Contemporary management of patients with atrial fibrillation in the Netherlands and Belgium: a report from the EORP-AF long-term general registry.

BACKGROUND: Contemporary data regarding the characteristics, treatment and outcomes of patients with atrial fibrillation (AF) are needed. We aimed to assess these data and guideline adherence in the EURObservational Research Programme on Atrial Fibrillation (EORP-AF) long-term general registry. METHODS: We analysed 967 patients from the EORP-AF long-term general registry included in the Netherlands and Belgium from 2013 to 2016. Baseline and 1­year follow-up data were gathered. RESULTS: At baseline, 887 patients (92%) received anticoagulant treatment. In 88 (10%) of these patients, no indication for chronic anticoagulant treatment was present. A rhythm intervention was performed or planned in 52 of these patients, meaning that the remaining 36 (41%) were anticoagulated without indication. Forty patients were not anticoagulated, even though they had an indication for chronic anticoagulation. Additionally, 63 of the 371 patients (17%) treated with a non-vitamin K antagonist oral anticoagulant (NOAC) were incorrectly dosed. In total, 50 patients (5%) were overtreated and 89 patients (9%) were undertreated. However, the occurrence of major adverse cardiac and cerebrovascular events (MACCE) was still low with 4.2% (37 patients). CONCLUSIONS: Overtreatment and undertreatment with anticoagulants are still observable in 14% of this contemporary, West-European AF population. Still, MACCE occurred in only 4% of the patients after 1 year of follow-up.

[Short version of recommendations for enhanced recovery program (ERP) for cystectomy: Technical measures]. / Version courte des recommandations de la récupération ameliorée après chirurgie (RAAC) pour la cystectomie : mesures techniques.

INTRODUCTION: The enhanced recovery program (ERP) is a management mode whose objective is to reduce the risk of complications and allow the patient to recover more quickly all its functional capacities and to reintegrate at most quickly and safely in his usual environment. This intentionally synthetic document aims to disseminate in the urological community the main points of the ERP recommendations for cystectomy. This work, coordinated by AFU, involves several other partners. The full document is available on the "Urofrance" website. Another article will follow on organizational measures. METHOD: The development of the recommendations is based on the method "formalized consensus of experts" proposed by the HAS. The report is based on a systematic review of the literature (January 2006-May 2017), two rounds of iterative quotations and a national proofreading. Levels of proof of conclusions and gradation of recommendations are based on the HAS grid. RESULTS: The bibliographic strategy made it possible to retain 298 articles. Only the recommendations that obtained a strong agreement after the two rounds of iterative listing were retained. The recommendations presented here are in chronological form (before, during, after hospitalization). Twenty-six key points on the technical and organizational measures of ERP have been identified. CONCLUSION: The result of the literature review, supplemented by expert opinion, suggests a significant clinical interest in the application and dissemination of ERP for cystectomy, despite the limited data available for this indication.

[Morbi-mortality of transurethral resection of the prostate in patients aged 75 and over]. / Morbi-mortalité de la résection trans-urétrale de la prostate par courant monopolaire chez les patients âgés de 75 ans et plus.

INTRODUCTION: Monopolar transurethral resection of the prostate is one of standard surgical treatment of benign prostatic hyperplasia. The objective of this study was to evaluate early postoperative complications in patients aged 75 years old and more using a standardized classification. MATERIAL AND METHODS: We included all patients aged at least 75 on the day of surgery between 1 January 2008 and 31 December 2013. The reporting of complications was carried from the Clavien-Dindo classification. RESULTS: One hundred and seventy-six patients were included in this study. A total of 47.2% of patients experienced at least one complication. The majority of patients (79.5%) had complications grade 1 or 2 according to Clavien-Dindo classification. One patient died postoperatively at day 27. Most complications were urological (55%). A high Charlson score and low plasma hemoglobin levels have been identified as a risk factor for complications. CONCLUSION: Monopolar transurethral resection of the prostate is followed by significant morbidity in older patients, higher than in the general population. LEVEL OF EVIDENCE: 4.

Air pollution and ST-elevation myocardial infarction: A case-crossover study of the Belgian STEMI registry 2009-2013.

BACKGROUND: Previous studies have shown that air pollution particulate matter (PM) is associated with an increased risk for myocardial infarction. The effects of air pollution on the risk of ST-elevation myocardial infarction (STEMI), in particular the role of gaseous air pollutants such as NO2 and O3 and the susceptibility of specific populations, are still under debate. METHODS: All patients entered in the Belgian prospective STEMI registry between 2009 and 2013 were included. Based on a validated spatial interpolation model from the Belgian Environment Agency, a national index was used to address the background level of air pollution exposure of Belgian population. A time-stratified and temperature-matched case-crossover analysis of the risk of STEMI was performed. RESULTS: A total of 11,428 STEMI patients were included in the study. Each 10µg/m3 increase in PM10, PM2.5 and NO2 was associated with an increased odds ratio (ORs) of STEMI of 1.026 (CI 95%: 1.005-1.048), 1.028 (CI 95%: 1.003-1.054) and 1.051 (CI 95%: 1.018-1.084), respectively. No effect of O3 was found. STEMI was associated with PM10 exposure in patients ≥75y.o. (OR: 1.046, CI 95%: 1.002-1.092) and with NO2 in patients ≤54y.o. (OR: 1.071, CI 95%: 1.010-1.136). No effect of air pollution on cardiac arrest or in-hospital STEMI mortality was found. CONCLUSION: PM2.5 and NO2 exposures incrementally increase the risk of STEMI. The risk related to PM appears to be greater in the elderly, while younger patients appear to be more susceptible to NO2 exposure.

Organ donation and transplantation statistics in Belgium for 2012 and 2013.

BACKGROUND: The 2012 and 2013 solid organ transplantation statistics were presented during the annual meeting of the Belgian Transplant Society. METHODS: All data presented were collected from Eurotransplant International Foundation and/or from all individual Belgian transplant centers. RESULTS: It was demonstrated that the highest number of deceased donors detected (1310) from which 47.8% were an effective organ donor that corresponded to 29 per million inhabitants (pmi) in 2012 and 27.4 pmi in 2013. Out of 626 effective deceased organ donors, 491 (79%) were donors after brain death (DBD) and 135 (21%) donors after circulatory death (DCD), respectively. The majority (125/135; 93%) of DCD donors were DCD Maastricht category III donors and there were 7 (5%) donations following euthanasia. Family refusal tended to be lower for DCD (10.4%) compared to DBD donors (13.4%). Despite the increasing DCD donation rate, DBD donation remains stable in Belgium. The donor age is still increasing, reaching a median age of 53 years (range 0-90). Spontaneous intracranial bleeding (39.3%) and cranio-cerebral trauma (25%) remained the most frequent reasons of death. The number of living related kidney transplantations (57 in 2012 and 63 in 2013) followed the international trend albeit in Belgium it is still very limited. Nevertheless this activity could explain that the number of patients waiting for kidney transplantation (770) reached an absolute minimum in 2013. Except the reduced waiting list for lung transplantation (from 119 patients in 2011 to 85 in 2013), the waiting list remained stable for the other organs but almost 200 patients still died while on the waiting list. CONCLUSIONS: Belgium demonstrated the highest number of effective organ donors that corresponded to 29 per million inhabitants (pmi) in 2012 and 27.4 pmi in 2013. Thus far, and in contrast with other countries, there is no erosion of DBD in the DCD donor organ pool, but it is the important responsibility of all transplant centers and donor hospitals to avoid a substitution from DBD by DCD donors.

Belgian modified classification of Maastricht for donors after circulatory death.

BACKGROUND: "Non-heart-beating donors," or, in a more recent and international definition, "donors after circulatory death," are a potential and additional group of deceased persons who are able to add organs to the pool. METHODS: A new classification is proposed on the basis of the result of a consensus of experts issued from all Belgian transplant centers. RESULTS: The first level of definition is simple and based on whether the situation is uncontrolled (categories I and II) or controlled (categories III, IV, and V). In category I, the patient is declared "dead on arrival" and, in category II, there is an "unsuccessful resuscitation" whether it occurred out or in the hospital for both situations. Category III is the most usual situation in which the treating physician and family are "awaiting cardiac arrest" to declare the death of the patient. Category IV is always characterized by "cardiac arrest during brain death." The special situation of the Belgian law allowing the euthanasia is elaborated in category V, "euthanasia," and includes patients who grant access to medically assisted circulatory death. Organ donation after euthanasia is allowed under the scope of donation after circulatory death. CONCLUSIONS: This classification conserves the skeleton of the Maastricht one, as it is simple and clear, but classifies easily the different donors after circulatory death types by processes for ethical issues and for the non-medical or non-specialized reader interested in the field. This is also an argument for public consideration and trust in the difficult field of organ donation.

Another case of "European hantavirus pulmonary syndrome" with severe lung, prior to kidney, involvement, and diagnosed by viral inclusions in lung macrophages.

Puumala virus (PUUV) is considered a classic Old World etiologic agent of nephropathia epidemica (NE), or hemorrhagic fever with renal syndrome (HFRS). HFRS is considered to be distinct from hantavirus (cardio-)pulmonary syndrome (HPS or HCPS), described in the New World. Here, we report a severe case, which fulfilled most, if not all, Centers for Disease Control and Prevention (CDC) criteria for HPS, needing non-invasive ventilation and subsequent acute hemodialysis. However, the etiological agent was PUUV, as proved by serological testing, real-time polymerase chain reaction (PCR), and sequencing. Viral antigen was detected by specific anti-PUUV immunostaining, showing, for the first time, greenish intracytoplasmic inclusions in bronchoalveolar lavage (BAL) macrophages. This case definitely confirms that HPS can be encountered during PUUV infections. Interestingly, special findings could render the diagnosis easier, such as greenish homogeneous cytoplasmic inclusions, surrounded by a fine clear halo in BAL macrophages. Therefore, although the diagnosis remains difficult before the onset of renal involvement, the occurrence of severe respiratory failure mimicking community-acquired pneumonia must alert the clinician for possible HPS, especially in endemic areas.

Polyurethane does not protect better than polyvinyl cuffed tracheal tubes from microaspirations.

BACKGROUND: Mechanically ventilated patients are prone to develop ventilator associated pneumonia due to microaspirations of subglottic secretions around the endotracheal tube cuff (usually constructed of polyvinyl material). A novel polyurethane cuff has been designed to minimize these leakages. The aim of the study was to compare the tracheal sealing capacities between the two tubes. METHODS: Twenty-nine consecutive patients from whom tracheal intubation was necessary as part of their care were randomized to receive either a polyvinyl HI-LO Evac® or a polyurethane SEALGUARD Evac® endotracheal tube. Patients requiring emergency intubation, with unstable hemodynamics or history of tracheal/laryngeal disease were excluded. For the entire study, cuff pressure was set at 30 cmH2O, and ventilator parameters were adjusted for a plateau pressure ≤30 cmH2O; Patients were fasting, placed in a strict 45° position during 12 hours and sedated if needed. After injection of 74 MBq 99mTc-DTPA diluted in 5 mL 0.9% NaCl just above the cuff, tracheal radioactivity was assessed sequentially (hourly from T0 to T6, then T8 and T12 hours) using a scintillation camera. RESULTS: Sixteen polyurethane and 13 polyvinyl tubes were compared. Leakages were observed in 11/29 patients (38%) (5/16 polyurethane and 6/13 polyvinyl tubes [P=NS]). Leakages occurred more often in female (7/8) than in male patients (4/21) (P<0.001). Microaspirations were decreased with larger tubes (size 9 vs. ≤8.5: 24% vs. 75%; P=0.01), whatever the cuff membrane. CONCLUSION: These preliminary results suggest that both tubes are poorly effective in preventing microaspirations.

Correlations between cyclosporine concentrations at 2 hours post-dose and trough levels with functional outcomes in de novo lung transplant recipients.

BACKGROUND: Although the reliability of cyclosporine (CyA) concentration at 2 (C2) hours postdosing has been established for kidney, liver, and heart transplant recipients, its use in lung cases remains to be validated. We investigated the relationship between CyA dual time point monitoring and long-term functional outcomes after lung transplantation. METHODS: We included data from 38 lung transplant recipients receiving CyA, azathioprins, and steroids in the study. CyA dosages were based on the trough concentrations. CyA concentrations at 0 (C0) and 2 (C2) hours postdosing were obtained at 1, 2, 3, 6, 9, 12, 15, 18, and 24 months postoperative. We retrospectively compared average CyA level (C0 and C2) during the first 3 posttransplantation months with forced expiratory volume in 1 second (FEV1), forced vital capacity (FVC), forced expiratory flow 25%-75 % (FEF 25-75), creatinine, systolic blood pressure (SBP), and diastolic blood pressure (DBP) using regression analysis via generalized estimating equations (GEE). RESULTS: Only improvement in FVC (P = .033) and deterioration of SBP (P < .001) were related to C0 monitoring. No correlation was observed between C0 and FEV1 (P = .13), FEF 25-75 (P = .48), creatinine (P = .07), and DBP (P = .97). Nor was any relationship observed between C2 concentrations and FEV1 (P = .64), FVC (P = .38), FEF 25-75 (P = .09), creatinine (P = .95), SBP (P = .73), or DBP (P = .51). CONCLUSION: There was a lack of a relationship between CyA concentrations (C0 and C2) and functional outcomes among de novo lung transplantations except for a positive correlation of 0 value with long-term improved FVC and increased SBP. This study suggested that C2 determinations may not improving lung recipient management.

A French observational study of botulinum toxin use in the management of children with cerebral palsy: BOTULOSCOPE.

BACKGROUND: Dystonia and spasticity are common symptoms in children with Cerebral Palsy (CP), whose management is a challenge to overcome in order to enable the harmonized development of motor function during growth. AIM: To describe botulinum toxin A (BTX-A) use and efficacy as a treatment of focal spasticity in CP children in France. METHODS: This prospective observational study included 282 CP children mostly administered according to French standards with BTX-A in lower limbs. Realistic therapeutic objectives were set with parents and children together before treatment initiation and assessed using the Visual Analogue Scale (VAS). Child management was recorded and the efficacy of injections was assessed during a 12-month follow-up period by physicians (Modified Ashworth Scale, joint range of motion, Physician Rating Scale, Gillette Functional Assessment Questionnaire and Gross Motor Function Measure-66) and by patients/parents (Visual Analogue Scale). RESULTS: BTX-A treatment was administered in different muscle localizations at once and at doses higher than those recommended by the French Health Authorities. Children were treated in parallel by physiotherapy, casts and ortheses. Injections reduced spasticity and improved joint range of motion, gait pattern and movement capacity. Pain was reduced after injections. BTX-A administration was safe: no botulism-like case was reported. The log of injected children who were not included in the study suggested that a large population could benefit from BTX-A management. CONCLUSIONS: We showed here the major input of BTX-A injections in the management of spasticity in CP children. The results are in favor of the use of BTX-A as conservative safe and efficient treatment of spasticity in children, which enables functional improvement as well as pain relief.

[Implementation of reperfusion therapy in ST segment elevation myocardial infarction (STEMI). A policy statement from the Belgian Society of Cardiology and its working group of acute cardiology and interventional cardiology]. / Mise en pratique du traitement de reperfusion dans les infarctus du myocarde avec élévation du segment ST (STEMI). Recommandations actualisées de la Société Belge de Cardiologie et de ses groupes de travail: le Groupe Interdisciplinaire de Cardiologie Aiguë (BIWAC) et le Groupe de Cardiologie Interventionnelle (BWGIC).

Myocardial infarction remains a major healthcare problem. Reperfusion therapy has been shown to influence favourably short- and long-term patient survival. The authors reviewed the data of early recognition of STEMI (ST Elevation Myocardial Infarction), the reperfusion modalities including a flowchart management, as proposed by the Belgian working groups (BIWAC and BWGIC), and the lessons learned from European and American registries. Primary PCI often remains the treatment of choice. A national policy is still required to implement the guidelines and improve clinical practice for our STEMI patients.

[Implementation of reperfusion therapy in ST segment elevation myocardial infarction (STEMI). A policy statement from the Belgian Society of Cardiology and its working group of acute cardiology and interventional cardiology]. / Mise en pratique du traitement de reperfusion dans les infarctus du myocarde avec élévation du segment ST.

Myocardial infarction remains a major healthcare problem. Reperfusion therapy has been shown to influence favourably short- and long-term patient survival. The authors reviewed the data of early recognition of STEMI (ST Elevation Myocardial Infarction), the reperfusion modalities including a flowchart management, as proposed by the Belgian working groups (BIWAC and BWGIC), and the lessons learned from European and American registries. Primary PCI often remains the treatment of choice. A national policy is still required to implement the guidelines and improve clinical practice for our STEMI patients.

Effects of curing sodium nitrite additive and natural meat fat on growth control of Listeria monocytogenes by the bacteriocin-producing Lactobacillus curvatus strain CWBI-B28.

In realistic model meat systems, the separate and combined effects of fat content and sodium nitrite on the antilisterial activity of the bacteriocin of Lactobacillus curvatus CWBI-B28 were studied. In laboratory fermentations where Listeria monocytogenes was co-cultured at 4 degrees C with bacteriocin-producing CWBI-B28 in lean pork meat (fat content: 13%) without added nitrite, a strong antilisterial effect was observed after one week. The effect was maintained for an additional week, after which a slight and very gradual rebound was observed. Both added nitrite (20 ppm) and a high-fat content (43%) were found to antagonise this antilisterial effect, the Listeria cfu count reached after six weeks being 200 times as high in high-fat meat with added nitrite than in lean meat without nitrite. This antagonism could not be attributed to slower growth of the bacteriocin-producing strain, since CWBI-B28 grew optimally in fat-rich meat with 20 ppm sodium nitrite. Bacteriocin activity was also measured in the samples. The observed activity levels are discussed in relation to the degree of antilisterial protection conferred.

[When should the pulmonologist consider lung transplantation? Criteria for referral and transplantation in COPD and idiopathic pulmonary fibrosis]. / Quand le pneumologue doit-il envisager la transplantation pulmonaire pour un de ses patients? Critères d'inscription en liste d'attente. BPCO, fibrose pulmonaire idiopathique.

INTRODUCTION: The definition of the optimal timing of referral for lung transplantation in cases of chronic obstructive pulmonary disease or idiopathic pulmonary fibrosis remains a difficult question. BACKGROUND: The decision is based on the balance between the risk of death associated with the transplant procedure on one hand and, on the other, the risk of death associated with the natural course of the disease and/or the severity of the disability. It is not surprising, therefore, that the selection criteria take into account the main prognostic factors identified in both diseases. Recommendations for the selection criteria based on these prognostic indices have been published recently by a panel of international experts. In case of chronic obstructive pulmonary disease, the main indication for lung transplantation worldwide, the decision is not simple. The course of the individual patient is not easy to determine as, on an individual basis, some patients have a prolonged survival. In the case of idiopathic pulmonary fibrosis, the survival benefit provided by lung transplantation is well documented and it is therefore recommended to refer the patient to a transplantation centre once the diagnosis is established. This evaluation for lung transplantation does not prevent the subsequent inclusion of the patients in therapeutic protocols. CONCLUSION: International guidelines assist the pulmonologist to define the optimal moment of referral but these guidelines are not absolute and therefore, in case of doubt, physicians should not hesitate to refer the patient to a transplant centre.

Basiliximab as an alternative to antithymocyte globulin for early immunosuppression in lung transplantation.

OBJECTIVE: Basiliximab (BAS), an interleukin-2 monoclonal antibody, has shown promising results as induction therapy in liver and kidney transplantation. We compared the efficacy and patient tolerability of BAS and antithymocyte globulin (ATG) in lung transplantation (LTx). PATIENTS AND METHODS: The study included 37 patients in two groups (ATG and BAS, respectively). The indication for LTx was emphysema in 62.6% of patients in group 1 and 57.1% of patients in group 2. Mean (SD) patient characteristics compared in the two groups included age (52.0 [9.8] vs 54 [10.6] years), height (172.0 [10.1] vs 169 [7.55] cm), and weight (73.9 [15.3] vs 64.4 [14.2] kg) (P = .049). Induction therapy after LTx in the two groups was as follows: in 16 transplantation procedures (eight single and eight bilateral) performed between April 1998 and December 2002, ATG, 3 mg/kg/d for 3 days, was administered, and in 21 transplantation procedures (15 single and 6 bilateral) performed between January 2003 and July 2005, BAS, 20 mg on days 0 and 4, was given. Dosages of cyclosporine, azathioprine, and steroids for maintenance therapy were equivalent in the two groups. We retrospectively compared patient tolerability, occurrence of acute rejection or infection (from bacteria, cytomegalovirus [CMV], or Aspergillus), and outcomes between the two groups during 2 years of follow-up. RESULTS: No cytokine-mediated reaction was observed in either group; however, there was a difference in hematologic effects. On day 2, mean (SD) platelet count was significantly lower in the ATG group at 113,500 (56,400)/mm(3) vs 151,900 (57,300)/mm(3) (P = .046). Because of severe thrombocytopenia, three patients could not be given ATG on day 3. The overall incidence of aspergillosis (18.8% vs 19.0%) and CMV infection (31% vs 57%) was similar in the two groups. However, when the recipient was CMV-positive and the donor was CMV-negative, the there was a clear trend (33.3% vs 88.9%). The number of acute rejection episodes was similar (43.8% vs 52.4%). Survival increased by 20% in the BAS group (P = .03). CONCLUSION: In LTx, safety of BAS use is as good as or better than that of ATG, with no difference in acute rejection episodes or infections, with the possible exception of increased reactivation of CMV infection. Thus, BAS could be an alternative to ATG as induction therapy in LTx; however, further studies are necessary.

Prenatal isolated mild ventriculomegaly: outcome in 167 cases.

OBJECTIVE: To define the contribution of prenatal investigation and evaluate the prognosis of isolated mild ventriculomegaly (IMV). DESIGN: Retrospective study. SETTING: University hospital between January 1992 and December 2002. POPULATION: One hundred and sixty-seven cases of prenatal unilateral or bilateral IMV without any associated anomaly at the time of initial diagnosis. METHODS: Complementary investigations were performed: amniocentesis with karyotyping, screening for viruses and acetylcholinesterase electrophoresis, magnetic resonance imaging (MRI), and ultrasonography every 3-4 weeks. MAIN OUTCOME MEASURES: Results of prenatal investigations, pregnancy outcome, and postnatal psychomotor development. RESULTS: IMV was diagnosed around 26.5 weeks. Amniocentesis revealed four chromosomal anomalies and two cytomegalovirus infections. MRI diagnosed brain-associated anomalies in 15 cases and ultrasonographic monitoring highlighted malformations not initially diagnosed in 28 cases. Termination of pregnancy (TOP) was considered in 21 pregnancies (12.6%). Indications were aneuploidy, fetal infectious disease or associated malformations. In women for whom a TOP was considered, consanguinity, fetus of female sex and frontal horn enlargement were statistically more frequent, ventriculomegaly was more often bilateral and asymmetrical, atrial width, and the rate of progressive ventricular enlargement were significantly higher. One hundred and one children with prenatal IMV were assessed between 19 and 127 months (mean age 54.68 +/- 2.87 months). Twelve children had neurological disease or psychomotor delay and 89 children had a normal psychomotor development. Poor neurological outcome was more often associated with atrial width greater than or equal to 12 mm, asymmetrical bilateral enlargement, and progression of the ventriculomegaly. CONCLUSION: The detection of IMV raises the question of the child's psychomotor development and justifies meticulous prenatal investigation. In addition to associated anomalies, three criteria are often associated with an unfavourable outcome: atrial width greater than 12 mm, progression of the enlargement, and asymmetrical and bilateral ventriculomegaly.

[Aicardi-Goutieres syndrome: an oft unrecognised familial early-onset encephalopathy]. / Le syndrome d'Aicardi-Goutières: une maladie neurologique infantile, à transmission familiale, encore méconnue.

INTRODUCTION: Aicardi-Goutieres syndrome, first described in 1984, is a progressive infantile familial encephalopathy featuring cerebral calcifications, mainly of the basal ganglia, cerebral white matter abnormalities and cerebrospinal fluid lymphocytosis. Most of the patients present with severe developmental retardation, microcephaly, abnormal eye movements, pyramidal tract signs, and prominent dystonic movements. An elevated level of interferon-alpha in the CSF is a constant feature, particularly during the first stages of the disease course. One locus has been mapped on chromosome 3p21 in about half of the families so far studied. PATIENTS: and results. We report two new French cases and discuss the limits of the clinical syndrome, the differential diagnosis and issues raised by the pathophysiological mechanisms involved. The major concern is to separate this condition from intrauterine infections because of the genetic and therapeutic consequences. A number of other questions remain unanswered. For example, we still do not know today at what age the absence of features like CSF lymphocytosis, and possibly absence of calcifications, rules out the diagnosis of the condition. The origin of the vasculitis lesions is not known, but seems to be related to dysregulation of interferon production and secretion. CONCLUSION: Currently about 75 patients have been reported, even though many more probably exist. The study of this syndrome can contribute to the understanding of some mechanisms of CNS calcification and in a broader perspective to that of chronic encephalopathies with dysregulation of immune mechanisms.

Is the management of hepatitis C patients appropriate? A population-based study.

BACKGROUND: In order for hepatitis C patients to receive antiviral treatment, they must reach medical care. AIM: To assess the proportion of patients reaching medical care after hepatitis C diagnosis in a general population (1 006 171 inhabitants) in France. METHODS: Between 1994 and 1999, 1508 cases were diagnosed, of which 1251 were eligible for the study. RESULTS: Two-hundred and two patients did not have any medical care; among them, 55.4% had normal alanine transferase, 58.4% had risk factors related to lifestyle and 22.8% were alcoholics. Amongst the 1049 other patients, 41.6% had a liver biopsy, 25.0% were treated. Treatment was more often carried out in males than in females (OR: 1.59; P = 0.001), and in patients under 65 than in older patients (OR: 2.22; P < 0.008). Among non-treatment reasons, alcoholism (P = 0.001), drug-addiction (P = 0.04) and escaping monitoring (P = 0.04) were more frequent in males than in females, whereas normal alanine transferase was more frequent in females than in males (P = 0.004). Amongst 278 patients with a Metavir score >A1F1, 71 (25.5%) did not undergo treatment. CONCLUSION: In a general population, one patient in six did not receive on-going health care; a quarter of patients with a Metavir score >A1F1 did not receive any treatment. These results showed insufficient clinical management, which could compromise the effectiveness of treatment in general population.

Left thoracoscopic thymectomy in children.

BACKGROUND: In the management of autoimmune myasthenia, thymectomy is recognized as effective surgical therapy. The necessity of complete radical thymectomy to achieve maximal improvement has been emphasized. Video-assisted thoracoscopic surgery has been successfully used for thymectomy in adults, and more recently in children, and has been described as achieving the same radicality and functional improvement as median sternotomy or as transcervical thymectomy. The aim of this work is to report our first thoracoscopic experience in this indication. METHODS: Patients with myasthenia gravis on anticholinesterase drugs and/or steroids are discussed for surgery in case of clinical deterioration despite increasing doses of medication or in case of no improvement. We decided to perform thoracoscopic thymectomies by a left-sided approach. Preoperative localization of thymic tissue is done by a thoracic CT exam. Patients are placed on their right side with a thoracic tilt under the thorax. Four thoracoscopic ports are used, a 10-mm for the camera and three 5-mm operating ports. The left lung was collapsed by selective intubation (double-lumen endotrachial intubation). RESULTS: Two boys, 7.5 and 14 years old, were addressed by the department of neurology for radical thymectomy. They presented an ocular myasthenia gravis for 2 years and a mild general myasthenia gravis for 7 years. The operative times were 120 and 240 min. There was no intraoperative or postoperative complication. Duration of thoracic drainage was 2 days. The children were discharged on the third postoperative day. For the second procedure, an ultrasound exam during surgery was necessary to localize the thymus exactly, thus enabling its complete resection without the need for a conversion. The follow-up is 19 and 7 months with a clinical improvement enabling the diminution of medication for both children, the end of ptosis for the first child, and the general improvement of muscle strength for the second. CONCLUSIONS: Thoracoscopic thymectomy in children with juvenile myasthenia gravis seems to offer a complete surgical resection, as do open techniques. In case of difficulties in finding the thymus, an ultrasound exam is feasible to enable complete resection. The left-sided thoracoscopic approach gives a good mediastinal and cervical exposition. Furthermore, being less painful in the postoperative period, it presents a less pronounced impairment of pulmonary function, and it presents good cosmetic effect.

[Practical considerations for the treatment of acute rhythm disturbances. Report of Belgium Interdisciplinary Working Group on Acute Cardiology]. / Recommandations pratiques pour le traitement d'arythmies aiguës. Rapport du Groupe Interdisciplinaire Belge de Cardiologie Aiguë.

Acute arrhythmia is a condition covering a wide variety of rhythm disturbances. The aim of the article is to give practical recommendations for the approach and the treatment of the patient presenting with an acute arrhythmia. We discuss bradycardia and tachycardia. Tachycardias are divided into the small QRS complex tachycardias and the wide QRS complex tachycardias. Another important distinction with immediate therapeutic consequences is that between the hemodynamic stable and unstable patient. Flowcharts with diagnostic means and therapeutic schemes are added and a table with practical considerations for electrical cardioversion.