Inner segment ellipsoid band length is a prognostic factor in retinitis pigmentosa associated with EYS mutations: 5-year observation of retinal structure.

PurposeTo evaluate whether the length of the inner segment ellipsoid (ISe) band can be used as a prognostic factor for disease course in retinitis pigmentosa (RP) patients with EYS mutations by observation over a period of 5 years.MethodsTwelve RP patients with EYS mutations were studied. The horizontal and vertical ISe length of the right eye was manually measured at five time points annually, using spectral domain optical coherence tomography. A regression line through the five points from baseline to the final measurement was drawn and the ratio of the length (%) at each point to the baseline length was calculated; the slope was defined as the rate of ISe shortening (%/year). The correlation between the rate of ISe shortening and age, visual acuity, and mean deviation (MD) value were evaluated. The intraclass correlation coefficient (ICC) for the measurements was calculated.ResultsThe mean rate of ISe shortening was -4.65±2.89% per year and the decline was statistically significant. The rate of shortening was significantly negatively correlated with the baseline length (P=0.046, r=0.58), but not with the baseline age, visual acuity, and MD value. The ICC (2, 1) was 0.999.ConclusionsISe of all RP patients with EYS mutations shortened during the 5 years of annual observation. The measurement of the length of ISe is a simple and convenient method with high repeatability, and the length is a sensitive prognostic factor for the rate of ISe shortening in RP patients with EYS mutations.

Remnant-like lipoprotein particles as risk factors for coronary artery disease in elderly patients.

Although remnant-like lipoprotein particles (RLPs) are known to be atherogenic, the relationship between serum RLP-cholesterol (RLP-C) level and coronary artery disease (CAD) has not as yet been evaluated. This clinical study was aimed at investigating the pathological significance of serum RLP-C among several coronary risk factors with a clear focus on elderly patients. We took fasting venous blood samples to determine lipid profiles including RLP-C from 188 patients with angiographically identified CAD and 68 control patients. Overall analysis showed that the RLP-C/HDL-C ratio was higher in both single-vessel CAD group (n = 67; p < 0.01) and multi-vessel CAD group (n = 121; p < 0.001) compared to controls. Further, multiple logistic regression analysis indicated that the diabetes, HDL-C and the RLP-C/HDL-C ratio could discriminate CAD patients from controls. In patients younger than 65 years, diabetes, HDL-C, LDL-C and the LDL-C/HDL-C ratio as well as the RLP-C/HDL-C ratio could discriminate CAD. In patients 65 aged years or older, however, diabetes, triglyceride and RLP-C as well as the RLP-C/HDL-C ratio could discriminate CAD, whereas LDL-C and the LDL-C/HDL-C ratio could not. These results led us to believe that the contribution of a given risk factor to the development of CAD in elderly patients may be different from that in younger patients. In elderly patients, RLP-C rather than LDL-C was strongly associated with the development of CAD. Accordingly, serum RLP-C levels may serve as a convenient and reliable index for assessing CAD.

Acute myocardial infarction associated with myocardial bridge and coronary artery vasospasm.

A 65-year-old man was admitted to our hospital with acute myocardial infarction (MI). Emergency coronary angiography showed no significant organic lesions, but a myocardial bridge was found at the mid-left anterior descending artery An acetylcholine provocation test revealed 90% spastic stenosis just proximal to the myocardial bridge. His acute MI could have been caused by both a coronary spasm and the myocardial bridge.

Clinical significance of antibody against oxidized low density lipoprotein in patients with atherosclerotic coronary artery disease.

OBJECTIVES: This study was designed to establish the clinical significance of antibodies against oxidized low density lipoprotein (anti-Ox-LDL) titer in atherosclerotic coronary artery disease (CAD). BACKGROUND: Oxidative modification of LDL, which plays a key role in the development of atherosclerosis, induces immunogenic epitopes in the LDL molecule, and the presence of anti-Ox-LDL has been demonstrated in human sera. METHODS: Anti-Ox-LDL titer was measured by enzyme-linked immunosorbent assay in 108 patients who had angiographically verified CAD, and 31 patients who had chest pain but no significant CAD, as controls. RESULTS: The anti-Ox-LDL titer was higher (p < 0.01) in patients with multivessel CAD (19.4 +/- 10.1 AcU/ml, n = 68) than in the controls (9.8 +/- 4.1). However, no significant difference was shown between the single-vessel CAD group (15.1 +/- 6.4, n = 40) and the controls, or between the multivessel CAD group and the single-vessel CAD group. The titer was higher in patients with unstable angina (21.5 +/- 11.8 AcU/ml, n = 20, p < 0.01), or in patients with acute myocardial infarction (23.1 +/- 12.0, n = 20, p < 0.01) than in patients with stable-effort angina or old myocardial infarction (12.2 +/- 8.6, n = 68). Multiple logistic regression analysis indicated that the anti-Ox-LDL titer most powerfully discriminated CAD patients from controls (odds ratio [OR]: 1.20, 95% confidence interval [CI]: 1.07-1.33, p = 0.0006) and acute coronary syndrome from chronic CAD (OR: 1.09, 95% CI: 1.04-1.14, p = 0.0008). CONCLUSIONS: Serum anti-Ox-LDL titer not only can predict a presence of atherosclerotic CAD but also may be a marker of plaque instability. Low density lipoprotein oxidation may play an important role in the development of plaque instability.

Significance of platelet aggregability immediately after blood sampling and effect of cigarette smoking.

A novel type platelet aggregometer, a WBA Analyzer, has enabled us to obtain the platelet aggregability data immediately after blood sampling, which is considered to closely reflect in vivo platelet function. Using this analyzer, we measured the platelet aggregatory threshold index (PATI) 5 min after blood sampling and compared it with that 60 min after blood sampling in 20 healthy male volunteers (10 smokers and 10 non-smokers). In the non-smokers, PATI was 10.3+/-2.3 microM 5 min after blood sampling, and it decreased to 4.7+/-1.5 (P<0.001) 60 min after blood sampling. In the smokers, the PATI was 7.7+/-2.9 microM 5 min after blood sampling, and it decreased to 3.8+/-1.5 (P<0.001) at 60 min after blood sampling. In the smokers, the PATI 5 min after blood sampling increased after a 4-week cessation of smoking (10.4+/-2.9, P<0.01), although the PATI 60 min after blood sampling did not change (4.2+/-1.6 microM). The measurement of platelet aggregability immediately after blood sampling using a WBA Analyser may be useful to evaluate not only platelet function in various thrombotic disorders, but also the effects of various anti-platelet drugs. Cessation of smoking should also be encouraged in the light of the adverse effects on platelet function.

Unusual coronary artery aneurysm and acute myocardial infarction in a middle-aged man with systemic lupus erythematosus.

A 55-year-old man developed acute myocardial infarction (AMI) related to a large coronary artery aneurysm and a distal coronary stenotic lesion after steroid therapy for systemic lupus erythematosus (SLE). Only 13 SLE patients with AMI caused by coronary artery aneurysms have been reported, 11 of whom were young or middle-aged women and the 2 remaining were young men. This is the first report of a middle-aged man with multiple coronary lesions.

Antibody against oxidized low density lipoprotein may predict progression or regression of atherosclerotic coronary artery disease.

OBJECTIVES: This study aimed to elucidate whether an antibody against oxidized low density lipoprotein (anti-Ox-LDL) could predict short-term coronary artery atherosclerotic lesion progression. BACKGROUND: It is still controversial whether higher levels of the anti-Ox-LDL titer are associated with atherosclerotic coronary artery disease. METHODS: In 52 patients undergoing coronary angioplasty and six-month follow-up angiography, we performed quantitative coronary angiographic analysis of a lesion on a branch away from the intervention site vessel and assessed lesion progression or regression using the Progression-Regression score calculated as the baseline minimal lumen diameter minus the follow-up minimal lumen diameter. The serum anti-Ox-LDL titer was measured using an enzyme-linked immunosorbent assay method just before the initial angiography in all patients. RESULTS: The anti-Ox-LDL titer was 16.6+/-1.5 AcU/ml in the progression group (Progression-Regression score >0.15 mm; n = 20), which was significantly higher (p < 0.001) than the value of 9.5+/-1.2 in the regression group (< or =-0.15 mm; n = 14) and also higher (p < 0.01) than the value of 11.4+/-1.3 in the no-change group (-0.15 to 0.15 mm; n = 18). The Progression-Regression score was correlated with the antibody titer in all patients (r = 0.56, p < 0.001). Multiple regression analysis showed that the Progression-Regression score was independently correlated with the antibody titer (r = 0.44, p < 0.01) as well as lipoprotein (a) (r = 0.33, p < 0.05). CONCLUSIONS: Anti-Ox-LDL may be an independent predictor of coronary atherosclerotic lesion progression in the short term.

Immunological response to oxidized LDL occurs in association with oxidative DNA damage independently of serum LDL concentrations in dyslipidemic patients.

Oxidative modification of LDL induces immunogenic epitopes in the LDL molecule, and the presence of antibodies against oxidized LDL (anti-Ox-LDL) has been demonstrated in human sera. However, little is known about the clinical significance of anti-Ox-LDL. To elucidate a clinical relationship between the immunological response to oxidized LDL and cellular oxidative stress, we measured serum titers of anti-Ox-LDL in 45 unselected patients with hypercholesterolemia and serum 8-hydroxy-2'-deoxyguanosine (8-OHdG), considered a biomarker of the oxidative damage to DNA. The anti-Ox-LDL titer was not correlated with the serum LDL-C concentration, but was correlated with the 8-OHdG concentration (r = 0.300, P < 0.05) in a simple linear regression. Multiple regression analysis indicated that 8-OHdG was independently correlated with anti-Ox-LDL (r = 0.429, P < 0.05), but no other variables, including LDL-C concentrations and smoking habit, were correlated with anti-Ox-LDL. In 16 subgroup patients, the concentrations of TC, TG and LDL-C decreased and the HDL-C concentration increased after cholesterol-lowering therapy with fluvastatin. In addition, both the anti-Ox LDL titer (14.0 +/- 9.5 to 11.4 +/- 6.6 AcU/ml, P < 0.05) and the 8-OHdG concentration (1.19 +/- 0.41 to 0.85 +/- 0.43 ng/ml, P < 0.05) also decreased after fluvastatin therapy. The immunological response to LDL oxidation on vascular wall tissues or cells appear to occur in association with oxidative DNA damage. The measurement of anti-Ox-LDL may be a useful indicator for lipid-lowering therapy.

Serum prostaglandin D synthase level after coronary angioplasty may predict occurrence of restenosis.

Lipocalin-type prostaglandin D synthase (L-PGDS), which is responsible for the biosynthesis of PGD2, has recently been found to be present in the atherosclerotic plaque of the human coronary artery and also to be secreted in human serum. We measured the serum L-PGDS level and compared it with the expressions of the platelet membrane surface glycoprotein and neutrophil adhesion molecule in patients undergoing PTCA. The L-PGDS level significantly decreased (P < 0.01) and the platelet surface expression of CD62P (P-selectin) significantly increased (P < 0.01) immediately after PTCA in the coronary sinus blood. Both changes were inversely correlated (R = -0.72, P < 0.001). Although the L-PGDS level in the coronary sinus blood remained equivalent to the baseline level in patients who experienced restenosis, the level increased over the baseline level (P < 0.01) at 48 h after PTCA in patients without restenosis. Neutrophil surface expression of CD11b (alpha subunit of Mac-1) significantly increased at 24 h (P < 0.01) to 48 h (P < 0.001) after PTCA in the coronary sinus blood in patients with restenosis but the change showed less significant in patients without restenosis. The changes in the L-PGDS level and the CD11b expression at 48 h after PTCA were inversely correlated (R = -0.55, P < 0.05). An increased serum L-PGDS level at 48 h after PTCA possibly predicts the avoidance of late restenosis. It is suggested that reduction in PGD2 synthesis triggers platelet activation and that a subsequent increase in the PGD2 synthesis suppresses inflammatory reaction at the intervention site indicated by neutrophil activation and inhibits development of restenosis. Pharmacological or biological intervention that increases endogenous PGD2 synthesis should be tested as a new strategy to prevent restenosis.

Cardiac pacing as emergency care for serious bradyarrhythmia with circulatory shock.

Three cases of bradyarrhythmia with serious illness of extracardiac organs are reported. Case 1 had sick sinus syndrome. He was admitted to our hospital complaining of syncope and developed apnea. Case 2 had complete atrioventricular block and serious hepatic failure in the hospital. Case 3 had paroxysmal atrioventricular block. He complained of syncope which followed convulsions. Their symptoms might be due to circulatory shock caused by a lazy lower pacemaker from the ventricle. Emergent temporary pacing successfully improved the extracardiac organ dysfunction. Although their bradyarrhythmias were transient, permanent pacemakers were implanted to inhibit the recurrence. A quick temporary pacing should be indicated in patients with critical bradyarrhythmia like our cases for survival.

Comparison of activation process of platelets and neutrophils after coronary stent implantation versus balloon angioplasty for stable angina pectoris.

The pathophysiologic features of stent-induced cellular responses of platelets and leukocytes have not been established. This study was designed to clinically investigate the activation of platelets and neutrophils after coronary stenting and to identify its effects on the long-term results of coronary stents. Forty-eight consecutive patients with left anterior descending coronary artery disease indicating coronary intervention were randomly assigned to either a balloon angioplasty group or a coronary stent group. Flow cytometric analysis demonstrated that the transcardiac gradient (the value of coronary sinus blood minus the value of peripheral blood) of platelet surface expression of CD62P (p < 0.001) and CD63 (p < 0.01) increased immediately after coronary stenting, but increased less significantly immediately after balloon angioplasty (CD62P, p < 0.01; CD63, p < 0.05). These increases were persistently observed after coronary stenting but transiently after balloon angioplasty alone during a 48-hour observation period after the procedures. The gradient for neutrophil surface expression of CD11b increased, and that of CD62 L decreased 48 hours after coronary stenting (CD11b, p < 0.001; CD62 L, p < 0.05), but these changes showed less significance 48 hours after balloon angioplasty alone (CD11b, p < 0.05; CD62 L, p = NS). The gradients 48 hours after the procedures for both CD62P (r = 0.39, p < 0.05) and CD11b (r = 0.44, p < 0.01) were independently correlated with the late loss in the stent group, whereas the correlation was seen only for CD11b (r = 0.38, p < 0.05) in the balloon angioplasty group. Both platelet and neutrophil activation was greater after coronary stenting than after balloon angioplasty. Cellular interactions between platelets and neutrophils may be related to the progression of neointimal proliferation leading to restenosis after coronary stent implantation.

Reflex sympathetic dystrophy following transbrachial cardiac catheterization.

We experienced a rare case of complication by reflex sympathetic dystrophy (RSD) following transbrachial cardiac catheterization which may have been caused by poorly executed hemostasis using a hemostatic device. The symptoms of RSD markedly limited the patientOs daily work activities. Although the transbrachial approach is a useful procedure for cardiac catheterization, interventionalists should be aware that RSD may cause serious complications.

Idiopathic adrenal hemorrhage.

A case of idiopathic adrenal hemorrhage is reported. A 76-year-old woman exhibited a left adrenal tumor, 3 cm in diameter, on abdominal computed tomography. The patient was receiving aspirin medication for atrial fibrillation. There was no evidence of increased adrenal hormones. The mass enlarged to 6 cm in diameter within 18 months, and malignancy was suspected. The mass was diagnosed as adrenal hematoma by operative findings.

Correlation between interventricular septal motion and left ventricular systolic-diastolic function in patients with left bundle branch block.

The echocardiographic correlation between abnormal interventricular septal motion including systolic paradoxical, flat motion and early diastolic notch and ejection fraction, mean ventricular circumferential fiber shortening and early diastolic mitral filling velocity was studied in 46 patients with complete left bundle branch block. Systolic normal interventricular septal motion was used as the control. Ejection fraction was significantly smaller in the paradoxical(0.44 +/- 0.13, p < 0.02) and flat motion groups(0.38 +/- 0.09, p < 0.001) than in the normal group(0.54 +/- 0.1). Mean ventricular circumferential fiber shortening was significantly smaller in the flat motion group than in the normal group(0.72 +/- 0.19 vs 0.99 +/- 0.23 circ/sec, p < 0.002). The size of the notch was significantly smaller in the flat motion group than in the normal group (2.3 +/- 0.2 vs 4.8 +/- 0.8 mm, p < 0.0001). The deceleration rate of the notch was significantly slower in the paradoxical and flat motion groups than in the normal group(37.3 +/- 12.2, 31.3 +/- 8.1 vs 69.1 +/- 2.5 cm/sec, p < 0.0001). Early diastolic mitral filling velocity was slower in the flat motion group than in the other 2 groups. In conclusion, systolic flat interventricular septal motion showed more severe disturbances of left ventricular systolic and diastolic function than paradoxical interventricular septal motion in patients with complete left bundle branch block.

Insulin resistance affects endothelium-dependent acetylcholine-induced coronary artery response.

AIMS: This study was designed to investigate the relationship between insulin resistance and the acetylcholine-induced endothelium-dependent coronary artery response in patients without angiographically significant atherosclerotic coronary artery disease and to elucidate the pathophysiological significance of insulin resistance in the early stages of coronary atherosclerosis. METHODS AND RESULTS: Insulin resistance was calculated from fasting plasma glucose and insulin concentration using homeostasis model assessment in 40 patients suspected of having ischaemic heart disease, but without angiographic evidence of atherosclerotic coronary artery disease defined as a discrete stenosis or intimal irregularity. They were selected for an acetylcholine provocation test in both left and right coronary arteries. The homeostasis model assessment level was higher in 16 acetylcholine-positive patients than in 24 acetylcholine-negative patients (1.84+/-1.24 vs 0.72+/-0.62, P<0.01). Comparisons of the percentage change in vessel lumen diameter after the acetylcholine test in each of proximal, mid and distal segments of three coronary arteries among the three groups of low (less than 0.7; n=13), intermediate (0.7 to 1.4; n=13), and high homeostasis model assessment level (more than 1.4; n=14) revealed that a higher level resulted in a worse acetylcholine-induced constrictive response in coronary arteries. CONCLUSION: These results suggest that there is an association between high insulin resistance and coronary vascular endothelial cell dysfunction, and that insulin resistance may be an indicator of early stage coronary artery atherosclerosis not detectable by angiography.

Brugada syndrome characterized by the appearance of J waves.

We describe a patient with Brugada syndrome. The ST-segment elevation in precordial leads was revealed during admission, but the appearance of J waves was characteristic before ventricular fibrillation (VF), rather than ST-segment elevation. J waves have been reported to be associated with the presence of an Ito-mediated prominent action potential notch in the epicardium. It is considered that one of the mechanisms of this VF is due to heterogeneous distribution of the refractory period according to changes in K+ channels including Ito.

Serum N-acetyl-beta-D-gulucosaminidase activity increases in association with insulin resistance in patients with coronary artery disease.

N-acetyl-beta-D-glucosaminidase (NAG) is released from lysosomes, but the clinical significance of its serum activity in the pathogenesis of coronary artery disease has not been well understood. We measured serum NAG activity by a colorimetric method in consecutive 168 patients suspected of having coronary artery disease who underwent diagnostic coronary angiography. In addition, we evaluated the relationship between the activity and severity of coronary artery disease, as well as various coronary risk factors. Serum NAG activity was higher in the multi-vessel disease group than in the no stenotic lesion group (9.2+/-2.3 vs. 7.8+/-1.8 U/l, P<0.01) and in the single-vessel disease group (vs. 8.2+/-2.2 U/l, P<0.05). In all patients, Gensini score was closely correlated with the serum NAG activity (r = 0.39, P<0.001). Multiple regression analysis showed that serum NAG activity was correlated with plasma insulin level (r = 0.49, P<0.01), but not correlated with other coronary risk factors. In 126 patients without apparent diabetes mellitus, serum NAG was also correlated with plasma insulin level (r = 0.37, P<0.01) and additionally with insulin resistanc determined by homeostasis model assessment (r = 0.47, P<0.01). Our results suggested that serum NAG activity correlates with the severity of coronary artery disease in relation to plasma insulin level and insulin resistance, and thus can be an indicator of coronary artery disease based upon abnormalities of glucose metabolism.

Air embolism in the right coronary artery occurring during the left coronary angioplasty using the guiding catheter with a side hole.

Coronary air embolism is one of the inadvertent complications of coronary angioplasty. We report two rare cases of complicating air embolism in the right coronary artery occurring during control left coronary angiography using a guiding catheter with a side hole, just prior to a coronary intervention procedure for a left coronary artery lesion. The air seemed to be injected into the right coronary artery through the side hole. When we use an angiographic or guiding catheter with a side hole, we should be aware that an air embolism can occur in the contralateral coronary artery and should carefully and repeatedly perform aspiration of the catheter.

Direct discrimination and full-day disclosure of ventricular parasystole on new heart rate tachograms.

INTRODUCTION: To discriminate ventricular parasystole from fixed coupling interval ventricular premature complexes (VPCs), we developed a new diagnostic method using a dot distribution pattern corresponding to VPCs recorded on a heart rate tachogram using ambulatory ECG monitoring data. We tested our hypothesis that widely scattered VPC dots on instantaneous heart rate tachograms indicate a constant VPC-VPC interval compatible with parasystole. METHODS AND RESULTS: Patients with frequent VPCs > 5,000/day) were divided into two groups depending on the tachogram dot distribution patterns: group S (n = 10, aged 61 +/- 16 years) showed widely scattered VPC dot distribution, whereas group F (n = 10, 60 +/- 17 years) showed fixed VPC dot distribution limited to a narrow zone. Using digitized R-R interval data, full-day heart rate tachograms and VPC-VPC intervals were depicted simultaneously. Group S demonstrated constant basic VPC-VPC intervals (1,285 to 2,052 msec, mean 1,738 +/- 219), with a coefficient of variation (CV) of 0.061 +/- 0.018. Their VPC coupling intervals were markedly variable (651 +/- 113 msec; CV = 0.193 +/- 0.034). Each patient's basic VPC-VPC intervals showed small diurnal alterations (minimum -13% +/- 3% to maximum +15% +/- 6%). VPC-VPC intervals in group F were not constant and showed marked variation. Group F VPC coupling intervals were shorter and constant (480 +/- 30 msec, P = 0.0002; with CV = 0.076 +/- 0.013, P < 0.0001). CONCLUSION: Ventricular parasystole with constant VPC-VPC intervals consistently became evident based on VPC dot patterns recorded on heart rate tachograms.

[A ruptured aneurysm of the anterior and posterior inferior cerebellar artery: a case report].

We report a case of an anterior and posterior-inferior cerebellar artery (AICA-PICA) aneurysm. The patient was a 62-year-old woman who suffered from sudden onset of severe headache and nuchalgia. Computed tomography (CT) scan revealed subarachnoid hemorrhage in the ambient cistern and blood clots in the 3rd and 4th ventricles. Vertebral angiography demonstrated an aneurysm located at the distal segment of the left AICA-PICA. Three demensional CT scan was very useful for the decision concerning surgical strategy. The patient underwent bilateral occipital craniectomy and the aneurysm was clipped successfully via the midline suboccipital approach. Her postoperative course was uneventful. Postoperative angiography showed successful clipping of the aneurysm. Distal AICA-PICA aneurysm is a very rare disease and only one case has been reported in the literature. The clinical features, CT findings, and surgical approach of distal AICA-PICA aneurysms are briefly discussed while reviewing the literature.