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Objective: To compare a physical therapy program to usual care on disability of systemic sclerosis (SSc) patients. Methods: A 12-month follow-up, parallel-group randomized controlled trial involving a modified Zelen design was conducted in 4 tertiary-care hospitals. Patients were enrolled if they had a disability rating≥0.5 on the Health Assessment Questionnaire Disability Index (HAQ-DI) or complaints of decreased mouth opening or limited range of motion of at least one joint. The experimental intervention (n=112, 110 analyzed) was a 1-month personalized supervised physical therapy program provided by trained care providers followed by home sessions. The comparator (n=108, 108 analyzed) was usual care that could include ambulatory physical therapy. The primary outcome was the HAQ-DI score. Results: There was no statistically significant difference in disability at 12 months (HAQ-DI between-group difference, -0.01; 95% CI -0.15 to 0.13; p=0.86). Disability was reduced at 1 month for patients in the physical therapy group (HAQ-DI between-group difference -0.14; 95% confidence interval [CI] -0.24 to -0.03; p=.01), at 6 months the HAQDI scores between-group difference was -0.12; 95% CI, -0.23 to 0.01; p = 0.054. There was a statistically significant difference for hand mobility and function, and pain, at 1 month. Microstomia was lower in the physical therapy group at 1, 6 and 12 months (between-group difference at 12 months, 1.62; 95% CI 0.32 to 2.93; p=0.01). No differences in adverse effects were observed. Conclusion: A personalized physical therapy program did not reduce disability at 12 months but had short-term benefits for patients with SSc. This article is protected by copyright. All rights reserved.

Gaucher disease (GD) is a rare genetic lysosomal storage disorder caused by a beta-glucocerebrosidase deficiency and responsible for a lysosomal storage disorder. GD is characterized by haematological, visceral and bone involvements. The aim of this study was to describe the diagnostic journey of type 1 GD patients as well as the role of the internist.A retrospective multicentric study involving type 1 GD patients has been conducted in 16 centers, between 2009 and 2011.Fifty-five type 1 GD patients were included, under the care of an internist or an haematologist. They were originally hospitalized in 8 different specialized units. Diagnosis was established by bone-marrow aspiration in 22 patients (40%), by enzymatic assay of glucocerebrosidase activity in 15 patients (27%), and by bone-marrow biopsy in 9 patients (16%). The use of enzymatic assay became more frequent after 1990. The delay between first hospitalization due to GD symptoms and definitive diagnosis was less than one year for 38 patients. Patients with suspected GD were mainly referred to an internist physician.GD seems to be better recognized and quickly diagnosed since 1990 in spite of the multiplicity of journeys. The role of the internist seems important.

Resume Introduction La foudre touche 240 000 personnes par an dans le monde et sa consequence sur le handicap est importante. Les atteintes les plus frequemment retrouvees sont cerebrales mais il existe une minorite d’atteintes medullaires et peripheriques. Observations Cette etude reprend 5 observations de patients foudroyes ayant presente des atteintes tres differentes. Une patiente a presente des paresthesies douloureuses des deux membres superieurs resolutives en 18 mois, secondaire a une atteinte plexique bilaterale. Une patiente a presente un deficit moteur proximal du membre superieur gauche en rapport avec une atteinte myogene. Deux patients ont presente une atteinte motrice aux membres inferieurs avec une atteinte de la corne anterieure. La derniere patiente a presente une tetraplegie brutale resolutive en 5 minutes, rentrant dans le cadre de la keraunoparalysie. Discussion Les atteintes liees a la foudre sont multiples et repondent a des mecanismes differents. La foudre correspond le plus souvent a une lesion tres focale et sans extension secondaire. En ce qui concerne le motoneurone, le mecanisme est probablement celui d’une myelopathie post-traumatique. Nous discutons le parallele avec l’association sclerose laterale amyotrophique–electrocution longtemps discutee dans la litterature. Conclusion Le systeme nerveux peripherique ainsi que la moelle peuvent etre le siege de lesions a plusieurs niveaux (motoneurone, plexus, muscle, parenchyme cerebral) avec des presentations cliniques atypiques. Leur evolution est le plus souvent favorable.

L’epigenetique etudie les effets potentiels de l’environnement sur l’expression des genes a travers un ensemble de mecanismes moleculaires. Il est admis que les modifications epigenetiques sont reversibles. S’il est indeniable que des facteurs genetiques sont impliques dans les troubles psychiatriques; ceux-ci ne suffisent pas a eux seuls a expliquer le phenotype. Par exemple, on a montre que les souris alcoolo-preferentes ont une histone desacetylase fortement exprimee et donc un BDNF peu acetyle, induisant une diminution de la proteine CREB via une baisse de son niveau d’ARNm. Ce profil serait retrouve chez les sujets alcoolodependants. Par ailleurs, dans le cadre des psychoses, l’etude de jumeaux homozygotes montre que l’un des jumeaux presente 50 % de risque d’etre atteint si son frere jumeau l’est deja, mais cette proportion n’atteint pas 100 %, alors qu’ils possedent le meme code genetique. Ceci nous a conduit a nous interesser aux interactions gene–environnement (G x E). On retrouve des differences significatives de methylation de genes impliques dans les systemes GABA-ergiques et glutaminergiques chez les patients atteints de psychoses par rapport aux sujets sains. Des differences de methylation sont egalement observees dans les promoteurs des genes 5HTR1 et 5HTRA2, et il est maintenant connu que la dysregulation du systeme serotoninergique est impliquee dans le developpement de psychoses. Concernant la depression, le transporteur de la serotonine 5-HTT regule sa concentration dans la synapse. Sa fonction est modulee par la region polymorphe du promoteur du gene SLC6A4: 5-HTTLPR. Les sujets depressifs ont une augmentation de la methylation du promoteur 5-HTTLPR et donc une diminution de son expression. Une autre etude comparant les niveaux de methylation avant et apres six semaines de traitement antidepresseur a montre qu’une augmentation de la methylation de l’ADN au site CpG de SLC6A4 etait associee a une meilleure reponse therapeutique chez les ces patients. L’epigenetique est un domaine en plein essor mais insuffisamment etudie a ce jour en psychiatrie. Le diagnostic, le pronostic et la decision therapeutique reposent sur des criteres cliniques. Il est possible qu’a l’avenir l’epigenetique nous apporte des perspectives en tant que facteurs predictifs et donc de decision therapeutique.

Resume Propos Determiner les maladies associees a un taux plasmatique eleve de vitamine B12 et mesurer la force de l’association. Patients et methodes Enquete retrospective incluant les sejours hospitaliers du premier mai 2005 au 30 avril 2008 dans les services du pole UMAG de notre hopital (urgences, medecine interne, geriatrie aigue et reanimation medicale) et ayant eu un dosage de vitamine B12. L’association entre chacun des codes Programme de medicalisation des systemes d’information (PMSI) (cancers solides, hemopathies malignes et maladies renales) et une valeur basse ou elevee de vitamine B12 (B12) a ete mesuree par l’ odds ratio (OR) a partir d’une regression logistique prenant en compte les hospitalisations multiples, avec ajustement sur l’âge et l’indice de comorbidite de Charlson pondere. Resultats Parmi les 3702 sejours, 12 % avaient une B12 superieure a 820 pg/mL, 10,4 % une B12 inferieure a 180 pg/mL et 77,6 % une B12 normale. Apres ajustement sur l’âge et l’indice de Charlson pondere, une B12 elevee etait associee aux maladies interstitielles renales (OR 2,7 ; (IC95 % : [1,7–4,2]) et aux cirrhoses ou hepatites (OR 4,3 ; [(2,9–6,4]). Apres ajustement supplementaire sur ces parametres, la B12 restait independamment associee aux cancers (OR 1,8 ; [1,2–2,6]), aux hemopathies malignes (OR 2,1 ; |1,3–3,5]), aux metastases OR 2,9 (1,5–5,9), aux metastases hepatiques (OR 6,2 ; [2,7–14,5]), aux carcinomes hepatocellulaires (CHC) (OR 3,3 ; [1,1–10,4]), aux cancers du foie hors CHC (OR 4,7 ; [1,2–17,9]) et aux lymphomes (OR 3,2 ; [1,6–6,4]) mais pas au myelome (OR 1 ; [0,6–1,4]). Un taux bas de B12 etait associee au myelome (OR 2,9 ; [1,3–6,6]). Conclusion La decouverte d’un taux eleve de vitamine B12 doit conduire a rechercher de maniere systematique une maladie hepatique ou tumorale, et en premier lieu une localisation tumorale hepatique.

Objectives Systemic sclerosis (SSc) is a heterogeneous autoimmune disease associated with several antinuclear autoantibodies useful to diagnosis and prognosis. The aim of the present multicentric study was to determine the clinical relevance of anti-fibrillarin autoantibodies (AFA) in patients with SSc. Methods The clinical features of 37 SSc patients positive for AFA (AFA+) and 139 SSc patients without AFA (AFA-) were collected retrospectively from medical records to enable a comparison between AFA- and AFA+ patients. Anti-fibrillarin autoantibodies were screened by an indirect immunofluorescence technique using HEp2 cells and identified by an in-house Western blot technique and/or an EliA test. Results Comparing AFA+ and AFA– patients, AFA+ patients were significantly younger at disease onset (36.9 vs. 42.9; p=0.02), more frequently male (p=0.02) and of Afro-Caribbean descent (65% vs. 7.7%; p

Vi sono tre tipi di singhiozzo: il singhiozzo isolato, una contrazione organizzata dei muscoli respiratori, breve, unica, involontaria e quotidiana, che passa spesso inosservata. E un fenomeno quotidiano, fisiologico ad ogni eta; il singhiozzo acuto, caratterizzato da scosse ripetitive che durano meno di 48 ore, di cui il soggetto e cosciente. E un fenomeno che disturba ma senza gravita, che ognuno conosce e che fa sorridere; e un’esperienza banale che si osserva ad ogni eta e la cui prognosi e eccellente. Esso richiede poca o nessuna attivita medica e regredisce spontaneamente; il singhiozzo cronico, caratterizzato da scosse ripetitive che durano piu di 48 ore, con una prognosi riservata; infatti, puo essere il sintomo di una malattia o di una complicanza patologica sottostante, prima di tutto esofagea, ed e spesso refrattario. La sua prognosi e la prosecuzione a lungo termine delle scosse a frequenza variabile, spesso con una periodicita di alcuni giorni o di alcune settimane al mese. Esso e all’origine di un’invalidita significativa. Spesso rivelatore di una patologia grave sottostante, e, quindi, il solo singhiozzo che implica una vera gestione medica la cui priorita e la ricerca della causa per tentare un trattamento eziologico. Le patologie esofago-gastriche, dominate dall’esofagite da reflusso, sono state riconosciute come eziologia principale del singhiozzo cronico. A molta distanza seguono i singhiozzi di causa toracica, addominale, cerebrale o, anche, psichica.

Resume Introduction La dystrophie musculaire de Becker est habituellement symptomatique des l’enfance. Apparentee a la dystrophie musculaire de Duchenne, elle est caracterisee par une plus grande heterogeneite, ainsi qu’une moindre severite dans ses aspects cliniques. Observation Nous rapportons une observation de myopathie de Becker a revelation tardive chez un homme de 54 ans et analysons 12 autres observations a debut tardif de la litterature. L’âge de debut des symptomes varie entre 30 et 65 ans. La deletion genetique porte majoritairement (7/13) sur le domaine des exons 45 a 55 avec un phenotype variable : hypertrophie musculaire distale (4/13), atteinte cardiaque (electrocardiographie, radiographique ou echographique) chez six des 12 patients explores. Le retentissement fonctionnel semble egalement moindre, seulement trois des dix patients dont on dispose d’un suivi entre sept et 20 ans sont en fauteuil roulant. Conclusion Comme les formes pediatriques, de loin les plus nombreuses, les myopathies de Becker a revelation tardive, au dela de 30 ans, ont un phenotype variable mais elles s’en distinguent par une severite moindre.

Little is known about systemic sclerosis (SSc)-related myopathy. We aimed to compare the clinical and immunological features of SSc patients with or without associated myopathy.Forty SSc patients with myopathy, defined by myalgia or muscle weakness associated with creatine kinase (CK) more than five times the upper limit range or myopathic electromyography (EMG) or abnormal myopathology, were identified from the records of four French hospital centres. For each patient, we selected two SSc controls matched for cutaneous SSc form, sex, age at SSc onset, and disease duration. We performed a case-control study testing clinical and immunological SSc-related features for association with myopathy by conditional logistic regression.Muscle and SSc features of patients with myopathy did not differ significantly among the four centres of origin. Only four (10%) patients with SSc-associated myopathy had anti-polymyositis-scleroderma (PM-Scl) antibodies. Case-control univariate analysis revealed that reduced forced vital capacity (FVC) [odds ratio (OR) 3.0, 95% confidence interval (CI) 1.3-34.9], heart involvement, defined as clinical congestive heart failure, left ventricular ejection fraction (LVEF)60%, arrhythmia or conductive abnormalities (OR 2.9, 95% CI 1.3-6.5), and scleroderma renal crisis (OR 3.0, 95% CI 1.3-34.9) were significantly more frequent in patients with myopathy than in controls. Two autoantibodies were more frequent in patients with myopathy: anti-PM-Scl (OR 5.0, 95% CI 1.1-23.9) and anti-RNP (OR 6.9, 95% CI 1.1-64.4). Multivariate analysis retained two variables associated positively with myopathy [reduced FVC (OR 3.1, 95% CI 1.3-9.8) and heart involvement (OR 2.5, 95% CI 1.1-7.1)], while anti-centromere antibodies were associated negatively (OR 0.11, 95% CI 0.03-0.53).Heart monitoring of SSc patients with myopathy should be undertaken regularly because of the association of myocardial and skeletal myopathies in such patients.

Resume Introduction Parmi les manifestations cliniques des infections a Neisseria meningitidis, la meningococcemie chronique est singuliere quant a sa semiologie et son profil evolutif. Observation Nous rapportons l’observation d’un homme de 32 ans hospitalise pour exploration d’une fievre evoluant depuis 15 jours associee a une eruption cutanee maculopapuleuse diffuse et fugace, sans aucun signe de syndrome meninge ou de sepsis severe. Une hemoculture revenait positive a N. meningitidis (serotype B). L’evolution etait favorable sous antibiotherapie. Conclusion La reconnaissance precoce de cette entite a partir de ses caracteristiques cliniques est essentielle pour une prise en charge efficace du patient et des sujets contacts. La meningococcemie chronique doit etre evoquee devant la triade fievre, eruption cutanee et arthralgies, et ne doit pas etre confondue avec une vascularite systemique car la prescription inadequate d’une corticotherapie peut engendrer des complications severes.

Resume Introduction La chimiokine CXCL10 induite par l’interferon γ serait impliquee dans la physiopathologie de la pneumopathie interstitielle diffuse (PID) de la sclerodermie systemique (ScS). L’objectif de cette etude etait d’evaluer les taux seriques de CXCL10 chez des patients ayant une ScS. Methodes CXCL10 a ete dosee par methode Elisa dans le serum de 23 volontaires sains (mediane : 60,0 ans ; 58,0–67,3) et 29 patients atteints de ScS (63,1 ans ; 60,1–69,4). Ces derniers ont egalement eu des explorations fonctionnelles respiratoires, un examen tomodensitometrique du thorax et une echographie cardiaque. Les taux de CXCL10 (mediane et interquartile 25–75 %) ont ete compares entre patients et volontaires sains, et entre patients avec et sans PID. Resultats Les patients avaient un taux serique median de CXCL10 plus eleve que les volontaires sains (110,0 pg/ml ; 60,8–223,8 pg/ml contre 52,0 pg/ml ; 41,3–65,8 ; p Conclusion Ces resultats montrent l’implication de CXCL10 dans la physiopathologie de la ScS et suggerent plus particulierement une relation a la PID rencontree au cours de cette maladie.

Background Localized scleroderma also called morphea is a skin disorder of undetermined cause. The widely recognized Mayo Clinic Classification identifies 5 main morphea types: plaque, generalized, bullous, linear and deep. Whether each of these distinct types has a particular clinical course or is associated with some patient-related features is still unclear. Methods We report here a retrospective series of patients with localized scleroderma with an attempt to identify features related to the type of lesion involved. The medical records of all patients with a diagnosis of localized scleroderma were reviewed by skilled practitioners. Lesions were classified according to the Mayo Clinic Classification. The relationship between each lesion type and various clinical features was tested by non-parametrical methods. Results The sample of 52 patients included 43 females and 9 males. Median age at onset was 30 y (range 1–76). Frequencies of patients according to morphea types were: plaque morphea 41 (78.8%) (including morphea en plaque 30 (57.7%) and atrophoderma of Pasini–Pierini 11 (21.1%)), linear scleroderma 14 (26.9%). Nine patients (17.3%) had both types of localized scleroderma. Median age at onset was lower in patients with linear scleroderma (8 y (range 3–44)) than in others (36 y (range 1–77)) (p = 0.0003). Head involvement was more common in patients with linear scleroderma (37.5%) than in other subtypes (11.1%) (p = 0.05). Atrophoderma of Pasini–Pierini was never located at the head. Systemic symptoms, antinuclear antibodies and the rheumatic factor were not associated with localized scleroderma types or subtypes. Conclusion These results suggest that morphea types, in adults are not associated with distinct patient features except for age at disease onset (lower) and the localization on the head (more frequent), in patients with lesions of the linear type.

Resume Introduction Dans le spectre des desordres microangiopathiques de la grossesse, le syndrome catastrophique des antiphospholipides (SCAP) tient une place particuliere de par sa severite mettant en jeu le pronostic fœtal et maternel. Son atteinte multisystemique, en cas de presentation hepatique predominante, peut etre confondue avec un syndrome HELLP qui survient habituellement plus tard au cours de la grossesse. Observation Nous rapportons une observation de SCAP probable avec atteinte hepatique chez une femme enceinte a 13 semaines d’amenorrhee, porteuse d’un syndrome des antiphospholipides (SAPL), et presentant des stigmates biologiques de syndrome HELLP. L’imagerie de l’atteinte hepatique, non specifique, bien documentee chez notre patiente a permis une prise en charge therapeutique precoce. Conclusion Le SCAP et le syndrome HELLP sont des pathologies microthrombotiques severes pouvant etre associees. La distinction nosologique ne modifie pas la prise en charge qui constitue une urgence materno-fœtale, mais leur association impose un traitement immediat plus agressif.

Objective There is now growing evidence that connective tissue diseases, including systemic sclerosis (SSc), share a common genetic background. Microarray studies support a pivotal role of type I interferon (IFN) in the pathophysiology of connective tissue diseases. Interferon regulatory factors coordinate the expression of type I IFNs, and the IRF5 gene has been identified as a susceptibility gene of systemic lupus and Sjogren's syndrome. The aim of this study was to determine whether the IRF5 rs2004640 single-nucleotide polymorphism is associated with SSc. Methods The IRF5 rs2004640 (GT) functional polymorphism was genotyped in 1,641 subjects of French European Caucasian origin: a discovery set comprising 427 patients with SSc and 380 control subjects and a replication set comprising 454 patients with SSc and 380 control subjects. Results In both the discovery set and the replication set, the TT genotype was significantly more common in patients with SSc than in control subjects, with an odds ratio (OR) for the combined populations of 1.58 (95% confidence interval [95% CI] 1.18–2.11 [P for trend 0.002]). Analyses of the whole SSc population showed a significant association between homozygosity for the T allele and the presence of antinuclear antibodies (corrected P [Pcorr] = 0.04, OR 1.59, 95% CI 1.16–2.17) and fibrosing alveolitis (Pcorr = 0.001, OR 2.07, 95% CI 1.38–3.11). In a multivariate analysis model including the diffuse cutaneous subtype of SSc and positivity for anti–topoisomerase I antibodies, the IRF5 rs2004640 TT genotype remained associated with fibrosing alveolitis (P = 0.029, OR 1.92, 95% CI 1.07–3.44). Conclusion The IRF5 rs2004640 GT substitution is associated with susceptibility to SSc. These data provide new insight into the pathogenesis of SSc, including clues to the mechanisms leading to fibrosing alveolitis.

Resume Le syndrome de Munchausen est defini par la triade : troubles factices d’allure aigue conduisant a des investigations ou des traitements inutiles, peregrinations d’un hopital a l’autre et fabulations infiltrant la biographie. Nous rapportons l’observation d’une femme de 20 ans, vue a la consultation de medecine interne de notre hopital avec des resultats biologiques evoquant une leucemie. La numeration avec formule sanguine de controle et le myelogramme par ponction sternale etaient normaux. L’analyse comparative des feuilles d’examen permit de conclure a une falsification des resultats faisant porter le diagnostic de syndrome de Munchausen. Avec Internet, qui permet un acces quasi illimite a l’information et celui des logiciels de traitement de texte ou de retouche photographique, il devient aise de falsifier des resultats biologiques dans le cadre d’un syndrome de Munchausen non pas clinique mais « paraclinique », particulierement difficile a deceler lors d’une simple consultation.

Objective To determine whether genetic variants of the PTPN22 gene, including the R620W (1858C>T) missense single-nucleotide polymorphism (SNP), are associated with systemic sclerosis (SSc). Since PTPN22 is involved in multiple autoimmune diseases, we also examined the occurrence of a concomitant autoimmune disease. We then conducted a meta-analysis of the most recent studies of SSc in order to verify the association or lack of association between the PTPN22 1858C>T variant and SSc. Methods Seven PTPN22 SNPs were analyzed in a French Caucasian cohort of 659 SSc patients and 504 healthy controls. All SSc patient sera were tested for the presence of autoantibodies against topoisomerase I (anti–topo I) and for anticentromere antibodies (ACAs). Results The co-occurrence of an autoimmune disease was observed in 22% of the 416 SSc patients who were exhaustively screened. In 33 of the 416 patients (8%), the concomitant autoimmune disease was known to be associated with PTPN22 1858T; these patients were excluded prior to analysis. No association was detected for any of the SNPs tested. PTPN22 haplotype analysis identified a strong association between SSc and the presence of a risk haplotype carrying the 1858T allele (P = 1.52 × 10–7) and a protective haplotype carrying the 1858C allele (P = 2.20 × 10–16) in our French Caucasian population. The meta-analysis provided evidence that the PTPN22 1858T allele is involved in the genetic susceptibility to SSc in Caucasian (P = 8.39 × 10–3, OR 1.08 [95% CI 1.02–1.15]) and mixed (P = 3.11 × 10–3, OR 1.09 [95% CI 1.04–1.16]) populations, particularly in the anti–topo I–positive subset. Conclusion Our results indicate that PTPN22, a shared genetic factor of multiple autoimmune diseases, also contributes to the genetic background of SSc.

Objectives: There is increasing concern about heart and pulmonary vascular involvement in systemic sclerosis (SSc). One of the most severe complications of SSc is pulmonary arterial hypertension (PAH). There has been an increased awareness of left ventricular (LV) diastolic abnormalities in SSc patients. However, previous studies have generally been conducted in small populations. The aims of this study were to prospectively screen for PAH and to describe echocardiographic parameters in a large group of SSc patients. Methods: This prospective study was conducted in 21 centres for SSc in France. Patients without severe pulmonary function abnormalities, severe cardiac disease and known PAH underwent Doppler echocardiography performed by a reference cardiologist. Results: Of the 570 patients evaluated, PAH was suspected in 33 patients and was confirmed in 18 by right heart catheterisation. LV systolic dysfunction was rare (1.4%). LV hypertrophy was found in 22.6%, with LV diastolic dysfunction in 17.7%. These LV abnormalities were influenced by age, gender and blood pressure. We identified a small group of 21 patients with a restrictive mitral flow pattern in the absence of any other cardiopulmonary diseases, suggesting a specific cardiac involvement in SSc. Conclusions: Left and right heart diseases, including PAH, LV hypertrophy and diastolic dysfunction, are common in SSc. However, a small subset of patients without any cardiac or pulmonary diseases have a restrictive mitral flow pattern that could be due to primary cardiac involvement of SSc. The prognostic implications of the LV abnormalities will be evaluated in the 3-year follow-up of this cohort.

Resume Propos La valeur pronostique des parametres biochimiques a l'admission est encore mal connue en medecine interne. Nous avons examine dans une population retrospective de medecine interne, les relations entre mortalite hospitaliere ou duree de sejour et la concentration de plusieurs parametres biochimiques courants a l'admission. Methodes Pour chaque sejour hospitalier non programme dans notre service en 2004 nous avons collige huit parametres biochimiques sanguins courants (sodium, potassium, chlore, reserve alcaline, trou anionique, uree, creatinine, protides totaux), mesures au cours du jour precedant ou suivant l'admission hospitaliere. Un modele de Cox mixte evaluait le risque de mortalite associe aux valeurs des parametres biochimiques. Un modele lineaire mixte testait la relation entre ces derniers et la duree de sejour. Resultats En 2004, le service totalisait 1199 sejours soit 1054 patients distincts (âge : 69,9 ± 19,2 ans, femmes : 59,2 %) parmi lesquels 59 decedes en cours de sejour. Les parametres biochimiques d'interet etaient disponibles pour 977 (81,5 %) de ces sejours (duree de sejour : 17,5 ± 16,0 jours). La mortalite hospitaliere etait significativement associee aux concentrations plasmatiques initiales du potassium, des protides, a la valeur du trou anionique ainsi qu'au rapport uree/creatinine. Chez les survivants, la duree de sejour etait significativement associee aux dosages initiaux de la natremie, de la chloremie ainsi qu'a la valeur du trou anionique. Conclusions Les examens biochimiques d'entree fournissent une information precoce sur le pronostic a court terme des patients hospitalises en medecine interne et meritent a ce titre une attention particuliere.

Objectives. Systemic sclerosis (SSc) has an heterogenous clinical pattern, with variable organ involvement and degrees of severity. Like in other rheumatic diseases, the self-questionnaires have been used to evaluate SSc globally. The aim of the study is as to evaluate the quality of life (QoL) in patients with either diffuse or limited SSc, and to examine the impact of pain on the QoL scores. Methods. Patients with SSc, eitheir diffuse or limited SSc, were included in a cross-sectional study. The QoL was evaluated with the short-form 36 (SF-36) and the functional repercussion with the SSc-modified Health Assessment Questionnaire (S-HAQ). Results. A total of 89 patients (67 with diffuse and 22 with limited SSc) were included. The SF-36 score values were lower in SSc patients than those reported in the general population. The physical component scores (PCS) of the SF-36 was significantly worse in diffuse compared with limited SSc (P < 0.05). The PCS was significantly negatively related to the number of clinical manifestations (ANOVA, P < 0.0001). The mental component score (MCS) was not influenced by the type of SSc or the number of clinical manifestations presented by the patient. The QoL of SSc patients was highly correlated with pain (R ¼ 0.69) and disability (R ¼ 0.70). Interestingly, the QoL of SSc patients was only slightly correlated with cutaneous (R ¼ 0.42) and pulmonary involvement (R ¼ 0.57). Conclusion. The QoL of patients with SSc is strongly influenced by the type of SSc, the burden of clinical manifestations, the functional disability and by the pain, whatever its cause. The treatment of pain should be considered as priority to improve the QoL of SSc patients.

Resume Propos et objectifs L’hypertension arterielle pulmonaire (HTAP) represente l’une des graves complications menacant la vie des patients atteints de sclerodermie systemique (ScS). En France, les modalites de depistage et de gestion de l’HTAP associee a la ScS ne sont pas encore bien codifiees et ne font pas l’objet d’un consensus. Nous avons mene une enquete aupres des medecins inscrits sur la liste du Groupe de recherche francais sur la sclerodermie (GRFS) pour faire un etat des lieux de la prise en charge de l’HTAP associee a la ScS. Methodes Nous avons envoye en 2002 un questionnaire a 160 medecins membres du GRFS, pour evaluer l’epidemiologie et la clinique des patients ScS, ainsi que les modalites de depistage et de gestion de l’HTAP chez ces patients. Resultats Quatre-vingt-huit medecins dans 71 centres ont repondu a ce questionnaire. Chaque centre suivait en moyenne 33 ScS, avec une repartition globale de 53 % de ScS limitees et de 47 % de ScS diffuses. Ces medecins voyaient en moyenne 5 nouveaux cas de ScS par an. Les patients leur etaient adresses par les confreres de ville (53 %) ou d’hopital (47 %). Le nombre moyen de patients ScS + HTAP etait de 5,1 par medecin (1,5 nouveaux patients ScS + HTAP par an). La quasi-totalite (65/67) des centres ayant repondu depistaient systematiquement l’HTAP chez les patients ScS, par echo-Doppler cardiaque, en moyenne tous les 1,3 ans. La majorite (41/63) des centres collaboraient, pour la gestion de leurs patients atteints d’HTAP, avec un service specialise. Environ un tiers de ces centres traitaient ces patients par inhibiteurs calciques (82 %) et/ou prostacycline (90 %). Le suivi des patients se faisait par l’echographie Doppler cardiaque dans tous les cas. La majorite des medecins (72 %) etaient interesses par un protocole de recherche sur ce sujet et auraient pu chacun y inclure 4 patients, soit un total de 160. Conclusion L’HTAP, complication severe de la ScS, est depistee par les medecins du GRFS avec l’echographie cardiaque a une frequence se rapprochant des recommandations de l’OMS (1,3 versus 1 fois/an).

The modified Scleroderma Health Assessment Questionnaire (SSc HAQ) is a functional score to assess systemic sclerosis (SSc) comprising the HAQ disease index (HAQ-DI) plus five specific visual analogue scales (VAS). Since it was validated in English-speaking patients only, its general use in any other language necessitates prior cross-cultural adaptation and validation. We designed this study to assess its value in French-speaking patients and to validate the French version according to international recommendations. We elaborated a French version using the "forward-backward" method. We then validated its psychometric properties with 100 consecutive SSc French-speaking patients who had undergone simultaneous clinical and paraclinical examination. In addition, we calculated the SSc HAQ score, a new outcome measure, which is obtained by pooling the eight domains from the HAQ-DI with the five organ VAS. Our study confirmed the psychometric properties of the SSc HAQ in non-English-speaking patients with (a) structural validity: the major component analysis, performed on the HAQ-DI and the five VAS, yielding a two-factor structure; (b) convergent validity: with high correlation coefficients between the SSc HAQ score and the physical component score of the SF-36 (r=-0.74, p0.0001); (c) discriminant validity: the SSc HAQ score was better in patients with limited than with diffuse SSc (0.5+/-0.5 vs 1.1+/-0.7, respectively, p0.0001) in relation to the number of clinical involvements; (d) reproducibility was high using the test-retest procedure (r=0.98). This study showed the value of the SSc HAQ, which is a simple, discriminant, reproducible self-administered questionnaire to evaluate French-speaking SSc patients. In addition, we suggest the use of a new outcome measure, the SSc HAQ score, to assess this systemic disease more accurately.

Resume Propos. – Evaluer l’utilisation du Medical Outcome Study Short Form 36 (SF-36), score evaluant la qualite de vie, dans la sclerodermie systemique et apprecier l’impact de cette maladie sur la qualite de vie. Methodes. – Etude transversale de 86 patients atteints de sclerodermie systemique (66 femmes et 20 hommes, 64 formes diffuses, 22 formes limitees). La qualite de vie a ete evaluee par le score SF-36. La severite de la maladie a ete evaluee par l’examen clinique, des tests paracliniques et le score de retentissement fonctionnel S–HAQ (HAQ modifie pour la sclerodermie). Resultats. – Les scores du SF-36 sont plus bas (moins bonne qualite de vie) dans les formes diffuses que dans les formes limitees de sclerodermie systemique. La composante physique du score de la qualite de vie est plus alteree en presence qu’en l’absence des atteintes cliniques, et ce d’autant plus que le nombre d’atteintes augmente. La qualite de vie des patients atteints de sclerodermie systemique est correlee au retentissement fonctionnel de la maladie. Conclusion. – La qualite de vie des patients atteints de sclerodermie systemique est correlee a la severite de l’atteinte clinique de la maladie. Le SF-36 est un outil utile pour l’evaluation clinique des patients atteints de sclerodermie systemique.

Objective – Multiple sclerosis (MS) is by far the most popular diagnosis for patients with multifocal neurological disease. Owing to demyelinating inflammatory non-necrotic plaques of the white matter, MS can give remitting symptoms of virtually every part of the central nervous system. Corticosteroids are usually helpful. Devic's neuromyelitis optica (DNMO) is a neurological disease involving only the optic nerves and the spinal cord, where demyelination evolves towards necrosis and atrophy; the prognosis is poor and no satisfactory treatment is known. The objectives of this study are to describe clinical, biological, pathological and radiological data of patients with DNMO and to differentiate DNMO from MS. Material and methods – We studied the files of 14 patients diagnosed with possible DNMO in three French hospitals between 1980 and 1999 and reviewed the literature. Results – Nine patients were included as definite DNMO. Five were excluded because they did not fulfil the diagnostic criteria. For the nine patients with definite DNMO, DNMO was either monophasic or multiphasic. The prognosis was generally poor: two patients died and five others developed severe disability such as blindness, para or quadriplegia or both. Cerebrospinal fluid study and neuroimaging were essential to confirm the diagnosis of DNMO. Various immunosuppressive treatments generally failed to benefit the patients. Conclusion – In the literature (as well as our 14 initial patients) only a few cases of patients described as suffering from DNMO fulfilled the diagnostic criteria. The others showed evidence that another disease like MS was involved. We stress that inclusion and exclusion criteria have to be kept in mind to differentiate clearly DNMO from MS and other central nervous system white matter diseases.

The cannabinoid hyperemesis syndrome (CHS) is characterized by cyclic episodes of nausea, vomiting, and abdominal pain, and occurs in young adults with long-term cannabis use. The feature of this syndrome is the relief of symptoms with hot showers. We report here six cases report.Three women and 3 men, chronic consumers of cannabis, presented with typical features of the CHS, but the syndrome remained undiagnosed until then: nausea, vomiting, abdominal pain with morning ascendancy and loss of weight occurring in adult's of less than 50 years. The symptoms were improved by taking repeated hot showers. The medical investigations were negative. After addictologist care and cannabis weaning, digestive symptoms disappeared.The CHS is based on a clinical diagnosis. When undiagnosed, clinical presentation leads to a medical wandering and to the realization of repeated, expensive, and sometimes invasive exams. The physiopathology is not clear. The treatment relies on the definitive cannabis weaning.

Introduction The purpose of this study was to estimate the prevalence of monoclonal immunoglobulin (MIg) among patients with systemic sclerosis (SSc) according to the capillary electrophoresis or immunofixation method of detection and to search for any related clinical correlations. Patients and methods Retrospective multicenter comparison of capillary electrophoresis and immunofixation results in SSc patients and of the characteristics of patients with and without MIg. Results The study included 244 SSc patients (216 women and 28 men, mean age: 55 ± 14 years). Median time since SSc diagnosis was 51 months [0–320]; disease was diffuse in 48% of cases. Ten percent of patients had cancer, including Waldenstrom macroglobulinemia (n = 1) and multiple myeloma (n = 3). Capillary electrophoresis showed a γ-globulin anomaly in 41% of cases, and immunofixation in 18%: MIg (13.5%) and restriction of heterogeneity (4.5%). Capillary electrophoresis failed to detect 60% of the 33 MIg patients. Measurable MIg concentrations were obtained from 7 patients. MIg patients were significantly older at SSc diagnosis than those without MIg (p = 0.002), had a lower diffusing capacity (p = 0.002), a higher prevalence of pulmonary hypertension and cancer (p = 0.002) and were more frequently positive for anti-mitochondrial and anti-beta2-glycoprotein-I antibodies (p = 0.03 and p = 0.02, respectively). Multivariate analyses showed that only age at test [hazard ratio 1.03 (95% CI, 1.00–1.07, p = 0.04)] and presence of cancer [hazard ratio 4.46 (95% CI, 1.6–12.4, p = 0.004)] were associated with MIg. Conclusion Immunofixation detected a high prevalence of MIg among SSc patients especially in patients aged 50-years or older. MIg was not detected by the standard capillary electrophoresis in 60% of cases and was significantly associated with cancer.

Periungueal capillaroscopy is a simple and reliable non-invasive technique allowing evaluation of cutaneous microcirculation. It was promoted for decades in patients with Raynaud's phenomenon in order to differentiate between the benign primary Raynaud's phenomenon and the secondary form in connective tissue diseases, especially systemic sclerosis. Nevertheless, the value of this procedure has also been shown in numerous pathologies such as diabetes or cardiovascular diseases. This literature review points to the versatility of this useful exam and its results in a large spectrum of diseases with microvascular involvement.

Inclusion body myositis, although rare, is the commonest cause of myopathy in patients aged over 55 years. We report a 62-year-old man presenting a glycogen storage disease type 2 mimicking an inclusion body myositis.

Cholesterol crystal embolism is observed in polyvascular patients usually following a medical procedure. We report a 93-year-old patient with lower limb mononeuropathy in whom cholesterol crystal embolism was confirmed by neuromuscular biopsy. Previously, an ophthalmoscopy and a livedo biopsy had been performed and the results were normal. Mononeuropathy simplex or multiplex is a clinical expression for cholesterol crystal embolism that may thus be confounded with polyarteritis nodosa. Neuromuscular biopsy can provide accurate diagnosis if other investigations are inconclusive.

We report a 74-year-old man with severe chronic primary neutropenia (neutrophil count: 390 per millimeter cube) uncovered following surgery for perianal abscess collection. Clinical, laboratory and roentgenographic findings revealed no abnormality. Antineutrophil antibodies were positive in two consecutive serum samples. Under cyclosporine, neutrophil count reached 1970 per millimeter cube. However, this therapy was discontinued due to new onset of severe renal failure. After six weeks, neutrophil count was 950 per millimeter cube and sirolimus was started, resulting in renal function improvement and resolution of neutropenia.

BACKGROUND: Acquired hemophilia is a rare disease caused by the development of auto-antibodies against factor VIII. SUBJECTS AND METHODS: We studied the characteristics and outcomes of 34 patients (19 women and 15 men) with acquired hemophilia from 1980 to 1997. RESULTS: The mean age of the patients was 61 years (range, 22–93 years). An underlying disease was observed in 18 (53%) patients: 5 patients had cancer, 4 an autoimmune disorder, 2 a dermatologic disorder, 3 asthma, 3 were postpartum, and 1 had an adverse reaction to ampicillin. Factor VIII level was 10 Bethesda units) in 23 (69%) patients. Bleeding requiring transfusions was reported in 25 (75%) patients. Human factor VIII was given to 14 patients and porcine factor VIII to 5. Six patients received prothrombin complex concentrates and one desmopressin. Several immunosuppressive treatments were used, mainly corticosteroids, cyclophosphamide, and intravenous immunoglobulin. Bleeding stopped in all but one patient within 2 weeks. Most patients achieved complete remission, although two relapses were observed subsequently. CONCLUSION: This large study helps to clarify the presentation and clinical course of acquired hemophilia. Prospective studies are needed to determine the efficacy of treatment.

La cinetique tres rapide de l’oxydation interne rend difficile le passage a l’oxydation externe.La complexite de l’oxydation des alliages concentres ne permet pas l’obtention d’une interface metal-oxyde bien definie. La formation de « bandes » est expliquee par une evolution comprenant des stades de deformation suivis de stades de relaxation. L’oxydation d’alliages dilues, sous tres faibles pressions d’oxygene, est la meilleure solution pour realiser une couche continue d’oxyde externe.

Severe strongyloidiasis, caused by Strongyloides stercoralis, is a preventable life-threatening disease that can occur in any corticosteroid-treated patient who has travelled to a country with infested soil, even if the contact occurred up to 30 years previously. This diagnosis should be considered in corticosteroid-treated patients who experience either unusual gastrointestinal or pulmonary symptoms, or who suffer from unexplained sepsis caused by Gram-negative bacilli. Peripheral eosinophilia is not observed systematically and, even if present, is moderate in most cases. Ivermectine is the best prophylactic and therapeutic option, and thiabendazole should no longer be used. However, guidelines for the prevention and management of S. stercoralis infection in such patients have not yet been established.

In this paper, we report kinetics of sulphur surface segregation obtained by Auger electron spectroscopy (AES) in pure iron and in FeMo (0.5, 2 and 3.5 at%) alloys over the temperature range 500–630°C. Moreover, we investigate sulphur equilibrium surface adsorption at 800°C using radioactive 35 S. We show first that the maximum sulphur amount increases with bulk Mo content at 800°C and second, that sulphur strongly segregates in the temperature range [550–600°C] on Fe and FeMo alloys even if the maximum S segregated quantities are always lower than the maximum sulphur amount obtained in adsorption experiments. At high temperature, the S segregation kinetics observed are governed in a first stage by grain boundary and pipe diffusion. A first equilibrium state corresponding to the S and Mo surface cosegregation on FeMo alloys (comparable with S segregation on iron) has to be considered. 2D (Fe, Mo) sulphides in which the most of Mo atoms are located near the surface (and not in the substitutional sites of the first layer) are formed in place of 2D (Fe) sulphide. The second point concerns nitrogen which is a residual impurity in FeMo alloys. At low temperature (520°C), a superficial 2D Mo nitride (second equilibrium state) is formed. However, as sulphur is the most active species in the FeMo alloys, non equilibrium states and site competition are observed in an intermediate temperature range.

Every year, 240,000 people are struck by lightning worldwide, causing injuries leading to significant handicaps. Most of the symptoms involve brain lesions; neuromuscular sequelae and myelopathy are less common.We describe five cases of patients struck by lightning with various clinical presentations. The first patient presented painful paresthesias in both upper limbs that disappeared 18 months later; the injury was a plexopathy. The second patient developed proximal weakness in the upper-left limb due to a myopathy. Two patients presented with various motor weaknesses in the lower limbs due to motor neuron disease and myelopathy. The last patient had a transient tetraplegy, which resolved in 5minutes; the diagnosis was keraunoparalysis.Lightning injuries can have many consequences depending on the different mechanisms involved. The clinical presentation is often due to a very focal lesion without any secondary extension. Motor neuron disease probably results from post-traumatic myelopathy. We discuss the ALS-electrocution association, frequently described in the literature.Various peripheral nerve and spinal cord lesions can be seen in lightning strike victims involving myelopathy, motor neuron, muscle and plexus. Clinical syndromes are often atypical but outcome is often favorable.

This work concerns an experimental study of sulphur surface segregation in FeMo (0.5, 2 and 3.5 at%) alloys with nitrogen as residual impurity. Auger electron spectroscopy and a radiochemical technique are used. At high temperature (T > 560°C), an increase of the maximum of segregated sulphur with bulk Mo content is explained by a cosegregation effect probably with the formation of 2D (Fe, Mo) sulphides. At low temperature, N and Mo cosegregate which can be explained by the tendency to form 2D Mo nitrides and sulphur segregation takes place for very long segregation treatments.

To identify the diseases that are associated with a high plasma concentration of vitamin B12 and to measure the strength of this association.Retrospective study including all admissions between 1st May, 2005 and 30th April, 2008 in the UMAG pole departments (emergency, internal medicine, acute geriatrics and medical intensive care) with a test for plasma vitamin B12. The association between each of medical information system codes (solid tumors, malignant hematologic process, and renal disease) and a high or low vitamin B12 concentration was measured by odds ratios (OR) from logistic models taking into account repeated admissions, with adjustment for age and the weighted Charlson index.Among 3702 admissions, 12% had a B12 more than 820pg/ml, 10.4% a B12 less than 180pg/ml and 77.6% a normal B12 concentration. After adjustment for age and the weighted Charlson index, high concentration of vitamin B12 was associated with interstitial renal diseases (OR 2.7; 95% CI: [1.7-4.2]), and cirrhosis or hepatitis (OR 4.3; [2.9-6.4]). After additional adjustment for these parameters, it was still associated with tumors (OR 1.8; [1.2-2.6]), malignant hematologic diseases (OR 2.1; [1.3-3.5]), metastasis (OR 2.9; [1.5-5.9]), liver metastasis (OR 6.2; [2.7-14.5]), liver carcinoma (LC) (OR 3.3; [1.1-10.4]), liver tumors other than LC (OR 4.7; [1.2-17.9]) and lymphoma (OR 3.2; [1.6-6.4]) but not with myeloma (OR 1.9; [0.6-1.4]). Low concentration of B12 was associated with myeloma (OR 2.9; [1.3-6.6]).Finding a high plasma concentration of vitamin B12 should lead to a systematic search for a hepatic disease or a tumor, and particularly for a hepatic localization of a tumor.

Pulmonary embolism is a frequent disorder with a diagnostic approach based on probability estimation. Nevertheless, in some cases, prognosis may be impaired by delayed diagnosis resulting from atypical presenting manifestations.We report a 37-year-old woman, admitted for a seizure as the presenting manifestation of pulmonary embolism, and review nine additional similar cases reported in the literature since 1945. Seizures were always generalized tonico-clonic in nature without a past medical history of epilepsy in any case. Tachycardia was noted in nine patients over ten. Prognosis was usually severe leading to death by cardiovascular deficiency in 70% of cases.These observations suggest a systematic suspicion of pulmonary embolism in the presence of generalized convulsion with persistent tachycardia after resolution of the episode and no past medical history of seizures. More research is necessary to assess the role of d-dimer testing in these situations.

In patients treated with systemic glucocorticoids (GCs), it is unknown if beliefs about the treatment are associated with level of reported adherence.Cross-sectional study conducted in two departments of internal medicine during a six-month period. All patients receiving long-term GCs therapy were asked to fill in a questionnaire regarding their beliefs about (specific scale of the Beliefs about Medicines Questionnaire) and their adherence to (four-item Morisky-Green scale) GCs. Logistic regression analysis was used to assess association between beliefs about GCs and adherence to treatment.One hundred and eighty one questionnaires were analysed (women: 79%, median age [IQR]: 47 [33-61] years, median duration of treatment: 18 [7-72] months, median daily dosage of prednisone equivalent: 10 [6-20] mg). Among these 181 patients, 83 (46%) reported a "concern" score equal to or higher than the "necessity" score. Nineteen percent of patients reported a low adherence level. In multivariate analysis, these patients were significantly younger (OR: 0.96 [0.93-0.98] per increasing year of age, P=0.002) and reported more frequently a "concern" score higher than a "necessity" score (OR: 3.08 [1.27-7.46], P=0.01) as compared to patients reporting a high adherence level.Informing patients about the "necessity" of GCs and taking into account their "concerns" about adverse events or their fear of becoming dependent on the medication may improve their adherence to treatment.

Fabry disease (FD) is an X-linked lysosomal storage disorder due to α-galactosidase A deficiency. It is associated with a broad range of clinical symptoms, resulting in frequent misdiagnosis and diagnostic delay, which may impact on patient outcomes. This retrospective observational study of 58 FD patients referred to 10 internal medicine departments in France aimed to review differential diagnoses received prior to diagnosis and examines diagnostic delay. The average age at the time of diagnosis was 27.6 years (range: 10-60) and 42.2 years (range: 9-77) among the 23 males and 35 females analyzed, respectively. Most common symptoms that led to FD diagnosis were family history of FD (12 males and 27 females), followed by pain in extremities (10 males and 5 females), and angiokeratoma (8 males and 4 females). Eighteen patients had received alternative diagnoses prior to FD diagnosis, including a female patient with four previous diagnoses. Four case reports are presented, which illustrate the diagnostic 'odyssey' and delayed diagnosis often experienced by patients. Clinicians should consider a diagnosis of FD when presented with a wide range of symptoms, thus helping to shorten the diagnostic delay and facilitating early therapy with enzyme replacement therapy to improve patient outcomes.

International audience; Objective. The nonsynonymous polymorphism rs763361 of the CD226 gene, which encodes DNAX accessory molecule 1, which is involved in T cell co-stimulation pathways, has recently been identified as a genetic risk factor for autoimmunity. The purpose of this study was to test for association of the CD226 rs763361 polymorphism with systemic sclerosis (SSc) in European Caucasian populations. Methods. CD226 rs763361 was genotyped in 3,632 individuals, consisting of a discovery sample (991 SSc patients and 1,008 controls) and a replication sample (999 SSc patients and 634 controls). All study subjects were of European Caucasian origin. Expression of CD226 was assessed on peripheral blood mononuclear cells obtained from 21 healthy donors genotyped for CD226 rs763361. Results. The CD226 rs763361 T allele was found to be associated with SSc in both the discovery and the replication samples, showing the following results in the combined populations: odds ratio (OR) 1.22 (95% confidence interval [95% CI] 1.10-1.34), P = 5.69 x 10(-5). The CD226 T allele was also associated with various SSc subsets, highlighting a potential contribution to disease severity. The most remarkable associations of the CD226 TT risk genotype were observed with the diffuse cutaneous SSc subtype, the anti-topoisomerase I antibody-positive, and SSc-related fibrosing alveolitis subsets: OR 1.86 (95% CI 1.42-2.43), P = 5.15 x 10(-6), OR 1.82 (95% CI 1.38-2.40), P = 2.16 x 10(-5), and OR 1.61 (95% CI 1.25-2.08), P = 2.73 x 10(-4), respectively. CD226 expression was not significantly influenced by CD226 rs763361 genotypes whatever the T cell subtype investigated. Conclusion. Our results establish CD226 as a new SSc genetic susceptibility factor underlying the contribution of costimulation pathways in the pathogenesis of SSc. Further work is nevertheless needed to define the causal variant at the CD226 locus as well as the functional consequences.

Lattice parameters of Ag-Mg (0.5 and 1 at.%) were measured. A comparison between these results and data previously obtained by gravimetry shows that (a) a high dilatation is due mainly to excess oxygen by comparison with the amount necessary to form stoichiometric MgO oxide, and (b) the earliest stage of Mg oxidation occurs in a non-expanded layer beneath the expanded one, with the formation of substoichiometric species (O/Mg < 1). The formation of substoichiometric species is explained by taking account of strain fields close to O and Mg atoms in the silver lattice. Indeed, the strong deformation introduced by oxygen in interstitial position delays the addition of oxygen on the MgO* and Mg2O* initial species and favors the formation of substoichiometric species.

Hiccup is a forceful, involuntary inspiration commonly experienced by fetuses, children and adults. Its purpose is unknown and its pathophysiology still poorly understood. Short hiccup bouts are mostly associated with gastric distention or alcohol intake, resolve spontaneously or with simple folk remedies and do not require medical attention. In contrast, prolonged hiccup is a rare but disabling condition which can induce depression, weight loss and sleep deprivation. A wide variety of pathological conditions can cause chronic hiccup: myocardial infarction, brain tumour, renal failure, prostate cancer, abdominal surgery etc. Detailed medical history and physical examinations will often guide diagnostic investigations (abdominal ultrasound, chest or brain CT scan...). Gastric and duodenal ulcers, gastritis, oesophageal reflux and oesophagitis are commonly observed in chronic hiccup patients and upper gastrointestinal investigations (endoscopy, pH monitoring and manometry) should be included in the diagnostic evaluation systematically. Etiological treatment is not always available and chronic hiccup treatment has classically relied on metoclopramide and chlorpromazine. Recently, baclofen (LIORESAL) has emerged as a safe and often effective treatment.