Implementing shared decision-making on acute psychiatric wards: a cluster-randomized trial with inpatients suffering from schizophrenia (SDM-PLUS).

AIMS: Although shared decision-making (SDM) has the potential to improve health outcomes, psychiatrists often exclude patients with more severe mental illnesses or more acute conditions from participation in treatment decisions. This study examines whether SDM is facilitated by an approach which is specifically adapted to the needs of acutely ill patients (SDM-PLUS). METHODS: The study is a multi-centre, cluster-randomised, non-blinded, controlled trial of SDM-PLUS in 12 acute psychiatric wards of five psychiatric hospitals addressing inpatients with schizophrenia or schizoaffective disorder. All patients fulfilling the inclusion criteria were consecutively recruited for the trial at the time of their admission to the ward. Treatment teams of intervention wards were trained in the SDM-PLUS approach through participation in two half-day workshops. Patients on intervention wards received group training in SDM. Staff (and patients) of the control wards acted under 'treatment as usual' conditions. The primary outcome parameter was the patients' perceived involvement in decision-making at 3 weeks after study enrolment, analysed using a random-effects linear regression model. RESULTS: In total, 161 participants each were recruited in the intervention and control group. SDM-PLUS led to higher perceived involvement in decision-making (primary outcome, analysed patients n = 257, mean group difference 16.5, 95% CI 9.0-24.0, p = 0.002, adjusted for baseline differences: ß 17.3, 95% CI 10.8-23.6, p = 0.0004). In addition, intervention group patients exhibited better therapeutic alliance, treatment satisfaction and self-rated medication compliance during inpatient stay. There were, however, no significant improvements in adherence and rehospitalisation rates in the 6- and 12-month follow-up. CONCLUSIONS: Despite limitations in patient recruitment, the SDM-PLUS trial has shown that the adoption of behavioural approaches (e.g. motivational interviewing) for SDM may yield a successful application to mental health. The authors recommend strategies to ensure effects are not lost at the interface between in- and outpatient treatment.Trial registration: The trial was registered at Deutsches Register Klinischer Studien (DRKS00010880).

Rare splicing defects of FAS underly severe recessive autoimmune lymphoproliferative syndrome.

Autoimmune lymphoproliferative syndrome (ALPS) is a prototypic disorder of impaired apoptosis characterized by autoimmune features and lymphoproliferation. Heterozygous germline or somatic FAS mutations associated with preserved protein expression have been described. Very rare cases of homozygous germline FAS mutations causing severe autosomal recessive form of ALPS with a complete defect of Fas expression have been reported. We report two unrelated patients from highly inbred North African population showing a severe ALPS phenotype and an undetectable Fas surface expression. Two novel homozygous mutations have been identified underlying rare splicing defects mechanisms. The first mutation breaks a branch point sequence and the second alters a regulatory exonic splicing site. These splicing defects induce the skipping of exon 6 encoding the transmembrane domain of CD95. Our findings highlight the requirement of tight regulation of FAS exon 6 splicing for balanced alternative splicing and illustrate the importance of such studies in highly consanguineous populations.

Drug reaction with eosinophilia and systemic Symptoms (DRESS) Syndrome in children: a case report

No disponible

Draft Genome Sequence of Serratia sp. Strain ATCC 39006, a Model Bacterium for Analysis of the Biosynthesis and Regulation of Prodigiosin, a Carbapenem, and Gas Vesicles.

Serratia sp. strain ATCC 39006 is a Gram-negative bacterium and a member of the Enterobacteriaceae that produces various bioactive secondary metabolites, including the tripyrrole red pigment prodigiosin and the ß-lactam antibiotic 1-carbapenen-2-em-3-carboxylic acid (a carbapenem). This strain is the only member of the Enterobacteriaceae known to naturally produce gas vesicles, as flotation organelles. Here we present the genome sequence of this strain, which has served as a model for analysis of the biosynthesis and regulation of antibiotic production.

[Acute transverse myelitis and Lyme borreliosis: a case report]. / Myélite transverse aiguë et maladie de Lyme: à propos d'un cas.

Lyme disease is an infectious disease caused by a spirochete of the Borrelia sensu lato group. Its incidence has greatly increased in recent years. The main vector is a tick of the Ixodes family. Clinical manifestations are multiple and show the multi-organ character of the disease. In terms of frequency, joint and neurological presentations, respectively more frequent in North America and Europe, are the main manifestations after cutaneous symptoms, of which erythema migrans is the most common, followed by cardiac and ocular signs. Other signs exist but are anecdotal. Neuroborreliosis manifests itself most often with peripheral facial palsy, but there are other clinical forms, which include acute myelitis (4-5% of neuroborreliosis). We present here the case of a 16-year-old teenager with acute myelitis and meningeal involvement due to Lyme disease, who presented with atypical symptoms (massive and rapid weight loss, vomiting). MRI showed localized marrow edema as well as leptomeningeal and root enhancement. Lumbar puncture showed lymphocytic pleocytosis. Lyme serology was positive both in blood and cerebrospinal fluid. Even if acute myelitis remains exceptional among neuroborreliosis manifestations, this diagnosis has to be thought of when a child presents with unexplained neurological symptoms.

[Pediatric Crohn's disease in Tunisia]. / La maladie de Crohn de l'enfant en Tunisie.

UNLABELLED: Crohn's disease occur mainly in adults. However, pediatric onset forms are not rare and have many characteristics. AIM: to study clinical, diagnostic, therapeutic and evolutive characteristics of crohn's disease in tunisian children. METHODS: Retrospective multicenter study conducted in 10 pediatric departments on a period of 10 years (2000-2008) RESULTS : 43 children were included. The sex-ratio was 1.68. The mean age at the onset of the symptoms was 11+/-2.3 years (5-16 years). The age of onset was inferior to 10 years in 25 % of the children. The delay before management was superior to 1 year in 25% of cases. The initial symptoms were dominated by diarrhea (95%). Perineal manifestations were present at diagnosis in 30% of children and extra-digestive manifestations in 53%. Ileocolonic localization was the most frequent (46%). The initial disease flare was moderate in 83% of cases. The treatment was medical in 77 % of cases, nutritional and medical in 18.5%. Maintenance therapy was instituted in 86% of cases and consisted essentially in azathioprin (62%). The mean follow-up was 3 years and 4 months. 60% of the children had at least one acute flare. During evolution, 7% of children had anoperineal surgery and 11% an intestinal resection. CONCLUSION: Crohn's disease seems rare in Tunisia. The time of diagnosis is often delayed. The management is based on immunosuppressive therapy and nutritional support.

[Longevity from the medical point of view--an increasing problem for life insurers]. / Langlebigkeit aus medizinischer Sicht--ein zunehmendes Problem für Lebensversicherer.

Longevity is one of the most challenging processes for the developed societies in western countries. Social security systems and private insurance companies calculate their promises on the basis of demographic facts. The article will give an overview about the development of longevity in recent years, the most important studies and theories and the causes from a medical perspective.

Draft genome sequence of the thermoalkaliphilic Caldalkalibacillus thermarum strain TA2.A1.

The genes and molecular machines that allow for a thermoalkaliphilic lifestyle have not been defined. To address this goal, we report on the improved high-quality draft genome sequence of Caldalkalibacillus thermarum strain TA2.A1, an obligately aerobic bacterium that grows optimally at pH 9.5 and 65 to 70°C on a wide variety of carbon and energy sources.

[The re-emergence of pertussis in Tunisia]. / La réémergence de la coqueluche en Tunisie.

OBJECTIVE: The authors had for aim to analyze pertussis epidemiology in Tunisia by studying nasopharyngeal specimens of infants hospitalized in Tunis. METHODS: Between march 2007 and march 2008, clinical nasopharyngeal samples were collected from infants with a suspected diagnosis of whooping cough, pertussoid cough, or pertussis-like syndrome, admitted at the Tunis children's hospital. The laboratory diagnostic criteria were culture isolation of Bordetella species on Bordet-Gengou medium and real-time PCR. RESULTS: Fifty-nine percent of the 74 investigated children with suspected pertussis were less than two months of age. The diagnosis of pertussis was proved positive by real-time PCR for 41%. Culture was negative in all cases. CONCLUSIONS: Whooping cough is still prevalent in Tunisia despite an important vaccination coverage. Real-time PCR is an invaluable tool for the rapid diagnosis of pertussis, however culture must also be associated.

Nucleotide sequence of the Serratia entomophila plasmid pADAP and the Serratia proteamaculans pU143 plasmid virulence associated region.

Some strains of Serratia entomophila and S. proteamaculans cause amber disease of the New Zealand grass grub Costelytra zealandica (Coleoptera: Scarabaeidae), an important pasture pest in New Zealand. The disease determinants of S. entomophila, are encoded on a 153,404-bp plasmid, termed pADAP for amber disease associated plasmid. The S. proteamaculans strain 143 (Sp143) exhibits an unusual pathotype, where only 60-70% of C. zealandica larvae infected with the bacterium succumb to disease. DNA sequence analysis of the Sp143 pU143 virulence associated region identified high DNA similarity to the pADAP sep virulence associated region, with DNA sequence variation in the sepA gene and the variable region of the sepC component. No pADAP anti-feeding prophage orthologue was detected in the Sp143 genome. The region of pADAP replication was cloned and found to replicate in S. entomophila but not in Escherichia coli. DNA sequence analysis of the plasmid pSG348 repA gene from the French isolate of Serratia grimesii, identified 93% DNA identity to the pADAP repA gene. A comparison of the pU143 virulence associated region with the completed pADAP nucleotide sequence is given.

Yersinia entomophaga sp. nov., isolated from the New Zealand grass grub Costelytra zealandica.

A Gram-negative, rod-shaped, non-spore-forming bacterium (MH96(T)) was isolated from diseased larvae of the New Zealand grass grub, Costelytra zealandica (Coleoptera: Scarabaeidae). On the basis of 16S rRNA gene sequence similarity, strain MH96(T) is a member of the genus Yersinia, which is a member of the class Gammaproteobacteria. The most similar 16S rRNA gene sequence to that of MH96(T) is that of the type strain of Yersinia mollaretii (98.5 % similarity) followed by those of the type strains of Yersinia aldovae, Y. frederiksenii and Y. rohdei (all 98.4 % similarity). Multilocus sequence typing of five housekeeping genes (dnaJ, glnA, gyrB, groEL and recA) identified Yersinia ruckeri (81-92 % similarity) as the closest relative. The results of DNA-DNA hybridization and physiological and biochemical tests allowed genotypic and phenotypic differentiation of strain MH96(T) from the four most closely related Yersinia species with validly published names, including a Y. ruckeri isolate. Strain MH96(T) therefore represents a novel species, for which the name Yersinia entomophaga sp. nov. is proposed, with the type strain MH96(T) ( = DSM 22339(T)  = ATCC BAA-1678(T)).

[Cystic pulmonary malformations: clinical and radiological polymorphism. A report on 30 cases]. / Les malformations pulmonaires kystiques : polymorphisme clinique et radiologique. A propos de 30 observations.

OBJECTIVES: This report describes different clinical pictures of cystic pulmonary malformation (CPM) and problems in diagnosis. PATIENTS AND METHODS: Cases of CPM between 01 January 1994 and 31 December 2004 diagnosed in our institution were reviewed. RESULTS: Thirty-three cases of CPM were diagnosed in 30 children. They consisted of 17 boys and 13 girls ranging from 20 days to 16 years of age at the time of the diagnosis. The CPM included: 17 cases of congenital lobar emphysema (CLE), seven bronchogenic cysts (BC), five cystic adenomatoid malformations (CAM) and four pulmonary sequestrations (PS). Three patients presented two associated lung malformations. The mean ages at the time of diagnosis varied from 2 to 88 months. The symptoms consisted of respiratory distress (n=14, 46.6%); recurrent attacks of respiratory embarrassment (n=6, 20%); pulmonary infection (n=8, 26.6%) associated with haemoptysis in two cases; haemothorax (n=1) and a chance discovery (n=1). Radiological investigations led to the diagnosis in all cases of CLE and CAM although it contributed less to the diagnosis of BC and PS. Twenty-nine patients required chirurgical treatment involving lobectomy (n=22), pneumonectomy (n=2) and cystectomy (n=8). The histopathological examinations confirmed the diagnosis in all cases and rectified the preoperative diagnosis in four cases. Except for one patient with CLE, who died a few days after a lobectomy due to acute nosocomial pneumonia, the postoperative period was uneventful in 26 children with a mean of follow-up of 24 months (4 months to 7 years). Three patients developed transient and episodic attacks of dyspnoea. CONCLUSION: CPM may be responsible for many clinical and radiological pictures that present difficulties in their diagnosis. Polymorphism is related to the type of malformation, its topography and the evolutive complications.

[Use of the rapid antigen detection test in group A streptococci pharyngitis diagnosis in Tunis, Tunisia]. / Utilisation d'un test de diagnostic rapide des angines à streptocoque bêta-hémolytique du groupe A, auprès d'un échantillon d'enfants à Tunis, Tunisie.

The aims of this study were to determine the contribution of commercial rapid antigen detection test (RADT) in the rapid diagnosis of pharyngitis caused by group A streptococci. A total of 292 children with pharyngitis was included. A duplicate throat swabs was taken simultaneously from each patient. One of them was used for RADT achievement and the other for culture. When cultures were positive, a semi-quantitative evaluation was done. Throat culture and RADT were positive in 59 and 72 cases respectively. Fifty four specimens were positive simultaneously with RADT and culture and 215 specimens were negative using both methods. Eighteen specimens were positive only with RADT, 5 of them were treated by amoxicillin. Regarding to the group of patients with a negative RADT and a positive culture (5 cases), all of them had a culture evaluation at "+" or "++". Specificity and sensibility of the RADT were 92.2% and 91.5% respectively. RADT use by physicians can reduce antibiotic prescription among paediatric population. Because of the high specificity of RADT, therapeutic decisions can be made on the basis of a positive test.

Serine proteases identified from a Costelytra zealandica (White) (Coleoptera: Scarabaeidae) midgut EST library and their expression through insect development.

Costelytra zealandica larvae are pests of New Zealand pastures causing damage by feeding on the roots of grasses and clovers. The major larval protein digestive enzymes are serine proteases (SPs), which are targets for disruption in pest control. An expressed sequence tag (EST) library from healthy, third instar larval midgut tissue was constructed and analysed to determine the composition and regulation of proteases in the C. zealandica larval midgut. Gene mining identified three trypsin-like and 11 chymotrypsin-like SPs spread among four major subgroups. Representative SPs were examined by quantitative PCR and enzyme activity assayed across developmental stages. The serine protease genes examined were expressed throughout feeding stages and downregulated in nonfeeding stages. The study will improve targeting of protease inhibitors and bacterial disruptors of SP synthesis.

[Central diabetes insipidus: diagnostic difficulties]. / Diabète insipide central: difficultés diagnostiques.

UNLABELLED: Central diabetes insipidus is rare in children. Characteristic features include polyuria and polydipsia due to arginine vasopressin deficiency. The differential diagnosis of polyuric states may be difficult. Etiologic diagnosis of central diabetes insipidus may be an equally difficult task. OBJECTIVE: To specify the difficulties encountered in the diagnosis of central diabetes insipidus and to point out features of the etiologic work-up and of long-term follow-up of children with idiopathic central diabetes insipidus. METHODS: A retrospective study of 12 children admitted with a polyuria/polydipsia syndrome to the pediatric - consultation and emergency unit of the children's hospital of Tunis between 1988 and 2005. Children with acquired nephrogenic central diabetes insipidus were excluded. Fourteen-hour fluid restriction test and/or desmopressin test were used without plasma vasopressin measurement. RESULTS: Eight patients were classified as having central diabetes insipidus, which was severe in seven children and partial in one girl. One patient was classified as having primary polydipsia. The diagnosis remains unclear in three patients. The etiological work-up in eight patients with central diabetes insipidus enabled the identification of Langerhan's-cell histiocytosis in two patients and neurosurgical trauma in one. The cause was considered idiopathic in five patients. The median follow-up of the five patients with idiopathic central diabetes insipidus was five years two months plus or minus six years seven months (range five months, 14.5 years). During this follow-up, neither brain magnetic resonance imaging scans findings nor anterior pituitary function have changed. CONCLUSION: Fluid restriction and desmopressin tests did not enable an accurate distinction between partial diabetes insipidus and primary polydipsia. Regular surveillance is warranted in patients with idiopathic central diabetes insipidus to identify potential etiologies.

Ccr1 deficiency reduces inflammatory remodelling and preserves left ventricular function after myocardial infarction.

Myocardial necrosis triggers inflammatory changes and a complex cytokine cascade that are only incompletely understood. The chemokine receptor CCR1 mediates inflammatory recruitment in response to several ligands released by activated platelets and up-regulated after myocardial infarction (MI). Here, we assess the effect of CCR1 on remodelling after MI using Ccr1-deficient (Ccr1(-)(/-)) mice. MI was induced in Ccr1(-/-) or wild-type mice by proximal ligation of the left anterior descending (LAD). Mice were sacrificed and analysed at day 1, 4, 7, 14 and 21 after MI. While initial infarct areas and areas at risk did not differ between groups, infarct size increased to 20.6+/-8.4% of the left ventricle (LV) in wild-type mice by day 21 but remained at 11.2+/-1.2% of LV (P<0.05) in Ccr1(-/-) mice. This attenuation in infarct expansion was associated with preserved LV function, as analysed by isolated heart studies according to Langendorff. Left ventricular developed pressure was 84.5+/-19.8 mmHg in Ccr1(-/-) mice compared to 49.0+/-19.7 mmHg in wild-type mice (P<0.01) and coronary flow reserve was improved in Ccr1(-/-) mice. An altered post-infarct inflammatory pattern was observed in Ccr1(-/-) mice characterized by diminished neutrophil infiltration, accelerated monocyte/lymphocyte infiltration, decreased apoptosis, increased cell proliferation and earlier myofibroblast population in the infarcted tissue. In conclusion, functional impairment and structural remodelling after MI is reduced in the genetic absence of Ccr1 due to an abrogated early inflammatory recruitment of neutrophils and improved tissue healing, thus revealing a potential therapeutic target.

[Cardiotoxicity of n-methyl-glucamine antimoniate (Glucantime). A case report]. / Toxicité cardiaque de l'antimoniate de méglumine (Glucantime). A propos d'une observation.

The pentavalent antimonial meglumine (Glucantime) is the drug of choice in treatment of cutaneous leishmaniasis in Tunisia. It may create severe adverse effects. A ten year-old girl was treated by Glucantime for cutaneous leishmaniasis. On the eighth day of treatment, she developed palpitations and precordialgia. The ECG showed T wave inversion prolongation of corrected QT interval. Drug therapy was stopped. Within a few days, she recovered and her elctrocardiographic changes came back to normal. The cardio toxicity of Glucantime may be severe. Electrocardiographic changes are the primary signs. Long term ECG follow-up is necessary.