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51.
d'Amati G Bagattin A Bauce B Rampazzo A Autore C Basso C King K Romeo MD Gallo P Thiene G Danieli GA Nava A 《Human pathology》2005,36(7):761-767
We report on a family with a history of sudden death and effort-induced polymorphic ventricular arrhythmias. The index case was a 17-year-old boy who died suddenly and at postmortem had evidence of fibrofatty replacement in the right ventricular free wall, consistent with arrhythmogenic right ventricular cardiomyopathy, as well as calcium phosphate deposits within the myocytes. A molecular genetics investigation carried out in the paraffin-embedded myocardium of the subject and in blood samples of family members disclosed a missense mutation in exon 3 (230C-->T; A77V) of the cardiac ryanodine receptor type 2 gene. The carriers showed effort-induced polymorphic ventricular tachycardia in the setting of normal resting electrocardiogram and trivial echocardiographic abnormalities, consistent with catecholaminergic polymorphic ventricular tachycardia. The observation of both arrhythmogenic right ventricular cardiomyopathy type 2 and catecholaminergic polymorphic ventricular tachycardia in the same family suggests that the two entities might correspond to different degrees of phenotypic expression of the same disease. This experience underscores the importance of a precise autopsy diagnosis in the case of sudden cardiac death, including molecular genetics, and the mission of pathologists to guide further clinical investigation of family members. 相似文献
52.
Six-month multicenter study on invasive infections due to Streptococcus pyogenes and Streptococcus dysgalactiae subsp. equisimilis in Argentina
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Lopardo HA Vidal P Sparo M Jeric P Centron D Facklam RR Paganini H Pagniez NG Lovgren M Beall B 《Journal of clinical microbiology》2005,43(2):802-807
During a 6-month period, 95 invasive infections due to Streptococcus pyogenes and group C or group G Streptococcus dysgalactiae subsp. equisimilis were recorded from 40 centers of 16 cities in Argentina. We describe here epidemiologic data available for 55 and 19 patients, respectively, associated with invasive infections due to S. pyogenes and S. dysgalactiae subsp. equisimilis. The associated isolates and 58 additional pharyngeal isolates were genotyped and subjected to serologic and/or antibiotic susceptibility testing. Group A streptococcal emm type distribution and strain association with toxic shock appeared to differ somewhat from results found within the United States; however, serologic characterization and sof sequence typing suggested that emm types found in both countries are reflective of shared clonal types. 相似文献
53.
Silviene Fabiana De Oliveira Eliane Barbosa Dos Santos Patrícia Jeanne De Souza Mendona Daniela Cristiane Da Cruz Rocha Sidney Emanuel Batista Dos Santos 《American journal of human biology》2001,13(6):718-720
The group‐specific component (GC) system is of interest in anthropological genetic studies because the distribution of its subtypes distinguishes among major ethnic groups. The GC system was analyzed in Curiaú and Pacoval, two remnant Quilombo populations (African‐derived populations) from the Brazilian Amazon. There was no significant statistical difference in allelic frequencies between the two populations or between them and three other African‐derived Brazilian populations (Mimbó, Sítio Velho, and Gaucinha in Northeastern Brazil). These populations share similarities among themselves and with African populations (high frequencies of GC*1F and lower frequencies of GC*1S), which may reflect the influence of a high level of African contribution to their formation, but there is a clear difference between them and Europeans and South American Indians. It is suggested that the GC system is a useful marker for studying relationships between single populations and major ethnic groups, but does not discriminate between populations which share the same parental stock. Am. J. Hum. Biol. 13:718–720, 2001. © 2001 Wiley‐Liss, Inc. 相似文献
54.
The Vi capsular antigen of Salmonella enterica serotype Typhi reduces Toll-like receptor-dependent interleukin-8 expression in the intestinal mucosa 总被引:5,自引:1,他引:5
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Raffatellu M Chessa D Wilson RP Dusold R Rubino S Bäumler AJ 《Infection and immunity》2005,73(6):3367-3374
Human infections with nontyphoidal Salmonella serotypes, such as S. enterica serotype Typhimurium, are characterized by a massive neutrophil influx in the colon and terminal ileum. In contrast, neutrophils are scarce in intestinal infiltrates of typhoid fever patients. Here, we show that in S. enterica serotype Typhi, the causative agent of typhoid fever, expression of the Vi capsular antigen reduced expression of the neutrophil chemoattractant interleukin-8 (IL-8) in host cells. Capsulated bacteria elicited IL-8 expression in polarized human epithelial cells (T84) and human macrophage-like cells (THP-1) in vitro at significantly reduced levels compared to noncapsulated bacteria. Experiments with a human cell line (HEK293) transfected with human Toll-like receptors (TLRs) demonstrated that in the presence of TLR5 or TLR4/MD2/CD14, a noncapsulated serotype Typhi mutant was able to induce the expression of IL-8, while this host response was significantly reduced when cells were infected with the capsulated serotype Typhi wild type. The relevance of these in vitro observations for the interaction of serotype Typhi with its human host was further studied ex vivo using human colonic tissue explants. Expression of IL-8 was detected in human colonic tissue explants infected with serotype Typhimurium or a noncapsulated serotype Typhi mutant. In contrast, infection with the serotype Typhi wild type did not elicit IL-8 expression in colonic tissue explants. Collectively, these data suggest that the scarcity of neutrophils in intestinal infiltrates of typhoid fever patients is due to a capsule-mediated reduction of TLR-dependent IL-8 production in the intestinal mucosa. 相似文献
55.
F. Doricchi Daniela Perani Chiara Incoccia Franco Grassi Stefano F. Cappa Valentino Bettinardi Gaspare Galati Luigi Pizzamiglio Ferruccio Fazio 《Experimental brain research. Experimentelle Hirnforschung. Expérimentation cérébrale》1997,116(1):50-62
Regional cerebral blood flow changes related to the performance of two oculomotor tasks and a central fixation task were
compared in ten healthy human subjects. The tasks were: (a) performance of fast-regular saccades; (b) performance of voluntary
antisaccades away from a peripheral cue; (c) passive maintenance of central visual fixation in the presence of irrelevant
peripheral stimulation. The saccadic task was associated with a relative increase in activity in a number of occipitotemporal
areas. Compared with both the fixation and the saccadic task, the performance of antisaccades activated a set of areas including:
the superior and inferior parietal lobules, the precentral and prefrontal cortex, the cingulate cortex, and the supplementary
motor area.
The results of the present study suggest that: (a) compared with self-determined saccadic responses the performance of fast
regular, reflexive saccades produces a limited activation of the frontal eye fields; (b) in the antisaccadic task the inferior
parietal lobes subserve operations of sensory-motor integration dealing with attentional disengagement from the initial peripheral
cue (appearing at an invalid spatial location) and with the recomputation of the antisaccadic vector on the basis of the wrong
(e.g., spatially opposite) information provided by the same cue.
Received: 20 May 1996 / Accepted: 28 January 1997 相似文献
56.
Doris Škorić‐Milosavljević Fleur V. Y. Tjong Julien Barc Ad P. C. M. Backx Sally‐Ann B. Clur Karin van Spaendonck‐Zwarts Roelof‐Jan Oostra Najim Lahrouchi Leander Beekman Regina Bökenkamp Daniela Q. C. M. Barge‐Schaapveld Barbara J. Mulder Elisabeth M. Lodder Connie R. Bezzina Alex V. Postma 《American journal of medical genetics. Part A》2019,179(9):1836-1845
The first human mutations in GATA6 were described in a cohort of patients with persistent truncus arteriosus, and the phenotypic spectrum has expanded since then. This study underscores the broad phenotypic spectrum by presenting two patients with de novo GATA6 mutations, both exhibiting complex cardiac defects, pancreatic, and other abnormalities. Furthermore, we provided a detailed overview of all published human genetic variation in/near GATA6 published to date and the associated phenotypes (n = 78). We conclude that the most common phenotypes associated with a mutation in GATA6 were structural cardiac and pancreatic abnormalities, with a penetrance of 87 and 60%, respectively. Other common malformations were gallbladder agenesis, congenital diaphragmatic hernia, and neurocognitive abnormalities, mostly developmental delay. Fifty‐eight percent of the mutations were de novo, and these patients more often had an anomaly of intracardiac connections, an anomaly of the great arteries, and hypothyroidism, compared with those with inherited mutations. Functional studies mostly support loss‐of‐function as the pathophysiological mechanism. In conclusion, GATA6 mutations give a wide range of phenotypic defects, most frequently malformations of the heart and pancreas. This highlights the importance of detailed clinical evaluation of identified carriers to evaluate their full phenotypic spectrum. 相似文献
57.
Francesca Cirulli Daniela Santucci Giovanni Laviola Enrico Alleva Seymour Levine 《Developmental psychobiology》1994,27(5):301-316
These studies investigated behavioral and hormonal responses to stress in developing mice. Experiment 1 examined the effects of 24-hr maternal deprivation on corticosterone (CORT) secretion and ultrasonic vocalization (UVZ) rate in 4-, 8-, and 12-day-old mice. At these ages, exposure to a novel environment resulted in minimal changes in CORT secretion. Maternal deprivation increased pups′ CORT secretion in an age-dependent fashion but did not affect their UVZ rate. The aim of experiment 2 was to test the effects of cholordiazepoxide (CDP), an anxyolytic compound, on CORT secretion and UVZ in both normally reared and in maternally deprived 8-day-old mice. CDP administration elevated CORT increases in deprived (DEP) animals. CDP affected UVZ only in nondcprived (NDEP) animals: UVZ ratewas decreased by high CDP doses Overall, these findings demonstrate that the infant mouse shows a period of stress hyp9oresponsiveness similar to the rat and that maternal presence contributes to inhibit adrenocorticalactivity. CDP administration, butnot novelty exposure, increased CORT secretion in 8-day- old normally reared mice suggesting that during the stress hyporesponsive period, the HPA axis is capable of responding only to specific stimuli. Changes in HPA axis activity and UVZ rateresulting from maternal deprivation and/or CDP challenge do not seem to be directly related. ©1994 John Wiley & Sons, Inc. 相似文献
58.
Chiara Fenoglio Daniela Galimberti Laura Piccio Diego Scalabrini Paola Panina Cecilia Buonsanti Eliana Venturelli Carlo Lovati Gianluigi Forloni Claudio Mariani Nereo Bresolin Elio Scarpini 《Neuroscience letters》2007
Triggering Receptor Expressed on Myeloid cells (TREM)2 deficiency originates a genetic syndrome characterized by bone cysts and presenile dementia, named Nasu-Hakola disease (NHD). Early onset dementia and marked involvement of frontal regions are features characterizing both NHD and other kinds of neurodegenerative disorders, such as Frontotemporal Lobar Degeneration (FTLD), and, in some cases, Alzheimer's disease (AD). Three Single Nucleotide Polymorphisms (SNPs) in TREM2 coding region were screened by allelic discrimination in a population of probable AD patients as well as FTLD patients as compared with age-matched controls. In addition, mutation scanning of the coding region of TREM2 gene was carried out in 7 patients with early onset AD (EOAD), 16 FTLD, and 20 controls. None of the SNPs analyzed was present, either in patients or controls. Moreover, mutation scanning of the five exons of TREM2 failed to detect the presence of novel polymorphisms. These data demonstrate that TREM2 coding region is highly conserved, implying a crucial role of this receptor. Further studies, including a functional analysis, are certainly required to clarify the role of TREM2 in neurodegenerative processes. 相似文献
59.
The ultrastructure of the myoneural junctions in the body wall muscles has been studied in Branchiobdella pentodonta Whit. A single junctional type has been found. Within the terminal axon there are two types of vesicles, which differ in number, size and electron opacity. The junctional gap contains the basement membrane. The post-junctional membrane displays concave patches on which slender projections arising from the outer lamina of the sarcolemma extend into the junctional gap. These concave patches overlie a cytoplasmic lamina made up of electron dense material. 相似文献
60.
CpG-containing oligodeoxynucleotides (CpG ODNs) act on Toll-like receptor 9 (TLR9) that is expressed on B cells and plasmacytoid dendritic cells (pDCs) to stimulate the innate immune system, however, different types of CpG ODNs induce distinct responses. Recent papers suggest some CpG ODNs could require a second receptor or cofactor to signal. The different signaling complexes assembled might impact on the affinity with which CpG ODNs signal to TLR9 or activate additional pathways that lead to distinct immune responses. 相似文献