Plasma globotriaosylsphingosine (lyso-Gb3) is a promising secondary screening biomarker for Fabry disease. Here, we examined its applicability as a primary screening biomarker for classic and ...late-onset Fabry disease in males and females.
Between 1 July 2014 and 31 December 2015, we screened 2,360 patients (1,324 males) referred from 169 Japanese specialty clinics (cardiology, nephrology, neurology, and pediatrics), based on clinical symptoms suggestive of Fabry disease. We used the plasma lyso-Gb3 concentration, α-galactosidase A (α-Gal A) activity, and analysis of the α-Gal A gene (GLA) for primary and secondary screens, respectively.
Of 8 males with elevated lyso-Gb3 levels (≥2.0 ng ml–1) and low α-Gal A activity (≤4.0 nmol h–1 ml–1), 7 presented a GLA mutation (2 classic and 5 late-onset). Of 15 females with elevated lyso-Gb3, 7 displayed low α-Gal A activity (5 with GLA mutations; 4 classic and 1 late-onset) and 8 exhibited normal α-Gal A activity (1 with a classic GLA mutation and 3 with genetic variants of uncertain significance).
Plasma lyso-Gb3 is a potential primary screening biomarker for classic and late-onset Fabry disease probands.
The Relationship Between Coronary Plaque Characteristics and Small Embolic Particles During Coronary Stent Implantation Takahiro Kawamoto, Hiroyuki Okura, Yuji Koyama, Iku Toda, Haruyuki Taguchi, ...Koichi Tamita, Atsushi Yamamuro, Yuki Yoshimura, Yoji Neishi, Eiji Toyota, Kiyoshi Yoshida In 44 patients who underwent elective coronary stenting, lesion plaque characteristics were identified with virtual histology intravascular ultrasound, and small embolic particles during stenting were detected as high-intensity transient signals (HITS) with a Doppler guidewire. Dense calcium and necrotic core areas were significantly larger in the highest HITS count tertile, and multivariate analysis revealed only necrotic core area was an independent predictor of a high HITS count (odds ratio 4.41, p = 0.045). The HITS counts negatively correlated with coronary flow velocity reserve after stenting (r = −0.35, p = 0.017). Necrotic core component is an indicator for high risk of micro-emboli during stenting.
The present study investigated the clinical value of myocardial contrast-delayed enhancement (DE) with multidetector computed tomography (MDCT) without iodine re-injection immediately after primary ...percutaneous coronary intervention (PCI) for predicting future cardiovascular events after acute myocardial infarction (AMI). We performed a prospective study in which 263 consecutive patients with first AMI successfully treated with primary PCI were enrolled. Sixty-four-slice MDCT without the re-injection of contrast medium was performed immediately after PCI. Myocardial DE was considered to be transmural when involving myocardial thickness ≥ 75% (Group A;
n
= 104), subendocardial (< 75%, Group B;
n
= 108), or normal (Group C;
n
= 51). A semiquantitative scale score was defined for 17 left ventricular segments to investigate the extent of the DE area assessed. We examined the relationship between the presence or absence of transmural DE and long-term cardiovascular event rates. The median follow-up period was 3.5 years. Kaplan–Meier survival curves showed that patient prognosis was poorer in the group with Group A than that in the group with Group B, which was equivalent to that with Group C. A multivariate analysis identified the presence of transmural DE as the strongest predictor for future cardiovascular events (hazard ratio: 3.7;
P
= 0.023). Transmural myocardial DE immediately following primary PCI without an iodine re-injection for AMI is a major risk factor for future cardiovascular events.
Summary A 59-year-old male with an abdominal mass that showed a diffuse large B cell lymphoma underwent extirpation of the tumor and chemotherapy. He subsequently received high-dose chemotherapy ...containing cyclophosphamide (1.5 g/m2 /day × 2 days), followed by autologous peripheral blood stem cell transplantation. He developed congestive heart failure 5 days after administration of cyclophosphamide. His electrocardiogram showed extremely low voltage with ST segment change and echocardiogram showed diffusely increased left ventricular wall thickness, an increase in myocardial echogenicity, pericardial effusion, and generally decreased systolic function. Congestive heart failure progressed rapidly and he died the following day. Post-mortem examination of the heart revealed myocardial hemorrhage, yellowish brown pericardial effusion, and fibrinous pericarditis. His liver was atrophic and focal necrosis was observed histologically. Cyclophosphamide-induced cardiotoxicity occurred, even though the patient had both shown normal cardiac function before high-dose chemotherapy and had received a lower dose of cyclophosphamide. Concomitant administration of cytarabine might have affected his liver function and there might have been interaction between the drugs.
Recent studies have demonstrated that newly diagnosed glucose intolerance is common among patients with acute myocardial infarction (AMI). The purpose of this study was to assess the long-term ...clinical cardiovascular outcomes in participants with AMI with abnormal fasting glucose compared with normal fasting glucose and an abnormal oral glucose tolerance test (OGTT) compared with a normal OGTT.
A prospective study was performed in 275 consecutive patients with AMI, 85 of whom had pre-diagnosed diabetes mellitus (DM). Those without DM were divided into two groups based on the 75 g OGTT at the time of discharge. Abnormal glucose tolerance (AGT) was defined as 2 h glucose ≥140 mg/dl; 78 patients had normal glucose tolerance (NGT) and 112 had AGT. The same patients were also reclassified into the normal fasting glucose group (NFG; n=168) or the impaired fasting glucose group (IFG; n=22). The association between the glucometabolic status and long-term major adverse cardiovascular event rates was evaluated.
Kaplan-Meier survival curves showed that the AGT group had a worse prognosis than the NGT group and an equivalent prognosis to the DM group (p<0.0005). Cox proportional hazard model analysis showed that the HR of AGT to NGT for major adverse cardiovascular event rates was 2.65 (95% CI 1.37 to 5.15, p=0.004) while the HR of DM to NGT was 3.27 (1.68 to 6.38, p=0.0005). However, Cox HR of IFG to NFG for major adverse cardiovascular event rates was 1.83 (0.86 to 3.87), which was not significant.
In patients with AMI, an abnormal OGTT is a better risk factor for future adverse cardiovascular events than impaired fasting blood glucose.
BackgroundDrug-eluting stent-induced vasospastic angina (DES-VSA) has emerged as a novel complication in the modern era of percutaneous coronary intervention (PCI). Although beta blockers (BBs) are ...generally recommended for coronary heart disease, they may promote incidence of DES-VSA. This study aimed to compare the effects of calcium channel blockers (CCBs) perceived to be protective against DES-VSA and BBs on subsequent coronary events after second-generation drug-eluting stent implantation.MethodsIn this multicentre prospective, randomised study, 52 patients with coronary artery disease who underwent PCI for a single-vessel lesion with everolimus-eluting stent placement were randomised into post-stenting BB (N=26) and CCB (N=26) groups and followed for 24 months to detect any major cardiovascular events (MACE). A positive result on acetylcholine provocation testing during diagnostic coronary angiography (CAG) at 9 months was the primary endpoint for equivalence. MACE included all-cause death, non-fatal myocardial infarction, unstable angina, cerebrovascular disease or coronary revascularisation for stable coronary artery disease after index PCI.ResultsAt 9 months, 42 patients (80.8%) underwent diagnostic coronary angiography and acetylcholine provocation testing. Among them, seven patients in each group were diagnosed with definite vasospasm (intention-to-treat analysis 26.9% vs 26.9%, risk difference 0 (−0.241, 0.241)). Meanwhile, the secondary endpoint, 24-month MACE, was higher in the CCB group (19.2%) than in the BB group (3.8%) (p=0.01). In detail, coronary revascularisation for stable coronary artery disease was the predominant endpoint that contributed to the greater proportion of MACE in the CCB group (CCB (19.2%) vs BB (3.8%), p=0.03).ConclusionsThe incidence of acetylcholine-induced coronary artery spasms did not differ between patients receiving BBs or CCBs at 9 months after PCI. However, a higher incidence of 2-year MACE was observed in the CCB group, suggesting the importance of BB administration.Trial registration numberThis study was registered at the Japanese University Hospital Medical Information Network (UMIN) Clinical Trial Registry (The Prospective Randomized Trial for Optimizing Medical Therapy After Stenting: Calcium-Beta Trial; UMIN000008321, https://upload.umin.ac.jp/cgi-open-bin/ctr_e/ctr_view.cgi?recptno=R000009536).
Background Newly-diagnosed diabetes mellitus (DM) and impaired glucose tolerance are common among patients with acute myocardial infarction (AMI). However, its significance on long-term clinical ...outcomes in those patients remains unclear. The present study was designed to determine whether such abnormalities after AMI affect long-term clinical outcomes. Methods and Results Two-hundred and seventy-five AMI patients were prospectively enrolled. Eighty-five had diagnosed DM, while the other 190 did not. According to oral glucose tolerance tests at discharge, non-DM patients were divided into 2 groups: 78 patients with normal glucose tolerance and 112 patients with abnormal glucose tolerance. Patients were followed until they reached the primary endpoint: cardiovascular death or unplanned hospitalization due to major adverse cardiovascular events. The median follow-up period was 5.3 years. Kaplan-Meier survival curves for the abnormal glucose tolerance group were poorer than for normal glucose tolerance, and were equivalent to the pre-diagnosed DM group in prognosis (p<0.0005). Glucometabolic status was the strongest predictor for future cardiovascular events (hazard ratio to normal glucose tolerance; 2.65; confidence interval: 1.37-5.15; p=0.004 in abnormal glucose tolerance and 3.27:1.68-6.38; p=0.0005 in DM). Conclusions Abnormal glucose tolerance in patients with AMI is a major risk factor for future cardiovascular events and may critically distinguish high-risk individuals. (Circ J 2007; 71: 834 - 841)
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