Descriptive epidemiology of roe deer mortality in Sweden

A retrospective epidemiologic study was conducted to examine causes of mortality of 985 wild roe deer (Capreolus capreolus) submitted to the National Veterinary Institute (SVA; Uppsala, Sweden) from January 1986 to December 1995. Age, sex, body condition, and geographic distribution as related to disease conditions are reported herein. The most common causes of mortality in roe deer were trauma (19%), winter starvation (18%), gastritis/enteritis (15%), bacterial infections (11%), parasitic infection (11%), systemic diseases (11%), neoplasia (2%), congenital disorders (1%), and miscellaneous causes (6%). Cause of death was not determined in 6% of the cases. The distribution of causes of death reported in this study differ from previous works in Sweden in that infectious and parasitic diseases were more common than winter starvation. The pathologic findings in studies like this do not necessarily represent what is occurring in the natural environment, but they do provide a good indication of distribution of diseases over time as well as age and sex structure in relation to disease conditions. Further research and more detailed studies are in progress to better understand specific mortality factors as well as etiologies of certain described diseases in roe deer in Sweden.     

Cardiovascular causes of sudden unexpected death in children and adolescents (0–17 years)

Background

Little is known about the causes of unexpected death in minors (0–17 years). In young adults an important cause is cardiovascular disease, with primary arrhythmogenic disorders, atherosclerotic events, cardiomyopathies and myocarditis as main contributors. The aim of this autopsy study was to determine the contribution of cardiovascular disease to unexpected death in minors.

Methods and results

In the Netherlands, systematic investigation of all cases of unexplained death in minors was compulsory in a nationwide governmental project during a 15-month period. Autopsies were performed according to a standardised protocol (autopsy rate 85%). A cardiovascular cause of death was found in 13/56 cases (23%). In the group <1 year, the main cardiovascular causes were various congenital defects (n?=?3) and myocarditis (n?=?2). In the 1–9 year group, no cardiovascular causes were found. In the 10–14 year group, hypertrophic cardiomyopathy (n?=?1) and ruptured ascending aortic aneurysm (n?=?1) were among the observed cardiovascular causes. In 14/56 (25%) cases autopsy revealed no structural abnormalities that could explain the sudden death, mostly in the group <1 year.

Conclusion

This national cohort with a high autopsy rate reveals a high incidence (23%) of cardiovascular diseases as the pathological substrate of sudden unexpected death in children. Another high percentage of minors (25%) showed no structural abnormalities, with the possibility of a genetic arrhythmia. These findings underline the importance of systematic autopsy in sudden death in minors, with implications for cardiogenetic screening of relatives.

     

Mortality factors and diseases in free-ranging Eurasian cranes (Grus grus) in Germany

Detailed postmortem examinations were performed on 167 free-ranging Eurasian Cranes (Grus grus) from Germany, collected between September 1998 and December 2008 to evaluate causes of death and diseases. The most common causes of mortality were traumatic injuries (n=105, 62.9%) from collisions with power lines (n=39, 23.4%) and wire fences (n=12, 7.2%). A group of 28 Eurasian Cranes (16.8%) died from organophosphate intoxication. Predation by White-tailed Sea Eagles (Haliaeetus albicilla) and red foxes (Vulpes vulpes) occurred in four cases (2.4%). Pathologic changes due to infectious diseases were associated with Aspergillus spp. (n=7, 4.2%), endoparasites (n=7, 4.2%), avian poxvirus (n=6, 3.6%), Mycobacterium spp. (n=2, 1.2%), and adenovirus infection (n=1, 0.6%). A severe Strigea spp. infection (n=1, 0.6%) and a leiomyosarcoma (n=1, 0.6%) were newly recognized diseases in Eurasian Cranes in this study.     

Etiologie des infertilités secondaires de l’homme

Introduction

Secondary infertility is defined as the inability to achieve a new pregnancy for a couple that has been able to procreate in the past.

Material and methods

Over a 16-year period, 49 patients consulted for male secondary infertility. Clinical, laboratory or ultrasound assessment demonstrated the cause of infertility.

Results

These patients had a mean age of 33.6 years (range: 26 to 44 years). These couples had an average of 1 living child per family, corresponding to a girl in two-thirds of cases and a boy in one-third. In these patients, infertility was due to the presence of varicocele in 15 patients (31% of cases), infection in 14 patients (29%), and varicocele associated with infection in 11 cases (22%).

Conclusion

This assessment revealed two main aetiologies, but some causes remain obvious, such as surgery with resection of the prostate or testicles, or certain cytotoxic therapies.     

Cri-du-chat syndrome mimics Silver-Russell syndrome depending on the size of the deletion: a case report

Background

Silver-Russell Syndrome (SRS) is a rare growth-related genetic disorder mainly characterized by prenatal and postnatal growth failure. Although molecular causes are not clear in all cases, the most common mechanisms involved in SRS are loss of methylation on chromosome 11p15 (≈50%) and maternal uniparental disomy for chromosome 7 (upd(7)mat) (≈10%).

Case presentation

We present a girl with clinical suspicion of SRS (intrauterine and postnatal growth retardation, prominent forehead, triangular face, mild psychomotor delay, transient neonatal hypoglycemia, mild hypotonia and single umbilical artery). Methylation and copy number variations at chromosomes 11 and 7 were studied by methylation-specific multiplex ligation-dependent probe amplification and as no alterations were found, molecular karyotyping was performed. A deletion at 5p15.33p15.2 was identified (arr[GRCh37] 5p15.33p15.2(25942–11644643)×?1), similar to those found in patients with Cri-du-chat Syndrome (CdCS). CdCS is a genetic disease resulting from a deletion of variable size occurring on the short arm of chromosome 5 (5p-), whose main feature is a high-pitched mewing cry in infancy, accompanied by multiple congenital anomalies, intellectual disability, microcephaly and facial dysmorphism.

Conclusions

The absence of some CdCS features in the current patient could be due to the fact that in her case the critical regions responsible do not lie within the identified deletion. In fact, a literature review revealed a high degree of concordance between the clinical manifestations of the two syndromes.

     

Sarcoglycanopathies: Clinical,Molecular and Genetic Characteristics,Epidemiology, Diagnostics and Treatment Options

Sarcoglycanopathies are a group of autosomal recessive limb-girdle muscular dystrophies (LGMD) caused by mutations in sarcoglycan genes: SGCA (LGMD 2D, MIM 600119), SGCB (LGMD 2E, MIM 604286), SGCG (LGMD 2C, MIM 353700), and SGCD (LGMD 2F, MIM 601287). These genes encode four transmembrane sarcoglycan subunits participating in formation of the large sarcolemmal dystrophin- glycoprotein complex. Clinical symptoms of sarcoglycanopathies resemble the ones in Duchenne/Becker muscular dystrophy and several autosomal recessive LGMD, which causes difficulties in the differential diagnostics between these diseases. This review covers the main aspects of sarcoglycanopathies, such as etiology, spectrum of mutations, clinical features and diagnostics. In addition, we review the fundamental pathogenesis mechanisms leading to sarcoglycanopathies, which can also help to understand the potential options for treatment for patients with muscular dystrophies.     

Structure-activity relationships of monomeric and dimeric synthetic ACTH fragments in perifused frog adrenal slices

The effect of synthetic monomeric and dimeric ACTH fragments on spontaneous and ACTH(1-39)-evoked steroidogenesis in frog interrenal tissue was studied in vitro. Infusion of ACTH fragment 11-24 (10(-6) M) or its dimeric conjugates, attached either by their N-terminal, Glu(11-24)2, or their C-terminal amino acid, (11-24)2Lys, had no effect on the spontaneous release of corticosteroids. The monomer ACTH(11-24) and the dimer Glu(11-24)2 were also totally devoid of effect on the steroidogenic response to ACTH(1-39) (10(-9)M). In contrast, the (11-24)2Lys conjugate (10(-6)M) significantly decreased ACTH-induced stimulation of corticosterone and aldosterone (-63 and -62%, respectively). The dimeric conjugate of the fragment ACTH(7-24), linked through the C-terminal ends, (7-24)2Lys (10(-6)M), was also completely devoid of effect on basal steroidogenesis but caused a marked decrease of ACTH-evoked corticosterone and aldosterone release (-72 and -80%, respectively). Conversely, infusion of the dimer (1-24)2Lys gave rise to a dose-related stimulation of corticosterone and aldosterone release. The time-course of the steroidogenic response to the dimer was similar to that of ACTH(1-24). The 1-24 conjugate was 70 times less potent than the monomers ACTH(1-24) and ACTH(1-39). These results suggest that amphibian adrenocortical cells contain only one class of ACTH receptor which recognizes the 11-24 domain of ACTH with an affinity which depends on the presence of a strong potentiator segment, located at the N-terminus end of ACTH(1-39). Since the ACTH-dimers are thought to induce cross-linking of the receptors, our results suggest that aggregation of ACTH receptors causes a down-regulation of the receptors.     

Identification of 3-deoxy-manno-2-octulosonic acid, 3-deoxy-5-O-methyl-manno-2-octulosonic acid and 3-deoxy-lyxo-2-heptulosaric acid in the cell wall (theca) of the green alga Tetraselmis striata Butcher (Prasinophyceae)

The main constituent of the cell wall complex carbohydrate of the scaly green alga Tetraselmis striata Butcher is shown to be 3-deoxy-manno-2-octulosonic acid (42%). In addition two other 2-keto-sugar acids are present, namely, 3-deoxy-5-O-methyl-manno-2-octulosonic acid (7%), the first methylated derivative of 3-deoxy-manno-2-octulosonic acid found in nature, and 3-deoxy-lyxo-2-heptulosaric acid (11%). The characterization of the three 2-keto-sugar acids has been carried out on the corresponding methyl ester methyl glycosides using GLC-MS and 500-MHz 1H-NMR spectroscopy, and on the corresponding reduced alditol acetates using GLC-MS. Other monosaccharides occurring in the cell wall are D-galacturonic acid (14%), D-galactose (4%), D-gulose (2%), D-glucose (1%) and L-arabinose (1%).     

Row for Your Life: A Century of Mortality Follow-Up of French Olympic Rowers

Background/Aim

Strenuous endurance training required to participate in the highest sports level has been associated with deleterious effects on elite athletes'' health and cardiac abnormalities. We aimed to describe overall mortality and main causes of deaths of male French rowers participating in at least one Olympic Game (OG) from 1912 to 2012 in comparison with the French general population.

Methods

Identity information and vital status of French Olympic rowers were validated by National sources from 1912 to 2013 (study''s endpoint) among 203 rowers; 52 out of 255 (20.3%) were excluded because their vital statuses could not be confirmed. Main causes of deaths were obtained from the National registry from 1968 up to 2012. Overall and disease-specific mortalities were calculated through standardised mortality ratios (SMRs) with its 95% confidence intervals (CIs). The overall mortality was calculated for the whole rowers'' cohort (PT) and for two periods apart: (P1) including rowers from 1912 to 1936 OG, a cohort in which all rowers have deceased and (P2) considering rowers from 1948 to 2012 OG.

Results

Among the 203 rowers analysed, 46 died before the study''s endpoint, mainly from neoplasms (33%), cardiovascular diseases (21%) and external causes (18%). PT demonstrates a significant 42% lower overall mortality (SMR: 0.58, 95% CI: 0.43–0.78, p<0.001), P1 a 37% reduction (SMR: 0.63, 95% CI: 0.43–0.89, p = 0.009) and P2 a 60% reduction (SMR: 0.40, 95% CI: 0.23–0.65, p<0.001) compared with their compatriots. Mortality due to cardiovascular diseases is significantly reduced (SMR: 0.41, 95% CI: 0.16–0.84, p = 0.01) among rowers.

Conclusions

French Olympic rowers benefit of lower overall mortality compared with the French general population. Among rowers'' main causes of death, cardiovascular diseases are reduced in relation to their compatriots. Analytical studies with larger samples are needed to understand the reasons for such reductions.     

<Emphasis Type="Italic">Spirosoma pomorum</Emphasis> sp. nov., isolated from apple orchard soil

A Gram-negative, motile, rod-shaped, aerobic bacterial strain, designated S7-2-11^T, was isolated from apple orchard soil from Gyeongsangnam-do Province, Republic of Korea, and was characterized taxonomically using a polyphasic approach. 16S rRNA gene sequence analysis indicated that strain S7-2-11^T belongs to the family Cytophagaceae in phylum Bacteroidetes, and is closely related to Spirosoma luteolum 16F6E^T (94.2% identity), Spirosoma knui 15J8-12^T (92.7%), and Spirosoma linguale DSM 74^T (91.0%). The G + C content of the genomic DNA of strain S7-2-11^T was 49.8 mol%. Strain S7-2-11^T contained summed feature 3 (C_16:1 ω7c/C_16:1 ω6c; 35.1%), C_16:1 ω5c (22.4%), C_15:0 iso (13.9%), and C_17:0 iso 3-OH (10.6%) as major cellular fatty acids, and MK-7 as the predominant respiratory quinone. The main polar lipids were phosphatidylethanolamine, an unidentified aminophospholipid, and two unidentified polar lipids. Phenotypic and chemotaxonomic data supported the affiliation of strain S7-2-11^T with the genus Spirosoma. The results of physiological and biochemical tests showed the genotypic and phenotypic differentiation of the isolate from recognized Spirosoma species. On the basis of its phenotypic properties, genotypic distinctiveness, and chemotaxonomic features, strain S7-2-11^T represents a novel species of the genus Spirosoma, for which the name Spirosoma pomorum sp. nov. is proposed. The type strain is S7-2-11^T (= KCTC 52726^T = JCM 32130^T).     

Interaction between smoking and functional polymorphism in the TGFB1 gene is associated with ischaemic heart disease and myocardial infarction in patients with rheumatoid arthritis: a cross-sectional study

Introduction

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease. Cardiovascular disease (CVD) is common and a major cause of mortality. Studies on cardiovascular morbidity are abundant, whereas mortality studies focusing on cardiovascular outcomes are scarce. The aim of this study was to investigate causes of death and baseline predictors of overall (OM), non-vascular (N-VM), and specifically cardiovascular (CVM) mortality in SLE, and to evaluate systematic coronary risk evaluation (SCORE).

Methods

208 SLE patients were included 1995-1999 and followed up after 12 years. Clinical evaluation, CVD risk factors, and biomarkers were recorded at inclusion. Death certificates and autopsy protocols were collected. Causes of death were divided into CVM (ischemic vascular and general atherosclerotic diseases), N-VM and death due to pulmonary hypertension. Predictors of mortality were investigated using multivariable Cox regression. SCORE and standardized mortality ratio (SMR) were calculated.

Results

During follow-up 42 patients died at mean age of 62 years. SMR 2.4 (CI 1.7-3.0). 48% of deaths were caused by CVM. SCORE underestimated CVM but not to a significant level. Age, high cystatin C levels and established arterial disease were the strongest predictors for all- cause mortality. After adjusting for these in multivariable analyses, only smoking among traditional risk factors, and high soluble vascular cell adhesion molecule-1 (sVCAM-1), high sensitivity C-reactive protein (hsCRP), anti-beta2 glycoprotein-1 (abeta2GP1) and any antiphospholipid antibody (aPL) among biomarkers, remained predictive of CVM.

Conclusion

With the exception of smoking, traditional risk factors do not capture the main underlying risk factors for CVM in SLE. Rather, cystatin C levels, inflammatory and endothelial markers, and antiphospholipid antibodies (aPL) differentiate patients with favorable versus severe cardiovascular prognosis. Our results suggest that these new biomarkers are useful in evaluating the future risk of cardiovascular mortality in SLE patients.     

Adhesion-mediating molecules of human monocytes

Adhesion of monocytes to each other and to T cells and substrates is increased by phorbol esters. In the presence of these compounds monocyte aggregation was almost completely inhibited (greater than 90%) by monoclonal antibody 60.3. This antibody recognizes GP90 (CD18), a leukocyte surface glycoprotein which is separately and noncovalently associated to either GP160 (CD11a), GP155 (CD11b), or GP130 (CD11c). Anti-LFA-1 antibody (CD11a) was only partially inhibitory (35%) while antibodies 60.1 (CD11b) and anti-Leu-M5 (CD11c) had a minimal inhibitory effect (10%). Antibody LB-2 recognizing a single glycoprotein distinct from the GP90-GP160 complex and expressed on activated B and T cells, monocytes, and vascular endothelial cells was partially inhibitory (22%). Monoclonal antibodies anti-C3bR (CD35), T29/33 (CD45, leukocyte common antigen 200). TA-1 (CD11a), OKM1 (CD11b), F10-44-2 (brain-leukocyte antigen), OKM5 (monocyte-endothelial cell antigen) and to class I or class II molecules exerted no inhibition on the monocyte aggregation. Fab fragments of antibody 60.3 efficiently inhibited not only monocyte aggregation in the absence or presence of phorbol esters but also adhesion of these cells to autologous or allogeneic T lymphocytes and, to a lesser extent, to plastic surfaces. It is thus concluded that GP90, either alone or associated to the larger glycoproteins, and LB-2 antigen mediate monocyte adhesion.     

Jaundice revisited: recent advances in the diagnosis and treatment of inherited cholestatic liver diseases

Background

Jaundice is a common symptom of inherited or acquired liver diseases or a manifestation of diseases involving red blood cell metabolism. Recent progress has elucidated the molecular mechanisms of bile metabolism, hepatocellular transport, bile ductular development, intestinal bile salt reabsorption, and the regulation of bile acids homeostasis.

Main body

The major genetic diseases causing jaundice involve disturbances of bile flow. The insufficiency of bile salts in the intestines leads to fat malabsorption and fat-soluble vitamin deficiencies. Accumulation of excessive bile acids and aberrant metabolites results in hepatocellular injury and biliary cirrhosis. Progressive familial intrahepatic cholestasis (PFIC) is the prototype of genetic liver diseases manifesting jaundice in early childhood, progressive liver fibrosis/cirrhosis, and failure to thrive. The first three types of PFICs identified (PFIC1, PFIC2, and PFIC3) represent defects in FIC1 (ATP8B1), BSEP (ABCB11), or MDR3 (ABCB4). In the last 5 years, new genetic disorders, such as TJP2, FXR, and MYO5B defects, have been demonstrated to cause a similar PFIC phenotype. Inborn errors of bile acid metabolism also cause progressive cholestatic liver injuries. Prompt differential diagnosis is important because oral primary bile acid replacement may effectively reverse liver failure and restore liver functions. DCDC2 is a newly identified genetic disorder causing neonatal sclerosing cholangitis. Other cholestatic genetic disorders may have extra-hepatic manifestations, such as developmental disorders causing ductal plate malformation (Alagille syndrome, polycystic liver/kidney diseases), mitochondrial hepatopathy, and endocrine or chromosomal disorders. The diagnosis of genetic liver diseases has evolved from direct sequencing of a single gene to panel-based next generation sequencing. Whole exome sequencing and whole genome sequencing have been actively investigated in research and clinical studies. Current treatment modalities include medical treatment (ursodeoxycholic acid, cholic acid or chenodeoxycholic acid), surgery (partial biliary diversion and liver transplantation), symptomatic treatment for pruritus, and nutritional therapy. New drug development based on gene-specific treatments, such as apical sodium-dependent bile acid transporter (ASBT) inhibitor, for BSEP defects are underway.

Short conclusion

Understanding the complex pathways of jaundice and cholestasis not only enhance insights into liver pathophysiology but also elucidate many causes of genetic liver diseases and promote the development of novel treatments.

     

Performance and mortality of farmed hares

Performance and mortality of hares were evaluated for 2 consecutive years in a large farm in Veneto Region (Northern Italy). On average, fertile reproductive pairs (n=318) gave birth 4.8 times and produced 11.4 live leverets, weaned 8.4 leverets and produced 7.0 growing hares (60 days) every year. Mean mortality was 3.6%, 22.9%, 9.7% and 2.5% in newborn (0 to 2 days of age), suckling (3 to 25 days), growing (26 to 60 days) and sub-adult (61 days until sale) hares, respectively. The main causes of mortality were enteric diseases (75.5%, 75.9% and 12.1% in suckling, growing and sub-adult hares, respectively), followed by respiratory diseases (3.4%, 8.0% and 36.2% in suckling, growing and sub-adult hares, respectively), starvation (11.3% and 8.8% in suckling and growing hares, respectively) and trauma (7.1%, 2.3% and 30.2% in suckling, growing and sub-adult hares, respectively). In reproducing hares, mortality was 24.7% and 15.4% in 2010 and 2011, respectively. Respiratory diseases (34.8%) and ulcerative pododermatitis (18.9%) were the most common pathological changes detected in reproducing hares. Farmed hares seem to be affected by diseases resembling those of rabbits reared under intensive conditions. It seems necessary to improve the husbandry of hares to reach satisfactory technical standards and to preserve their health.     

The load of genetic and partially genetic disease in man. IV. Severe visual handicaps and profound childhood deafness in Hungarian school-age children

In Hungary, the school-age prevalences of severe visual handicaps and of profound childhood deafness have been estimated to be about 6/10⁴ and 10/10⁴, respectively. Most of these conditions have onset at birth or in early childhood and are aetiologically heterogeneous.

Severe visual handicaps are grouped under 11 aetiological categories, their relative contributions to the prevalence being: perinatal damage syndrome (20%; half of this is due to retinopathy of premature infants), cataracts (15%), choroidoretinal degenerations (15%), congenital abnormalities of the eye (15%), syndromes (10%), high myopia ± retinal detachment (7%), postnatal causes (5%), nystagmus (5%), optic atrophy (4%), bilateral retinoblastoma (2%) and prenatal causes (2%). Overall, Mendelian conditions (included under many of the above) account for about 50% with relatively more autosomal dominant than autosomal recessive and sex-linked entities, and acquired causes account for about 40% of the cases studied. No actiology could be assigned in 10% of the cases.

For profound childhood deafness, the rank order of the aetiological categories is: autosomal recessive entities (34%), postnatal causes (22%), perinatal causes (19%), autosomal dominant entities (17%), prenatal causes (5%) and unknown causes (3%).

Severe childhood visual handicaps are responsible for about 60 years of loss of life per 10⁴ live births and about 400 years of impaired life per 10⁴ live births. Genetic causes account for one-quarter of lost life years and three-quarters of impaired life years. The comparable estimates for profound childhood deafness are: about 240 years of life loss per 10⁴ live births (again, about one-quarter due to genetic causes) and about 640 years of impaired life per 10⁴ live births (about one-half due to genetic causes). In all these calculations, it has been assumed that the average life expectancy at birth for an individual in the population is 70 years.     

Phylogenetic comparison of Epstein-Barr virus genomes

Technologies used for genome analysis and whole genome sequencing are useful for us to understand genomic characterization and divergence. The Epstein-Barr virus (EBV) is an oncogenic virus that causes diverse diseases such as Burkitt’s lymphoma (BL), nasopharyngeal carcinoma (NPC), Hodgkin’s lymphoma (HL), and gastric carcinoma (GC). EBV genomes found in these diseases can be classified either by phases of EBV latency (type-I, -II, and -III latency) or types of EBNA2 sequence difference (type-I EBV, type-II EBV or EBV-1, EBV-2). EBV from EBV-transformed lymphoblastoid cell line (LCL) establishes type-III latency, EBV from NPC establishes type-II latency, and EBV from GC establishes type-I latency. However, other important factors play key roles in classifying numerous EBV strains because EBV genomes are highly diverse and not phylogenetically related to types of EBV-associated diseases. Herein, we first reviewed previous studies to describe molecular characteristics of EBV genomes. Then, using comparative and phylogenetic analyses, we phylogenetically analyzed molecular variations of EBV genomes and proteins. The review of previous studies and our phylogenetic analysis showed that EBV genomes and proteins were highly diverse regardless of types of EBV-associated diseases. Other factors should be considered in determining EBV taxonomy. This review will be helpful to understand complicated phylogenetic relationships of EBV genomes.     

Biventricular stimulation therapy in patients with heart failure and tachycardic arrhythmias. Indications--results--prospects]

Biventricular pacing (BV-P) therapy is a new therapeutic approach in patients (pts) with drug refractory congestive heart failure; the beneficial effects of implantable cardioverter defibrillator (ICD) without BV-P therapy in patients (pts) with life-threatening ventricular tachyarrhythmias and impaired left ventricular (LV) function is associated with a relatively high cardiac and total mortality. We studied the follow-up of 410 pts (368 males, 42 females, mean age 57 +/- 11 years) after ICD implant. The LV function was assessed by the New York Heart functional class of heart failure (NYHA). Fifty pts (12%) were in NYHA I-II, 151 pts (37%) in NYHA II, 117 pts (29%) in NYHA II-III and 92 pts (22%) in NYHA III. Epicardial ICD implantation was performed in 209 pts (51%) and 201 pts (49%) received nonthoracotomy ICDs. Perioperatively (within 30 days after implant), 12 pts (3%) died, significantly more frequent after epicardial (11 of 209 pts, 5%) than after transvenous ICD implant (1 of 201 pts, < 1%)(p < 0.05). During a mean follow-up of 28 + 24 months (range < 1 to 114 months), 90 pts (23%) died: 9 pts (2%) died from sudden arrhythmic death and 5 pts (1%) suddenly, but probably not from arrhythmic causes; 55 pts (14%) died from cardiac causes (congestive heart failure, myocardial reinfarction) and 21 pts (5%) from noncardiac causes. 338 pts (82%) received ICD shocks (mean incidence 21 +/- 43 shocks per pt). Our data show that pts with LV dysfunction benefit from ICD therapy and that these pts survive for a considerable time after the first shock. However, survival is clearly influenced by the degree of left ventricular dysfunction and, in addition to ICD therapy, aggressive treatment of heart failure is necessary. Therefore, BV-P is a very promising concept to improve the worse prognosis in pts with moderate or severe congestive heart failure.     

Bacterial DNA detected on pathologically changed heart valves using 16S rRNA gene amplification

Nowadays, dental diseases are one of the most common illnesses in the world. Some of them can lead to translocation of oral bacteria to the bloodstream causing intermittent bacteraemia. Therefore, a potential association between oral infection and cardiovascular diseases has been discussed in recent years as a result of adhesion of oral microbes to the heart valves. The aim of this study was to detect oral bacteria on pathologically changed heart valves not caused by infective endocarditis. In the study, patients with pathologically changed heart valves were involved. Samples of heart valves removed during heart valve replacement surgery were cut into two parts. One aliquot was cultivated aerobically and anaerobically. Bacterial DNA was extracted using Ultra-Deep Microbiome Prep (Molzym GmbH, Bremen, Germany) followed by a 16S rRNA gene PCR amplification using Mastermix 16S Complete kit (Molzym GmbH, Bremen, Germany). Positive PCR products were sequenced and the sequences were analyzed using BLAST database (http://www.ncbi.nlm.nih/BLAST). During the study period, 41 samples were processed. Bacterial DNA of the following bacteria was detected in 21 samples: Cutibacterium acnes (formerly Propionibacterium acnes) (n?=?11; 52.38% of patients with positive bacterial DNA detection), Staphylococcus sp. (n?=?9; 42.86%), Streptococcus sp. (n?=?1; 4.76%), Streptococcus sanguinis (n?=?4; 19.05%), Streptococcus oralis (n?=?1; 4.76%), Carnobacterium sp. (n?=?1; 4.76%), Bacillus sp. (n?=?2; 9.52%), and Bergeyella sp. (n?=?1; 4.76%). In nine samples, multiple bacteria were found. Our results showed significant appearance of bacteria on pathologically changed heart valves in patients with no symptoms of infective endocarditis.     

<Emphasis Type="Italic">Spirosoma flavus</Emphasis> sp. nov., a novel bacterium from soil of Jeju Island

A novel, Gram-staining negative, yellow pigmented bacterial strain, designated 15J11-2^T, was isolated from soil sample on Jeju Island, Republic of Korea. The strain was subjected to a taxonomic study using a polyphasic approach. The strain was able to grow at temperature range from 10°C to 30°C, pH 7–8, and in presence of 0–1% (w/v) NaCl. Comparative 16S rRNA gene sequence analysis showed that strain 15J11-2^T belongs to the genus Spirosoma and levels of 16S rRNA gene sequence similarity ranged from 91.5% to 89.8%. The genomic DNA G + C content of strain 15J11-2^T was 46.0 mol%. The isolate contained phosphatidylethanolamine and an unidentified aminophospholipid as the main polar lipids, menaquinone MK-7 as the predominant respiratory quinone, and summed feature 3 (C_16:1ω6c/C_16:1ω7c; 39.4%), C_16:1ω5c (27.1%), and C_16:0 (13.0%) as the major fatty acids, which supported the affiliation of strain 15J11-2^T to the genus Spirosoma. The results of physiological and biochemical tests allowed genotypic and phenotypic differentiation of strain 15J11-2^T from recognized Spirosoma species. On the basis of its phenotypic properties, genotypic distinctiveness, chemotaxonomic features, strain 15J11-2^T represents a novel species of the genus Spirosoma, for which the name Spirosoma flavus sp. nov. is proposed. The type strain is 15J11-2^T (= KCTC 52026^T = JCM 31998^T).     

Small heat shock proteins protect against alpha-synuclein-induced toxicity and aggregation

Protein misfolding and inclusion formation are common events in neurodegenerative diseases, such as Parkinson's disease (PD), Alzheimer's disease (AD) or Huntington's disease (HD). Alpha-synuclein (aSyn) is the main protein component of inclusions called Lewy bodies (LB) which are pathognomic of PD, Dementia with Lewy bodies (DLB), and other diseases collectively known as LB diseases. Heat shock proteins (HSPs) are one class of the cellular quality control system that mediate protein folding, remodeling, and even disaggregation. Here, we investigated the role of the small heat shock proteins Hsp27 and alphaB-crystallin, in LB diseases. We demonstrate, via quantitative PCR, that Hsp27 messenger RNA levels are approximately 2-3-fold higher in DLB cases compared to control. We also show a corresponding increase in Hsp27 protein levels. Furthermore, we found that Hsp27 reduces aSyn-induced toxicity by approximately 80% in a culture model while alphaB-crystallin reduces toxicity by approximately 20%. In addition, intracellular inclusions were immunopositive for endogenous Hsp27, and overexpression of this protein reduced aSyn aggregation in a cell culture model.