to the forearm and help children with terminal transverse deficiencies use orthotic devices. METHODS: This procedure involved an autologous, nonvascularized graft from the immature iliac crest. The graft was placed with its cartilaginous side facing the radial and ulnar articular surfaces. We studied the clinical, radiological, and functional outcomes of this technique in 2 pediatric cases 5 and 14 years after this procedure. RESULTS: The x-rays showed a mobile graft distally in the forearm. Magnetic resonance imaging showed a space between the graft and the radius and an intermediate signal on either side of the space highly suggestive of articular cartilage. These various elements were organized into a neo-articulation between the forearm and the graft. The functional ability at follow-up was greater compared with the absence of a graft. CONCLUSIONS: The results of basic research work on cartilage and chondrocytes may help explain the presence of persistent cartilaginous tissue on the articulated part of the graft. The good functional outcome provided by our surgical procedure may translate into reduced need for prosthesis acceptance. Our surgical procedure does not restore limb length, but does add a new functional ability. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Fungemia in the French department of Mayotte, Indian Ocean: A 10 years survey.

OBJECTIVE: This study aimed at providing original data on fungemia in the Centre Hospitalier de Mayotte in terms of prevalence, epidemiological characteristics of infected patients, yeast species distribution and profile of in vitro antifungals susceptibility. METHODS: A total of 223 positive blood cultures for yeasts were retrospectively reported during the period April 2010-April 2020. RESULTS: Ninety-five episodes were identified corresponding to an incidence rate of 3.7 cases/100,000 inhabitants. The average age of patients was 33.5 years, and 63.3% patients were hospitalized in intensive care unit. The main co-morbidities were surgery in the 30 days prior to fungemia (27.8%), neoplasia (22.8%), parenteral nutrition (17.7%), diabetes (16.5%) and immunosuppressive medications (31.6%). Candida spp accounted for the majority of isolates (92.4%) with a predominance of non-albicans species (55.8% vs 33.7%), including C. albicans (33.7%), C. tropicalis (30.5%) and C. parapsilosis (20%). The antifungal susceptibility profiles did not differ from expected results for each species and did not change significantly over time. DISCUSSION: Fungemia remain frequent hospital infections associated with high mortality in Mayotte. The vast majority of fungemia was due to Candida spp. Non-albicansCandida species reach half of the Candida isolates with a high percentage of C. tropicalis. Surprisingly, no case of candidemia due to C. glabrata were identified. The management of candidemia remains satisfactory and the treatment was adapted according to the international recommendations. However, the high susceptibility of Candida spp. isolates to fluconazole may invite to reconsider the use of this molecule as empirical and first-line treatment of candidemia in Mayotte.

[Children's soft tissue infections in tropical countries. Prospective study in Mayotte]. / Les infections des parties molles de l'enfant en milieu tropical. Etude prospective hospitalière à Mayotte.

In France and Europe, soft tissue infections are secondary to chickenpox infection. In tropical countries, soft tissue infections seem to be different and are more frequent. We conducted a prospective and descriptive study in children hospitalised for cellulitis. We studied characteristics of our population and we tried to individualize risk factors for deep soft tissue infections. 54 children were included over a six-month period. Blood cultures were positive in 10% and local culture in 62%. Pathogenic organisms to be found, were first Staphylococcus aureus (78%) and secondly alpha-haemolytic streptococcus. Average rate hospitalisation was 4.5 days (1-28). Despite intravenous antibiotherapy, more than one third of patients had had a deep soft tissue infection (myositis, abscess, or arthritis). As regards the overall population, deep soft tissue infections associated with cellulitis were more frequent in children over six. Association with arthritis was found only in children under two. Severe malnutrition seems to be a notable risk factor for myositis. Soft tissue infections are still frequent in tropical countries. Deep soft tissue infections are encountered in more than one third of the cases, specially in children over six, and with Staphylococcus aureus. These results justify a systematic hospitalisation. If severe malnutrition is present, association with myositis should be suspected.

The temporal relationship between speech auditory brainstem responses and the acoustic pattern of the phoneme /ba/ in normal-hearing adults.

OBJECTIVE: To investigate the temporal relationship between speech auditory brainstem responses and acoustic pattern of the phoneme /ba/. METHODS: Speech elicited auditory brainstem responses (Speech ABR) to /ba/ were recorded in 23 normal-hearing subjects. Effect of stimulus intensity was assessed on Speech ABR components latencies in 11 subjects. The effect of different transducers on electromagnetic leakage was also measured. RESULTS: Speech ABR showed a reproducible onset response (OR) 6ms after stimulus onset. The frequency following response (FFR) waveform mimicked the 500Hz low pass filtered temporal waveform of phoneme /ba/ with a latency shift of 14.6ms. In addition, the OR and FFR latencies decreased with increasing stimulus intensity, with a greater rate for FFR (-1.4ms/10dB) than for OR (-0.6ms/10dB). CONCLUSIONS: A close relationship was found between the pattern of the acoustic stimulus and the FFR temporal structure. Furthermore, differences in latency behaviour suggest different generation mechanisms for FFR and OR. SIGNIFICANCE: The results provided further insight into the temporal encoding of basic speech stimulus at the brainstem level in humans.

[Refractory behaviour of the electrically stimulated auditory nerve]. / Das Refraktärverhalten des elektrisch stimulierten Hörnervs.

INTRODUCTION: Electrically evoked compound action potentials (TECAP) of the auditory nerve can be recorded in cochlear implants. TECAP thresholds are used to predict threshold levels for speech processor maps. The auditory nerve's refractory properties can influence these levels. METHODS: Recovery functions were investigated at 84 stimulation sites in 14 patients who had Nucleus CI24 implants; neural response telemetry (NRT) and a modified forward-masking technique were used for these investigations, introducing the reference masker-probe interval (MPI). RESULTS AND CONCLUSION: An interval between 300 and 375 micros was found to be suitable as the reference MPI in our study. The median of the absolute refractory period was determined as 390 s and the median time constant of the recovery function, at 425 s. In practice, a reference MPI of 300 s is suggested for measurement of recovery and amplitude growth functions. As up to now the amplitude growth function has been measured at 500 s and thus mostly in a relatively refractory condition, the refractory behaviour should influence the TNRT. In addition, it was possible to explain the shape of standard forward-masking recovery functions with reference to the latency shift of the neural response.

Rehabilitation plasticity: influence of hearing aid fitting on frequency discrimination performance near the hearing-loss cut-off.

Several studies have already demonstrated that patients with steeply sloping hearing loss of cochlear origin exhibit an improvement in frequency discrimination performance at or around the cut-off frequency. This enhancement cannot be explained in terms of peripheral mechanisms and should rather be interpreted in terms of central reorganization: i.e., injury-induced cortical plasticity. However, the reversibility and time course of such reorganization has not yet been described. The main goal of the present study was therefore to investigate the occurrence of rehabilitation plasticity associated with hearing-aid fitting in human subjects. Nine subjects with steeply sloping hearing loss and who were candidates for auditory rehabilitation were tested. Discrimination-limen-for-frequency (DLF) enhancement was investigated at the frequency with the best DLF (bDLF) for each individual subject before and during auditory rehabilitation (at 1 month, 3 months and 6 months). From 1 month on, frequency discrimination performance decreased significantly at the bDLF frequency, while remaining stable at other frequencies. This normalization may reflect a new central reorganization reversing the initial injury-induced changes in the cortical map. A correlation between subject's age and alteration in DLF at 1 month was also found, suggesting that plasticity operates faster in younger patients.

Infectious complications following allogeneic HLA-identical sibling transplantation with antithymocyte globulin-based reduced intensity preparative regimen.

In the setting of reduced-intensity conditioning (RIC) regimens for allogeneic stem cell transplantation (allo-SCT), the epidemiology of transplant-related infections is still poorly defined. In 101 high-risk patients who received an HLA-identical sibling allo-SCT after RIC, including fludarabine, busulfan and antithymocyte globulin (ATG), we report during the first 6 months a cumulative incidence of positive CMV antigenemia of 42% (95% CI 32-52%), developing at a median of 37 (range 7-116) days without evidence of CMV disease (median follow-up, 434 days). The cumulative incidence of bacteremia was 25% (95% CI 17-33%), occurring at a median of 67 (range 7-172) days, while patients had recovered a full neutrophil count. In all, 65% of the bacteremia (95% CI 49-81%) were gram negative. The cumulative incidence of fungal infections was 8% (95% CI 3-13%), with a median onset of 89 (range 7-170) days. In multivariate analysis, stem cell source (bone marrow; P=0.0002) was significantly associated with the risk of positive CMV antigenemia, while higher doses of prednisone (>2 mg/kg) represented the major risk factor for bacteremia (P=0.0001). Infectious-related mortality was 5% (95% CI 1-9%), with aspergillosis being the principal cause. Collectively, these results suggest that prospective efforts are warranted to develop optimal antimicrobial preventive strategies after RIC allo-SCT.

The auditory acclimatization effect in sensorineural hearing-impaired listeners: evidence for functional plasticity.

The present study provides new data on perceptual and physiological modifications associated with hearing aid (HA) fitting. Eight sensorineural hearing-impaired (SNHI) listeners participated. They had symmetrical hearing loss and were being fitted with binaural HAs for the first time. Perceptual performances were measured four times during auditory rehabilitation, using an intensity discrimination task and a loudness-scaling task. Pure tones of two different frequencies were used, one well amplified by HAs and the other weakly amplified. Two intensity levels were also tested, one rated 'soft' by SNHI listeners and the other 'loud'. Auditory brainstem responses (ABRs) to click stimulation were recorded. All measures were performed without HA. Results were consistent with the auditory acclimatization effect: most modifications induced by HA fitting were found at loud intensity levels and at high frequency, i.e., for acoustic information that was newly available to the listener. While both ears had similar hearing loss and aided gains, some differences between ears appeared in both perceptual tasks and in ABRs. In the right ear, a shortening of wave V latency paralleled perceptual modifications. The present results suggest that HA-fitting induces functional plasticity at the peripheral level of the auditory system.

Lateralization of the effects of the benzodiazepine drug oxazepam on medial olivocochlear system activity in humans.

Benzodiazepines (Bzd) are known to interact with GABAergic inhibitory neurotransmission. Previous research on their effect on human auditory efferent pathways--through evoked otoacoustic emissions suppression by contralateral acoustic stimulation (CAS)--indicated a decrease in medial olivocochlear (MOC) efferent system inhibitory activity, after oral intake of oxazepam--representative of the Bzd drug class. To date, this pharmacological effect was only assessed in the right ear. Since a leftward asymmetry of Bzd receptors localization in human auditory cortex has been described recently, we explored in this study the hypothesis of an asymmetrical action of Bzd on MOC efferent functioning. The results revealed a significant difference of Bzd effect probing the right ear versus the left ear, with CAS-induced suppression being less effective in the right than left ear after oxazepam intake. This finding raises the question of possible neurochemical left-right asymmetry in the descending auditory pathways. The potential localization of this asymmetry is discussed.

A novel C202F mutation in the connexin26 gene (GJB2) associated with autosomal dominant isolated hearing loss.

Mutations in the GJB2 gene encoding connexin26 (CX26) account for up to 50% of cases of autosomal recessive hearing loss. In contrast, only one GJB2 mutation has been reported to date in an autosomal dominant form of isolated prelingual hearing loss. We report here a novel heterozygous 605G-->T mutation in GJB2 in all affected members of a large family with late childhood onset of autosomal dominant isolated hearing loss. The resulting C202F substitution, which lies in the fourth (M4) transmembrane domain of CX26, may impair connexin oligomerisation. Finally, our study suggests that GJB2 should be screened for heterozygous mutations in patients with autosomal dominant isolated hearing impairment, whatever the severity of the disease.

[Auditory neuropathy: clinical presentation of seven cases and review of the literature]. / Neuropathie auditive: clinique et revue de la littérature. A propos de 7 observations.

INTRODUCTION: Auditory Neuropathy (AN) is defined as a sensorineural hearing loss characterized by normal cochlear haircell function (assessed by recordable Otoacoustic Emissions) and absent or abnormal auditory brainstem evoked potentials (ABR) corroborated with absence of middle ear reflexes. PATIENTS AND METHODS: We report five cases with AN. We also report two others cases in which the presentation was different but suggestive of AN. For the majority of patients, the hearing loss had been detected during childhood. Hearing assessment of these patients included appropriate behavioral audiometric techniques (Pure Tone Audiometry - PTA, and speech audiometry), objective measures of middle ear function, acoustic reflex studies, Otoacoustic Emissions (OAE) and Auditory Brainstem Responses (ABR). RESULTS: Pure tone audiometry revealed mild-to-profound hearing loss. In patients with recordable PTA thresholds were less degraded than speech intelligibility. In all patients, tympanogram and OtoAcoustic Emissions were normal. The stapedius reflex and Auditory Brainstem Responses were absent or very degraded. CONCLUSIONS: AN can be diagnosed by the combined use of pure tone audiometry, speech audiometry, and objectives measures with the recording of OAE and ABR responses. Neonatal hearing loss OAE screening can miss babies with AN. The sooner the diagnosis is established the more successful the treatment, new opportunities being afforded by cochlear implantation.

Successful hemicondylar femoral allograft for traumatic bone loss: a paediatric case study with ten years of follow-up.

The management of massive traumatic defects of the knee joint is challenging, especially in children. Massive osteoarticular allograft may be an option in this kind of traumatic bone loss. We report on the case of a male patient who (at the age of 15) suffered an open grade III condylar femoral joint fracture, with a massive bone defect and a Schatzker V tibial plateau fracture. Ten years after first-line treatment with massive osteoarticular allograft of the lateral femoral condyle, the patient's knee was capable of full extension and 90° flexion. The patient reached a point of being pain free for nine years before he subsequently developed some pain with lateral arthritis progression.

Attention and otoacoustic emissions: a review.

The possible existence of an attention effect on the peripheral auditory system remains a controversial issue. The aim of the present study is to show the possible contribution of otoacoustic emissions towards demonstrating cognitive control of peripheral auditory processes via the auditory efferent fibers. This measurement technique allows investigation of a further part of the peripheral auditory pathways and of whether efferent fibers, presumably involved in cochlear neurosensory activity, could mediate an attention effect by the selection of auditory information.

Mutation in the zonadhesin-like domain of alpha-tectorin associated with autosomal dominant non-syndromic hearing loss.

A gene responsible for autosomal dominant non-syndromic hearing impairment in two families (DFNA8 and DFNA12) has recently been identified as TECTA encoding alpha-tectorin, a major component of the tectorial membrane. In these families, missense mutations within the zona pellucida domain of alpha-tectorin were associated with stable severe mid-frequency hearing loss. The present study reports linkage to DFNA12 in a new family with autosomal dominant high frequency hearing loss progressing from mild to moderate severity. The candidate region refined to 3.8 cM still contained the TECTA gene. A missense mutation (C1619S) was identified in the zonadhesin-like domain. This mutation abolishes the first of the vicinal cysteines (1619Cys-Gly-Leu- 1622Cys) present in the D4 von Willebrand factor (vWf) type D repeat. These results further support the involvement of TECTA mutations in autosomal dominant hearing impairment, and suggest that vicinal cysteines are involved in tectorial membrane matrix assembly.

GSR feedback and Schultz relaxation in tension headaches: a comparative study.

A comparative study of the use of GSR feedback (n = 16) and Schultz relaxation (n = 15) with patients presenting tension headaches showed that there was no significant improvement in the group treated by relaxation at the end of the treatment whereas the group treated by GSR feedback showed significant improvements with respect to frequency and intensity of headaches and to anxiety as measured by subjects' self-evaluation (P less than 0.05). Intergroup comparison demonstrated a greater improvement in headache intensity for the GSR feedback group than for the relaxation group (P less than 0.05) at the post-treatment stage. Likewise, the percentage of patients showing at least 50% improvement as to headache frequency was significantly higher (P less than 0.05) in the GSR feedback than in the relaxation group. High pre-treatment EMG and high pre-treatment weekly pain level indicate a good prognosis of improvement with respect to post-treatment headache frequency and intensity respectively in the case of the GSR feedback group. We found no prognostic factor for post-treatment clinical improvement in the relaxation group.

Influence of benzodiazepines on auditory perception.

The aim of this study was to test for an influence of benzodiazepine (BZD) on various perceptual and/or cognitive auditory processes. Loudness, auditory selective attention, and the ability of subjects to form perceptual streams out of alternating tone sequences were tested. Nine subjects were tested before, 1, 3, 7, and 24 h after a single-dose oxazepam vs placebo administration in a crossover design. A sample of blood allows us to measure plasma oxazepam concentration. The results revealed a significant reduction in stream segregation expressed as d' scores 1 h after oxazepam intake in the test subjects. No significant change occurred across time in the same subjects when they were administrated a placebo in another session. Furthermore, oxazepam had no substantial and systematic influence either on auditory selective attention or on loudness perception. Altogether, these results suggest that the perceptual organization of sound sequences involves inhibitory neural mechanisms, which can be affected by BZDs. This outcome is consistent with existing models of auditory stream segregation and may be paralleled with earlier findings on the effect of BZDs on perceptual binding in the visual modality.