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Background Vascular Ehlers-Danlos syndrome (vEDS) is a rare inherited connective tissue disorder, leading to mainly arterial complications, caused by COL3A1 pathogenic variations. Ong et al. (Lancet 2010) showed that the introduction of celiprolol significantly reduced arterial events in a predominantly symptomatic population on arterial level. We reported the benefit of full dose of Celiprolol in longitudinal study to prevent arterial events in Frank et al. (JACC 2019). To our knowledge, there is no data regarding the benefit of celiprolol in patients without any arterial event. Purpose The aim of this study was to assess the occurrence of arterial events during follow-up in vEDS patients without arterial event. Methods All patients – probands and relatives – with a pathogenic variation in COL3A1 diagnosed at the Referral Centre for Rare Vascular Diseases since 2001 and at least one arterial tree assessment were included. We then focused on vEDS patients without any arterial event. We retrospectively analyzed the duration of follow-up, the occurrence of arterial events during follow-up, and the introduction and dose of celiprolol, especially at the time of occurrence of an arterial event. Results Among the 230 patients included, 144 (63%) had at least one symptomatic arterial event, 44 (19%) had only silent arterial event and 67 (29%) had no arterial event at the first arterial tree assessment event. Patients with no arterial event were significantly younger at this visit compared with the two other groups with a median age of 23 vs. 38 and 39 years (silent and symptomatic) (p Conclusions Genetic testing of the relatives of probands with vEDS remains of utmost importance as one quarter presented with symptomatic arterial event and one fifth had silent arterial event at initial arterial tree assessment. For patients with no arterial event, those without celiprolol have a similar incidence of arterial events than those with celiprolol, however their follow-up duration was twice shorter suggesting a higher incidence rate or arterial event in the absence of celiprolol. Funding Acknowledgement Type of funding sources: None.

Sudden cardiac death, mostly related to ventricular arrhythmia, is a major public health issue, with still very poor survival at hospital discharge. Although coronary artery disease remains the leading cause, other etiologies should be systematically investigated. Exhaustive and standardized exploration is required to eventually offer specific therapeutics and management to the patient as well as his/her family members in case of inherited cardiac disease. Identification and establishing direct causality of the detected cardiac anomaly may remain challenging, underlying the need for a multidisciplinary and experimented team.

Resume Les pathologies genetiques sont la premiere cause d’atteinte anevrysmale et dissequante de l’aorte chez le sujet jeune, l’aorte ascendante etant touchee dans plus de 90 % des cas. Bien qu’hereditaires, ces pathologies sont genetiquement tres heterogenes, et peuvent paraitre sporadiques du fait de nombreux cas de mutations dites « de novo ». Elles sont classees en formes syndromiques et non syndromiques, l’examen clinique adapte etant un element cle de cette distinction. Les formes syndromiques sont majoritairement liees a un defaut de fonctionnement de la matrice extracellulaire. Elles sont parfois complexes a caracteriser cliniquement, car elles presentent une grande diversite d’atteintes y compris au sein d’une meme famille. Les formes non syndromiques sont de fait plus homogenes dans leur presentation, le mecanisme predominant etant un defaut de l’appareil contractile des cellules musculaires lisses aortiques. L’evaluation du risque genetique doit etre proposee devant tout atteinte aortique ascendante chez un patient de moins de 50 ans ou presentant peu de facteurs de risque cardiovasculaires, afin d’identifier la pathologie sous-jacente, assurer une surveillance et une prevention adaptee chez le patient et ses apparentes a risque. Une revue de ces syndromes avec leurs principales caracteristiques cliniques, physiopathologiques et genetiques est presentee, accompagnee de precisions sur les demarches genetiques a effectuer face a une suspicion diagnostique.

Background: Conflicting data exist regarding the benefit of urgent coronary angiogram and percutaneous coronary intervention (PCI) after sudden cardiac arrest, particularly in the absence of ST-segment elevation. We hypothesized that the type of lesions treated (stable versus unstable) influences the benefit derived from PCI. Methods: Data were taken between May 2011 and 2014 from a prospective registry enrolling all sudden cardiac arrest in Paris and suburbs (6.7 million inhabitants). Patients undergoing emergent coronary angiogram were included. Decision to perform PCI was left to the discretion of local teams. We assessed the impact of emergent PCI on survival at discharge according to whether the treated lesion was angiographically unstable or stable, and we investigated the predictive factors for unstable coronary lesions. Results: Among 9265 sudden cardiac arrests occurring during the study period, 1078 underwent emergent coronary angiogram (median age: 59.6 years, 78.3% males): 463 (42.9%) had an unstable lesion, 253 (23.5%) only stable lesions, and 362 (33.6%) no significant lesions. Emergent PCI was performed in 478 patients (91.4% of unstable and 21.7% of stable lesions). At discharge, PCI of unstable lesions was associated with twice-higher survival rate compared with untreated unstable lesions (47.9% versus 25.6%, P =0.013), while stable lesions PCI did not improve survival (25.5% versus 26.3%, P =1.00). After adjustment, PCI of unstable coronary lesions was independently associated with improved survival (odds ratio, 2.09 [95% CI, 1.42–3.09], P P =0.824). Angina, initial shockable rhythm, ST-segment elevation, and absence of known coronary artery disease were independent predictors of unstable lesions. Conclusions: Emergent PCI of unstable lesions is associated with improved survival after sudden cardiac arrest, contrary to PCI of stable lesions. Accordingly, early PCI should only be performed in patients with unstable lesions. Four factors (chest pain, ST-elevation, absence of coronary artery disease history, and shockable initial rhythm) could help identify patients with unstable lesions who would, therefore, benefit from emergent coronary angiogram.

Introduction Since a large proportion of patients resuscitated from out-of-hospital sudden cardiac arrest (SCA) die in the intensive care unit (ICU), early systematic investigation towards identifying etiology may be crucial to ensure targeted therapy and appropriate future prevention among relatives, especially when the index case is young. Purpose We hypothesized that etiologic investigations were not initiated in a timely manner in a significant proportion of young SCA patients, alive at ICU admission, prior to death. Methods In this prospective, ongoing, multicenter, population-based registry (6.7 million inhabitants), data from all SCA over a 5-year period were analyzed, in collaboration with all the 48 hospitals of the area, with a specific focus on young patients ( Results Of the 18,622 out-of-hospital cardiac arrests from May 2011 to May 2016, 3,028 were admitted alive to ICU. Among them, 2,190 (72.3%) died in ICU, including 367 (16.8%) young cases ( Conclusion More than half of young SCA cases who died in ICU remained unexplained. There was significant underuse of core cardiac investigations. Efforts to promote prompt and systematic investigation through better collaboration between intensivist and cardiologist may improve both acute management and future targeted preventive strategies for family members.

Introduction et but de l’etude Au CHIV, la demande institutionnelle de consultation dietetique dans les services se fait par fax, prealablement remplie par le medecin. Le Clan a realise une evaluation des pratiques professionnelles(EPP) en UGA de fevrier a mai 2018 afin de renseigner les pourcentages de prescription de regimes, de CNO ainsi que leur dispensation et consommation. Puis nous avons apporte des axes d’amelioration et nous avons fait un 2e tour d’audit. Materiel et methodes Il s’agit d’une etude prospective avant-apres moncentrique. Nous avons etudie 38 dossiers de patients en UGA sur les items precedents. Puis devant les resultats obtenus, nous avons choisi des axes d’amelioration : – arret du fax au profit d’un item informatique cree sur le logiciel Pharma. Les medecins vont renseigner les antecedents du patient et demander un regime dietetique particulier ainsi que des CNO. La dieteticienne peut visualiser la prescription de son bureau, sur son ordinateur, cibler alors le patient, venir le voir et conseiller le type de CNO, – creation pour les paramedicaux, d’items sur Pharma renseignant la date et l’heure de delivrance ainsi que la quantite consommee, – nous avons dispense un cours a tous les personnels sur les consequences de la denutrition, son interet de la depister, et avons explique l’utilisation des nouveaux outils informatiques. Nous avons refait un 2e tour d’audit 2 mois plus tard sur 20 patients denutris. Resultats et analyse statistique Nous constatons que le taux de denutrition est de 55 % en UGA. La prescription de regime par fax est de 55 % et passe a 95 % quand il y a un support informatique. La delivrance des CNO ainsi que leurs horaires et leurs consommations sont respectivement de 0 %, 0 %, 0 % ! Avec la creation d’items sur Pharma, la delivrance est notee a 60 %, les horaires a 55 % et la consommation a 15 %. 100 % des medecins prescripteurs sont satisfaits de ce mode de prescription. Conclusion Nous avons donc abandonne le fax. Le nouvel outil de prescription se deploie dans tous les services, appuyes par un cours sur les consequences de la denutrition, une intervention par la dieteticienne du service, et la demonstration informatique par la referente paramedicale du logiciel. Les equipes sont satisfaites car la tracabilite est manifeste et la surveillance plus aisee. Mais il faut etre vigilant et revenir dans les services afin de sensibiliser encore et toujours sur les mefaits de la denutrition.

Introduction Since a large proportion of patients resuscitated from out-of-hospital sudden cardiac arrest (SCA) die in the intensive care unit (ICU), early systematic investigation towards identifying etiology may be crucial to ensure appropriate future prevention among relatives, especially when the index case is young. Purpose We hypothesized that etiologic investigations were not initiated in a timely manner in a significant proportion of young SCA patients prior to death. Methods In this prospective ongoing multicenter, population-based registry (6.7 million inhabitants), data from all SCA over a 5-year period were analyzed with a specific focus on young patients ( Results Of the 18,622 out-of-hospital cardiac arrests from May 2011 to May 2016, 3,028 were admitted alive to ICU. Among them, 2,190 (72.3%) died in ICU, including 367 (16.8%) young cases ( Conclusion More than half of young SCA cases who died in ICU remained unexplained. Efforts to promote early systematic investigation through better collaboration between intensivist and cardiologist may improve future targeted preventive strategies for family members.

Introduction/Background The use of myoelectric prosthetics in upper limb amputees introduced compensations on the residual shoulder and trunk which could be the cause of musculoskeletal disorders. Task/work involving movements in abduction of shoulder (> 60°) more than two hours per day in cumulative have been identified to be responsible of musculoskeletal disorders. The aim of the study is to evaluate the shoulder compensations in upper limb amputees with the use of the new myoelectric multi-articulated prosthetic hands compared to tri-digital hands during different functional tasks. Material and method Five unilateral trans-radial upper limb amputees (4 male, 1 female; age 45.6 ± 13.8 years old) participated in this prospective crossover study. Evaluations were realized by each participant with their own tri-digital myoelectric prosthesis and 2 multigrip prosthesis: I Limb Ultra® (IL) without a wrist mobility and Michelangelo® (MA) with a wrist mobility, after specific rehabilitation and one month of home use. Mean of internal rotation, mean of abduction shoulder, percent time in abduction higher than 60° and its extrapolation in hours were calculated by an optoelectronic system (VICON) for the both sides (amputated - non-amputated) during the Box and Block Test (BBT) and 2 common daily life tasks (cut meat, make a knot). Results Whatever the tasks, there is any significant difference in internal rotation with the different prosthetic devices. Conclusion New types of grip of innovative prosthetic hands and wrist mobility seems to decrease shoulder abduction, considered as pathogenic compensations. However, the compensation strategy is different across patients, which involve a personalized analysis for his rehabilitation program and for the prosthetic device choice. Further studies are necessary to evaluate the impact of trunk compensations.

Patellofemoral pain is likely due to compressive force acting on the patella related in turn to knee extension moment. The latter variable was assumed to be (i) reduced during short-distance free walking in case of patellofemoral pain syndrome and (ii) increased after therapeutic pain reduction. Peak knee extension moment at beginning of stance phase was recorded by three-dimensional gait analysis in 22 controls and in 23 patients with patellofemoral pain syndrome before and after rehabilitation of knee extensors and flexors to reduce the pain. Pain would occur mainly in stressful activities such as stair negotiation or squatting and was quantified by the anterior knee pain scale. Peak knee extension moment was significantly reduced in all the patients before treatment (n = 23) compared to controls, although no one had pain during free walking. In the 17 patients who experienced significant postrehabilitation pain reduction in their stressful activities, the peak knee extension moment was significantly reduced before treatment compared to controls and significantly increased after treatment, reaching values similar to control values. The peak knee extension moment during free walking appears to be a good kinetic variable related to a compensatory mechanism limiting or avoiding anterior knee pain and may be of interest in assessing knee dynamics alteration in patients with PFPS.

The authors had for aim to make an inventory of antibiotic treatment for severe community-acquired Streptococcus pneumoniae pneumonia and compare local practices to the local and national guidelines.An audit was conducted retrospectively in the Versailles hospital ICU between January 2006 and April 2009. Forty patients were included.Ninety-three percent had major risk factors for pneumonia. Ninety-eight percent were treated, with the usual empirical treatment (69%) or treatment active against Pseudomonas aeruginosa (31%). Eighty-five percent of empirical treatment complied with the French national guidelines issued by the SPILF and 49% with the local ICU protocol, more restrictive for the choice of the agent and dose. Early de-escalation to amoxicillin was applied to 41% of patients after obtaining results for pneumococcal and Legionella antigen and results of respiratory sample direct examination. For all patients, empirical treatment was reassessed according to culture results: 81% were prescribed amoxicillin. Evaluation showed that 92% of treatment complied with SPILF guidelines and 65% with the local ICU protocol that required adaptation of amoxicillin doses according to MICs; adaptation to severity and BMI was necessary for ten patients. Mortality remained high, at 37%, despite using antibiotics still effective against S. pneumoniae.This survey revealed a satisfactory adhesion to recommendations and prompt responsiveness of the team for adjustment of antibiotic therapy. The audit allowed updating the local ICU protocol.

Introduction Invasive aspergillosis is a serious disease, the lethality of which is important among hematology patients. Early diagnosis is crucial for treatment options and the prognosis. Detection of the antigen galactomannan is the most frequently used microbiological tools. But galactomannan detection may be falsely positive, and this false positivity has been associated with piperacillin-tazobactam treatment, the main antibiotic combination used in clinical hematology. Objective The purpose of our study, carried out from January 2009 to December 2010 at the Versailles hospital on in-patients with hematological disorders, was to evaluate the association between false galactomannan positivity and administration of piperacillin-tazobactam, and to study a possible variability of products issued by three manufacturers. Patients and method We noted that 207 patients were included (n = 207), accounting for 69 false positive and 138 true negative results. The intrinsic galactomannan values in the study were sensitivity 100%, specificity 68%, positive and negative predictive values respectively 16%, 100%, and a likelihood positive and negative test at respectively 3.12, and 0. Results The statistical analysis did not determine any association between false positivity in galactomannan and piperacillin-tazobactam issued by two manufacturers (P = 0.87 and P = 0.94). But, there was a significant association between false galactomannan positivity and piperacillin-tazobactam issued by the third manufacturer (P = 0.02). Four of the 25 batches issued by this manufacturer were tested and negative “in vitro” for galactomannan. Discussion This study results suggest that the association between false galactomannan positivity and piperacillin-tazobactam is not longer systematic, but can still prevail depending on the manufacturers. It also confirmed the positive contribution of testing piperacillin-tazobactam batches “in vitro” before using the antibiotic.

This article describes the design considerations for low-power short-range radio transceivers with a focus on the 2.4 GHz PHY layer defined as part of the IEEE 802.15.4 standard. The specification requirements for IEEE 802.15.4-compliant transceivers, and the design challenges and practical implementation of a highly-integrated low-power 2.4 GHz transceiver are subsequently discussed. The transceiver uses a direct-conversion receiver with switched antenna diversity and a transmitter using direct closedloop VCO modulation. It integrates a mask-programmable radio controller capable of autonomously performing timing-critical MAC functions, and a sleep timer. Implemented in 0.18 mum RFCMOS technology with a chip area of less than 6 mm2, the transceiver achieves a link margin of 99 dB while drawing 16.8 mA and 18 mA from a 1.8 V supply in receive and transmit mode, respectively.

Les tumeurs intramedullaires sont rares. Le dernier rapport de la SNCLF remonte a 1994 sur une periode de 25 ans. Il exposait les progres de la chirurgie face a ces tumeurs. Dans le meme temps, les progres de l’imagerie etaient expose en particulier par IRM. Dans les recentes publications sur le sujet, la chirurgie reste essentielle, biopsie ou exerese. Aussi, au debut de ce siecle, peut-on eviter la chirurgie et proposer un suivi medical en l’absence de troubles neurologiques ? Patients et methode Nous rapportons notre experience sur 4 cas, suivi depuis plusieurs annees par la clinique et l’imagerie par IRM. Ces tumeurs ont ete decouvertes suite a une cervicalgie chez 3 patients et un cas de dorsalgie. L’IRM a mis en evidence des lesions tumorales non clivables intramedullaires. Le suivi medical sur 8 ans et plus montre une parfaite stabilite de l’examen clinique sans troubles neurologiques. Resultats Deux patients sont en retraite, l’un est en invalidite et le dernier travaille mais evite les efforts. Pour ce dernier patient, le diagnostic d’astrocytome de bas grade est le plus vraisemblable, pour les autres cas aucun diagnostic precis ne peut etre fourni. En l’absence d’evolution, faut-il continuer la surveillance, a quel rythme ? Dans toutes les series, les risques chirurgicaux restent importants, ce qu’il faut garder a l’esprit. Dans la litterature, aucun cas de suivi medical de tumeur intramedullaire n’est rapporte. Nous sommes interpelles par des lesions etendues intramedullaires bien tolerees. L’un des patients, pompier professionnel a continue de travailler jusqu’a sa retraite sans probleme et son imagerie est toujours stable. Conclusion En l’absence de troubles neurologiques, sachant que chez tous ces patients aucun geste d’exerese complete ne peut-etre propose, il peut etre licite de proposer un suivi rigoureux annuel clinique et par imagerie chez ces patients pendant les 6 premieres annees puis tous les 2 ans jusqu’a 10 ans. Ensuite, je leur propose un suivi medical simple.

Background In gradient echo magnetic resonance imaging (MRI), intravascular thrombi (IT) can appear as vascular susceptibility artifacts, linked to local presence of intra-arterial deoxyhaemoglobin, and called susceptibility vessel signs (SVS). Aims Our objectives were to evaluate the sensitivity of susceptibility-weighted sequences, such as T2* weighted angiography (SWAN) in the visualization of SVS compared with T2*, to consider whether it enabled a better understanding of the importance of SVS, and to compare cerebral circulation regulation profiles according to the localization of the SVS (i.e. proximal or distal). Methods We prospectively studied the clinical and imaging data of 78 consecutive patients admitted for acute cerebral ischemia to the stroke unit of Besançon University Hospital between 1 April 2009 and 31 January 2010. Results An SVS was visualized in 44/78 (56%) patients using SWAN and in 13/78 (16%) patients using T2*. All the SVS visible using T2* were also visible on the SWAN. The interobserver kappa score was 0·72 [CI (0·53–0·91)] for T2*, 0·72 [CI (0·57–0·87)] for SWAN, and weighted kappa was 0·77 [CI (0·61–0·92)] for both T2* and SWAN. When an MCA occlusion was visible on MRA imaging (22/78 patients), a SVS was visualized in 7/22 cases (31·8%) using T2* and in 20/22 cases (91%) using SWAN. When the occlusion was visible in the M1 or M2 segments (17/78 patients), an SVS was visualized in 6/17 cases (35·3%) using T2* and in 15/17 cases (88·2%) using SWAN. When the occlusion was visible in the M3 segment (5/78 patients), an SVS was visualized in 1/5 cases (20%) using T2* and in 5/5 cases (100%) using SWAN. Presence of SVS was not associated with cardioembolic etiology of the stroke. Conclusions SWAN was more sensitive than T2* in the visualization of SVS in the intracranial arteries during the acute phase of ischemic stroke. Our study shows that the low number of SVS visualized using T2* in previous studies is probably related to a lack of sensitivity of the sequence, rather than to the nature or age of the thrombus. The greater sensitivity of SWAN seems to be linked to the visualization of SVS in cases of small thrombi.

The thermal inactivation of Salmonella enteritidis phage type 4 and Escherichia coli O157:H7 as affected by temperature (54.5-64.58C), pH (4.2-9.6 with HCl or NaOH) and NaCl concentration (0.5-8.5% w / w) was studied. Cell suspensions in modified tryptone soya broth were heated in a submerged-coil heating apparatus and survivors were enumerated on tryptone soya agar incubated aerobically. For most thermal inactivation data there was a logarithmic decrease in the viable cell concentration over the initial 4-6 log reduction and D-values were fitted. In some cases, tailing of the survivor curves was 10 observed with cells surviving longer than the D-values predicted. Models describing the effect of temperature, pH and NaCl concentration on the thermal inactivation of S. enteritidis and E. coli O157:H7 were produced. For both organisms, predicted z-values of 4.6-7.0 C8 were obtained depending on conditions, with larger z-values at higher levels of NaCl. Optimum survival occurred between pH 5 and pH 7 and increasing acidity or alkalinity caused a decrease in the predicted D-values. At equivalent pH, acetic acid and lactic acid (at 0.5, 1 and 2% w / w) generally had a similar, or increased, lethal effect compared with HCl, whereas in most cases citric acid had a less lethal effect. For E. coli O157:H7, increasing NaCl concentration had a protective effect up to the maximum tested (8.5% w / w), while for S. enteritidis optimal survival at a NaCl concentration of 5-7% w / w was predicted. The models were validated in foods by comparing predictions with published data. Most (80%) of the predicted D-values from the S. enteritidis model were within the 95% confidence interval (within 2.45-fold of the published data) for different Salmonella serotypes in whole egg, egg albumen, egg yolk, beef and milk. Most (93%) of the predicted D-values from the E. coli O157:H7 model were larger than the limited published data for this organism in meat, poultry, milk and apple juice with 42% within the 95% confidence interval (within 2.05-fold of the published data). The D-value models were incorporated into Version 1, and subsequent versions, of the predictive microbiology software program, Food MicroModel. © 1997 Elsevier Science B.V.

Summary Background and purpose Transfontanellar contrast enhanced ultrasound (TCEUS) in infants with neurological diseases has not been previously reported. Thus, the objective of our study was to describe the imaging findings of transfontanellar contrast enhanced ultrasound (TCEUS) performed in various neurological conditions in infants and to compare the findings with non-enhanced transfontanellar ultrasound (TFUS) and MRI. Methods Local institutional review board approval was obtained and, because of the need to catheterize children for contrast media administration, written informed consent of parents was obtained prior to all performed TCEUS. Twelve infants who underwent 12 TCEUS were included in this study from June 2009 to June 2012. Second generation contrast material was used (Bracco). TCEUS imaging findings were compared with those of conventional transfontanellar ultrasound in each case and with MRI. Results In 10 out of the 12 performed examinations, TCEUS showed abnormalities which were not depicted on non-enhanced TFUS. Accurate diagnosis of TCEUS compared with MRI was found in 10 out of 12 initial TCEUS. No adverse events during or immediately after contrast media injection occurred. Conclusion TCEUS appears to be a potential bedside accessible non-ionizing alternative imaging modality in the assessment of neonatal brain injury. It provides additional information when compared to non-enhanced transfontanellar US, especially in the field of brain perfusion assessment. Moreover, the information provided seems to be accurate when compared with those of MRI.

A patient who developed severe sleepiness and sleep apnoea in association with adult acquired retrognathia and subluxation of the cervical spine at the level of C3-C4, both resulting from rheumatoid arthritis, is described. The possible causative factors of the association between sleep apnoea and rheumatoid arthritis include reduction of the size of the upper airway by temporomandibular joint destruction, brainstem compression due to rheumatoid arthritis affecting the cervical spine, sleep fragmentation, and drug effects.

Objectifs Le syndrome d’encephalopathie posterieure reversible (PRES) est une entite clinico-radiologique due a un desordre de l’auto regulation cerebrale. Les causes sont nombreuses. Notre observation rapporte un cas d’hypotension intracrânienne responsable d’un PRES syndrome. Patient et resultats Une patiente de 22 ans sans antecedents particuliers est admise initialement pour torticolis febrile. La ponction lombaire s’avere normale. Le bilan scanographique montre un phlegmon amygdalien retro-pharynge gauche qui est traite par chirurgie. Les suites immediates sont simples. A 48 h la patiente presente 2 crises comitiales tonicocloniques generalisees avec phase post-critique comateuse (Glasgow 6). La tension arterielle etait normale. Un scanner fait en urgence ne montre pas d’arguments pour une thrombose veineuse. L’IRM realisee immediatement apres, montre des lesions caracteristiques d’un PRES syndrome, associees a des ventricules de petite taille, des veines corticales tres dilatees et un elargissement du sinus coronaire inferieur, evoquant une hypotension intracrânienne associee. L’EEG etait normal. Trois tentatives de ponction lombaire apres l’IRM sans obtention de liquide cerebrospinal. L’IRM de controle a 72 h montrait une regression complete des signes de PRES. A j15 normalisation complete clinique et radiologique avec disparition des signes d’hypotension intracrânienne. Discussion Selon la litterature, deux mecanismes peuvent expliquer l’association PRES et hypotension intracrânienne : soit l’augmentation du volume sanguin cerebral par stagnation veineuse, soit l’attraction de l’ampoule de Galien et du sinus droit, entrainant un obstacle au retour veineux et, par consequence, une distension du systeme veineux, une extravasation et des lesions de PRES syndrome. Conclusion Une hypotension intracrânienne responsable d’un PRES syndrome est une observation tres rare avec peu de cas rapportes dans la litterature. Le diagnostic precoce est primordial car en traitant la cause, les lesions sont en general reversibles avec un bon pronostic.

Idiopathic hypereosinophilic syndrome is an uncommon disease often associated with diverse non-specific skin manifestations. Mucosal ulcerations suggest a myeloproliferative from with poor prognosis due to possible progression to malignant hemopathy or visceral complications.A 28-year-old man presented idiopathic hypereosinophilia with isolated mucosal ulcerations involving the buccal and genital areas. Laboratory results (hematology, CD25) suggested a myeloproliferative form. Treatment with alpha interferon (18 months) led to regression of the mucosal lesions and a decrease in the markers of eosinophil toxicity. There was no visceral involvement.Immunosuppression with/without high-dose alpha interferon is usually used for the treatment of hypereosinophilic syndrome. In our case favorable outcome was obtained with lower doses of alpha interferon than those reported in the literature. There was objective decrease in eosinophil toxicity (regular counts of hypodense eosinophils, CD25 or interleukin 2 soluble receptor) and no progression (malignant hemopathy, mortal visceral involvement).

Some skin changes in acromegaly belong to the classical dysmorphic syndrome. Furthermore, other minor skin changes can be associated.We report 3 patients consulting in the department of Dermatology for various skin changes that have lead to the diagnosis of acromegaly. However these patients have also typical dysmorphic syndrome that they have failed to recognize. The fourth patient associated a cutaneous mastocytosis and acromegaly, as reported only once in literature. The evolution of the minor skin changes was parallel with the endocrinopathy. Moreover in one case, they have preceded the recurrence of the acromegaly.Our observations demonstrate the importance of the minor skin changes that lead the patient to consult more than the dysmorphic syndrome. Moreover these changes that depend of growth hormone and other associated growth factors can be evolutive signs for endocrinopathy.

To optimize the funds devoted to health care, and to objectively assess the tools used in monitoring patients with cerebral malaria, we determined two parameters, care charges and direct costs due to hospitalization. During this longitudinal study conducted at the Albert Royer Pediatric Hospital in Dakar from October 15, 1991 to October 15, 1992 with 76 cases, malaria represented 5.2% of the febrile cases and 3.4% of the hospitalized cases. The lethality rate was 3.4%. Clinical features were not correlated with care charges and thus were not good indicators of worker's labor. The average cost of a cerebral malaria hospitalization was estimated at 35,710 F CFA (In October 1992, 1 F CFA = 0.02 FF). This cost is very high depending the limited resources of the region. Rapid and proper treatment of malaria cases may lead to a significant reduction of costs.

Right to left shunts in the adult in their usual location are either intraarticular or intrapulmonary, and are often congenital. The acquired intrapulmonary shunt of the cirrhotic is now well defined by the hepatopulmonary syndrome. The major complication of right to left shunts are paradoxical pulmonary emboli which are often more severe than haemoptysis or chronic hypoxia. The diagnosis of intracardiac shunts as well as intrapulmonary shunts is now easier, thanks to transoesophageal ultrasound associated with contrast. Pulmonary angiography enables the treatment of arteriovenous fistula to be carried out by embolisation, and also avoids any surgery.

Objectifs Evaluation prospective par sequence de diffusion en IRM 3T des ischemies cerebrales silencieuses apres catheterisme des vaisseaux a destinee cerebrale dans un but diagnostique ou therapeutique. Etudier le degre d’installation et la reversibilite de ces lesions et degager les facteurs de risques eventuels. Materiels et methodes Suivi par sequences de diffusion de 3 et 5 mm d’epaisseur en IRM 3Tesla et en sequences T2 FLAIR de 20 patients ayant beneficie d’un catheterisme cerebral a visee diagnostique ou therapeutique. Resultats Nous rapportons le nombre, la taille et la topographie des hypersignaux observes sur les sequences de diffusion 3 et 5 mm d’epaisseur en IRM 3 Tesla et nous les avons comparees avec la sequence T2 FLAIR pour juger de l’evolution a distance de ces hypersignaux. Les hypersignaux vus en sequence de diffusion ont ete confrontes aux donnees cliniques avant et apres la procedure de catheterisme. Conclusion L’etude par sequence de diffusion en IRM 3 Tesla des ischemies cerebrales silencieuses apres catheterisme cerebral semble tres utile pour determiner la frequence de ces ischemies silencieuses et envisager leur prevention.

The effects of extrahepatic cholestasis upon adrenergic regulation of glycogenolysis and upon the numbers of adrenoceptors in rat liver were studied using isolated hepatocytes and plasma membranes, respectively. A 60% decrease in the number of alpha 1 adrenoceptors (285 vs. 680 fmol/mg protein) and a simultaneous 2.7-fold increase in the number of beta adrenergic sites (67 vs. 25 fmol/mg protein) were observed beginning 36 h after bile flow obstruction and persisted for at least 68 h. The reciprocal modification of the numbers of alpha 1 and beta adrenoceptors was accompanied by a change in the manner of stimulation of glycogen phosphorylase by catecholamines in hepatocytes; originally alpha 1 adrenergic in normal rats (phenylephrine Ka = 0.9 microM, isoproterenol Ka = 7.1 microM), the stimulation became predominantly beta adrenergic in cholestatic animals (phenylephrine Ka = 3.7 microM, isoproterenol Ka = 0.06 microM). In normal rats, activation of the enzyme by epinephrine was inhibited by the alpha blocker phentolamine, without inhibition by the beta blocker propranolol. In contrast, propranolol was more effective than phentolamine in cholestatic rat hepatocytes. Modification of the regulation of glycogenolysis after cholestasis did not seem to be secondary to an alteration in the metabolism of thyroid hormones or in the action of glucocorticoids. However, cholestasis provoked a 10-fold increase in the number of hepatic mitoses and in the incorporation of thymidine into liver DNA of cholestatic animals. Similar changes were observed in regenerating livers, following two-thirds hepatectomy. We propose that the changes following extrahepatic cholestasis might, as well, be explained by a regenerative process.

Glucagon and adrenaline exert their action upon the liver via the cyclic AMP synthetizing system located in the plasma membrane. The enzyme adenylate cyclase is further regulated by guanyl nucleotides. It has been recently shown that the rat liver plasma membrane system could respond to GTP by simultaneous increase in the cyclase activity in response to glucagon and by the dissociation of this hormone from its binding sites (1). Unambiguous relationship between the activating effect of GTP upon the cyclase and its action upon glucagon binding has not been determined yet (2). This problem was approached using the in vitro action of epinephrine as a model. When 1 to 100 muM GTP or DGP were added to rat liver plasma membranes isolated from adrenalectomized animals, they increased markedly the response of the cyclase system to epinephrine. These effects could be observed in the absence of an ATP-regenerating system and were mimicked by 5'-guanylyl diphosphonate; GTP and GDP were the most active compounds followed by ITP, CTP and by a series of guanyl derivatives. UTP, as well as guanosine, GMP, cyclic GMP and ppGpp were inactive. Guanyl nucleotides did not increase the affinity of the cyclase system for the activating hormones, but enhanced the affinity for ATP-Mg and also the Vmax of the reaction. Finally, GTP, ATP, CTP, UTP but not GDP displaced epinephrine bound to plasma membranes by a mere chelation phenomenon. It is concluded that 1) guanyl nucleotides do not act primarily by influencing the binding of hormones to the membranes; 2) they act directly upon the catalytic subunit of the cyclase; 3) the low concentrations of GTP required for its action strongly suggest that this nucleotide plays a role in the physiological regulation of the intrahepatic cyclic AMP level.