European Consensus Statement Research Articles

442 European consensus statement on phenotypes of pustular psoriasis

442 European consensus statement on phenotypes of pustular psoriasis

Experience in Cochlear Reimplantation. Descriptive Study of a 20-Year Period

Introduction and objectivesThe cochlear implant is a surgical procedure that has increased substantially, because the paediatric population is diagnosed and implanted early and because there are increased potential indications. This device has the inherent risk of failure in performance, as dies any active medical device, which is the most common cause of implant removal. Our goal was to understand what the causes that produced removal in our series were, and confirm if these conformed to reality as reviewed in the literature. MethodsThis was a retrospective, descriptive, observational study of 859 cochlear implant surgeries carried out between October 1991 and May 2011. The causes of implant removal were classified according to the European Consensus Statement on Cochlear Implant Failures and Explantations. ResultsThe reimplantation rate was 6.16% (n=51). The most common reason for removal was technical device failure (45.5%), followed by infection/rejection (23.6%) and upgrade (12.7%). Less common causes: there were 3 cases (5.6%) of electrode misplacement, 2 cases (3.6%) of labyrinthine ossification, 2 (3.6%) as a result of head trauma, 2 (3.6%) from need for nuclear magnetic resonance imaging and 1 case (1.8%) from psychiatric illness. ConclusionsCochlear reimplantation is a safe procedure, with a low complication rate. In our centre, it reaches an overall rate of 6.16%. Technical device failure remains the most common cause of this procedure, although there is a significant percentage of reimplantation for device update

Easing the wheeze.

Easing the wheeze.

Expert consensus document: European Consensus Statement on congenital hypogonadotropic hypogonadism--pathogenesis, diagnosis and treatment.

Congenital hypogonadotropic hypogonadism (CHH) is a rare disorder caused by the deficient production, secretion or action of gonadotropin-releasing hormone (GnRH), which is the master hormone regulating the reproductive axis. CHH is clinically and genetically heterogeneous, with >25 different causal genes identified to date. Clinically, the disorder is characterized by an absence of puberty and infertility. The association of CHH with a defective sense of smell (anosmia or hyposmia), which is found in ∼50% of patients with CHH is termed Kallmann syndrome and results from incomplete embryonic migration of GnRH-synthesizing neurons. CHH can be challenging to diagnose, particularly when attempting to differentiate it from constitutional delay of puberty. A timely diagnosis and treatment to induce puberty can be beneficial for sexual, bone and metabolic health, and might help minimize some of the psychological effects of CHH. In most cases, fertility can be induced using specialized treatment regimens and several predictors of outcome have been identified. Patients typically require lifelong treatment, yet ∼10-20% of patients exhibit a spontaneous recovery of reproductive function. This Consensus Statement summarizes approaches for the diagnosis and treatment of CHH and discusses important unanswered questions in the field.

Comparative analysis between Sendai guidelines and the European experts consensus statement on BD-IPMN of the pancreas

Introduction: There is still room for discussion, from the point of view of pancreatic fistula (PF) prevention, how to dissect pancreas and treat pancreatic stump in distal pancreatectomy (DP). Standardization of DP is favorable. Aims: Here we discuss on standardization of DP by reviewing our own cases. Patients & methods: Ninety-four patients who underwent DP in our hospital betweenMay 2000 and September 2013were involved. According to usage of automatic suture devices (ASD), we defined four terms mentioned below. PF incidence was compared among these 4 terms; Term 1: each operator chose in his preference whether using ASD or by handsewing (HS), Term 2: no usage of ASD, Term 3: basically usage of ASD, Term 4: standardization of ASD usage. Standardization is as follows; Usage of ASD, Soft and gradual compression of pancreas parenchyma, Gradual firing of ASD, No additional suture for the cut end. Results: In Term 1, PF incidence in ASD group (n1⁄429)was 58.6%, whereas 25% in HS group (n1⁄48). In Term 2, all cases were classified into HS group (n1⁄48), and PF incidence was 43.5%. In Term 3, PF incidence in ASD group (n1⁄415)was46.7%,whereas 25% inHSgroup (n1⁄44). InTerm4, all inASDgroup (n1⁄415), PF incidence decreased lower to 13.3%. There was no significance on which type of ASD was the best from the point of view of PF incidence. Conclusion:When dividing pancreas in DP, we standardize to use ASD, with soft compression of the pancreas parenchyma and gradual firing.

Experiencia en reimplantación coclear. Estudio descriptivo durante un período de 20 años

Introduction and objectivesThe cochlear implant is a surgical procedure that has increased substantially, because the paediatric population is diagnosed and implanted early and because there are increased potential indications. This device has the inherent risk of failure in performance, as dies any active medical device, which is the most common cause of implant removal. Our goal was to understand what the causes that produced removal in our series were, and confirm if these conformed to reality as reviewed in the literature. MethodsThis was a retrospective, descriptive, observational study of 859 cochlear implant surgeries carried out between October 1991 and May 2011. The causes of implant removal were classified according to the European Consensus Statement on Cochlear Implant Failures and Explantations. ResultsThe reimplantation rate was 6.16% (n=51). The most common reason for removal was technical device failure (45.5%), followed by infection/rejection (23.6%) and upgrade (12.7%). Less common causes: there were 3 cases (5.6%) of electrode misplacement, 2 cases (3.6%) of labyrinthine ossification, 2 (3.6%) as a result of head trauma, 2 (3.6%) from need for nuclear magnetic resonance imaging and 1 case (1.8%) from psychiatric illness. ConclusionsCochlear reimplantation is a safe procedure, with a low complication rate. In our centre, it reaches an overall rate of 6.16%. Technical device failure remains the most common cause of this procedure, although there is a significant percentage of reimplantation for device update

Recommendations for the management of patients with homozygous familial hypercholesterolaemia: Overview of a new European Atherosclerosis Society consensus statement

Although homozygous familial hypercholesterolaemia (HoFH) is rare, patients with this disease have a poor prognosis, even when they receive the best available treatment, including pharmacotherapy and apheresis. The current therapeutic gap emphasizes the potential impact of new and developmental treatment options, which include lomitapide, mipomersen, anti-PCSK9 monoclonal antibodies and CETP inhibitors. It is imperative that patients with HoFH receive the most appropriate treatment as early as possible and clinical guidance is needed to provide clinicians with the information they require to expedite diagnosis and initiate effective treatment. Until now, however, guidance on the management of (HoFH) has generally been included as part of broader guidelines on dyslipidemia, FH or low-density lipoprotein (LDL)-apheresis and even in guidelines specifically on FH, HoFH has been under-represented. A consensus statement on recommendations for the management of HoFH has recently been published by a working group of the European Atherosclerosis Society. An outline of the content of the statement is presented in the current paper.

THU0520 Interferon (IFN)-Lambda1/3, but not IFN-Alpha, is A Marker of Poor Response to Treatment in Lupus Nephritis

BackgroundLupus nephritis (LN) is a severe manifestation of systemic lupus erythematosus (SLE) affecting up to 60% of the patients. IFN-α is a central cytokine in the pathogenesis for LN, and...

Evolution of MRI changes in Rasmussen's encephalitis

We studied the MRI findings in 16 patients with Rasmussen's encephalitis (RE), further analysed serial MRI changes in 11 of them and correlated it with clinical features. The diagnosis of RE was based on the European consensus statement (Brain, 128, 2005, 454). Details related to demographical, clinical, MRI observations were analysed. Forty MRIs of brain of 16 patients were reviewed. Eleven patients had undergone serial brain MRIs ranging from two to five occasions. All the patients had unihemispheric focal cortical atrophy, predominantly in the perisylvian region (n = 13). Other features were white matter signal changes (n = 14), and ipsilateral caudate (n = 6) and putamen (n = 4) atrophy. Signal alterations in putamen and caudate were noted in four each. In all the 11 patients with serial MRI, there was progression of cerebral atrophy and a trend towards increase in MRI staging. The MRI signal changes remained same in five patients, resolved in three patients, differential change in two patients and increased in one patient. Diffusion-weighted imaging showed facilitated diffusion (n = 5), and MR spectroscopy showed reduced N-acetyl-aspartate and elevated lactate (n = 2). Pattern recognition of MRI findings and the changes in serial MRI might serve as a surrogate marker of disease viz. unihemispheric progressive focal cortical atrophy and signal changes predominantly in the perisylvian distribution and caudate followed by putamen involvement. This might assist in understanding and monitoring of the disease progression.

Consensus statement on the diagnosis, treatment and follow‐up of patients with primary adrenal insufficiency

Primary adrenal insufficiency (PAI), or Addison's disease, is a rare, potentially deadly, but treatable disease. Most cases of PAI are caused by autoimmune destruction of the adrenal cortex. Consequently, patients with PAI are at higher risk of developing other autoimmune diseases. The diagnosis of PAI is often delayed by many months, and most patients present with symptoms of acute adrenal insufficiency. Because PAI is rare, even medical specialists in this therapeutic area rarely manage more than a few patients. Currently, the procedures for diagnosis, treatment and follow-up of this rare disease vary greatly within Europe. The common autoimmune form of PAI is characterized by the presence of 21-hydroxylase autoantibodies; other causes should be sought if no autoantibodies are detected. Acute adrenal crisis is a life-threatening condition that requires immediate treatment. Standard replacement therapy consists of multiple daily doses of hydrocortisone or cortisone acetate combined with fludrocortisone. Annual follow-up by an endocrinologist is recommended with the focus on optimization of replacement therapy and detection of new autoimmune diseases. Patient education to enable self-adjustment of dosages of replacement therapy and crisis prevention is particularly important in this disease. The authors of this document have collaborated within an EU project (Euadrenal) to study the pathogenesis, describe the natural course and improve the treatment for Addison's disease. Based on a synthesis of this research, the available literature, and the views and experiences of the consortium's investigators and key experts, we now attempt to provide a European Expert Consensus Statement for diagnosis, treatment and follow-up.

Evaluation of universal newborn hearing screening in Switzerland 2012 and follow-up data for Zurich

The European Consensus Statement of Neonatal Hearing recommended universal newborn hearing screening (UNHS) in 1998. UNHS was introduced in Switzerland in 1999 under the auspices of a "Swiss Working Group Hearing Screening in Newborns". The aim of this study was to evaluate the number of newborns being screened and consequently followed-up in Switzerland for the year 2012. Postal questionnaires were sent to all registered maternity clinics and birth-centres in Switzerland. To evaluate follow-up of newborns failing the screening process, a retrospective consecutive cohort analysis of newborns failing screening at the University Hospital Zurich between 2005 and 2010 was performed. A total of 102/110 (92.7%) maternity clinics and 1/14 (7.1%) birth-centres routinely performed UNHS. When weighted according to the number of births in the varying locations, 97.9% of all newborn received hearing screening. At the University Hospital of Zurich, 253/12,080 (2.1%) newborns failed the screening test and in 15/253 (6%) a relevant bilateral hearing impairment was found. This makes an overall incidence of congenitally relevant hearing loss of 0.12%. Unfortunately, 33/253 (13%) of newborns with failed screening were lost to follow-up. UNHS is well-established in Switzerland and the vast majority of newborns are screened. However, follow-up of failed screens is disappointing. Further measures need to be taken to improve follow up.

AB0379 Long-term renal outcome after remission induction with cyclophosphamide pulse therapy in patients with lupus nephritis – a single centre experience

BackgroundCyclophosphamide (Cyc) pulse therapy is the treatment of choice in patients with proliferative lupus nephritis (LN). Dependent on its definition, a remission of LN is achieved in 54 – 90...

European multidisciplinary consensus statement on the use and monitoring of metal-on-metal bearings for total hip replacement and hip resurfacing

There is an ongoing debate about the optimal use of metal-on-metal (MoM) bearings in total hip replacement, since there are uncertainties about local and systemic adverse effects due to wear and corrosion of these bearings. Despite various national recommendations, efforts to achieve international harmonization of specific evidence-based recommendations for best practice are still lacking. An international consensus study group should be able to develop recommendations on the use and monitoring of MoM bearings, preferably at the European level, through a multidisciplinary approach, by integrating the perspectives of various stakeholders. Twenty-one experts representing three stakeholder groups and eight countries participated in this European consensus study, which consisted of a consensus meeting, subsequent structured discussion, and consensus voting. The current statement defines first of all benefits, local and systemic risks, as well as uncertain issues related to MoM bearings. Safety assessment after implantation of MoM comprises all patients. A closer follow-up is recommended for large head MoM (≥36mm) and resurfacing. In these implants basic follow-up should consist of x-rays and metal ion measurement of cobalt in whole blood, performed with GF-AAS or ICP-MS. Clinical and/or radiographic abnormality as well as elevated ion levels needs additional imaging (ultrasound, CT-scan and/or MARS-MRI). Cobalt values less than 2 μg/L are probably devoid of clinical concern, the threshold value for clinical concern is expected to be within the range of 2-7 μg/L. This is the first multinational, interdisciplinary, and multiprofessional approach for developing a recommendation for the use and monitoring of MoM bearings in total hip replacement. The current recommendations are in partial agreement with previous statements regarding the extent of follow-up and imaging techniques. They however differ from previous communications regarding measurement of metal ions and especially the investigated medium, technique, and eventual threshold levels. Level V, expert opinion/agreement conference.

European experts consensus statement on cystic tumours of the pancreas

Cystic lesions of the pancreas are increasingly recognized. While some lesions show benign behaviour (serous cystic neoplasm), others have an unequivocal malignant potential (mucinous cystic neoplasm, branch- and main duct intraductal papillary mucinous neoplasm and solid pseudo-papillary neoplasm). European expert pancreatologists provide updated recommendations: diagnostic computerized tomography and/or magnetic resonance imaging are indicated in all patients with cystic lesion of the pancreas. Endoscopic ultrasound with cyst fluid analysis may be used but there is no evidence to suggest this as a routine diagnostic method. The role of pancreatoscopy remains to be established. Resection should be considered in all symptomatic lesions, in mucinous cystic neoplasm, main duct intraductal papillary mucinous neoplasm and solid pseudo-papillary neoplasm as well as in branch duct intraductal papillary mucinous neoplasm with mural nodules, dilated main pancreatic duct >6mm and possibly if rapidly increasing in size. An oncological partial resection should be performed in main duct intraductal papillary mucinous neoplasm and in lesions with a suspicion of malignancy, otherwise organ preserving procedures may be considered. Frozen section of the transection margin in intraductal papillary mucinous neoplasm is suggested. Follow up after resection is recommended for intraductal papillary mucinous neoplasm, solid pseudo-papillary neoplasm and invasive cancer.

Long-term follow-up in lupus nephritis patients treated with rituximab--clinical and histopathological response

To investigate the long-term clinical, histological and serological affects of B-cell-depleting therapy (BCDT) in patients with LN refractory to conventional treatment. Twenty-five patients, followed for a mean time of 36 months (9-95 months), were included. Renal disease activity was evaluated with the BILAG index and renal response was determined according to the LN European consensus statement. Renal biopsies were performed for histological evaluation at baseline and follow-up. Partial response (PR) or complete renal response (CR) was observed in 22 of 25 after a median of 12 months. Sixteen patients achieved CR after a median of 24 months. Six patients experienced a renal relapse. Proteinuria decreased significantly (P = 0.0002) from baseline to 36 months. A noteworthy histological improvement was seen in nearly all patients with a significant reduction in activity index (P = 0.01). Longer depletion time and low baseline values of IgM were indicative of achieving clinical remission during the first year after treatment (P = 0.03 and P = 0.04, respectively). In therapy-resistant LN, BCDT induced clinical and histological improvements in the majority of patients. Transition from PR to CR was mainly seen during the second year of follow-up. Patients with longer depletion time and low baseline levels of IgM were more likely to gain a faster remission, suggesting that the clinical benefit may be linked to suppression of autoreactive plasmablasts. Although formal evidence of BCDT in LN is lacking, our data may provide guidance to clinicians considering therapeutic options in patients with refractory LN.

Mycophenolate mofetil versus intravenous cyclophosphamide for induction treatment of proliferative lupus nephritis in a Japanese population: a retrospective study

Objectives: Recent studies have shown that mycophenolate mofetil (MMF) is similar to intravenous cyclophosphamide (IVC) for the treatment of lupus nephritis (LN), but that treatment response may vary according to location and race/ethnicity. Moreover, no studies have been conducted to compare the efficacy of MMF with that of IVC for a Japanese population. We therefore conducted a retrospective study to clarify the efficacy and safety of MMF compared with that of IVC for induction therapy for active LN, classes III and IV, in a Japanese population of 21 patients, 11 of whom received MMF and 10 IVC.Methods: The primary endpoint was expressed as the percentage of responders, who in turn were defined as the patients who met complete or partial response criteria according to the European consensus statement. The secondary endpoints comprised the renal activity component and serological activity.Results: The primary endpoint was achieved in nine (81.8 %) patients receiving MMF and in four (40.0 %) receiving IVC, with no significant difference between the two groups (p = 0.081), while there was also no significant difference between them in terms of secondary endpoints. However, the MMF group suffered significantly fewer hematologic toxic effects than the IVC group.Conclusions: MMF may be used as an alternative to IVC for inducing renal remission of LN in Japanese patients.

Inhaler competence in asthma: Common errors, barriers to use and recommended solutions

Whilst the inhaled route is the first line administration method in the management of asthma, it is well documented that patients can have problems adopting the correct inhaler technique and thus receiving adequate medication. This applies equally to metered dose inhalers and dry powder inhalers and leads to poor disease control and increased healthcare costs. Reviews have highlighted these problems and the recent European Consensus Statement developed a call to action to seek solutions. This review takes forward the challenge of inhaler competence by highlighting the issues and suggesting potential solutions to these problems. The opportunity for technological innovation and educational interventions to reduce errors is highlighted, as well as the specific challenges faced by children. This review is intended as a policy document, as most issues faced by patients have not changed for half a century, and this situation should not be allowed to continue any longer. Future direction with respect to research, policy needs and practice, together with education requirements in inhaler technique are described.

Hybrid Thoracoscopic Surgical and Transvenous Catheter Ablation of Atrial Fibrillation

The purpose of this study was to evaluate the feasibility, safety, and clinical outcomes up to 1 year in patients undergoing combined simultaneous thoracoscopic surgical and transvenous catheter atrial fibrillation (AF) ablation. The combination of the transvenous endocardial approach with the thoracoscopic epicardial approach in a single AF ablation procedure overcomes the limitations of both techniques and should result in better outcomes. A cohort of 26 consecutive patients with AF who underwent hybrid thoracoscopic surgical and transvenous catheter ablation were followed, with follow-up of up to 1 year. Twenty-six patients (42% with persistent AF) underwent successful hybrid procedures. There were no complications. The mean follow-up period was 470 ± 154 days. In 23% of the patients, the epicardial lesions were not transmural, and endocardial touch-up was necessary. One-year success, defined according to the Heart Rhythm Society, European Heart Rhythm Association, and European Cardiac Arrhythmia Society consensus statement for the catheter and surgical ablation of AF, was 93% for patients with paroxysmal AF and 90% for patients with persistent AF. Two patients underwent catheter ablation for recurrent AF or left atrial flutter after the hybrid procedure. A combined transvenous endocardial and thoracoscopic epicardial ablation procedure for AF is feasible and safe, with a single-procedure success rate of 83% at 1 year.

Bleeding risk assessment and management in atrial fibrillation patients. Key messages for clinical practice from the European Heart Rhythm Association position statement

The prevention of thromboembolism is the main therapeutic goal in patients with atrial fibrillation (AF). Vitamin K antagonists have been proved highly effective in preventing thromboembolic events in patients with AF and despite recent advances in oral anticoagulation they remain the most widely used agents. Anticoagulation increases the incidence of bleeding; however, in the field of stroke prevention in AF the clinical benefit of vitamin K antagonists clearly outweighs potential risks. The annual incidence of major bleeding among individuals with AF on oral anticoagulation varies widely, ranging from 1.3% to 7.2%. Several factors affect bleeding risk including the intensity of anticoagulation, the efficacy of monitoring modalities, and patient characteristics. This multifactorial etiology makes prediction of bleeding risk complex, necessitating the derivation and validation of clinical prediction tools for the estimation of total bleeding risk in clinical practice. The present review summarizes data on definition, risk prediction, prevention, and management of oral anticoagulation‑associated bleeding as reflected by the recent European Heart Rhythm Association consensus statement.

Mycophenolate mofetil versus intravenous cyclophosphamide for induction treatment of proliferative lupus nephritis in a Japanese population: a retrospective study

Recent studies have shown that mycophenolate mofetil (MMF) is similar to intravenous cyclophosphamide (IVC) for the treatment of lupus nephritis (LN), but that treatment response may vary according to location and race/ethnicity. Moreover, no studies have been conducted to compare the efficacy of MMF with that of IVC for a Japanese population. We therefore conducted a retrospective study to clarify the efficacy and safety of MMF compared with that of IVC for induction therapy for active LN, classes III and IV, in a Japanese population of 21 patients, 11 of whom received MMF and 10 IVC. The primary endpoint was expressed as the percentage of responders, who in turn were defined as the patients who met complete or partial response criteria according to the European consensus statement. The secondary endpoints comprised the renal activity component and serological activity. The primary endpoint was achieved in nine (81.8%) patients receiving MMF and in four (40.0%) receiving IVC, with no significant difference between the two groups (p=0.081), while there was also no significant difference between them in terms of secondary endpoints. However, the MMF group suffered significantly fewer hematologic toxic effects than the IVC group. MMF may be used as an alternative to IVC for inducing renal remission of LN in Japanese patients.