Imaging findings in infants with recurrent vomiting after pyloromyotomy: a pictorial review.

Hypertrophic pyloric stenosis (HPS) is a common entity with an incidence of 2-4 per 1000 live births. Current definitive treatment is with pyloromyotomy, which is usually performed laparoscopically. The procedure is generally well tolerated with resolution of the patient's symptoms. In a small percentage of patients, however, there is recurrent vomiting which warrants further investigation. In this pictorial review, the expected post-operative appearance of the pylorus will be described, and the imaging findings and clinical course of two patients with recurrent vomiting after pyloromyotomy will be presented.

Contribution of increased ISG15, ISGylation and deregulated type I IFN signaling in Usp18 mutant mice during the course of bacterial infections.

Host genetics has a key role in susceptibility to Salmonella Typhimurium infection. We previously used N-ethyl-N-nitrosourea (ENU) mutagenesis to identify a loss-of-function mutation within the gene ubiquitin-specific peptidase 18 (Usp18(Ity9)), which confers increased susceptibility to Salmonella Typhimurium. USP18 functions to regulate type I interferon (IFN) signaling and as a protease to remove ISG15 from substrate proteins. Usp18(Ity9) mice are susceptible to infection with Salmonella Typhimurium and have increased expression and function of ISG15, but Usp18(Ity9) mice lacking Isg15 do not show improved survival with Salmonella challenge. Type I IFN signaling is increased in Usp18(Ity9) mice and inhibition of type I IFN signaling is associated with improved survival in mutant mice. Hyperactivation of type I IFN signaling leads to increased IL-10, deregulated expression of autophagy markers and elevated interleukin (IL)-1ß and IL-17. Furthermore, Usp18(Ity9) mice are more susceptible to infection with Mycobacterium tuberculosis, have increased bacterial load in the lung and spleen, elevated inflammatory cytokines and more severe lung pathology. These findings demonstrate that regulation of type I IFN signaling is the predominant mechanism affecting the susceptibility of Usp18(Ity9) mice to Salmonella infection and that hyperactivation of signaling leads to increased IL-10, deregulation of autophagic markers and increased proinflammatory cytokine production.

Psychotherapeutic follow up of out patients with traumatic brain injury: preliminary results of an individual neurosystemic approach.

UNLABELLED: Psychotherapy for affective/behaviour disorders after traumatic brain injury (TBI) remains complex and controversial. The neuro-systemic approach aims at broadening the scope in order to look at behaviour impairments in context of both patient's cognitive impairments and family dysfunctioning. OBJECTIVE: To report a preliminary report of a neuro-systemic psychotherapy for patients with TBI. PATIENTS AND METHODS: All patients with affective/behaviour disorders referred to the same physician experienced in the neuro-systemic approach were consecutively included from 2003 to 2007. We performed a retrospective analysis of an at least 1-year psychotherapy regarding the evolution of the following symptoms: depressive mood, anxiety, bipolar impairment, psychosis, hostility, apathy, loss of control, and addictive behaviours as defined by the DSM IV. Results were considered very good when all impairments resolved, good when at least one symptom resolved, medium when at least one symptom improved, and bad when no improvement occurred, or the patient stopped the therapy by himself. RESULTS: Forty-seven patients, 35 men and 12 women, with a mean age of 33.4 years, were included. Most suffered a severe TBI (mean Glasgow coma score: 6.4) 11 years on average before the inclusion. At the date of the study, 11 patients (23%) had a poor outcome, 23 (48%) suffered Moderate disability and 13 (27%) had a Good recovery on the GOS scale. All therapy sessions were performed by the same physician, with 10 sessions on average during 13.5 months. Results were classified very good in six cases (13%), good in 18 others (38%), medium in 10 patients (21%) and bad in 13 cases (27%). We observed a significant improvement of affective disorders, namely anxiety (P<0.001) depressive mood (P<0.001) and hostility (P<0.01). However, bipolar symptomatology, apathy, loss of control and addictive disorders did not improve. DISCUSSION/CONCLUSION: From our best knowledge, this is the first clinical report of neuro-systemic psychotherapy for affective/behaviour disturbances in TBI patients. This kind of therapy was shown to be feasible, with a high rate of compliance (72%). Psycho-affective disorders and hostility were shown to be more sensitive to therapy than other behaviour impairments. These preliminary findings have to be confirmed by prospective trials on broader samples of patients.

Impact of Usp18 and IFN signaling in Salmonella-induced typhlitis.

In humans, Salmonella infection causes two major clinical diseases, typhoid fever and a self-limiting gastro-enteritidis. Salmonella transmission occurs by the fecal-oral route and the interactions between the bacteria and the digestive tract epithelium are central to the outcome of the infection. Using a mouse model of typhoid fever, we previously identified a mutation in USP18 affecting type I interferon (IFN) signaling resulting in increased susceptibility to systemic Salmonella infection. In this study, we demonstrate the effects of this mutation during the early response to Salmonella using a model of typhlitis. Mutant Usp18 mice showed a minimal inflammatory response early after Salmonella Typhimurium infection that was associated with low pathologic scores and low IFN-γ production. This resulted in an increased interaction of Salmonella with the cecal epithelium and earlier systemic dissemination of the bacteria. The global transcriptional signature in the cecum of mouse during Salmonella infection showed normal expression of tissue specific genes and upregulation of type I IFN pathway in mutant mice. In control mice, there was a significant over-representation of genes involved in cellular recruitment and antibacterial activity paralleling the histopathological features. These results show the impact of USP18 in the development of Salmonella-induced typhlitis.

[Outcome of the Aquitaine Unit for Evaluation, Training and Social and Vocational Counselling (UEROS) at 5-year follow-up in young adults with brain damage]. / Programme d'aide à la réinsertion sociale et professionnelle de jeunes adultes cérébrolésés: résultats à cinq ans du réseau UEROS-Aquitaine.

INTRODUCTION: Difficulties in social and vocational adjustment are common in adults with brain damage. A French government-funded program, UEROS (Unit for Evaluation, Training and Social and Vocational Counselling), was developed to improve cognitive adjustment, social autonomy and return to work for these people. OBJECTIVES: To describe the outcome and satisfaction with life after 5 years for patients who participated in the UEROS-Aquitaine network program. MAIN OUTCOME MEASURES: EBIS Document to evaluate people with traumatic brain injury. RESULTS: Seventy-five of the 102 patients participating in UEROS from 1997 to 1999 were assessed during a phone-structured interview based on the EBIS Document. The sample consisted of young adults (mean age 28.5 years), most (65, 85%) with brain injury and moderate disability (Glasgow Outcome Scale 2: 57%) or severe disability (Glasgow Outcome Scale 3: 42%). On entering the program, 33% of the subjects needed no help physically or cognitively. None were employed. At the end of the program, 9% were students or were learning a job, 8% worked full-time, 16% were in sheltered conditions, and 13% had volunteer activities. At 5-year follow-up, 50% of the subjects were independent, 4% were in school, 10.6% worked full-time, and 26.7% were in sheltered conditions. Playing sports was associated with good social adjustment. The professional status at 5 years was significantly correlated with following the UEROS program (r=0.30, P<0.01) and status at the end of the program (r=0.29, P<0.05). However, 41.3% of the subjects were still unsatisfied with their conditions of life. DISCUSSION-CONCLUSION: In patients with brain damage, the UEROS-Aquitaine network program improves independence in daily living and allows for nearly one inactive adult in two (42%) to be engaged in an activity or a job.

[Indications, efficacy and tolerance of drug therapy in view of improving recovery of consciousness following a traumatic brain injury]. / Indications, efficacité et tolérance des traitements médicamenteux susceptibles d'améliorer la reprise de conscience après un coma traumatique.

OBJECTIVE: To review the literature about the indications, efficacy, limits and tolerance problems of drugs used with the aim of improving recovery of consciousness after a traumatic coma. METHODS: Query using Medline, Embase, Neurosciences and Pascal databases. Thirty-two references were selected, including:articles proposing a synthesis of recent knowledge concerning neurotransmitters involved in vigilance, the pathophysiological mechanisms of impairment and the related pharmacology;clinical studies examining the efficacy of large categories of pharmacological agents (dopaminergic drugs, psychostimulants, tricyclic antidepressants and others) on vigilance and on alteration of consciousness following brain lesions. RESULTS: The synthesis provides evidence about the theoretical actions and efficacy of the available pharmacological agents. The clinical studies are less convincing: indications and therapeutic choices are empirical. Studies report often single cases. Randomised studies are rare, often heterogeneous concerning the aetiology of the brain lesions. The evaluation scales are varied and too wide. In this context, amantadin, amphetamine, methylphenidate and bromocryptin showed some positive effects. Despite a pessimist general feeling, some cases of unexpected recovery as well as relapses during breaks in treatment were arguments in favour of the drug that was administered. DISCUSSION/CONCLUSION: This review suggests that drug treatments for awakening might become a useful and perhaps even indispensable, complement in case management. It is therefore urgent to design multicentre studies in order to set rational indications and to develop realistic therapeutic protocols.

[Life satisfaction and psychosocial outcome in severe traumatic brain injuries in Aquitaine]. / Satisfaction de vie et devenir psychosocial des traumatisés crâniens graves en Aquitaine.

OBJECTIVE: To assess late outcome and satisfaction of life of patients with severe traumatic brain injury (TBI) patients who received inpatient rehabilitation in Aquitaine. DESIGN AND PATIENTS: Seventy-nine consecutive patients out of the 158 who were hospitalized for rehabilitation in 1993 were asked for in 2000 by a phone interview including standardised scales and free talk as well. RESULTS: The results showed that nine years on average after their injury, 65 to 85% of patients were independent for daily living, whereas 35 to 55% only were independent in social life. Most of them were satisfied with their autonomy (67%), family life (66%) and financial status (41%), but they were dissatisfied with leisure (36%), vocational adjustment (28%) and sexual life (32%). CONCLUSION: Return to work, leisure and sexuality are major parameters of satisfaction of life after a severe TBI, and should be emphasized in goal-directed rehabilitation programs.

Regulation of NRAMP1 gene expression by 1alpha,25-dihydroxy-vitamin D(3) in HL-60 phagocytes.

The natural resistance-associated macrophage protein 1 (Nramp1) is a proton-dependent transporter of divalent metals. We studied NRAMP1 expression during HL-60 differentiation induced by VD and VD agonists. NRAMP1 and CD14 gene expression differed in kinetics of induction, mRNA levels and stability, and response to VD combined with PMA, whereas a combination of VD and IFN-gamma induced similar up-regulation. NRAMP1 protein expression paralleled the accumulation of mRNA and was localized in the phagosomal membrane after phagocytosis. A promoter construct extending 647 bp upstream of NRAMP1 ATG showed myeloid-specific transcription in transient transfection assays, which was up-regulated by VD in HL-60. In HL-60 clones stably transfected with this construct, transcription was apparently induced through indirect VD genomic effects, and there was accordance between the levels of reporter transcription and endogenous NRAMP1 mRNA in response to VD but not to IFN-gamma. Thus, VD genomic effects stimulate NRAMP1 transcription and protein expression in maturing phagocytes.

Satisfaction of life and late psycho-social outcome after severe brain injury: a nine-year follow-up study in Aquitaine.

In view of assessing their late outcome and satisfaction of life, 79 out of 158 severe traumatic brain injury (STBI) patients who received inpatient rehabilitation in Aquitaine in 1993 were followed by phone interview. Results showed that 9 years on average after their injury, 65 to 85% of these patients were independent for daily living, whereas 35 to 55% only were independent in social life. Most of the patients were satisfied with their autonomy (67%), family life (66%) and financial status (41%), but they were dissatisfied with leisures (36%), vocational adjustment (28%) and sexual life (32%). Satisfaction of life was mostly related to age, gender, physical autonomy, need of help because of cognitive impairment and functional outcome as assessed by the Glasgow Outcome Scale. Severe traumatic brain injury (STBI) stands in industrialised countries as a major Public Health problem and a dreadful human drama for the patients, their families and the community [2]. A great number of STBI patients survive with severe disability, most of them being young adults. The most severely impaired may live only with their parents or in high-cost nursing homes. From a psychological point of view, STBI causes a total and sudden breakdown of the mental states, personality and conditions of life. Life plans and projects are definitively disrupted, satisfaction of life is deeply changed. Rehabilitation aims at improving functional outcome of persons with STBI, and at improving their overall quality of life. Planning for rehabilitation and re-entry into community of STBI patients need to be provided with precise data on their late outcome and disability level. Despite that the concepts of quality and satisfaction of life are difficult to define and moreover to assess, these are also major factors to take into account. The aims of the present study were to assess the late psycho-social outcome of patients hospitalized in Aquitaine for rehabilitation of a STBI 7 to 10 years after their injury, and to ask for their satisfaction of life and subjective feeling of quality of life.

Dysautonomia syndrome in the acute recovery phase after traumatic brain injury: relief with intrathecal Baclofen therapy.

In the initial phase of severe head injury, dysautonomic abnormalities are frequent. Within the framework of a prospective study, evaluating the efficacy of continuous intrathecal Baclofen therapy (CIBT) on hypertonia during the initial recovery phase of severe head injury, the authors report on the preliminary results of this treatment on paroxysmal dysautonomia about four patients. Continuous intrathecal Baclofen infusion was first delivered, for a test period, continuously for 6 days. If a relapse of dysautonomia occurred at the end of the test period, an implantation of a continuous intrathecal infusion pump delivering Baclofen was performed. Results were assessed with four continuous variables; duration (days), dose of Baclofen per day (microg/d), number of dysautonomic paroxysmal episodes per day, and initial recovery evaluated by a scale of the first initial stages of head injury coma recovery. For three patients: (1) the number of dysautonomic paroxysmal episodes per day and the doses of Baclofen during the follow-up period were correlated (p = 0.02, p < 0.001, p = 0.008, respectively, distribution-free test of Spearman), (2) during the test period and the relapse after the test period, the number of paroxysmal episodes and the Baclofen dose are correlated to p < 0.05, p = 0.03, p = 0.04, respectively (distribution-free test of Spearman). The second statistical test was used to prove that Baclofen doses and number of paroxysmal dysautonomic episodes are correlated independently of the duration of follow-up. The fourth patient improved with CIBT without any recurrence at the end of the treatment test period. For the four patients, recovery score increased during the overall follow-up. In the authors' experience CIBT is very efficient to control paroxysmal dysautonomia during the initial recovery phase in severe head injury, and seems to facilitate recovery.

Bone material ultrasound velocity is predictive of whole bone strength.

In humans, bone strength is assessed indirectly by the noninvasive measurement of structure or mass. Recent clinical application of an ultrasonic critical-angle reflectometry technique (UCR) has demonstrated the measurement of the regional and directional distribution of mechanical stiffness. This study investigates the specific question: are these measurements of a local material level property predictive of the strength of whole bone? Maximum values of pressure wave velocity and breaking strength were recorded at two locations (midshaft and base of neck) on rat femurs from growing rats. The results demonstrate a strong empirical relationship between material-level ultrasound (US) velocity and whole bone mechanical strength. However, the US velocity at a specific bone site can be used to assess bone strength at that site only, explaining discrepancies in other published studies that negate a relationship between strength and US velocity. The results indicate an important role for US velocity measurement in clinical evaluation of bone health.

Polyphyletic origins of bacterial Nramp transporters.

The redox-active metals iron and manganese are required for energy metabolism, protection against oxidative stress and defense against infections. In eukaryotes, both divalent metals are transported by Nramp transporters. The sequence of these transporters was remarkably conserved during evolution. Several bacterial Nramp homologs (MntH) are also proton-dependent manganese transporters. Here, we present phylogenetic evidence for the polyphyletic origins of three groups of MntH proteins and for possible Nramp horizontal gene transfer with eukaryotes. We propose that the evolution of the MntH/Nramp family is related to adaptation to oxidative environments, including those arising during infection of animals and plants.

Rehabilitation after traumatic brain injury in adults.

PURPOSE: Traumatic brain injury (TBI) stands as a major public health problem and one of the most important challenges for neurological rehabilitation. This review discusses advances that have occurred in the past 10 years in rehabilitation after severe TBI in adults. METHOD: First, theoretical concepts, goals of rehabilitation and organization of resources are reviewed. Then specific questions that arise in the rehabilitation of severe TBI patients are considered. RESULTS: Three phases are distinguished in post-traumatic evolution. Acute rehabilitation takes place during coma and arousal states. Specific aims are to prevent orthopaedic and visceral complications, and to provide sensory stimulations with the hope of accelerating arousal. Secondly subacute (generally inpatient) rehabilitation is designed to facilitate and accelerate recovery of impairments, and to compensate for disabilities. Motility, cognition, behaviour, personality and affect should be simultaneously addressed in an holistic approach. Physical as well as psychological independence and self-awareness are the major goals to emphasize. A third, post-acute rehabilitation phase includes outpatient therapy for achieving physical, domestic and social independence, reduction of handicaps and re-entry into the community. CONCLUSIONS: Problems with returning home, obtaining financial independence, driving, returning to work, participating in social relationships and leisure activities, and the importance of psychosocial adjustment and self-acceptance, are outlined. Questions about economic aspects and rehabilitation in the future are addressed.

[Deep cerebral stimulation in patients with post-traumatic vegetative state. 25 cases]. / Stimulation cérébrale profonde chez des patients en état végétatif post-traumatique. 25 observations.

In 25 cases of post traumatic vegetative state persisting 3 months after the initial injury, deep brain stimulation (DBS) has been used with the aim to activate the cortex and the hope to produce some degree of functional recovery. Electrodes were stereotaxically implanted in the centrum medianum-parafascicularis complex. Bipolar stimulation was provided daily from 8 a.m. to 8 p.m. In 12 cases no changes occurred in the clinical features and overall behaviour. DBS was given up after 2 months. All these patients with a follow-up of 1 to 10 years, remained in a permanent vegetative state, 4 of them eventually deceased. In 13 cases, following 1 to 3 weeks of DBS a definite improvement was obtained with recovery of some degree of consciousness and interpersonal relationship. However all these patients, with 1 to 12 years follow-up remained severely disabled. 2 of them deceased from intercurrent causes. The practical meaning of these clinical results are questioned. We consider that the absence of response to DBS is an important argument to predict the irreversibility of post traumatic vegetative state. It is not certain that DBS is directly responsible for positive changes observed, some degree of long-term spontaneous recovery being already documented in such patients. However it seems likely that DBS accelerates recovery and possibly improves the final level of performances.

[Electrophysiological development under thalamic stimulation of post-traumatic persistent vegetative states. Apropos of 25 cases]. / Evolution électrophysiologique sous stimulation thalamique des états végétatifs persistants post-traumatiques. A propos de 25 observations.

We have studied the electrophysiological evolution, with the aid of long-term, quantitative E.E.G. (Holter method) and sensory evoked potentials (visual, auditory and somatosensory) on 25 severe traumatic head injury patients (initial Glasgow scale--between 3 and 5) aged from 5 to 42 years having evolved towards a persistent vegetative state and being object of a protocol of thalamic stimulation. Electrophysiological exploration was practised before placement of the stimulation, then at 2 weeks, 1 month, 2 months and finally 3 months after. The electrophysiological parameters appear to have no predictive value on the clinical evolution after stimulation. However, changes such as long-term quantitative EEG as well as evoked potentials mostly precede clinical progress when present. Cortical evoked potentials are the first to change significantly (J15) followed by long-term quantitative EEG and brain stem evoked potentials (J30); the latter improving more completely. Also the consideration of the chronobiological aspects of the recordings of the long term EEG allow the proposal of "waking targeted" stimulations at the most favorable moments of the nyctohemeral period.

[Late postpartum eclampsia, myth or reality?]. / Eclampsie tardive du post-partum, mythe ou réalité?

It has never been formally established whether eclampsia can come on more than 48 hours after delivery. We report a case of a patient who had convulsions together with transitory raised blood pressure coming on 14 days after her delivery. MRI was carried out 24 hours after the attack and showed pathological sub-cortical images and there was a hyper signal at T2. This is now a well known feature and has been described in the course of typical eclampsia fits. On the other hand MRI is able to eliminate a certain number of differential diagnoses such as cerebral thrombophlebitis, cerebral vascular accidents and tumours. A cerebral blood flow study and a trans-cranial Doppler flow study showed (as far as we know for the first time) a lessening in perfusion and in speed of flow giving rise to lowered blood circulation in that part of the brain and suggestive of vascular spasm. This observation makes it very likely that eclamptic crises can occur two weeks after delivery and favours a vascular spasm being the origin of these crises.

[Ovarian abscess. Report of 2 cases]. / Abcès de l'ovaire. A propos de deux observations.

Ovarian abscess is very rare and is usually a complication of acute purulent salpingitis. It can however rarely be isolated. Wilson and Black found 28 cases of 75,262 women who were admitted to the Department of Gynaecology and Obstetrics in Philadelphia between 1948 and 1963. There are only 120 cases in the literature. We report here two cases of abscess of the ovary which were operated in our department at an interval of one month.