Electrophysiologic investigation of a diffuse and bizarre conduction abnormality in a patient with cardiomyopathy revealed profound slowing of the impulse in the right ventricular outflow region. The ...resultant 220 msec QRS duration is the longest reported in right bundle branch block.
Small bowel cancer (SBC) is the malignancy with the highest standardized incidence ratio in Lynch syndrome (LS) patients. Of all SBCs, about 50% are duodenal cancers (DCs), therefore being accessible ...by esophago-gastro-duodenoscopy (EGD) for surveillance. We asked whether early detection of DC is possible for LS patients undergoing surveillance by EGD and if surveillance should be limited to specific subgroups. Data for LS patients with DC were retrieved from the registry of the German Consortium for Familial Intestinal Cancer. Patients undergoing active surveillance by EGDs (surveillance group) were compared to those who did not (nonsurveillance group) regarding tumor stage at diagnosis. Union for International Cancer Control stages I-IIA were defined as early stage disease and IIB-IV as advanced stage disease. Statistical analysis was performed using Fisher's exact test. Among 2015 patients with pathogenic variants in any mismatch-repair-gene, 47 patients with 49 DCs were identified. In 10% of cases, patients were under 35 years at diagnosis; family and personal tumor history did not correlate with DC diagnosis. Pathogenic germline variants in MSH6, PMS2 or EPCAM were present in 10% of patients. Statistical analysis could be performed on 13 DC patients in the surveillance group and 14 in the nonsurveillance group. Early detection was possible for 71% of patients in the surveillance group and 29% of patients in the nonsurveillance group (P = .021). Early detection of DC by EGD in LS patients is feasible regardless of family history, mutational status and should start no later than 25 years of age.
In our study, we evaluated the effectiveness of upper gastrointestinal (GI) endoscopy as an instrument for early gastric cancer (GC) detection in Lynch syndrome (LS) patients by analyzing data from ...the registry of the German Consortium for Familial Intestinal Cancer. In a prospective, multicenter cohort study, 1128 out of 2009 registered individuals with confirmed LS underwent 5176 upper GI endoscopies. Compliance was good since 77.6% of upper GI endoscopies were completed within the recommended interval of 1 to 3 years. Forty-nine GC events were observed in 47 patients. MLH1 (n = 21) and MSH2 (n = 24) mutations were the most prevalent. GCs in patients undergoing regular surveillance were diagnosed significantly more often in an early-stage disease (UICC I) than GCs detected through symptoms (83% vs 25%; P = .0231). Thirty-two (68%) patients had a negative family history of GC. The median age at diagnosis was 51 years (range 28-66). Of all GC patients, 13 were diagnosed at an age younger than 45. Our study supports the recommendation of regular upper GI endoscopy surveillance for LS patients beginning no later than at the age of 30.
What's new?
Risk of gastric cancer (GC) is significantly increased among patients with Lynch syndrome (LS). GC screening in LS patients, however, is fraught with uncertainty, particularly regarding the use of esophagogastroduodenoscopy (EGD). The authors of this study investigated the use of EGD for regular GC surveillance in a German cohort of LS patients. Regular surveillance by EGD resulted in more frequent diagnosis and significant down-staging of GC, relative to detection via symptoms alone. In most cases, family history of GC was negative. This study supports recommendations for regular gastroscopic surveillance in LS patients starting by age 30.
Lynch syndrome (LS), Lynch-like syndrome (LLS) and familial colorectal cancer type X (FCCX) are different entities of familial cancer predisposition leading to an increased risk of colorectal cancer ...(CRC). The aim of this prospective study was to characterise and to compare the risks for adenoma and CRC in these three risk groups. Data was taken from the registry of the German Consortium for Familial Intestinal Cancer. Patients were prospectively followed up in an intensified colonoscopic surveillance programme that included annual examinations. Cumulative risks for adenoma and CRC were calculated separately for LS, LLS and FCCX, and then for males and females. Multivariate Cox regression was used to analyse the independent contributions of risk group, mismatch repair gene (within LS), sex and previous adenoma. The study population comprised 1448 individuals (103 FCCX, 481 LLS and 864 LS). The risks were similar for colorectal adenomas, but different for first and metachronous CRC between the three risk groups. CRC risk was highest in LS, followed by LLS and lowest in FCCX. Male sex and a prevalent adenoma in the index colonoscopy were associated with a higher risk for incident adenoma and CRC. In patients with LS, CRC risks were particularly higher in female MSH2 than MLH1 carriers. Our study may support the development of risk-adapted surveillance policies in LS, LLS and FCCX.
What's new?
While associations between colorectal cancer (CRC) risk and Lynch syndrome (LS) are well-described, less is known about CRC risks linked to the closely related Lynch-like syndrome (LLS) and familial colorectal cancer type X (FCCX). In this prospective follow-up study of patients with LS, LLS, and FCCX, risks were similar for colorectal adenomas but considerably different for first and metachronous CRCs. In addition, LS females who carried MSH2 mutations had notably higher CRC risks than female MLH1 mutation carriers. The identification of variations in carcinogenic pathways between LS, LLS, and FCCX could enable risk-adapted CRC surveillance for these syndromes.
This paper reports results on simultaneous measurements of the reaction channels pp -> pK+\Lambda and pp -> pK+\Sigma^0 at excess energies of 204, 239, and 284 MeV (\Lambda) and 127, 162, and 207 MeV ...(\Sigma^0). Total and differential cross sections are given for both reactions. It is concluded from the measured total cross sections that the high energy limit of the cross section ratio is almost reached at an excess energy of only about 200 MeV. From the differential distributions observed in the overall CMS as well as in the Jackson and helicity frames, a significant contribution of interfering nucleon resonances to the \Lambda production mechanism is concluded while resonant \Sigma^0-production seems to be of lesser importance and takes place only through specific partial waves of the entrance channel. The data also indicate that kaon exchange plays a minor role in the case of \Lambda- but an important role for \Sigma^0-production. Thus the peculiar energy dependence of the \Lambda-to-\Sigma^0 cross section ratio appears in a new light as its explanation requires more than mere differences between the p\Lambda and the p\Sigma^0 final state interaction. The data provide a benchmark for theoretical models already available or yet to come.
Hyperon production in the threshold region was studied in the reaction pp -> K+Lp using the time-of-flight spectrometer COSY-TOF. Exclusive data, covering the full phase-space, were taken at the ...three different beam momenta of p_beam=2.95, 3.20 and 3.30 GeV/c, corresponding to excess energies of epsilon=204, 285 and 316 MeV, respectively. Total cross-sections were deduced for the three beam momenta to be 23.9+/-0.8 +/-2.0 ub, 28.4+/-1.3 +/-2.2 ub and 35.0+/-1.3 +/-3.0 ub. Differential observables including Dalitz plots were obtained. The analysis of the Dalitz plots reveals a strong influence of the N(1650)-resonance at p_beam=2.95 GeV/c, whereas for the higher momenta an increasing relative contribution of the N(1710)- and/or of the N(1720)-resonance was observed. In addition, the pL-final-state interaction turned out to have a significant influence on the Dalitz plot distribution.
Decision analysis may be useful to people facing Alzheimer disease (AD) decisions. The use of decision analysis in three such cases is reported. The first case involved a middle-aged person worried ...about early-onset AD and deciding whether to seek genetic testing. The analysis let the participant reject testing and consider innovative care options. The second case involved a middle-aged person concerned about later-onset AD. The analysis for her was more complex, and led to the assignment of some limited value on genetic testing for her. The third case revolved around a caregiver's treatment decisions for a patient with severe AD. It led her to recognize the importance of factors she had not previously considered. In each of the three cases, the intensive process of decision analysis appears to have improved the subject's decision.
A systematic study of the production of omega-mesons in proton-proton-collisions was carried out in a kinematically complete experiment at three excess energies(epsilon= 92, 128, 173MeV). Both ...protons were detected using the large-acceptance COSY-TOF spectrometer at an external beam line at the Cooler Synchrotron COSY at Forschungszentrum J\"ulich. The total cross section, angular distributions of both omega-mesons and protons were measured and presented in various reference frames such as the overall CMS, helicity and Jackson frame. In addition, the orientation of the omega-spin and invariant mass spectra were determined. We observe omega-production to take place dominantly in Ss and Sp final states at epsilon = 92, 128 MeV and, additionally, in Sd at epsilon= 173 MeV. No obvious indication of resonant omega-production via N^*-resonances was found, as proton angular distributions are almost isotropic and invariant mass spectra are compatible with phase space distributions. A dominant role of ^3P_1 and ^1S_0 initial partial waves for omega-production was concluded from the orientation of the decay plane of the omega-meson. Although the Jackson angle distributions in the omega-p-Jackson frame are anisotropic we argue that this is not an indication of a resonance but rather a kinematical effect reflecting the anisotropy of the omega angular distribution. The helicity angle distribution in the omega-p-helicity frame shows an anisotropy which probably reflects effects of the omega angular momenta in the final state; this observable may be, in addition to the orientation of the omega decay plane, the most sensitive one to judge the validity of theoretical descriptions of the production process.
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