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PURPOSE For survivors of childhood cancer treated with doxorubicin, dexrazoxane is cardioprotective for at least 5 years. However, longer-term data are lacking. METHODS Within the Children's Oncology Group and the Dana Farber Cancer Institute's Childhood Acute Lymphoblastic Leukemia Consortium, we evaluated four randomized trials of children with acute lymphoblastic leukemia or Hodgkin lymphoma, who received doxorubicin with or without dexrazoxane, and a nonrandomized trial of patients with osteosarcoma who all received doxorubicin with dexrazoxane. Cumulative doxorubicin doses ranged from 100 to 600 mg/m2 across these five trials, and dexrazoxane was administered uniformly (10:1 mg/m2 ratio) as an intravenous bolus before doxorubicin. Cardiac function was prospectively assessed in survivors from these trials, plus a matched group of survivors of osteosarcoma treated with doxorubicin without dexrazoxane. Two-dimensional echocardiograms and blood biomarkers were analyzed centrally in blinded fashion. Multivariate analyses adjusted for demographic characteristics, cumulative doxorubicin dose, and chest radiotherapy determined the differences and associations by dexrazoxane status. RESULTS From 49 participating institutions, 195 participants were assessed at 18.1 ± 2.7 years since cancer diagnosis (51% dexrazoxane-exposed; cumulative doxorubicin dose 297 ± 91 mg/m2). Dexrazoxane administration was associated with superior left ventricular fractional shortening (absolute difference, +1.4% [95% CI, 0.3 to 2.5]) and ejection fraction (absolute difference, +1.6% [95% CI, 0.0 to 3.2]), and lower myocardial stress per B-type natriuretic peptide (–6.7 pg/mL [95% CI, –10.6 to –2.8]). Dexrazoxane was associated with a reduced risk of having lower left ventricular function (fractional shortening < 30% or ejection fraction < 50%; odds ratio, 0.24 [95% CI, 0.07 to 0.81]). This protective association was primarily seen in those treated with cumulative doxorubicin doses ≥ 250 mg/m2. CONCLUSION Among young adult-aged survivors of childhood cancer, dexrazoxane was associated with a cardioprotective effect nearly 20 years after initial anthracycline exposure.

To describe the epidemiology of pericardial effusion in hospitalized children and evaluate risk factors associated with the drainage of pericardial effusion and hospital mortality.A retrospective study of a national pediatric discharge database.We analyzed hospitalized pediatric patients from the neonatal age through 20 years in the Kids' Inpatient Database 2016, extracting the cases of pericardial effusion. Of the 6 266 285 discharged patients recorded, 6417 (0.1%) were diagnosed with pericardial effusion, with the highest prevalence of 2153 patients in teens (13-20 years of age). Pericardial effusion was drained in 792 (12.3%), and the adjusted risk of pericardial drainage was statistically low with rheumatologic diagnosis (OR, 0.485; 95% CI, 0.358-0.657, P .001). The overall mortality in children with pericardial effusion was 6.8% and 10.9% of those who required pericardial effusion drainage (P .001). The adjusted risk of mortality was statistically high with solid organ tumor (OR, 1.538; 95% CI, 1.056-2.239, P = .025) and pericardial drainage (OR, 1.430; 95% CI, 1.067-1.915, P = .017) and low in all other age groups compared with neonates, those with cardiac structural diagnosis (OR, 0.322; 95% CI, 0.212-0.489, P .001), and those with rheumatologic diagnosis (OR, 0.531; 95% CI, 0.334-0.846, P = .008).The risk of mortality in hospitalized children with pericardial effusion was higher in younger children with solid organ tumors and those who required pericardial effusion drainage. In contrast, it was lower in older children with cardiac or rheumatologic diagnoses.

Background To investigate the association between the central sensitization inventory (CSI), a screening tool for central sensitization, and the number of painful sites and the severity of pain in locomotive organs in an epidemiological study in the elderly. Methods A total of 379 individuals who underwent musculoskeletal disease screening were enrolled in this study. The CSI was used to assess symptoms of central sensitization. The number and location of painful sites and the severity of pain were evaluated using pain mapping and a numerical rating scale (NRS) at 37 sites. We investigated the association between the number of painful sites and CSI score, and the association between the severity of low back pain or knee pain and CSI score. Results There was a positive correlation between CSI score and the number of painful sites. The CSI score was significantly higher in those with significant low back pain than in those without pain, and the high-CSI group tended to have a greater number of painful sites. Comparison of CSI scores between participants with low back pain alone and those with low back pain and posterior lower leg pain showed that the latter group had a significantly higher CSI score than the former group. The CSI score in participants with radiographic evidence of knee osteoarthritis was significantly higher in those with knee pain than in those without pain. Conclusions The results of this study suggest that participants with significant low back pain and a higher number of painful sites are more susceptible to the influence of central sensitization. In addition, CSI score was higher in participants with low back pain and posterior lower leg pain than in those with low back pain alone, suggesting that the spread of pain may be due to central sensitization.

The incidence of airway obstruction in patients with complex CHD other than vascular rings and absent pulmonary valve syndrome is unknown. We reviewed pre-operative CT and clinical data of children with conotruncal abnormalities to assess for airway obstruction. Airway obstruction was common (41% of patients), often moderate to severe, of diverse aetiology, and most commonly associated with a right aortic arch. Patients with airway obstruction showed a trend towards a higher mortality rate. Patients with complex conotruncal abnormalities should be assessed for airway obstruction as it may help predict the need for additional interventions and assist with prognostication.

Background Longitudinal clinical surveillance by transthoracic echocardiography (TTE) is an established practice in children with repaired tetralogy of Fallot (TOF). Non-Invasive Imaging Guidelines recommends a list of reporting elements that should be addressed during routine TTE in this population. In this study, we assessed the adherence to these recommendations. Methods This was a multi-center (n = 8) retrospective review of TTE reports in children ≤11 years of age who have had complete TOF repair. We included 10 patients from each participating center (n = 80) and scored 2 outpatient follow-up TTE reports on each patient. The adherence rate was based on completeness of TTE reporting elements derived from the guidelines. Results We reviewed 160 TTE reports on 80 patients. Median age was 4.4 months (IQR 1.5-6.6) and 3.6 years (IQR 1.3-6.4) at the time of complete surgical repair and first TTE report, respectively. The median adherence rate to recommended reporting elements was 61% (IQR 53–70). Of the 160 reports, 9 (7%) were ≥80% adherent and 40 (25%) were ≥70% adherent. Quantitative measurements of right ventricular outflow tract (RVOT), right ventricular (RV) size and function, and branch pulmonary arteries were least likely to be reported. Conclusions Overall adherence to the most recent published imaging guidelines for surveillance of children with repaired TOF patients was suboptimal, especially for reporting of RVOT, RV size and function, and branch pulmonary arteries. Further studies are needed to explore the barriers to adherence to guidelines and most importantly, whether adherence is associated with clinical outcomes.

Although exercise is beneficial for chronic musculoskeletal pain (CMP), the optimal type and amount of exercise are unclear. This study aimed to determine the impact of circuit training that combines aerobic and resistance exercises on adult women with CMP. A total of 139 women with CMP underwent circuit training for 3 months and were asked to complete the following questionnaires at baseline and 3 months later: Numeric Rating Scale (NRS), Pain Catastrophizing Scale (PCS), Roland-Morris Disability Questionnaire (RDQ), Shoulder36, and Knee injury and Osteoarthritis Outcome Score (KOOS). Significant improvements were observed in NRS, PCS, RDQ, and KOOS activities of daily living (ADL) scores after the intervention relative to baseline (p

Graphical abstract, Highlights • The authors report successful palliation of APVS in DORV and right-dominant AVCD. • Fetal echocardiography assists in the prenatal diagnosis. • Prenatal cardiac MRI can measure lung volumes to assess bronchial compression.

Yoshinori Satake,1 Masashi Izumi,1 Koji Aso,1 Yoichi Igarashi,2 Nao Sasaki,3 Masahiko Ikeuchi1 1Department of Orthopaedic Surgery, Kochi Medical School, Kochi University, Kochi, Japan; 2Department of Orthopaedic Surgery, Tosa Municipal Hospital, Kochi, Japan; 3Medicines Development Unit Japan, Eli Lilly Japan K.K., Hyogo, JapanCorrespondence: Yoshinori SatakeDepartment of Orthopaedic Surgery, Kochi Medical School, Kochi University, Kohasu, Oko-cho, Nankoku-shi, Kochi 783-8505, JapanTel +81 88 880 2387Fax +81 88 880 2388Email y-satake@kochi-u.ac.jpPurpose: Patients with knee osteoarthritis (OA) complain of various types of pain, divided into two main categories: pain on movement and pain at rest. A thorough understanding of pain is essential for managing knee OA; however, few studies have investigated the mechanisms underlying the two different types of pain. This study aimed to clarify the predisposing factors for pain in patients with knee OA with a focus on differences between pain on walking and pain at rest.Patients and Methods: This study involved 93 patients, aged 44– 90 years, with knee OA, including 74 women. We assessed demographic variables (sex, age, body mass index [BMI], side), visual analogue scale (VAS) score on walking, VAS score at rest, Kellgren and Lawrence (KL) grade on radiograph, synovitis score and bone marrow lesion (BML) score on magnetic resonance imaging, and pressure pain threshold (PPT), and used univariate and multiple regression analyses to investigate factors predisposing patients to pain at rest or pain on walking.Results: In the univariate analyses, we found significant correlations between VAS score on walking and BMI (r=0.31, p< 0.01), KL grade (r=0.40, p< 0.01), synovitis score (r=0.26, p=0.01), and BML score (r=0.36, p< 0.01), whereas VAS score at rest correlated with PPT (r=− 0.23, p=0.02) and BMI (r= 0.26, p=0.01). Multiple regression analysis showed that significant explanatory factors for VAS score on walking were BMI (β=0.22, p=0.03) and KL grade (β=0.27, p=0.03). By contrast, PPT was the only significant explanatory factor for VAS score at rest (β=− 0.27, p=0.01).Conclusion: Predisposing factors were significantly different between pain on walking and pain at rest, indicating that different pain mechanisms exist in the two types of pain. Pain on walking was more strongly associated with mechanical and structural factors, while pain at rest was associated with mechanical hyperalgesia of the knee.Clinical Registration: University Hospital Medical Information Network Clinical Trials Registration number; 000041190.Keywords: knee osteoarthritis, pain at rest, hyperalgesia

A previously conducted placebo-controlled, randomized, phase 3 study of 353 Japanese patients with knee osteoarthritis (OA) showed significant improvements for duloxetine vs placebo in pain and health-related quality of life (HRQoL) (ClinicalTrials.gov Identifier: NCT02248480). Reported here are post hoc subgroup analyses evaluating the efficacy of duloxetine according to the pattern of prior nonsteroidal anti-inflammatory drug (NSAID) use.Patients with knee OA pain received once-daily duloxetine or placebo for 14 weeks. Pain was evaluated using the Brief Pain Inventory (BPI) and HRQoL was evaluated using the Western Ontario McMaster Universities Osteoarthritis Index (WOMAC). Patients were divided into four subgroups based on their prior NSAID use: (i) no prior NSAID use; (ii) low-frequency NSAID use (14 days/month); (iii) high-frequency transdermal NSAID use (transdermal NSAIDs only; ≥14 days/month for the 3 months before study entry); and (iv) high-frequency other NSAID use (eg, oral NSAIDs only, both oral and transdermal NSAIDs; ≥14 days/month for the 3 months before study entry).In each of the four prior NSAID use subgroups, there were greater reductions in BPI average pain severity score for duloxetine vs placebo at all timepoints during the 14-week treatment period; the treatment*prior NSAID use interaction was not statistically significant. In each subgroup, the proportion of patients achieving a ≥50% reduction in BPI average pain severity score was higher for duloxetine vs placebo. In each subgroup, there were greater reductions in WOMAC total score for duloxetine vs placebo at all timepoints; the treatment*prior NSAID use interaction was not statistically significant. In each subgroup, there were greater reductions at Week 14 in WOMAC pain, stiffness, physical function, and total scores for duloxetine vs placebo.Duloxetine was consistently effective with respect to pain relief and HRQoL in Japanese patients with knee OA pain, regardless of the pattern of prior NSAID use.

Kaname Ueda,1 Nao Sasaki,2 Amir Goren,3 Shawna R Calhoun,4 Katsuhiro Shinjo,5 Hiroyuki Enomoto,5 Takeshi Muneta6 1Health Outcomes/Health Technology Assessment/Real World Evidence, Eli Lilly Japan, Kobe, Japan; 2Bio-Medicines, Medicines Development Unit, Eli Lilly Japan, Kobe, Japan; 3Health Outcomes, Kantar Health, New York, NY, USA; 4Health Outcomes, Kantar Health, Horsham, PA, USA; 5Bio-Medicines, Medicines Development Unit, Eli Lilly Japan, Tokyo, Japan; 6Department of Orthopaedic Surgery, National Disaster Medical Center, Tokyo, Japan Purpose: Patient satisfaction is an important outcome in successful osteoarthritis (OA) treatment. The aim of this study was to evaluate treatment satisfaction for medication (TSM) in people with knee OA (KOA), identify the factors predictive of treatment satisfaction, and describe the burden of illness.Patients and methods: This cross-sectional, patient-reported study used an Internet-based survey and analyzed responses of respondents with KOA (N=400) on characteristics including pain sites and levels (including pain ratings using the Numerical Rating Scale and Short-Form McGill Pain Questionnaire), treatment satisfaction (Global, Effectiveness, and Convenience scores) based on the Treatment Satisfaction Questionnaire for Medication (TSQM-9), and quality of life (QoL; based on the Arthritis Impact Measurement Scale 2-Short Form). Respondents with only KOA (n=237) were compared with those having KOA and additional painful sites (KOA+; n=163). Factors predicting TSM were identified using multivariable linear regression analyses.Results: Respondents with KOA were more likely to report intermittent pain for 3 months or more compared with those with KOA+ (58.6% vs 48.5%, respectively; P=0.044), while those with KOA+ were more likely to report consistent pain for 3 months or more (P=0.022). Respondents with KOA+ also had more difficulty due to their knee pain while sleeping (P=0.022) and resting (P=0.015). Reported TSM did not differ significantly across KOA vs KOA+ groups, with both groups reporting low satisfaction; all domains of QoL were worse for those with KOA+. Knee pain reduction by medication predicted higher satisfaction across domains, while lower pre-medication pain and post-medication pain matching expectations predicted higher TSQM-9 Global and Effectiveness scores.Conclusion: Medication treatment satisfaction rates were low among Japanese respondents with KOA. Given that lower pain, greater pain reduction post-medication, and meeting pain management expectations were predictive of higher satisfaction, treatment strategies that can better address pain may prove beneficial for overall patient satisfaction. Keywords: quality of life, AIMS2-SF, SF-MPQ, TSQM-9, knee osteoarthritis

Background Surgical repair for neonatal arch hypoplasia with coarctation can be performed by a sternotomy or thoracotomy. Objectives This study retrospectively investigates the use of pre-surgical absolute aortic arch measurements in guiding the optimal approach for neonates with coarctation and aortic arch hypoplasia. Methods Patients ≤ 30 days old who underwent coarctation repair via a median sternotomy or lateral thoracotomy between January 2000 and July 2018 were retrospectively reviewed. Neonates requiring intracardiac surgery were excluded. Specific aortic arch measurements were obtained and 3 ratios calculated: distal transverse arch diameter/carotid artery diameter (DT/CA), distal transverse arch diameter/distance from the carotid to subclavian artery (CSA) and isthmus diameter/descending aorta diameter (I/D). Results Of 118 patients, 65 had a lateral thoracotomy and 53 had a median sternotomy. Distal transverse arch, the carotid artery and isthmus diameters correlated with surgical approach. The DT/CA ratio was statistically significant with an ROC curve (p Conclusion In neonates with coarctation, pre-surgical aortic arch measurements and ratios can be utilized to identify aortic arch hypoplasia and guide surgical approach. In our institution, neonates with a DT/CA ratio of 0.9 or less routinely underwent arch reconstruction by a median sternotomy. Re-intervention rates in the median sternotomy and lateral thoracotomy groups were comparable.

PURPOSE OF REVIEW Left-to-right shunts are commonly seen in congenital heart diseases. This review aims to review the anatomy, physiology, and imaging of the four most common left-to-right lesions in adults. RECENT FINDINGS Reported late complications of left-to-right lesions provide guidance in follow-up imaging. Use of three-dimensional echocardiography helps not only in the diagnosis of these defects but also in assistance during device closure. SUMMARY Noninvasive imaging plays a significant role in the diagnosis and management of left-to-right lesions.

OBJECTIVES: To longitudinally measure LV diastolic function in HIV-exposed but uninfected (HEU) children perinatally-exposed to ART. DESIGN: HEU children who were perinatally-exposed to antiretroviral therapy (ART) may be at risk for adverse cardiac effects. We have previously reported that those children have decreased left ventricular (LV) mass, dimension, and septal thickness with increased contractility. METHODS: Serial echocardiograms were obtained at specific times from birth to 48 months from two groups of HIV-uninfected children: 148 HIV-negative children who were perinatally-exposed to ART and 130 non-ART-exposed HIV-unexposed healthy controls. The following LV diastolic indices were obtained: mitral valve early and late diastolic velocity (E and A), tissue Doppler derived LV free wall and septal early diastolic velocity (LVe’ and sep e’). RESULTS: All echocardiographic indices were significantly different in ART-exposed children compared to ART-unexposed healthy controls. Both E and A were overall lower at all ages by 8.28 cm/sec (P = 0.0002) and 13.46 cm/sec (P < 0.0001) respectively. E/A ratio was higher by 0.27, 0.46, and 0.28 units at birth, 1 year and 2 years of age, respectively (all P ≤ 0.01). Moreover, LVe’ and sep e’ were overall lower at all ages by 0.84 cm/sec (P = 0.01) and 0.47cm/sec (P = 0.02) respectively. CONCLUSIONS: Children who were exposed to ART in utero have subclinical yet significant differences in specific LV diastolic indices. Follow-up with serial echocardiograms is recommended in this population to further assess the potential cardiac toxicity of perinatal exposure to ART.

<>は清代初期成書の全100 回に及ぶ長編章回小説である。作者不詳であるものの、使用する言語の基礎方言から、山東省の人だと判る。我々はこれに訳注をほどこし、今回、新たに[比較]等の項を設け研究成果も取り込む。現在熊本大学近代漢語研究班として「清代北方官話区の代表的作品」の全出現語彙を調査研究しているが、その一環として<>に取り組んでいる。即ち、最終的には「どの語彙が、どのように(意味)、どれだけ(数量)使用されているか」を明らかにする。そして出現回数を数字で表す言語学的計量研究へと結びつけたい。 部分的注釈は、胡適「<>考証」(1933)、黄粛秋(1981)等が役立つ。最近では、晁瑞『<>方言詞歴史演変研究』(2014)、植田均『<>方言語彙辞典』(2016)がある。なお、邦訳は左並旗男『醒世姻縁傳』(兄弟舎、2002)がある。, Xingshi Yinyuan Zhuan is a full-length novel with 100 chapters, written in Shangdong dialect. Its writer is from Shangdong Province but his name and life story still remain unknown. We annotate the words/ phrases the novel and include `comparison' this time. Modern Chinese Research Class students from Kumamoto University are investigating all the words/ phrases occurring in the representative works produced in northern mandarin area in Qing Dynasty and currently are working on all the words/ phrases from Xingshi Yinyuan Zhuan. That is to say, the essay aims to expound `the rate of a certain word/ phrase corresponding to its meaning', and researches on the quantitative linguistics with figures counting frequencies. Textual Research of Xingshi Yinyuan Zhuan from Hu Shi (1993) and Huang Suqiu (1981) contribute greatly to the annotation. Recently the Historical Evolution of the Dialect of Xingshi Yinyuan Zhuan (Chao Rui, 2014) and Dialect Vocabulary Dictionary of Xingshi Yinyuan Zhuan (Hitoshi Ueda, 2016) have been published one by one.

Xingshi Yinyuan Zhuan is a full-length novel with 100 chapters, written in Shangdong dialect. Its writer is from Shangdong Province but his name and life story still remain unknown. We annotate the words/phrases the novel and include ‘comparison’ this time. Modern Chinese Research Class students from Kumamoto University are investigating all the words/phrases occurring in the representative works produced in northern mandarin area in Qing Dynasty and currently are working on all the words/phrases from Xingshi Yinyuan Zhuan. That is to say, the essay aims to expound ‘the rate of a certain word/phrase corresponding to its meaning’, and researches on the quantitative linguistics with figures counting frequencies. Textual Research of Xingshi Yinyuan Zhuan from Hu Shi(1993) and Huang Suqiu (1981) contribute greatly to the annotation. Recently the Historical Evolution of the Dialect of Xingshi Yinyuan Zhuan (Chao Rui, 2014) and Dialect Vocabulary Dictionary of Xingshi Yinyuan Zhuan (Hitoshi Ueda, 2016) have been published one by one.

Objective Determine the accuracy of echocardiography to diagnose coronary anatomy in transposition of the great arteries and to evaluate the effect of accuracy on surgical outcomes and changes in accuracy over time. Design Retrospective chart review of neonates admitted February 1999 to March 2013 with transposition. Coronary pattern from the preoperative echocardiogram and operative reports were collected and compared with determine diagnostic accuracy. Coronary patterns were further confirmed by intraoperative images taken during surgery. Setting Tertiary care children's hospital. Patients Neonates with transposition of the great arteries and planned arterial switch operation with an echo and operative report or image describing the coronaries. Interventions Not applicable. Outcome Measures Accuracy of echocardiography to diagnose coronary anatomy in transposition, and to identify factors related to correct diagnosis. Results One hundred forty-two patients met inclusion criteria with 122 correctly diagnosed, 16 incorrect, and 4 inconclusive. Accuracy was 86%, with 95% accuracy in patients with typical coronary patterns, 85% with the most common variant (left coronary from the leftward sinus and right and circumflex from the rightward sinus), and 61% with less common patterns. Typical and common variants were more likely to be correct than atypical patterns (P .05). There was no difference in duration of cardiopulmonary bypass, cross-clamp times, length of stay, or postoperative stay between the correct and incorrectly diagnosed groups (P > .05). Conclusions In our center, accuracy of echocardiographic imaging of the coronary arteries in transposition was 86% without improvement over time, and perioperative outcomes were not affected by diagnostic accuracy. Further invasive imaging may not be necessary to determine the coronary pattern in this lesion.

10513 Background: Dexrazoxane (DRZ) has cardioprotective effects among doxorubicin (DOX) treated childhood cancer survivors up to 5 years after therapy. However, longer-term data are lacking. Methods: P9404, P9425, P9426, and DFCI 95-01 were randomized trials of acute lymphoblastic leukemia and Hodgkin lymphoma, where patients were randomly assigned to DOX±DRZ. P9754 enrolled osteosarcoma patients who all received DOX+DRZ. In all studies, DRZ was given as an intravenous bolus before DOX (10:1mg ratio). DOX doses ranged from 100-600 mg/m2 across these 5 trials. A subset of COG institutions prospectively assessed cardiac function in long-term survivors from these trials, plus a matched group of osteosarcoma survivors treated with DOX alone. Echocardiograms (left ventricular [LV] Biplane ejection fraction [EF], shortening fraction [SF]) and blood biomarkers (b-type natriuretic peptides [BNP], N-terminal [NT] proBNP) were all analyzed centrally, with DRZ status masked. Lower LV function was defined as EF2) 17.6±2.4y since cancer diagnosis, DRZ+ participants were slightly younger (27.8 vs 29.6y, p=0.02), but baseline characteristics otherwise did not differ significantly by DRZ status. DRZ status was associated with higher FS (34.7±3.6% vs 33.4±4.3%, p=0.04) and EF (63.4±5.4% vs 61.4±5.5%, p=0.01), and lower BNP (median 10.4 pg/mL [IQR 6.0-18.0] vs 13.0 [IQR 6.0-28.2], p=0.03) and NT-proBNP (median 30.8 pg/mL [IQR 18.9-58.2] vs 47.1 [IQR 23.0-83.1], p2 (vs 2). Results from multivariate models were similar: DRZ was associated with higher SF (1.4% [95% CI 0.2, 2.6]) and EF (2.7% [95% CI 0.8, 4.6]), and reduced BNP (-4.0 pg/mL [95% CI -7.6, -0.4]) and NT-proBNP (-20.7 pg/mL [95% CI -33.5, -7.9]). Overall, DRZ was associated with a reduced risk of having lower LV function (odds ratio 0.27 [95% CI 0.08-0.96]). Conclusions: After >17y, childhood cancer survivors treated with DOX+DRZ had better LV systolic function and less myocardial wall stress compared with those treated with DOX alone. DRZ may preferentially benefit females and those treated with greater DOX doses.

BackgroundEnlargement of the left atrium is a non-invasive marker of diastolic dysfunction of the left ventricle, a determinant of prognosis in children with cardiomyopathy. Similarly, N-terminal prohormone brain natriuretic peptide is a useful marker in the management of children with cardiomyopathy and heart failure. The aim of this study is to evaluate the association of left atrial pressures with left atrial volume and N-terminal prohormone brain natriuretic peptide in children with cardiomyopathy.MethodsThis was a retrospective study reviewing the medical records of patients ResultsThere was a linear correlation of left atrial pressure estimated in the cardiac catheterisation with indexed left atrial volume (r=0.63; pConclusionsLeft atrial volume measured non-invasively by echocardiography can be used as a surrogate for left atrial pressure in assessing diastolic dysfunction of the left ventricle in children with cardiomyopathy. The larger the size of the left atrium, worse is the diastolic function of the left ventricle.

Hiroyuki Enomoto,1 Shinji Fujikoshi,2 Jumpei Funai,3 Nao Sasaki,4 Michael H Ossipov,5 Toshinaga Tsuji,6 Levent Alev,7 Takahiro Ushida8 1Medical Science, Eli Lilly Japan K.K., Tokyo, 2Statistical Science, 3Science Communications, 4Medical Science, Eli Lilly Japan K.K., Kobe, Japan; 5Clinical Division, inVentiv Health, LLC, Blue Bell, PA, USA; 6Medical Affairs Department, Shionogi & Co., Ltd., Osaka, Japan; 7Medical Department, Lilly Turkey, Istanbul, Turkey; 8Multidisciplinary Pain Center, Aichi Medical University, Nagakute, Aichi, Japan Background: Comorbid depression and depressive symptoms are common in patients with chronic low back pain (CLBP). Duloxetine is clinically effective in major depressive disorder and several chronic pain states, including CLBP. The objective of this post hoc meta-analysis was to assess direct and indirect analgesic efficacy of duloxetine for patients with CLBP in previous clinical trials. Methods: Post hoc path analyses were conducted of 3 randomized, double-blind, clinical studies of patients receiving duloxetine or placebo for CLBP. The primary outcome measure for pain was the Brief Pain Inventory, average pain score. A secondary outcome measure, the Beck Depression Inventory-II, was used for depressive symptoms. The changes in score from baseline to endpoint were determined for each index. Path analyses were employed to calculate the proportion of analgesia that may be attributed to a direct effect of duloxetine on pain.Results: A total of 851 patients (400 duloxetine and 451 placebo) were included in this analysis. Duloxetine significantly improved pain scores compared with placebo (p

Background. Pediatric patients with chronic kidney disease (CKD) are at increased risk of early cardiovascular disease and premature death. Abnormalities in microvascular structure and function may presage end-organ damage including vascular calcification and myocardial ischemia associated with disordered mineral metabolism. Early detection of microvascular rarefaction (reduced density of capillaries) may identify at-risk patients and prompt timely therapeutic interventions. Our objective was to study capillary rarefaction in pediatric hemodialysis (HD) patients and to determine possible associations with mineral metabolism and cardiac risk biomarkers. Methods. Capillary density (CD) was measured by nailfold capillaroscopy in 19 pediatric HD patients and 20 healthy controls. Demographic and biochemical markers were collected at entry and 6-month follow-up. Results. CD was significantly decreased in HD patients compared with controls with a deficit of 24 and 31% at baseline and subsequent follow-up. Maximal CD correlated significantly with intact parathyroid hormone (iPTH) (r= −0.45; P = 0.005), serum calcium (r= −0.38; P = 0.02) and 25(OH) vitamin D levels (r= +0.36; P = 0.03) in HD patients. Capillary functional measures were similar to controls. By multivariate analysis, the primary negative determinants of CD were African American race and hyperparathyroidism; whereas, glomerular disease had a positive influence on capillary rarefaction (R 2 = 64.2% variance; P = 0.001). Conclusion. Pediatric HD patients demonstrate a ‘structural deficit’ in CD but show preserved ‘functional integrity’. Capillary rarefaction, an early risk factor of incipient vascular calcification, was strongly associated with biomarkers of altered mineral metabolism. Further studies are warranted to determine the impact of optimizing blood pressure and metabolic control on changes in capillary rarefaction in young CKD patients.

Background: This post hoc analysis of a Japanese phase 3 randomized study (ClinicalTrials.gov identifier: NCT01855919) investigated relationships between pain severity (assessed by the Brief Pain Inventory [BPI]) and disease-specific health-related quality of life (assessed by the 24-item Roland-Morris Disability Questionnaire [RDQ-24]) in duloxetine-treated patients with chronic low back pain (CLBP). Methods: Patients with CLBP duration >6 months and BPI average score ≥4 received duloxetine 60 mg/d (N = 230) or placebo (N = 226) for 14 weeks. Spearman rank correlation coefficients were calculated for (1) BPI change from baseline and RDQ item change from baseline and (2) BPI change from baseline and the RDQ item baseline score in duloxetine-treated patients. Results: Duloxetine treatment significantly improved the RDQ-24 total score compared with placebo; the greatest improvements were observed for RDQ02, RDQ17, and RDQ13. The strongest correlations between BPI change from baseline and RDQ item change from baseline were for RDQ13, RDQ23, and RDQ10. The correlation coefficients for the correlations between BPI change from baseline and the RDQ item baseline score were generally small. Discussion: This post hoc analysis suggested that improvement in pain severity was associated with improvement in the RDQ-24 total score and in some individual RDQ items in duloxetine-treated patients with CLBP. Furthermore, positive responses to duloxetine in terms of the RDQ13, RDQ23, and RDQ10 items may correlate with better pain responses. Clinical Trial Registry: The study described in this manuscript was registered at www.clinicaltrials.gov (NCT01855919).

Airway obstruction (AO) has been well described in patients with vascular rings and absent pulmonary valve syndrome, however its incidence in patients with other forms of complex congenital heart disease is unknown. We sought to assess the incidence of AO in patients with complex vascular anatomy in

Background. Children undergoing chronic hemodialysis are at risk of cardiovascular disease and often develop left ventricular hypertrophy (LVH). Twenty-four-hour ambulatory blood pressure monitoring (ABPM) is known to better predict cardiovascular morbidity than casual blood pressure (BP) measurement. Given the BP variability attributed to interdialytic fluid overload, 44-h ABPM should better delineate cardiovascular morbidity in pediatric hemodialysis patients. Methods. In this cross-sectional study, 17 children (16.7 ± 2.9 years) on chronic hemodialysis underwent 44-h interdialytic ABPM and routine echocardiogram. Left ventricular mass index (LVMI) was calculated by height-based equation; LVH was defined as an LVMI in the ≥95th percentile for height-age and gender. Hypertension was defined by the recommendations of the Fourth Report of the National High Blood Pressure Education Program for casual measurements, and by those of the American Heart Association for ABPM. Results. Twenty-four percentage of patients were hypertensive by casual post-dialytic systolic BP, whereas 59% were hypertensive by ABPM. Eighty-eight percentage of patients had abnormal cardiac geometry: 53% had LVH. Thirty-five percentage (6 of 17) had masked hypertension, including four with abnormal cardiac geometry, of which, three had LVH. LVMI correlated with ABPM, but not with casual measurements. Strongest correlations with an increased LVMI were with 44-h diastolic BP: at night (r= 0.53, P = 0.03) and total load (r= 0.57, P = 0.02). LVH was similarly associated with 44-h nighttime BP: systolic (P = 0.02), diastolic (P = 0.01) and mean arterial (P = 0.01). Conclusions. Casual BP measurement underestimates hypertension in pediatric hemodialysis patients and does not correlate well with indicators of cardiovascular morbidity. In contrast, 44-h interdialytic ABPM better characterizes hypertension, with nighttime parameters most strongly predicting increased LVMI and LVH.

Determine the accuracy of echocardiography to diagnose coronary anatomy in transposition of the great arteries and to evaluate the effect of accuracy on surgical outcomes and changes in accuracy over time.Retrospective chart review of neonates admitted February 1999 to March 2013 with transposition. Coronary pattern from the preoperative echocardiogram and operative reports were collected and compared with determine diagnostic accuracy. Coronary patterns were further confirmed by intraoperative images taken during surgery.Tertiary care children's hospital.Neonates with transposition of the great arteries and planned arterial switch operation with an echo and operative report or image describing the coronaries.Not applicable.Accuracy of echocardiography to diagnose coronary anatomy in transposition, and to identify factors related to correct diagnosis.One hundred forty-two patients met inclusion criteria with 122 correctly diagnosed, 16 incorrect, and 4 inconclusive. Accuracy was 86%, with 95% accuracy in patients with typical coronary patterns, 85% with the most common variant (left coronary from the leftward sinus and right and circumflex from the rightward sinus), and 61% with less common patterns. Typical and common variants were more likely to be correct than atypical patterns (P .001). Cases with ventricular septal defect were more likely to have correctly diagnosed coronaries than with an intact ventricular septum (94% vs. 79%, P = .01). There was no change in accuracy over time (P .05). There was no difference in duration of cardiopulmonary bypass, cross-clamp times, length of stay, or postoperative stay between the correct and incorrectly diagnosed groups (P .05).In our center, accuracy of echocardiographic imaging of the coronary arteries in transposition was 86% without improvement over time, and perioperative outcomes were not affected by diagnostic accuracy. Further invasive imaging may not be necessary to determine the coronary pattern in this lesion.

Restrictive cardiomyopathy (RCM) is characterized by irreversible diastolic dysfunction with preserved systolic function. The aim of this study was to investigate the presence of impaired ventricular contractility even in the presence of normal ejection fraction (EF) in children with RCM. Longitudinal Doppler tissue velocities were obtained from apical 4-chamber view at three locations—the left-ventricular (LV) lateral wall, the septum, and the right ventricle—in 8 children age 3–17 years old with RCM who had LV EF >55%. Peak systolic velocity (S′), acceleration during isovolumic contraction (IVA), and myocardial performance index (MPI) were measured. Data from the RCM group were compared with those from 24 age- and sex-matched controls. Both S′ and IVA were markedly lower at the septum (S′ 6.2 ± 1.7 vs. 9.2 ± 1.6, P < 0.001; IVA 1.8 ± 0.5 vs. 3.9 ± 1.5, P < 0.001). MPI, a measure of both diastolic and systolic function, was statistically significantly greater in the RCM group at all 3 locations (P < 0.005). S′ and IVA identify global subclinical systolic dysfunction in RCM with normal EF. These findings suggest that pre-ejection abnormality and subclinical systolic dysfunction coexist with diastolic dysfunction in children with RCM.

遠方視力検査は5mとされているが、1.1mの距離で検査を行えるスペースセイビングチャート®(SSC-330 Type II、ニデック社、以下SSC)の有用性を検討したので報告する。症例は小児22例、成人105例。小児は5～8歳、9～12歳の症例に分けて検討した。成人は屈折異常のみ、偽水晶体眼、白内障の症例の測定を行った。さらに白内障では、核白内障、皮質白内障、後嚢下白内障に分けて、それぞれ裸眼視力、矯正視力、等価球面値の差について比較検討した。その結果、各症例ともSSCと5m視力表における裸眼視力、矯正視力はよく一致しており、等価球面値の差も0.1D以下で調節介入は見られなかった。使用して感じた利点は1.省スペースとして有用、2.他人に視標が見えないためプライバシーの保護ができる点であった。一方、欠点は1.視標の数が0.1以下で少なく低視力者に使いにくい、2.正面でしか視標が見えないので視野が狭いと視標が見つけにくいのではないか、という点であった。

A 3-year-old girl with Alport syndrome presented with decompensated heart failure from hypertension-induced cardiomyopathy 6 months following renal biopsy. Selective renal angiography revealed a large left renal arteriovenous fistula (AVF) with poor perfusion to the left renal parenchyma. The AVF was treated by transcatheter embolization using an Amplatzer vascular plug. Her blood pressure normalized after embolization, and her cardiac function normalized over the following 4 months.

Purpose Carvedilol and biomarkers do not have established roles in pediatric HF. We reviewed our 14-year experience to assess the role of N-terminal pro Natriuretic peptide (NTproBNP) and use of carvedilol in children with dilated cardiomyopathy (DCM). Methods and Materials We reviewed dosage of medications, echocardiogram measurements of ejection fraction (EF), fractional shortening corrected by age (FSz), left ventricular end diastolic diameter corrected by BSA (LVEDDz) and NTproBNP to assess outcome and response to treatment. Results There were 106 children with DCM (58 male). Mean age at presentation was 7.4±6.5 years. Mean EF and FSz at presentation were 28.9±10 and −8.1±6.7. Carvedilol and ACE inhibitors were used in combination in 72% of patients. Digoxin was used in symptomatic patients. Average dose (mg/kg/day) of carvedilol was 0.95 (range 0.05-1.7), enalapril 0.64 (0.08-1.2) and captopril 3.4 (0.45-6.4). Average follow up was 6.2±5.5 years. Death/transplant (DT) occurred in 45% of children; 40% of those occurred within one year of presentation. In the survivors, EF and FSz improved from 29.3±9.6 to 53.8±9 (p Conclusions Mortality and transplant remain common outcomes for children with DCM, particularly in the first year following presentation. Carvedilol and ACE inhibitors are safely tolerated with long-term use, and at higher doses than previously reported. Hypoglycemia may be a serious side effect that can be avoided by taking carvedilol with food. Persistent NTproBNP≥1000 may indicate need for transplantation.

Medical treatment in pediatric patient with dilated cardiomyopathy (DCM) is not as well standardized as in the adult population in relation to medications and dosages; use of carvedilol remains controversial. We reviewed our experience over the past 14 years in treating children with DCM. We