Lead and generator dysfunction in children and adolescents with epicardial pacemaker and implantable cardioverter defibrillator systems: The challenge of early recognition.

BACKGROUND: A major issue of cardiac implantable electronic device therapy in pediatric patients is the high incidence of lead dysfunctions and associated reinterventions. This study aims to analyze the timing and mode of generator and lead dysfunction. METHODS: Retrospective single-center analysis of 283 children and young adults with an epicardial pacemaker or implantable cardioverter defibrillator therapy from 1998 to 2018. RESULTS: Mean age at implant was 6.1 years (SD ± 5.8 years) and median follow-up 6.4 years (IQR, 3.4-10.4 years) with a total of 1998.1 patient-years of cardiac device therapy. A total of 120 lead-related complications were observed in 82 patients (29.0%). They were detected by device interrogation (n = 86), symptoms (n = 13), intraoperative findings (n = 7), routine chest radiography (n = 5), routine ECG (n = 4), patient alert sound by device (n = 3), and physical examination (n = 2). It was possible to find the date of the event on the device memory in 21 out of 120 lead dysfunctions (18%) with a median time interval between occurrence and detection of 1.3 months (IQR, 0.2-5.0 months). Moreover, 20 generator-related complications were found in 13 patients. CONCLUSIONS: Early recognition of lead and generator dysfunction remains challenging in pediatric patients. As symptoms are relatively rare conditions in the context of PM and ICD dysfunction, close patient monitoring is mandatory, even in asymptomatic patients with a good clinical course. To further improve the safety of pediatric pacing systems, more durable epicardial electrodes are desirable.

Fungicide Resistance in Botrytis spp. and Regional Strategies for Its Management in Northern European Strawberry Production.

Grey mould, caused by Botrytis cinerea and other Botrytis spp., is a major cause of fruit rot in strawberries and other fruit crops worldwide. Repeated fungicide applications are essential in order to secure harvests. However, resistance to all currently registered single-site fungicides is widespread. The rising importance of strains with multiple resistance to most or all fungicides is of particular concern. These strains may be introduced into fields via contaminated nursery plants and/or by immigration from adjacent plots. On the basis of research conducted in northern German and Danish strawberry production, a concept to manage fungicide resistance under northern European conditions has been developed and put into regional strawberry production practice. This principally includes the testing of nursery plants for fungicide-resistant Botrytis strains prior to planting; the restricted and specific use of fungicides at flowering in the production fields, taking account of the resistance spectrum within the local Botrytis population; and crop sanitation measures such as the removal of rotting fruits at the beginning of harvest. Further options such as protected cultivation, reduced fertilisation and biological control are also discussed. The practical implementation of such a strategy in northern Germany and Denmark has been shown to reduce the occurrence of multi-resistant strains to a tolerable steady-state level.

Homologue Composition of Technical Chlorinated Paraffins Used in Several Countries over the Last 50 Years─SCCPs Are Still Out There.

Chlorinated paraffins (CPs) are widely produced chemicals, with certain CP subgroups facing global restrictions due to their environmental dispersion, persistence, bioaccumulation, and toxicity. To evaluate the effectiveness of these international restrictions, we assessed the homologue group contribution and the mass fraction of short-chain CPs (SCCPs: C10-C13), medium-chain CPs (MCCPs: C14-C17), and long-chain CPs (LCCPs: ≥C18) in 36 technical CP mixtures used worldwide over the last 50 years. Using low-resolution mass spectrometry (LC-ESI-MS/MS), we quantified 74 CP homologue groups (C10Cl4-C20Cl10). Additionally, high-resolution mass spectrometry (LC-ESI-QTOF-MS) screening was employed to identify unresolved CP contents, covering 375 CP homologue groups (C6Cl4-C30Cl30). Overall, 1 sample was mainly composed of

Target and non-target analysis of per- and polyfluoroalkyl substances in representative chrome mist suppressants on the Chinese market.

Fluorinated chrome mist suppressants (CMSs) have been widely used in the electroplating industry globally, including China. In compliance with the Stockholm Convention on Persistent Organic Pollutants, China has phased out perfluorooctane sulfonate (PFOS) as CMS, except for closed-loop systems, before March 2019. Since then, several alternatives have been introduced to replace PFOS, but many of them still belong to the per- and polyfluoroalkyl substances (PFASs) family. In this study, for the first time, we collected and analyzed CMS samples from the Chinese market in 2013, 2015, and 2021 to determine their PFAS composition. For products with relatively few PFAS targets, we performed a total fluorine (TF) screening test and suspect and non-target analysis. Our findings suggest that 6:2 fluorotelomer sulfonate (6:2 FTS) has become the primary alternative on the Chinese market. Surprisingly, we identified 8:2 chlorinated polyfluorinated ether sulfonate (8:2 Cl-PFAES) as the primary ingredient in a CMS product (F-115B), which is the longer chain modification of the classical CMS product (F-53B). Furthermore, we identified three novel PFASs as PFOS alternatives, including hydrogen-substituted perfluoroalkyl sulfonates (H-PFSAs) and perfluorinated ether sulfonates (O-PFSAs). We also screened and identified six hydrocarbon surfactants in PFAS-free products as the primary ingredients. Despite this, some PFOS-based CMSs remain on the Chinese market. To prevent the opportunistic use of PFOS for illegal purposes, it is essential to enforce regulations strictly and ensure that such CMSs are used only in closed-loop chrome plating systems.

Industrial source identification of polyhalogenated carbazoles and preliminary assessment of their global emissions.

Polyhalogenated carbazoles (PHCZs) are emerging global pollutants found in environmental matrices, e.g., 3000 tonnes of PHCZs have been detected in the sediments of the Great Lakes. Recognition of PHCZ emissions from ongoing industrial activities worldwide is still lacking. Here, we identify and quantify PHCZ emissions from 13 large-scale industries, 12 of which previously have no data. Congener profiles of PHCZs from investigated industrial sources are clarified, which enables apportioning of PHCZ sources. Annual PHCZ emissions from major industries are estimated on the basis of derived emission factors and then mapped globally. Coke production is a prime PHCZ emitter of 9229 g/yr, followed by iron ore sintering with a PHCZ emission of 3237 g/yr. China, Australia, Japan, India, USA, and Russia are found to be significant emitters through these industrial activities. PHCZ pollution is potentially a global human health and environmental issue.

Antiarrhythmic Medication in Neonates and Infants with Supraventricular Tachycardia.

Supraventricular tachycardia (SVT) is the most common arrhythmia in neonates and infants, and pharmacological therapy is recommended to prevent recurrent episodes. This retrospective study aims to describe and analyze the practice patterns, effectiveness, and outcome of drug therapy for SVT in patients within the first year of life. Among the 67 patients analyzed, 48 presented with atrioventricular re-entrant tachycardia, 18 with focal atrial, and one with atrioventricular nodal re-entrant. Fetal tachycardia was reported in 27%. Antiarrhythmic treatment consisted of beta-receptor blocking agents in 42 patients, propafenone in 20, amiodarone in 20, and digoxin in 5. Arrhythmia control was achieved with single drug therapy in 70% of the patients, 21% needed dual therapy, and 6% triple. Propafenone was discontinued in 7 infants due to widening of the QRS complex. After 12 months (6-60), 75% of surviving patients were tachycardia-free and discontinued prophylactic treatment. Patients with fetal tachycardia had a significantly higher risk of persistent tachycardia (p: 0.007). Prophylactic antiarrhythmic medication for SVT in infancy is safe and well tolerated. Arrhythmia control is often achieved with single medication, and after cessation, most patients are free of arrhythmias. Infants with SVT and a history of fetal tachycardia are more prone to suffer from persistent SVT and relapses after cessation of prophylactic antiarrhythmic medication than infants with the first episode of SVT after birth.

Enhancing Scientific Support for the Stockholm Convention's Implementation: An Analysis of Policy Needs for Scientific Evidence.

The Stockholm Convention is key to addressing the global threats of persistent organic pollutants (POPs) to humanity and the environment. It has been successful in identifying new POPs, but its national implementation remains challenging, particularly by low- and middle-income Parties. Concerted action is needed to assist Parties in implementing the Convention's obligations. This analysis aims to identify and recommend research and scientific support needed for timely implementation of the Convention. We aim this analysis at scientists and experts from a variety of natural and social sciences and from all sectors (academia, civil society, industry, and government institutions), as well as research funding agencies. Further, we provide practical guidance to scientists and experts to promote the visibility and accessibility of their work for the Convention's implementation, followed by recommendations for sustaining scientific support to the Convention. This study is the first of a series on analyzing policy needs for scientific evidence under global governance on chemicals and waste.

Brazilian overview of per- and polyfluoroalkyl substances listed as persistent organic pollutants in the stockholm convention.

PFAS are a group of organic chemicals, which some presents environmental persistence, dispersion and potential toxicity. Some of them have been listed in the Stockholm Convention as persistent organic pollutants (POPs) to have its production and use restricted, namely PFOS, its salts and PFOSF and PFOA, its salts and related substances. As a Party, Brazil has to comply with the Convention provisions regarding the control of POPs. In order to develop listed PFAS inventories in the country, the Convention guidance documents were used. Stakeholders were consulted and trade data was assessed. Complementary, a review on listed PFAS occurrence in Brazil was performed. From over 1000 queries sent, only 3 answers were received. International trade data showed an import of 93.7 tonnes of PFOSF from China and export of sulfluramid-base ant bait to other developing countries. Domestic trade data showed that around 28 t per year of EtFOSA is commercialized in Brazil. The EtFOSA sold internally could lead to emissions of up to 616 t of PFOS. With domestic and foreign trade data it was possible to estimate the production of EtFOSA from 2010 to 2018 to 40 t per year. Only 10 studies reported listed PFAS occurrence in Brazilian environmental matrices. All of them reported ubiquitous occurrence of listed PFAS in the country, being PFOS the predominant in terms of occurrence and concentration. Brazil needs to develop strategies to overcome the low engagement of stakeholders and enforce control over listed PFAS foreign trade.

Remobilization of pollutants during extreme flood events poses severe risks to human and environmental health.

While it is well recognized that the frequency and intensity of flood events are increasing worldwide, the environmental, economic, and societal consequences of remobilization and distribution of pollutants during flood events are not widely recognized. Loss of life, damage to infrastructure, and monetary cleanup costs associated with floods are important direct effects. However, there is a lack of attention towards the indirect effects of pollutants that are remobilized and redistributed during such catastrophic flood events, particularly considering the known toxic effects of substances present in flood-prone areas. The global examination of floods caused by a range of extreme events (e.g., heavy rainfall, tsunamis, extra- and tropical storms) and subsequent distribution of sediment-bound pollutants are needed to improve interdisciplinary investigations. Such examinations will aid in the remediation and management action plans necessary to tackle issues of environmental pollution from flooding. River basin-wide and coastal lowland action plans need to balance the opposing goals of flood retention, catchment conservation, and economical use of water.

Advanced Imaging and New Cardiac Biomarkers in Long-term Follow-up After Childhood Cancer.

OBJECTIVES: Pathologic ejection fraction (EF), shortening fraction (FS), and standard heart failure biomarkers (high sensitive troponin T and N-terminal brain natriuretic peptide) during follow-up after childhood cancer have been associated with irreversible cardiac damage. We aimed to evaluate strain imaging values by echocardiography and new biomarkers for heart failure with preserved ejection fraction (HFpEF) as potential more sensitive parameters for cardiac deterioration in childhood cancer survivors (CCS). MATERIALS AND METHODS: Prospective study with 50 CCS (median 16.2 y) at a median follow-up of 13 years. In addition to standard echo and laboratory parameters for heart failure, strain measurements and new biomarkers, including myocardial inflammation (interleukin 6), extracellular matrix (ECM) remodeling (C-telopeptide for type I collagen, intact N-terminal propeptide of type III procollagen), and other heart failure biomarkers (galectin 3, solutable ST2, growth differentiation factor 15), were obtained and compared with 50 healthy controls. RESULTS: No significant differences in EF, FS, high sensitive troponin T, N-terminal brain natriuretic peptide, interleukin 6, solutable ST2, and galectin 3 were found between study and control groups. In contrast, strain imaging showed significant differences between both groups (global longitudinal strainGLS -16.1% vs. -20.4%, P<0.0001; global circumferential strain -14.3 vs. -21.4%, P<0.0001), detecting 66% (global longitudinal strain) and 76% (global circumferential strain) of patients with pathologic values in contrast to 6% (EF) and 16% (FS) for standard parameters. Markers for disturbances of ECM remodeling (C-telopeptide for type I collagen, intact N-terminal propeptide of type III procollagen, each P<0.0001) and growth differentiation factor 15 (P<0.0001) were significantly different between the groups. CONCLUSION: Strain imaging and new cardiac biomarkers used in HFpEF focusing on ECM remodeling appear to be more sensitive in detecting early remodeling processes in CCS than standard echo and laboratory parameters.

Inventory approach for short-chain chlorinated paraffins for the Stockholm Convention implementation in Brazil.

Chlorinated paraffins (CPs) are chemicals with multipurpose applications. Their global production has increased despite their adverse impacts on the environment and human health. In 2017, short-chain chlorinated paraffins (SCCPs) were listed as persistent organic pollutants (POPs) in the Stockholm Convention. Yet, specific exemptions were granted for their applications despite the recycling prohibition for products containing SCCPs. Therefore, we aimed to produce the first Brazilian inventory of SCCPs following its respective guidance to evaluate the applicability of the SCCP inventory guidance and to provide technical insights regarding SCCPs in the update of the Brazilian National Implementation Plan (NIP). Moreover, we performed a review of SCCP occurrence in Brazil to fulfil data gaps in the inventory development. We identified and consulted nationwide stakeholders and assessed foreign trade data of CPs and products that might contain CPs in relevant amounts. The Brazilian production of CPs was discontinued in 1994. However, CPs are still imported and used in the country. CPs have been mostly applied as plasticizers, flame retardants and lubricants in Brazil. The import of products containing CPs also pose a significant route of CP entrance into Brazil. Thus, the current end-of-life management of CP-containing products is a bottleneck towards the Convention implementation. The guidance application was feasible and useful despite the low engagement of stakeholders. To assess foreign trade of CPs and CP-containing products, we recommend the use of more specific tracking codes. Besides, the review of SCCP occurrence is not a demanded part for an inventory but was a useful complementation.

Developing potency factors for thyroid hormone disruption by PFASs using TTR-TRß CALUX® bioassay and assessment of PFASs mixtures in technical products.

BACKGROUND: Over the last decade, per- and polyfluoroalkyl substances (PFASs) have become one of the most heavily investigated persistent organohalogen compound class of environmental concern. However, knowledge about their toxicology is still scarce, although PFASs as individual compounds and their industrial mixtures were shown to exert effects on the thyroid hormone system. METHODS: In vitro toxicity potency factors were established for thyroid hormone transport disruption potential using the novel TTR-TRß CALUX® bioassay for major PFASs. We assessed technical PFASs mixtures, including aqueous film-forming foam (AFFF) surfactants and chromium mist suppressants (CMS) applications with and without total oxidizable precursor (TOP) by TTR-TRß CALUX® assay for their thyroid hormone transport disrupting potential. RESULTS: All PFASs listed in the German guideline for drinking water (German Environment Agency, 2017) affected the T4 binding to TTR, an important plasma thyroid hormone transport protein. For all tested PFASs, potency factors based on PC80 values relative to PFOA could be obtained and ranged between PFBA (0.0018) and PFOS (2.0). Applying in vitro potency factors obtained from the present in vitro TTR-TRß CALUX® assay study and recently reported in vivo potency factors (Zeilmaker et al., 2018; Bil et al., 2021) on the above-mentioned German guideline for PFAS in drinking water, showed that the cumulative effect-based trigger values (in vivo and in vitro) are comparable (3.0 vs. 2.9 to 4.6 µg PFOA-EQ/l). Additionally, AFFF surfactants and CMS with and without TOP assay were tested. Highest activities were found in the older AFFF surfactants (2013/2014) due to higher PFOS/PFOA levels, which were already substituted with 6:2 FTS in 2019, resulting in much lower PFOA-EQ levels. As expected also the PFOA-EQ levels increased in the samples with TOP treatment compared to the original AFFF surfactants and CMS as confirmed here by biological and chemical PFOA-equivalents (PFOA-EQ) analysis. Additionally, CMS (which have been used in the electroplating chromium industry since the 1950s) as well as PFOS-free, but not PFAS-free fume suppressants (such as Fumetrol® 21) have been tested in the TTR-TRß CALUX® assay and showed much lower activity levels then the AFFFs, confirmed by the similar potency determination based on chemical PFASs analysis followed by transformation to PFOA-EQ for comparison. The potency factor of 6:2 FTS, which is the main substitute for PFOS in CMS, indicates that it is approximately 100-times less potent as a thyroid hormone disruptor as compared to PFOS. CONCLUSION: Potency factors based on PC80 values from TTR-TRß CALUX® relative to PFOA have been developed for major PFASs. In AFFF surfactants and CMS a trend of higher activities with higher amounts of PFOS and PFOA have been found. PFOA and PFOS showed high responses in the TTR-TRß CALUX® assay and had the largest contributions to the PFOA-EQs in the AFFF surfactants and CMS applications. Using potency factors as determined in the TTR-TRß CALUX® to convert PFASs assessed by chemical analysis to PFOA-EQ led to comparable results as compared to the results from PFASs measured directly by the TTR-TRß CALUX® assay. This study supports the claim that semiquantitative effect- and group-based in vitro CALUX bioanalysis tools can be applied effectively to assess industrial products containing complex mixtures with PFAS compounds for which no instrumental analysis are established, and for many compounds where in vitro toxicity data are not yet available.

Distribution and Emission Estimation of Short- and Medium-Chain Chlorinated Paraffins in Chinese Products through Detection-Based Mass Balancing.

Short- and medium-chain chlorinated paraffins (SCCPs and MCCPs, respectively) have raised environmental concern due to their potential for persistence, long-range transport, bioaccumulation, and toxicity. However, little is known about the production, use, and environmental emissions of SCCPs and MCCPs in China, the world's largest producer and consumer. In this study, we estimated the amounts of SCCPs and MCCPs produced and used in China in 2018-2019 based on a nationwide survey and measurements of concentrations in products, from which we estimated the environmental emissions of SCCPs and MCCPs in China. Our results show that 225.2 and 236.4 metric kilotons (kt) of SCCPs and 428.5 and 450.2 kt of MCCPs were used in China in 2018 and 2019, respectively, with poly(vinyl chloride) (PVC) products dominating SCCP and MCCP usage. Moreover, a total of 3.9 and 4.2 kt SCCPs and 3.8 and 4.1 kt MCCPs were emitted into China's environment in 2018 and 2019, respectively. Although less MCCPs are released into the air relative to SCCPs, their level exceeds the emission of SCCPs into soil. Finally, detailed mass balance calculation indicates that, although emissions from the use of PVC products dominate SCCP and MCCP inputs into the air, emissions from the use of polyurethane foam adhesives are more closely related to input into surface waters for SCCPs and MCCPs. For input into soil, the main emission sources are the use of polyurethane foam adhesives (for SCCPs) and rubber products (for MCCPs). This study provides a preliminary overview of the distributions of SCCPs and MCCPs in products and insight into the mass balance of SCCPs and MCCPs from their production and use to emission in China. This assessment also provides an important foundation for better understanding the environmental risks and fates associated with SCCPs and MCCPs in China and around the world.

[Postoperative hypocalcemia - the most common complication of endocrine head and neck surgery: acute management]. / Postoperative Hypokalzämie ­ die häufigste Komplikation der endokrinen Chirurgie der Halsregion: Akutes Management.

BACKGROUND: In Germany, 8000 patients are affected by postoperative hypoparathyroidism per year following surgery of the thyroid gland, parathyroidal glands and the larynx. Patients do not only suffer from paresthesia in the acute phase of this complication, but are also adversely affected by the fear of loss of control following episodes of tetany even years after the first episode. OBJECTIVES: Discussion of a diagnostic pathway and presentation of a management pathway for postoperative hypocalcemia. METHODS: Narrative review, analysis and discussion of current literature and expert recommendations. RESULTS: Early determination of calcium and parathyroid hormone allows timely diagnosis and treatment of postoperative hypoparathyroidism. Active vitamin D is pivotal for the resorption of calcium. Only the combined treatment with active vitamin D and calcium can mitigate or prevent the postoperative drop of calcium levels. CONCLUSIONS: A standard operating procedure (SOP) for postoperative hypoparathyroidism should be implemented in every surgical department. An SOP for diagnosis and treatment of postoperative hypoparathyroidism is proposed for institutional individualization and implementation.

Polybrominated diphenyl ethers (PBDEs) concentrations in soil and plants around municipal dumpsites in Abuja, Nigeria.

Polybrominated diphenyl ethers (PBDEs) are listed as persistent organic pollutants (POPs) in the Stockholm Convention. It has been established that PBDEs may be released into the environment during improper handling and disposal of e-waste and other products containing PBDEs that is prevalent in developing countries. This research work assessed the status of PBDE contamination at dumpsites in Nigeria. Soil and edible plant samples were collected from the dumpsites and control sites for analysis. The concentrations of ∑7PBDE in the topsoils around the dumpsites at 0-15 cm depth ranged from 112 to 366 ng/g dry weight (dw) while that of the topsoil of the control site 500 m from the dumpsite ranged from 26.8 to 39.7 ng/g dw. These high concentrations stem likely from open burning of waste including electronic waste on the landfills. Plant samples (bentgrass, spinach, tomatoes, pumpkin and sweet potatoes) around the dumpsites were found to be contaminated by PBDEs with levels ranging from 25.0 to 60.5 ng/g dw in plant roots and from 8.45 to 32.2 ng/g dw in plant shoots for ∑7PBDE. This suggests that consumption of vegetables by humans and ingestion of contaminated soils and feed by chickens and cows can transfer PBDEs into the human food chain around the dumpsites. The comparison of PBDE levels in soils and the PBDE levels in chicken eggs from the former study indicate that PBDE levels in the soils are sufficient to explain the levels in the chicken eggs with a reasonable carry-over rate for PBDEs of 0.28 on average. The PBDE contamination in the soil was sufficient to result in a relevant exposure of humans via accumulation in eggs. The study shows that a better management of end-of-life products containing PBDEs is needed to reduce PBDE exposure risk in Africa.

Correction to: Time­trend population analysis of the clinical and epidemiologic effect on pediatric infective endocarditis after change of antibiotic prophylaxis guidelines.

The original version of this article unfortunately contained a mistake. In the author list, the first and last names were tagged incorrectly. The corrected author list is given above.

Time-trend population analysis of the clinical and epidemiologic effect on pediatric infective endocarditis after change of antibiotic prophylaxis guidelines.

PURPOSE: In 2007, antibiotic prophylaxis (AP) guidelines for infective endocarditis (IE) changed, but the possible influence on the annual incidences of pediatric IE is unclear. METHODS: We studied the clinical and epidemiologic impact of AP change by comparing two time periods before and after change of AP guidelines in a tertiary care center as referral center for a total population of more than 4,500,000 inhabitants. RESULTS: After change of AP guidelines, twenty-five patients were diagnosed for IE at a median age of 6.9 years (range 0.1-19.4, female 48%). Modified Duke criteria were fulfilled for definite (12/25; 48%), or probable IE (13/25; 52%). The frequency of IE (cases per 1000 hospitalized patients) increased from 0.37% (1995-2005) to 0.59% (2006-2017) [p = 0.152], the annual incidence of IE (cases per 1000 CHD patients, < 20 years of age) increased from 0.195 ‰ to 0.399 ‰ [p = 0.072]. Postoperative IE (13/25; 52%), was associated mostly with prosthetic pulmonary valves (12/13; 92%). Pathogens were staphylococci spp. (8/25; 32%), streptococci spp. (7/25; 28%), HACEK (3/25; 12%), other (4/25; 16%), or culture-negative (3/25; 12%). Treatment included antibiotics (25/25; 100%), and cardiac surgery (16/25; 64%). The clinical findings and complications of pediatric IE including mortality (2/25; 8%) did not differ between the two time periods. CONCLUSIONS: Pediatric IE remains a severe cardiac disease with a comparable clinical picture. Unless increasing absolute case numbers of IE, the relative case number of IE remains stable despite AP change. The high number of prosthetic pulmonary valve associated IE needs further evaluation and therapeutic alternatives.

Marfan syndrome and related connective tissue disorders in the current era in Switzerland in 103 patients: medical and surgical management and impact of genetic testing.

INTRODUCTION: Marfan syndrome (MFS) and related connective tissue disorders (CTDs) are increasingly recognised. Genetic testing has greatly improved the diagnostic outcome/power over the last two decades. In this study we describe a multicentre cohort of adults with MFS and related CTDs, with a particular focus on results from genetic testing. METHODS: All patients with MFS and related CTDs were identified from the databases of five centres in the canton of Zurich. Echocardiographic and clinical findings including systemic Marfan score, use of medication and genetic results were retrospectively analysed. MFS was diagnosed using the revised Ghent criteria (including FBN1 genetic testing if available); other CTDs (Loeys-Dietz syndrome) were diagnosed by genetic testing only. RESULTS: A cohort of 103 patients were identified (62 index patients, 41 relatives of family members): 96 patients with MFS and 7 patients with other CTD, 54 males (52%), median age 23 years (range 1–75). The median systemic Marfan score was 5 (range 0–18). Only 40 patients (40/103, 39%) fulfilled criteria for systemic involvement (≥7 points). A history of aortic dissection was present in 14 out of 103 patients (14%). Echocardiographic data were available for all: aortic root enlargement (Z-score ≥2 in adults, Z-score ≥3 in children) was found in 49 patients (48%) and mitral valve prolapse in 64 (62%). Genetic testing had been performed in 80 patients (78%); FBN1 mutations were present in 69 patients (86%); other pathogenic mutations could be identified in seven patients (9%); no disease-causing mutation was found in four patients, three of them fulfilling the Ghent criteria of MFS. Of the mutation-positive patients, 33 had a systemic score of ≥7 and 43 had a systemic score of ≥5. Revised Ghent criteria were fulfilled in 70 patients: in 69 patients with FBN1 mutations and 1 patient with another CTD. Recommended treatment (beta-blocker, angiotensin receptor blocker) was taken by 63% of patients. CONCLUSIONS: In this cohort a high percentage of patients fulfilling the revised Ghent criteria for MFS underwent genetic testing, often leading to or confirming the diagnosis of MFS. Other CTDs could be discriminated best by genetic testing. With respect to the diagnosis of MFS and related CTDs, the usefulness of the systemic score is limited, showing the importance of genetic testing, which enabled definitive diagnosis in 95% of tested patients. Patient education on medical treatment still has to be improved. (Trial registration no: KEK-ZH-Nr. 2013-0241).

Pacemaker Implantation in Neonates and Infants: Favorable Outcomes with Epicardial Pacing Systems.

The implantation of pacemakers (PM) in neonates and infants requires particular consideration of small body size, marked body growth potential, and the decades of future pacing therapy to be expected. The aim of this study is to quantify the complications of implantation and outcome occurring at our center and to compare these with other centers. Retrospective analysis of 52 consecutive patients undergoing PM implantation at a single tertiary care center within the first year of life. PMs were implanted at a median age of 3 months (range 0-10 months). Structural heart defects were present in 44 of 52 patients. During a median follow-up time of 40.4 months (range 0.1-114 months), measurements for sensing, pacing thresholds, and lead impedance remained stable. No adverse pacing effect was observed in left ventricular function or dimensions over time. There were 20 reoperations in 13 patients at a median time of 4.7 years (range 0.05-8.2 years) after implantation, for end of battery life (n = 10), lead dysfunction (n = 3), device dislocation (n = 3), infection (n = 3), and diaphragmatic paresis (n = 1). No PM-related mortality occurred. Epicardial pacemaker implantation in neonates and infants is an invasive but safe and effective procedure with a relatively low risk of complications. Our current implantation technique and the use of bipolar steroid-eluting electrodes, which we prefer to implant on the left ventricular apex, lead to favorable long-term results.

Characterization of PCDD/Fs and heavy metal distribution from municipal solid waste incinerator fly ash sintering process.

A recycling and disposal technology for municipal solid waste incinerator (MSWI) fly ash using high temperature sintering process was evaluated in an industrial scale facility with daily disposal capacity of 100 t/d. The emission, mass balance and distribution characteristics of polychlorinated dibenzo-p-dioxins and dibenzofurans (PCDD/Fs) as well as heavy metals (HMs) were assessed during two test runs. The PCDD/Fs emission from stack varied in range of 0.019 to 0.025 ng I-TEQ /Nm3, below international standards. The PCDD/Fs in the sintered product was reduced to 0.002-0.008 µg I-TEQ/kg from 2.593 to 2.704 µg I- TEQ/kg of the original MSWI fly ash (Mix-FA). However high concentration of 14.3 µg I-TEQ/kg were found in the secondary fly ash (Sec-FA). Therefore, a large share of PCDD/Fs just desorbed from the ash and the destruction efficiency (DRE) for PCDD/Fs was only 8.9%. The distribution characteristics of investigated HMs were primarily dependent on the evaporative properties. The results of HMs leaching test for the sintered product were considerably below the Chinese legal limits. The HMs leaching toxicity test for Sec-FA indicated which is a hazardous waste presented high risk to the environment. The use of the sintered product as construction material need further assessment on its long term HMs leaching behavior.