The c.1617del variant of TMEM260 is identified as the most frequent single gene determinant for Japanese patients with a specific type of congenital heart disease.

Although the molecular mechanisms underlying congenital heart disease (CHD) remain poorly understood, recent advances in genetic analysis have facilitated the exploration of causative genes for CHD. We reported that the pathogenic variant c.1617del of TMEM260, which encodes a transmembrane protein, is highly associated with CHD, specifically persistent truncus arteriosus (PTA), the most severe cardiac outflow tract (OFT) defect. Using whole-exome sequencing, the c.1617del variant was identified in two siblings with PTA in a Japanese family and in three of the 26 DNAs obtained from Japanese individuals with PTA. The c.1617del of TMEM260 has been found only in East Asians, especially Japanese and Korean populations, and the frequency of this variant in PTA is estimated to be next to that of the 22q11.2 deletion, the most well-known genetic cause of PTA. Phenotype of patients with c.1617del appears to be predominantly in the heart, although TMEM260 is responsible for structural heart defects and renal anomalies syndrome (SHDRA). The mouse TMEM260 variant (p.W535Cfs*56), synonymous with the human variant (p.W539Cfs*9), exhibited truncation and downregulation by western blotting, and aggregation by immunocytochemistry. In situ hybridization demonstrated that Tmem260 is expressed ubiquitously during embryogenesis, including in the development of cardiac OFT implicated in PTA. This expression may be regulated by a ~ 0.8 kb genomic region in intron 3 of Tmem260 that includes multiple highly conserved binding sites for essential cardiac transcription factors, thus revealing that the c.1617del variant of TMEM260 is the major single-gene variant responsible for PTA in the Japanese population.

Genetic Risk Stratification of Primary Open-Angle Glaucoma in Japanese Individuals.

PURPOSE: To assess the impact of genetic risk estimation for primary open-angle glaucoma (POAG) in Japanese individuals. DESIGN: Cross-sectional analysis. PARTICIPANTS: Genetic risk scores (GRSs) were constructed based on a genome-wide association study (GWAS) of POAG in Japanese people. A total of 3625 Japanese individuals, including 1191 patients and 2434 controls (Japanese Tohoku), were used for the model selection. We also evaluated the discriminative accuracy of constructed GRSs in a dataset comprising 1034 patients and 1147 controls (the Japan Glaucoma Society Omics Group [JGS-OG] and the Genomic Research Committee of the Japanese Ophthalmological Society [GRC-JOS]) and 1900 participants from a population-based study (Hisayama Study). METHODS: We evaluated 2 types of GRSs: polygenic risk scores using the pruning and thresholding procedure and a GRS using variants associated with POAG in the GWAS of the International Glaucoma Genetics Consortium (IGGC). We selected the model with the highest areas under the receiver operating characteristic curve (AUC). In the population-based study, we evaluated the correlations between GRS and ocular measurements. MAIN OUTCOME MEASURE: Proportion of patients with POAG after stratification according to the GRS. RESULTS: We found that a GRS using 98 variants, which showed genome-wide significance in the IGGC, showed the best discriminative accuracy (AUC, 0.65). In the Japanese Tohoku, the proportion of patients with POAG in the top 10% individuals was significantly higher than that in the lowest 10% (odds ratio [OR], 6.15; 95% confidence interval [CI], 4.35-8.71). In the JGS-OG and GRC-JOS, we confirmed similar impact of POAG GRS (AUC, 0.64; OR [top vs. bottom decile], 5.81; 95% CI, 3.79-9.01). In the population-based study, POAG prevalence was significantly higher in the top 20% individuals of the GRS compared with the bottom 20% (9.2% vs. 5.0%). However, the discriminative accuracy was low (AUC, 0.56). The POAG GRS was correlated positively with intraocular pressure (r = 0.08: P = 4.0 × 10-4) and vertical cup-to-disc ratio (r = 0.11; P = 4.0 × 10-6). CONCLUSIONS: The GRS showed moderate discriminative accuracy for POAG in the Japanese population. However, risk stratification in the general population showed relatively weak discriminative performance. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Movement during the acquisition of a visual landmark may be necessary for rapid learning in ants.

We conducted laboratory experiments using Japanese carpenter ants (Camponotus japonicus) to investigate whether movement during visual learning can influence the learning performance of ant foragers. We performed three different experiments. In the first experiment, the ants could move freely in a straight maze during the visual learning. The ants in the experiments two and three were fixed to a certain position during the visual learning training. A distinct difference between these two experiments was that the ants in one experiment could perceive an approaching visual stimulus during the training, although they were fixed. After training phases, a Y-maze test was performed. One arm of the Y-maze had a visual stimulus presented to the ants during the training. We found that the ants in the first experiment showed rapid learning and correctly selected the landmark arm. However, the ants in the experiments two and three did not exhibit any preference for the chosen arm. Interestingly, we found differences in the time spent around a certain location in the Y-maze between the experiments two and three. These results suggest that movement during visual learning may influence the rapid learning of ant foragers.

Risk Factors for Progression of Primary Open-Angle Glaucoma with Lower Normal Intraocular Pressure.

INTRODUCTION: This study aimed to investigate intraocular pressure (IOP)-independent factors associated with the progression of primary open-angle glaucoma (POAG) with IOP ≤15 mm Hg. METHODS: POAG patients with maximum IOP ≤15 mm Hg at the Kyoto University Hospital between January 2011 and August 2021 were retrospectively enrolled. We evaluated effects of various factors on the rate of mean deviation (MD) changes in the visual field (VF) examinations using a linear mixed model. These factors included hypertension, diabetes mellitus (DM), hyperlipidemia (HL), cardiovascular disease, arrhythmia, disc hemorrhage, sleep apnea syndrome, orthopedic diseases, and malignant tumors. RESULTS: In total, 98 eyes from 68 patients were included. The baseline MD was -9.74 ± 7.85 dB. The mean rate of MD change and IOP during the observation period were -0.28 ± 0.04 dB/year and 11.8 ± 1.0 mm Hg, respectively. Comorbidity of DM or HL showed a significant positive association with the rate of MD change (ß = 0.35, p = 0.0006 and ß = 0.18, p = 0.036, respectively) in the model adjusted for age, sex, axial length, mean IOP, and standard deviation of IOP during the observation period. However, no significant association of DM or HL was found after adjusting for central corneal thickness. CONCLUSION: This study suggests that DM or HL is associated with VF deterioration in glaucoma with lower IOP, but the association may be due to differences in IOP characteristics.

Ongoing vascular inflammation evaluated by 18F-fluorodeoxyglucose positron emission tomography in patients long after Kawasaki disease.

BACKGROUND: This study aimed to determine whether ongoing vascular inflammation presents in patients who had coronary artery aneurysms (CAAs) caused by Kawasaki disease (KD). METHODS: Subjects were 26 patients with a history of KD; 15 had giant CAA (gCAA) ≥ 8.0 mm and 11 had smaller CAA (smCAA) < 8 mm in the acute phase. They underwent X-ray computed tomography and 18F-fluorodeoxyglucose positron emission tomography. We determined the maximum coronary target-to-background ratio (CaTBR) and the mean thoracic aorta TBR (TaTBR) in each patient. They were compared between groups, and their correlation with various variables was determined. RESULTS: CaTBR and TaTBR were significantly higher in gCAA than in smCAA (P < .005 for both values) and were significantly higher even in patients without any metabolic risk factor (P < .05 for both values). The CAA size in acute phase significantly positively correlated with CaTBR (R2 = 0.32) as well as TaTBR (R2 = 0.28). Also, TaTBR significantly positively correlated with CaTBR (R2 = 0.32) as well as cumulative number of metabolic risk factors (trend, P = .03). CONCLUSIONS: Ongoing vascular inflammation may present long after KD, especially in patients with severe inflammation expressed as gCAA in the acute phase.

Influence of Tumor Characteristics on Visual Field Outcomes After Pituitary Adenoma Surgery.

BACKGROUND: There were few reports about the influence of tumor characteristics on the postoperative visual field outcomes after transsphenoidal surgery for pituitary adenoma. The purpose of this study was to explore the tumor characteristics that influenced perioperative visual field changes. METHODS: Patients who underwent transsphenoidal surgery under a diagnosis of pituitary adenoma at the Kyoto University Hospital between April 2012 and December 2018 were retrospectively enrolled. Correlations among circumpapillary retinal nerve fiber layer thickness, preoperative and postoperative mean deviation (MD) of visual field, MD change after the surgery, and maximum tumor diameter were evaluated by measuring Pearson correlation coefficient. We evaluated the influences on postoperative MD using a generalized estimating equation for univariate and multivariate regression analyses. We also compared the characteristics of cystic and solid tumors. RESULTS: Thirty-two eyes of 18 patients were included in this study (9 male and 9 female patients). Postoperative MD positively correlated with maximum tumor diameter only in multivariate regression {ß = 0.22 (95% confidence interval [CI], 0.004-0.43), P = 0.046}, although maximum tumor diameter negatively correlated with postoperative MD in univariate regression (ß = -0.16 [95% CI, -0.58 to 0.26], P = 0.46). In the investigation of perioperative MD changes, eyes with cystic tumors showed significantly better improvement those with solid tumors (8.93 ± 7.85 vs 0.18 ± 6.56 dB, P = 0.007). CONCLUSIONS: Cystic and solid tumors show different characteristics of visual loss and visual field defects. The MD in eyes with cystic tumors improved significantly more than that in eyes with solid tumors.

Association between topical ß-blocker use and asthma attacks in glaucoma patients with asthma: a cohort study using a claims database.

PURPOSE: To investigate the association between the use of topical ß-blockers and subsequent asthma attacks in glaucoma patients with asthma. METHODS: This was a retrospective longitudinal cohort study using an administrative claims database. All patients aged 20 years or older who were registered in the health insurance claims database updated and managed by JMDC Inc. (Tokyo, Japan). Patients who were newly prescribed eye drops for glaucoma treatment were identified between 2011 and 2017. The patients with glaucoma were divided into two groups: ß-blocker users and non-ß-blocker users, based on the presence of a ß-blocker in the prescribed eye drops. We investigated whether the incidence of asthma attacks in patients with previously treated asthma differed between the two groups. RESULTS: We categorized 17,666 patients in the ß-blocker-user group and 12,609 patients in the non-ß-blocker-user group. A total of 580 patients in the ß-blocker group (3.28%) and 847 in the non-ß-blocker group (6.72%) underwent asthma treatment before the prescription of anti-glaucoma eye drops (P < 0.001). Furthermore, 94 patients in the ß-blocker-user group (0.53%) and 278 in the non-ß-blocker user group (2.20%) were undergoing current treatment for asthma (P < 0.001). The adjusted hazard ratios of asthma attacks were 0.73 (95% confidence interval, 0.46-1.16, P = 0.18) in patients with a history of asthma treatment and 1.22 (95% confidence interval, 0.56-2.70, P = 0.62) in patients with current asthma treatment, compared to the non-ß-blocker-user group. CONCLUSION: Our results clarified that several patients with asthma were prescribed topical ß-blockers for glaucoma treatment. However, asthma attacks may not be significantly attributed to topical ß-blockers, even in glaucoma patients under current asthma treatment. The administration of topical ß-blockers to asthma patients could be a treatment option in the absence of other treatment options, if adequate informed consent is obtained. Further studies are needed to draw a firm conclusion on this clinical question.

Atopic dermatitis as a risk factor for severe visual field loss in youth-a retrospective cohort study of glaucoma under steroid treatment.

PURPOSE: To assess the characteristics of glaucoma patients who received systemic or topical steroid treatment. METHODS: Patients who received steroid treatment were selected from a total of 4256 patients at our tertiary referral center of glaucoma management between August 2011 and October 2017. Clinical characteristics of the subjects were extracted from clinical records. To evaluate the factors influencing highest intraocular pressure during observation (max-IOP) or mean deviations (MDs) of visual field at the first and last visits, univariate and multivariate regression analyses were performed using a generalized estimating equation. RESULTS: Three hundred and eighty-two eyes of 196 patients were included in this study. The most frequent disease as the reason for steroid treatment was atopic dermatitis (58 eyes) followed by autoimmune diseases. The patients with atopic dermatitis were significantly younger (38.0 ± 11.2 years old, p < 0.001) and had lower MD (- 9.3 ± 9.1 dB at first visit, p = 0.01; - 10.6 ± 9.2 dB at last visit, p = 0.004) than those with other diseases. In multivariate regression analysis, age and MD at first visit, max-IOP, and atopic dermatitis were correlated with MD at last visit. CONCLUSIONS: The results of the present study suggest that patients with atopic dermatitis carry the risk of irreversible visual field loss even in youth. For earlier detection of high intraocular pressure, reinforcement of ophthalmological screening in management of atopic dermatitis should be recommended.

Simple method to distinguish the type of fetal premature contraction using arterial Doppler time interval measurements.

AIM: The purpose of this study was to establish a simple method to distinguish premature ventricular contractions (PVC) from premature atrial contractions (PAC) using a fetal Doppler ultrasound arterial pulse waveform to measure time intervals between sinus node restarting. METHODS: We retrospectively identified 14 fetuses with premature contraction (8 with PAC, 6 with PVC). We measured two distinct parts of time intervals using an arterial pulsed-wave Doppler: the two consecutive waveforms just before the premature contraction (2-V interval) and two consecutive waveforms including the premature contraction (XV interval) to measure time intervals between sinus node restarting. We then evaluated the time difference between the 2-V and XV intervals in PVC compared to PAC. RESULTS: For PVC, the difference between the 2-V interval and the XV interval was significantly shorter than that for PAC. A cut-off point of 33 ms, where a difference ≤33 ms was clearly shown to be associated with a PVC and a difference more than 33 ms signified a PAC was demonstrated. CONCLUSION: The 2-V and XV interval measurements, used to measure time intervals between sinus node restarting, could easily distinguish PVC from PAC in utero. Therefore, this study could potentially be a feasible and effective method for obstetricians or sonographers to employ usefully.

Intracardiac Echocardiography as a Guide for Transcatheter Closure of Patent Ductus Arteriosus.

BACKGROUND: Transcatheter closure of patent ductus arteriosus (TC-PDA), conventionally guided by aortography, has become the standard treatment of this disease. The purposes of this study were to evaluate whether intracardiac echocardiography (ICE) may be used for measuring PDA size and be used as a guide for TC-PDA. METHODS: This study had 2 phases. In phase 1, we compared the measurements of PDA size: pulmonary artery side diameter (PA-D), length, and aortic side diameter (Ao-D) of PDA, as measured by ICE with those measured by aortography or cardiac computed tomography (AoG/CCT) in 23 patients who underwent TC-PDA. In phase 2, we compared the demographics, fluoroscopic time, contrast volume, and complications of the TC-PDAs between 10 adult patients with ICE guidance and 16 without it. RESULTS: In phase 1, we found great correlation and agreement between ICE and AoG/CCT in PA-D (r = 0.985, bias -0.077 to 0.224), but moderate to poor correlation and agreement in length (r = 0.653, bias -0.491 to 3.065) and Ao-D (r = 0.704, bias 0.738 to 4.732), respectively. Nevertheless, all patients underwent successful TC-PDA with ICE guidance that allowed us to continuously monitor the whole process. In phase 2, TC-PDA required a significantly lower contrast volume with ICE guidance than without it, and there was no significant difference in the remaining variables between the 2 groups. CONCLUSION: ICE is comparable to AoG/CCT in providing accurate PA-D of the PDA and may be a safe alternative to guide TC-PDA as compared to conventional aortography.

Pregnancy and Delivery in Patients With Repaired Congenital Heart Disease　- A Retrospective Japanese Multicenter Study.

BACKGROUND: Although advances in cardiac surgery have led to an increased number of survivors with congenital heart disease (CHD), epidemiological data regarding the pregnancies and deliveries of patients with repaired CHD are scarce.Methods and Results:In this study, we retrospectively reviewed the clinical outcomes of pregnancies and deliveries of women with repaired CHD. Overall, 131 women with repaired CHD were enrolled and there were 269 gestations. All patients were classified as New York Heart Association (NYHA) Class I or II. The prevalence of cesarean sections was higher in patients with (CyCHD) than without (AcyCHD) a past history of cyanosis (51% vs. 19%, respectively; P<0.01). There were 228 offspring from 269 gestations and the most prevalent neonatal complication was premature birth (10%), which was more frequent in the CyCHD than AcyCHD group (15.7% vs. 5.6%, respectively; P<0.01). Five maternal cardiac complications during delivery were observed only in the CyCHD group (8%); these were classified as NYHA Class II and none was fatal. CONCLUSIONS: Delivery was successful in most women with repaired CHD who were classified as NYHA Class I or II, although some with CyCHD and NYHA Class II required more attention. Cesarean sections were more common in the CyCHD than AcyCHD group, and CyCHD may be a potential risk for preterm deliveries.

Analysis of Retinal Nerve Fibre Thickening in Progressive and Non-progressive Non-arteritic Anterior Ischaemic Optic Neuropathy Using Optical Coherence Tomography.

The study aims to investigate the longitudinal changes in the circumpapillary retinal nerve fibre layer thickness (cpRNFLT) in progressive and non-progressive non-arteritic anterior ischaemic optic neuropathy (NAION). This retrospective observational case series study analysed 17 eyes with NAION. Patients sustaining any additional visual loss (additional decrease in visual acuity (VA) ≥0.2 logMAR) within two months after initial onset of symptoms were classified as having progressive NAION. Of the 17 eyes with NAION, 13 (76.5%) were diagnosed as non-progressive and 4 (23.5%) were diagnosed as progressive. Compared with control eyes, eyes with non-progressive NAION showed greater cpRNFLT in all four optic disc quadrants at the initial visit (temporal and superior: P < .001; nasal and inferior: P = .002). In contrast, compared with control eyes, eyes with progressive NAION showed greater cpRNFLT in the superior and nasal quadrants (P = .004 and 0.028, respectively), but not in the temporal and inferior quadrants. During progression, eyes with progressive NAION showed a significant increase in cpRNFLT in the inferior quadrants; furthermore, there was significant increase in cpRNFLT in the nasal sector before visual loss developed after the initial visit. Progressive NAION showed development of the disc swelling from the superior to inferior portion of optic disc via the nasal swelling, suggesting that swollen axons in one ischaemic part may lead to secondary vascular infarction in another part of the optic disc. This enlargement could constitute the earliest sign of progressive NAION.

Multiple subretinal fluid blebs after pars plana vitrectomy for rhegmatogenous retinal detachment repair.

PURPOSE: To investigate the incidence of and clinical risk factors for multiple subretinal fluid (SRF) blebs after pars plana vitrectomy (PPV). METHODS: This study examined patients who underwent PPV (25- or 27-gauge) to repair a primary rhegmatogenous retinal detachment (RRD). Clinical characteristics, including age, sex, axial length, symptom duration, and postoperative best-corrected visual acuity (BCVA), were compared between eyes with and without multiple SRF blebs. Intentional drainage retinotomy and cryotherapy use were also performed. Main outcome measures were the effect of these parameters on multiple SRF bleb incidence 1 month after surgery. RESULTS: A total of 108 eyes of 106 patients (76 men and 32 women; mean age = 58.9 ± 9.0 years) were included. Multiple SRF blebs were observed in 8 eyes (7.4%). Logistic regression analysis showed that creation of intentional drainage retinotomy and 27-gauge PPV are risk factors for the development of multiple SRF blebs. The number and size of blebs spontaneously decreased over time, and SRF had completely resolved in 5 eyes (62.5%) 1 year after surgery. CONCLUSION: Multiple SRF blebs developed even after small gauge vitrectomy. The SRF did not affect postoperative BCVA and gradually absorbed without treatment.

Association of Bruch's membrane opening and optic disc morphology to axial length and visual field defects in eyes with primary open-angle glaucoma.

PURPOSE: To examine the morphology of Bruch's membrane opening (BMO), optic disc, and peripapillary atrophy (PPA) by scanning laser ophthalmoscopy (SLO) and spectral-domain optical coherence tomography (SD-OCT), and to determine their association with the axial length and visual field defects. METHODS: This was a cross-sectional study of 94 eyes of 56 subjects; 77 eyes were diagnosed with primary open-angle glaucoma and 17 eyes as normal. The margins of the optic disc were determined in the SLO images, and that of the BMO in the SD-OCT images. The ovality and area of the BMO and the optic disc were measured. The beta and gamma-PPA areas were also measured. The association of each parameter with the axial length and the mean deviation (MD) of the visual field tests was determined by generalized estimating equations (GEEs). RESULTS: The optic disc ovality was associated with the axial length and the MD (ß = -0.47, P = 7.6 × 10-4 and ß = 0.12, P = 0.040). The BMO ovality was not significantly associated with the axial length and the MD. The BMO area was associated with the axial length (ß = 0.30, P = 0.029). A larger BMO area was associated with a thinner BMO-based neuroretinal rim width (BMO-MRW) after adjustments for the MD (ß = -0.30, P = 2.1 × 10-4). The beta- and gamma-PPA areas were associated with the axial length (ß = 0.50, P = 7.4 × 10-5 and ß = 0.62, P = 4.2 × 10-6). CONCLUSIONS: The optic disc ovality was associated with both the axial length and MD, whereas BMO ovality was not. Attention should be paid to the influence of the axial length-related enlargement of the BMO.

Three Cases of KCNT1 Mutations: Malignant Migrating Partial Seizures in Infancy with Massive Systemic to Pulmonary Collateral Arteries.

KCNT1 mutations are gain-of-function mutations in potassium channels resulting in severe infantile epilepsy. Herein we describe 3 infants with malignant migrating partial seizures with KCNT1 mutations accompanied by massive systemic to pulmonary collateral arteries with life-threatening hemoptysis and heart failure.

Microstructure of Peripapillary Atrophy and Subsequent Visual Field Progression in Treated Primary Open-Angle Glaucoma.

PURPOSE: To investigate the relationship between the microstructure of ß-zone peripapillary atrophy (PPA) and the subsequent visual field (VF) progression in eyes with primary open-angle glaucoma (POAG), including highly myopic eyes. DESIGN: Retrospective cohort study. PARTICIPANTS: A total of 129 patients with POAG who had been followed up for a minimum of 2 years and had undergone at least 5 reliable standard automated perimetry tests after spectral-domain (SD) optical coherence tomography (OCT) examination. METHODS: ß-Zone PPA was evaluated from 3 SD OCT scans centered on the optic disc. Upper and lower scans were defined as scans at 30° above and below the horizontal scan, respectively. From 3 scans of each eye, ß-zone PPA was classified as PPA(+BM) or PPA(-BM) on the basis of the presence or absence of Bruch's membrane (BM), respectively. Eyes were classified into 3 groups according to the horizontal scan images: group A (only PPA(+BM)), group B (both PPA(+BM) and PPA(-BM)), and group C (only PPA(-BM)). Factors associated with the subsequent mean deviation (MD) slope after OCT examination were analyzed, and the hemifield total deviation (TD) slope was assessed in eyes with unilateral hemifield VF defects in the corresponding direction. MAIN OUTCOME MEASURES: Subsequent MD slope after OCT examination. RESULTS: The VF progression in group A was faster than in group C (P = 0.004). A larger PPA(+BM) width was associated with a faster MD slope in all eyes (P < 0.001) and highly myopic eyes (P < 0.001) and with a faster TD slope in eyes with superior or inferior hemifield VF defects in the corresponding direction (P = 0.002 and P = 0.035, respectively). A larger PPA(-BM) was correlated with a slower MD slope in all eyes (P = 0.030 and P = 0.034) but not in highly myopic eyes. CONCLUSIONS: There were significant differences in VF progression according to the microstructure of the ß-zone PPA in eyes with POAG. The PPA(+BM) width may be an important risk factor for VF progression in POAG, including high myopia, and the PPA(-BM) width may have a protective effect for VF progression in this subtype of POAG.

Serum Tenascin-C as a Novel Predictor for Risk of Coronary Artery Lesion and Resistance to Intravenous Immunoglobulin in Kawasaki Disease　- A Multicenter Retrospective Study.

BACKGROUND: Tenascin-C (TN-C) is an extracellular matrix glycoprotein that is heavily upregulated at sites of inflammation. We conducted a retrospective study to assess the utility of TN-C as a novel biomarker to predict the risk of developing coronary artery lesions (CAL) and resistance to intravenous immunoglobulin (IVIG) in patients with Kawasaki disease (KD).Methods and Results:We collected blood samples of 111 KD patients (IVIG-responder: 89, IVIG-resistant: 22; CAL: 8) and 23 healthy controls, and measured the serum levels of TN-C. TN-C levels on admission were significantly higher in patients than in healthy controls and in patients during convalescence after IVIG administration (69.6 vs. 20.4 vs. 39.7 ng/ml, respectively; P<0.001), and correlated positively with C-reactive protein (P<0.001), neutrophil (percentage; P=0.005), and ALT (P<0.001), and negatively with platelet count (P=0.023) and sodium level (P=0.025). On admission, TN-C levels in patients who later developed CAL were significantly higher than in those without CAL (P=0.010), and significantly higher in IVIG-resistant subjects than in IVIG-responders (P=0.003). The accuracy of TN-C testing for the prediction of IVIG resistance was comparable to that of the Kobayashi score. CONCLUSIONS: Serum TN-C could be a biomarker for predicting the risk of developing CAL and IVIG resistance during the acute phase of KD. (Circ J 2016; 80: 2376-2381).

Intra-cardiac echocardiography-guided stent implantation into stenosed superior vena cava in a patient with a history of contrast anaphylaxis.

A 37-year-old patient, who suffered from a repeated superior vena cava (SVC) syndrome, was scheduled for stent implantation into SVC, but suffered from contrast anaphylaxis. To monitor the procedure, we used intra-cardiac echocardiography and successfully implanted a stent. Placing an intra-cardiac echocardiographic catheter in the main pulmonary artery and facing towards the right, we could readily visualize stenosis in the SVC and inflation of the stent. Also looking up from right atrium, we noted proximal obstruction of the stent and confirmed the relief of obstruction after additional balloon dilation. This report leads to new application of intra-cardiac echocardiography for intervention of structural and vascular diseases other than inter-atrial septum.

Structural dissociation of optic disc margin components with optic disc tilting: a spectral domain optical coherence tomography study.

PURPOSE: To investigate the dissociation of the Bruch's membrane opening (BMO) from the scleral canal opening (SO) of the optic disc. METHODS: In this prospective, cross-sectional, observational study, 101 eyes from 101 patients or suspected subjects of primary open angle glaucoma were included. Enhanced depth imaging spectral domain optical coherence tomography images along the long axis of the optic disc were used to visualize better the deep structures around the optic disc on both the temporal and nasal sides. The distances between the BMO and SO were measured at the temporal and nasal sides of the optic disc, and their correlations with age, axial length, intraocular pressure, disc size, disc ovality index, disc torsion degree, and visual field mean deviation were investigated. RESULTS: The temporal and nasal distances of BMO from SO correlated significantly with each other (R = 0.632, P < 0.0001). By multiple linear regression analysis, significant correlations were found for disc ovality index (temporal: ß = -0.691, P < 0.0001; nasal: ß = -0.420, P < 0.0001) and axial length (temporal: ß = 0.224, P = 0.002; nasal: ß = 0.310, P = 0.001). The other factors did not show any significant correlation. CONCLUSION: Locations of the SO at not only the temporal, but also the nasal side of the optic disc are nasally shifted from the BMO with optic disc tilting and axial length elongation in glaucomatous eyes, and are significantly correlated to each other. The nasal shift of the deep structures of the optic disc should be considered especially when assessing myopic eyes with optic disc tilt.