Search

Background: We validated the prevalence of albuminuria and its association with kidney function and hemodynamics in pre-school children who underwent surgery for congenital heart disease (CHD). Methods: From 403 patients who had undergone surgery for CHD at least 6 months before pre-school and were admitted to our hospital between 2011 and 2015, 75 who underwent blood and urine tests and cardiac catheterization were included in this study. The urinary albumin-to-creatinine ratio (ACR) was quantified, and the relationship of ACR with physical and laboratory findings and hemodynamics assessed using cardiac catheterization was analyzed. Results: The study cohort was divided into three groups: Fontan group (n = 25), tetralogy of Fallot (TOF) group (n = 18), and control group (other biventricular CHDs; n = 32). The median age of patients was 5.9 years. ACR was higher in the Fontan group than in the TOF and control groups (median: 15.0 vs. 5.0 and 0.0 mg/g, p < 0.001). Moreover, albuminuria (ACR > 30 mg/g) was observed in 20.0% of Fontan patients, while ACR was associated with potential complicating factors of Fontan circulation: high central venous pressure, high mean pulmonary artery pressure, and worse than moderate atrioventricular regurgitation. ACR showed a moderate correlation with the cystatin C-based estimated glomerular filtration rate (r = − 0.725, p < 0.001). Conclusions: Measurement of albuminuria in Fontan patients before they join elementary school is useful because it reflects kidney function and hemodynamic factors that can worsen their condition. Identification and management of patients with albuminuria may facilitate early therapeutic intervention for worsening Fontan factors, eventually delaying the deterioration of kidney function. [ABSTRACT FROM AUTHOR]

Although the number of pediatric patients with long-term survival following cardiac surgery is increasing, concerns regarding chronic kidney disease (CKD) after surgery are growing. We examined the frequency of and risk factors for pediatric CKD development in patients with congenital heart disease (CHD) at least 2 years after cardiac surgery. This was a cross-sectional study of 147 patients who underwent open-heart surgery for CHD at Kagoshima University Hospital from April 2010 to March 2017. Data on demographics, acute kidney injury after cardiac surgery, cyanotic heart disease, Fontan circulation, medications in the perioperative period, and Risk Adjustment for Congenital Heart Surgery-1 (RACHS-1) category were recorded. CKD was defined using the current classification system described in the National Kidney Foundation's Kidney Disease Outcomes Quality Initiative and assessed during early childhood within 2–3 years of cardiac surgery. Statistical analyses were performed using SPSS Statistics for Windows version 25.0. We consecutively enrolled 147 patients, of whom 22 (15.0%) had CKD, all with stage-2 severity. Among patients with CKD, a higher proportion underwent Fontan surgery (P < 0.001), a higher proportion had cyanotic heart disease (P = 0.009), and the RACHS-1 category was high (P = 0.003). Patients with CKD appeared more frequently than patients without CKD in RACHS-1 categories 3, 5, and 6. It is essential to evaluate renal function longitudinally and monitor for CKD, given that patients who underwent Fontan surgery or complicated surgery in infancy have a high rate of developing postoperative CKD in early childhood. [ABSTRACT FROM AUTHOR]

The article presents a case study of a neonate with a rare condition of neonatal complicated right ventricular diverticulum (RVD) with effective contraction. It is reported that the baby, prenatally diagnosed with double chamber right ventricle and pulmonary valve stenosis, exhibited cyanosis at birth. It is further reported that echocardiography revealed an abnormal muscle bundle dividing the right ventricle, diagnosed as RVD.

The article presents case study of patient was a 14-day-old female neonate with no specific abnormalities, who had growth restriction during her fetal period. It mentions diagnosis with neonatal cerebral sinovenous thrombosis (CSVT) because of its lower risk of bleeding compared with unfractionated heparin.

Transitive inference (TI) is the ability to infer unknown relationships from previous information. To test TI in non-human animals, transitive responding has been examined in a TI task where non-adjacent pairs were presented after premise pair training. Some mammals, birds and paper wasps can pass TI tasks. Although previous studies showed that some fish are capable of TI in the social context, it remains unclear whether fish can pass TI task. Here, we conducted a TI task in cleaner wrasses (Labroides dimidiatus), which interact with various client fishes and conspecifics. Because they make decisions based on previous direct and indirect interactions in the context of cleaning interactions, we predicted that the ability of TI is beneficial for cleaner fish. Four tested fish were trained with four pairs of visual stimuli in a 5-term series: A-B+, B-C+, C-D+, and D-E+ (plus and minus denote rewards and non-rewards, respectively). After training, a novel pair, BD (BD test), was presented wherein the fish chose D more frequently than B. In contrast, reinforcement history did not predict the choice D. Our results suggest that cleaner fish passed the TI task, similar to mammals and birds. Although the mechanism underlying transitive responding in cleaner fish remains unclear, this work contributes to understanding cognitive abilities in fish. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 23-year-old woman with no significant medical history was diagnosed with Wolff–Parkinson–White (WPW) syndrome during pregnancy and her fetus was diagnosed with tetralogy of Fallot (TOF). Topics include the electrocardiogram has showed right axis deviation and short PR interval with a narrow QRS complex, the transthoracic echocardiogram has showed a ventricular septal defect, and the medications were not required during the neonatal period.

Background: Acute kidney injury (AKI) after cardiac surgery (CS-AKI) in children with congenital heart disease is a serious complication closely associated with high morbidity and mortality. Kidney Disease: Improving Global Outcomes (KDIGO) AKI staging demonstrates high sensitivity for detecting AKI and predicting associated in-hospital mortality. However, neonatal-modified KDIGO criteria (n-KDIGO), recently introduced as a standard diagnostic tool, for CS-AKI have not been fully validated. Here, we evaluated the incidence of risk factors and postoperative outcomes of neonatal CS-AKI. Methods: We retrospectively studied 114 consecutive neonates who underwent cardiac surgery at the Kagoshima University Hospital. CS-AKI was classified using the n-KDIGO criteria. Risk adjustment in congenital heart surgery (RACHS-1) score was used to predict the complexity-adjusted mortality and % fluid overload (%FO) was used to monitor fluid balance in pediatric cardiac surgery. Results: Among 81 patients, neonatal CS-AKI occurred in 57 (70.4%) patients according to n-KDIGO criteria. Of these, 28 (34.6%) patients reached n-KDIGO 1, 17 (21.0%) reached n-KDIGO 2, and 12 (14.8%) reached n-KDIGO 3. Patients with CS-AKI had significantly higher vasoactive-inotropic score levels, longer operative times, and higher %FO than patients without CS-AKI. Notably, increased duration of cardiopulmonary bypass times and %FO were risk factors for the development of neonatal CS-AKI. The n-KDIGO-based severe AKI grade had higher risk of in-hospital mortality; however, the n-KDIGO-based mild AKI grade was not associated with any postoperative outcomes. Conclusions: CS-AKI based on n-KDIGO criteria is common in neonates and is closely associated with higher mortality, especially in patients with severe CS-AKI. [ABSTRACT FROM AUTHOR]

Background. Syncope is a common symptom in children, many of which are benign and do not require treatment. Anomalous aortic origin of a coronary artery (AAOCA) is a rare congenital malformation but can be a risk for serious cardiovascular events, including sudden death as well as cardiogenic syncope. Case Report. We describe the case of a 14-year-old boy who suffered an initial syncope and afebrile seizure during a soccer game. A detailed medical history and imaging studies led to the diagnosis of the anomalous aortic origin of the left main coronary artery with an intramural course (AAOLCA-IM). Conclusion. Symptomatic AAOLCA-IM has the highest risk of sudden death among AAOCA, and surgical repair may be performed. Onset during exercise or preceding chest symptoms are suspicious signs of cardiogenic syncope and should be considered for cardiovascular imaging evaluation. [ABSTRACT FROM AUTHOR]

Aim: We aimed to validate the incidence of, risk factors for, and postoperative outcomes of acute kidney injury (AKI) according to the modified Kidney Disease Improving Global Outcomes (m‐KDIGO) criteria and compare this criteria with both the paediatric Risk, Injury, Failure, Loss, End‐stage disease (pRIFLE) and Acute Kidney Injury Network (AKIN) criteria in infants after cardiac surgery. Methods: We retrospectively enrolled 145 consecutive infants who underwent open‐heart surgery at Kagoshima University Hospital. Results: Acute kidney injury was present in 55 (37.9%), 111 (75.9%), and 95 (65.5%) patients according to the m‐KDIGO, pRIFLE, and AKIN criteria, respectively. Among these, 71.9% of patients pRIFLE Risk patients and 60.5% of AKIN 1 patients were categorized in the 'no‐AKI' group according to the m‐KDIGO criteria. Low body weight (m‐KDIGO odds ratio [OR], 0.73; P = 0.015; pRIFLE OR, 0.66; P = 0.001; AKIN OR 0.69, P = 0.002) and prolonged cross‐clamp time (m‐KDIGO OR, 1.02;

This study aimed to evaluate adverse cardiac events using dexmedetomidine in infants with trisomy 21 and those without (controls) and examined potential risk factors in infants after cardiovascular surgery. We conducted a single-center retrospective cohort study. The medical records of 124 consecutive infants who had undergone cardiovascular surgery between April 1, 2013, and October 31, 2015, were enrolled. Clinical characteristics, usage of dexmedetomidine, and perioperative medications were analyzed. Adverse cardiac events were assessed with the Naranjo score and World Health Organization-The Uppsala Monitoring Centre (WHO-UMC) criteria. In total, 124 consecutive infants (30 patients and 94 controls) met the inclusion criteria. Eight of 30 (26.7 %) patients with trisomy 21 and 12 of 94 (12.8 %) controls experienced adverse cardiac events (i.e., hypotension, transient hypertension, and bradycardia) during dexmedetomidine with median Naranjo score of 6, and causality categories of WHO-UMC criteria were 'certain' or 'probable.' Of those, the incidence of bradycardia occurred at a higher rate in patients with trisomy 21 than in controls ( P = 0.011). Multiple logistic regression analysis revealed that the presence of trisomy 21 was an independent risk factor for adverse cardiac events of dexmedetomidine after cardiovascular surgery (odds ratio 4.10, 95 % CI 1.17-11.19, P = 0.006). Dexmedetomidine is associated with an increased incidence of bradycardia in patients with trisomy 21 after surgery for congenital heart disease. Physicians using dexmedetomidine should know a great deal about the characteristics of patients with trisomy 21, and hemodynamic monitoring should be closely observed. [ABSTRACT FROM AUTHOR]

Purpose Few studies are available on withdrawal seizures about dexmedetomidine (DEX). Thus, we retrospectively evaluated the incidence of withdrawal seizures after discontinuation of DEX and examined potential risk factors in infants after cardiovascular surgery. Methods The medical records of 142 infants who had undergone cardiovascular surgery between April 2010 and November 2013 were examined. Clinical characteristics and usage of DEX were analyzed. DEX withdrawal seizures were evaluated using Withdrawal Assessment Tool - version 1 (WAT-1). All the patients and controls were categorized according to DEX discontinuation strategy, which was either gradual or abrupt. Results Nine patients (6.3%) developed generalized clonic or generalized tonic-clonic seizures accompanied by preceding fever of >38°C approximately four to eight hours following the discontinuation of DEX, and were clinically diagnosed as DEX withdrawal seizures with a median WAT-1 score of 3. Clinical characteristics and operative data were similar, but median cumulative dose and maximum temperature after discontinuation of DEX were significantly higher in infants with withdrawal seizures than in those without (P=0.007 and P< 0.001, respectively). Eight of the 9 patients with withdrawal seizures (88.9%) and 20 of the 133 patients (15.0%) with no withdrawal seizures had discontinued DEX abruptly (P< 0.001). Cumulative dose and abrupt discontinuation of DEX were significantly associated with DEX withdrawal seizures in infants after cardiovascular surgery (R=0.619, P=0.004). Conclusion sPhysicians should be aware that infants who received DEX after cardiovascular surgery had potential to cause withdrawal seizures accompanied by preceding pyrexia after discontinuation of DEX. Higher cumulative dose and abrupt discontinuation of DEX appears to increase the risk for these withdrawal seizures. [ABSTRACT FROM AUTHOR]

The article describes the case of a previously healthy, 11-year-old girl who presented with primary or lone atrial fibrillation (AF) with cardiac troponin T elevation. Topics covered include the results of the physical examination indicated and electrophysiological (EP) study which led to the diagnosis of unsuspected, orthodromic atrioventricular reciprocating tachycardia (AVRT), patient outcome following milrinone and dexmedetomidine therapy, and complications in the diagnosis of lone AF.

The article discusses case study of 5-year-old girl with no past medical history had dyspnea and fatigue during exercise. It mentions She developed a cough and was presented to a pediatric department. A chest radiograph showed a rightward deviation of the trachea and mediastinum with volume loss over the right lung but she underwent no further medical examination.

Kawasaki syndrome (KS) is an acute febrile vasculitis of childhood. Coronary artery abnormalities (CAA) are a significant problem in KS patients. High dose intravenous immunoglobulin (IVIG) is effective for reducing the occurrence of CAA. Clinical and histopathological findings suggest that vascular endothelial growth factor (VEGF) is involved in CAA. In circulating blood, newly activated platelets are the major source of VEGF, which is released in large amounts in vascular inflammation. The present study analysed 80 KS patients (69 IVIG responders and 11 IVIG non-responders) and evaluated the role of platelet VEGF in KS vasculitis. Serum VEGF and platelet VEGF levels were significantly higher in KS patients than controls ( P < 0·001). Platelet VEGF reflected the reactivity of IVIG treatment and was decreased in responders ( P < 0·001), but remained increased in non-responders ( P = 0·01). Platelet VEGF levels, but not serum VEGF levels, before IVIG were significantly correlated with the maximum CAA z-score ( r = 0·524, P = 0·02). Our findings demonstrate that platelet VEGF may reflect the severity of vasculitis related to the pathological development of CAA in KS. Platelet VEGF may be an important feature of KS pathophysiology. [ABSTRACT FROM AUTHOR]

A case study of a 36-year-old woman who reported of uterine contractions at 33 weeks of her first pregnancy is presented. It is noted that she had no history of arrhythmia, cardiovascular or endocrine disease, and no family history of arrhythmia or sudden death. The article discusses that ritodrine can cause maternal tachycardia and disturb the normal response of heart in physiologic status. It is notes that her child had a normal heart with no abnormalities.

The article presents a case study of an 11-year-old boy with nephrotic syndrome. The patient was given steroid and had complete remission but suffered a relapse at the age of five. Steroids did not improve his symptoms and was diagnosed with steroid-resistant nephrotic syndrome where his condition stabilized with cyclosporine. The patient suffered from repeated relapses and refractory nephrotic syndrome complicated by isolated thrombus in the right ventricle diagnosis was formed.

Neurocutaneous melanosis (NCM) is a rare, non-hereditary neurocutaneous disorder characterized by excessive melanocytic proliferation in the skin and central nervous system. As no major studies have covered the incidence of NCM among Japanese patients with congenital melanocytic nevi (CMN), we prospectively investigated the incidence of NCM among Japanese patients who underwent initial treatment for CMN. The relationship of CMN and NCM was also investigated. Japanese pediatric patients with CMN under 1 year of age were included between January 2020 and November 2022, and all patients underwent brain MRI to check for NCM in this study. NCM lesions were most frequently seen in the amygdala, followed by the cerebellum, brainstem, and cerebral hemispheres. NCM was diagnosed on brain MRI in 31.6% of the 38 patients with CMN and in 25.0% of patients with no prior examination or treatment. Distribution and size of CMN, number of satellite nevi, rugosity and nodules were strongly associated with the existence of NCM, and these findings may guide a future registry study with a large cohort of CMN patients. [ABSTRACT FROM AUTHOR]

Neurocutaneous melanosis (NCM) is a rare, non-hereditary neurocutaneous disorder characterized by excessive melanocytic proliferation in the skin and central nervous system. As no major studies have covered the incidence of NCM among Japanese patients with congenital melanocytic nevi (CMN), we prospectively investigated the incidence of NCM among Japanese patients who underwent initial treatment for CMN. The relationship of CMN and NCM was also investigated. Japanese pediatric patients with CMN under 1 year of age were included between January 2020 and November 2022, and all patients underwent brain MRI to check for NCM in this study. NCM lesions were most frequently seen in the amygdala, followed by the cerebellum, brainstem, and cerebral hemispheres. NCM was diagnosed on brain MRI in 31.6% of the 38 patients with CMN and in 25.0% of patients with no prior examination or treatment. Distribution and size of CMN, number of satellite nevi, rugosity and nodules were strongly associated with the existence of NCM, and these findings may guide a future registry study with a large cohort of CMN patients. [ABSTRACT FROM AUTHOR]

Disruption of endothelial cell homeostasis may be associated with the pathogenesis of coronary artery abnormalities (CAA) in Kawasaki disease (KD). We sought to clarify the poorly understood pathogenic role of endothelial cell survival and death in KD vasculitis. Human umbilical vein endothelial cells (HUVECs) stimulated with sera from KD patients, compared with sera from patients with bacterial infections, exhibited significant increases in cytotoxicity, high mobility group box protein 1 (HMGB-1), and caspase-3/7 and a decrease in phosphorylated Akt/Akt (pAkt/Akt) ratios. HUVECs stimulated with sera from KD patients treated with immunoglobulin (IG) showed significantly decreased cytotoxicity, HMGB-1, and caspase-3/7 levels and increased pAkt/Akt ratios, as compared with results for untreated HUVECs (P < 0.001, P = 0.008, P = 0.040, and P < 0.001, respectively). In HUVECs stimulated with sera from KD patients, the increased cytotoxicity levels and the suppression of increased pAkt/Akt ratios after subsequent IG treatment were closely related to the development of CAA (P = 0.002 and P = 0.035). Our data reveal that shifting the balance toward cell death rather than survival appears to perturb endothelial cell homeostasis and is closely related to the development of CAA. The cytoprotective effects of IG treatment appear to ameliorate endothelial cell homeostasis. [ABSTRACT FROM AUTHOR]

Background: Delirium is common in critically ill patients. Haloperidol has long been used for the treatment of delirium. Dexmedetomidine has recently been used to treat delirium among intubated critically ill patients. However, the efficacy of dexmedetomidine for delirium in non-intubated critically ill patients remains unknown. We hypothesize that dexmedetomidine is superior to haloperidol for sedation of patients with hyperactive delirium, and would reduce the prevalence of delirium among non-intubated patients after administration. We will conduct a randomized controlled trial to compare dexmedetomidine and haloperidol for the treatment of nocturnal hyperactive delirium in non-intubated patients in high dependency units (HDUs). Methods: This is an open-label, parallel-group, randomized controlled trial to compare the efficacy and safety of dexmedetomidine and haloperidol for nocturnal hyperactive delirium in non-intubated patients at two HDUs of a tertiary hospital. We will recruit consecutive non-intubated patients who are admitted to the HDU from the emergency room, and allocate them in a 1:1 ratio to the dexmedetomidine or haloperidol group in advance. The allocated investigational drug will be administered only when participants develop hyperactive delirium (Richmond Agitation-Sedation Scale [RASS] score ≥1 and a positive score on the Confusion Assessment Method for the ICU between 19:00 and 6:00 the next day) during the night at an HDU. Dexmedetomidine is administered continuously, while haloperidol is administered intermittently. The primary outcome is the proportion of participants who achieve the targeted sedation level (RASS score of between -3 and 0) 2h after the administration of the investigational drug. Secondary outcomes include the sedation level and prevalence of delirium on the day following the administration of the investigational drugs, and safety. We plan to enroll 100 participants who develop nocturnal hyperactive delirium and receive one of the two investigational drugs. Discussion: This is the first randomized controlled trial to compare the efficacy and safety of dexmedetomidine and haloperidol for sedation of non-intubated critically ill patients with hyperactive delirium in HDUs. The results of this study may confirm whether dexmedetomidine could be another option to sedate patients with hyperactive delirium. Trial registration: Japan Registry of Clinical Trials, jRCT1051220015, registered on 21 April 2022. [ABSTRACT FROM AUTHOR]

The 2-lead Holter ECG also showed a paroxysmal second-degree atrioventricular block, with a maximal R-R interval of 1.5 s, and several episodes of sinus bradycardia (Fig. Holter electrocardiography can be helpful in preterm infants to detect and define paroxysmal atrioventricular block. The heart rate abnormalities gradually improved, and a 2-lead Holter ECG obtained at 3 months of age showed sinus arrest, a maximal R-R interval of 1.9 s, and sinus bradycardic events (maximal R-R interval, 1.0 s) without paroxysmal atrioventricular block (PAVB). [Extracted from the article]

The article presents a case study of an 8-month-old boy who had high fever and lymphadenopathy which was treated with antibiotics but later developed into autoimmune neturopenia that led to the development of Kawasaki syndrome after being treated with granulocyte colony-stimulating factor (G-CSF). He was diagnosed with neutropenia due to the presence of anti-neutrophil antibody that was used for his lymphadenopathy. Plasma exchange (PE) therapy decreased dilation of his left coronary artery.

Background: The safety of robotic gastrectomy (RG) for gastric cancer in daily clinical settings and the process by which surgeons are introduced and taught RG remain unclear. This study aimed to evaluate the safety of RG in daily clinical practice and assess the learning process in surgeons introduced to RG. Methods: Patients who underwent RG for gastric cancer at Kyoto University and 12 affiliated hospitals across Japan from January 2017 to October 2019 were included. Any morbidity with a Clavien–Dindo classification grade of II or higher was evaluated. Moreover, the influence of the surgeon's accumulated RG experience on surgical outcomes and surgeon-reported postoperative fatigue were assessed. Results: A total of 336 patients were included in this study. No conversion to open or laparoscopic surgery and no in-hospital mortality were observed. Overall, 50 (14.9%) patients developed morbidity. During the study period, 14 surgeons were introduced to robotic procedures. The initial five cases had surprisingly lower incidence of morbidity compared to the following cases (odds ratio 0.29), although their operative time was longer (+ 74.2 min) and surgeon's fatigue scores were higher (+ 18.4 out of 100 in visual analog scale). Conclusions: RG was safely performed in actual clinical settings. Although the initial case series had longer operative time and promoted greater levels of surgeon fatigue compared to subsequent cases, our results suggested that RG had been introduced safely. [ABSTRACT FROM AUTHOR]

Chronic obstructive pulmonary disease (COPD) may coexist with lung cancer, but the impact on prognosis is uncertain. Moreover, it is unclear whether pharmacological treatment for COPD improves the patient's prognosis. We retrospectively investigated patients with advanced non-small-cell lung cancer (NSCLC) who had received chemotherapy at Kyoto University Hospital. Coexisting COPD was diagnosed by spirometry, and the association between pharmacological treatment for COPD and overall survival (OS) was assessed. Of the 550 patients who underwent chemotherapy for advanced NSCLC between 2007 and 2014, 347 patients who underwent spirometry were analyzed. Coexisting COPD was revealed in 103 patients (COPD group). The median OS was shorter in the COPD group than the non-COPD group (10.6 vs. 16.8 months). Thirty-seven patients had received COPD treatment, and they had a significantly longer median OS than those without treatment (16.7 vs. 8.2 months). Multivariate Cox regression analysis confirmed the positive prognostic impact of COPD treatment. Additional validation analysis revealed similar results in patients treated with immune checkpoint inhibitors (ICIs). Coexisting COPD had a significant association with poor prognosis in advanced NSCLC patients if they did not have pharmacological treatment for COPD. Treatment for coexisting COPD has the potential to salvage the prognosis. [ABSTRACT FROM AUTHOR]

Objectives: It is important to clarify the relationship between irreversible organ damage and the quality of life (QOL) by considering the unique factors of patients with systemic lupus erythematosus (SLE). We aimed to clarify their correlation using SLE-specific QOL assessment tools. We also aimed to identify which type of organ damage is adversely correlated with the QOL. Methods: We conducted a questionnaire-based survey of outpatients with SLE at Kyoto University Hospital and evaluated irreversible organ damage using the SLICC/ACR damage index (SDI). LupusPRO and the SLE symptom checklist (SSC) were employed as SLE-specific QOL tools, and the SF-36v2 was used as a conventional QOL tool. Multiple linear regression analyses were performed to examine the correlations between the total SDI score and each QOL score, and between each SDI item/system score and each QOL score. Results: We analyzed the data of 265 patients. The total SDI score was significantly correlated with physical (PCS) and role/social component summary (RCS) of the SF-36v2, health-related QOL (HRQOL) of LupusPRO, and SSC (p < 0.001). Among the SDI items, atrophy/weakness and osteoporosis with fracture/vertebral collapse were negatively correlated with PCS (β = −0.40, p < 0.001/β = −0.28, p < 0.001), RCS (β = −0.30, p < 0.001/β = −0.35, p < 0.001), and HRQOL (β = −0.34, p < 0.001/β = −0.31, p < 0.001), respectively. Among the SDI systems, musculoskeletal damage had higher negative correlations with PCS (β = −0.51, p < 0.001), RCS (β = −0.29, p < 0.001), and HRQOL (β = −0.40, p < 0.001). Conclusion: We demonstrated the QOL of patients with SLE is negatively correlated with irreversible organ damage. We also revealed musculoskeletal damage is adversely correlated with the health-related QOL, especially the physical and role/social QOL. [ABSTRACT FROM AUTHOR]

This study aimed to evaluate the predictions of dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) for prognosis of triple-negative breast cancer (TNBC), especially with residual disease (RD) after preoperative chemotherapy. This retrospective analysis included 74 TNBC patients who received preoperative chemotherapy. DCE-MRI findings from three timepoints were examined: at diagnosis (MRIpre), at midpoint (MRImid) and after chemotherapy (MRIpost). These findings included cancer lesion size, washout index (WI) as a kinetic parameter using the difference in signal intensity between early and delayed phases, and time-signal intensity curve types. Distant disease-free survival was analysed using the log-rank test to compare RD group with and without a fast-washout curve. The diagnostic performance of DCE-MRI findings, including positive predictive value (PPV) for pathological responses, was also calculated. RD without fast washout curve was a significantly better prognostic factor, both at MRImid and MRIpost (hazard ratio = 0.092, 0.098, p < 0.05). PPV for pathological complete remission at MRImid was 76.7% by the cut-off point at negative WI value or lesion size = 0, and 66.7% at lesion size = 0. WI and curve types derived from DCE-MRI at the midpoint of preoperative chemotherapy can help not only assess tumour response but also predict prognosis. [ABSTRACT FROM AUTHOR]

In the Results section, under the subheading "COPD and OS in patients with advanced NSCLC", "In the multivariate Cox proportional hazard model, TKI treatment, recurrence after curative treatment and better PS were significantly associated with better OS in patients with advanced NSCLC (Table 2)." now reads: "In the multivariate Cox proportional hazard model, after adjusting for clinicopathologic variables, TKI treatment, recurrence after curative treatment and better PS were significantly associated with better OS in patients with advanced NSCLC (Table 2)." In addition, in the Results section, under the subheading "COPD treatment and OS", "The median OS in patients with COPD treatment (8.2 months) was lower than that in patients without COPD treatment (16.8 months)." now reads: "The median OS in patients without COPD treatment (8.2 months) was lower than that in patients with COPD treatment (16.8 months).". [Extracted from the article]

Background: Low levels of serum immunoglobulin G (IgG) before intravenous immunoglobulin (IVIG) therapy for Kawasaki disease (KD) have been reported as one of the risk factors for coronary artery abnormalities (CAAs). This risk factor needs to be re-evaluated because the dosage of IVIG has changed from 0.2-0.4 g/kg/day for 5 days to a single high dose of 2 g/kg. Methods: We reviewed the clinical records of KD patients admitted to our hospital from January 2001 to August 2011. Patients who were given a single high dose of IVIG within 7 days of illness, and who had blood collected for serum immunoglobulin values before treatment, were selected. The serum immunoglobulin levels and coronary artery diameters measured by echocardiogram were transformed to z-scores. Results: The subjects were 197 KD patients, including 22 IVIG nonresponders and 16 patients with CAAs. Of these, 150 (76%) had a z-score for IgG (IgGz) of ≤0. There were no differences in IgGz values between patients with CAAs and those without CAAs. However, nonresponders had higher IgGz values than responders (median, 25th percentile and 75th percentile: −0.26, −0.83 and 0.34 vs. −0.79, −1.40 and −0.03; p = 0.020). Logistic regression analysis showed that the IgGz value was an independent risk factor for resistance to IVIG (OR 1.36, 95% CI 1.002-1.849; p = 0.048). Conclusions: Low IgGz values were not a risk factor for CAAs in this study. However, KD patients with relatively high IgGz values before treatment may have an increased risk of resistance to initial IVIG therapy. © 2014 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Background: A diagnosis of Kawasaki disease (KD) is established using six principal symptoms. Because the principal symptoms are deeply connected with KD, it is thus important to investigate the usefulness of the principal symptoms for evaluating the disease severity of KD. Methods: Patients with definite KD or suspicion of KD were retrospectively examined. Blood test data and the incidence of patients who failed to respond to the initial i.v. immunoglobulin treatment (non-responders) were compared between patients with six principal symptoms, including fever of ≤4 days, before treatment of KD (six-symptom patients), and those with five or fewer symptoms (five-symptom patients). Results: The study group of 207 patients who were treated with immunoglobulin consisted of 121 six-symptom patients and 86 five-symptom patients. The six-symptom patients were older and had higher neutrophil proportion and total bilirubin, and lower serum sodium at diagnosis than the five-symptom patients. Although the treatments did not differ between the groups, the six-symptom patients had a higher incidence of non-responders than the five-symptom patients (17% vs 5%; P= 0.008). Logistic regression analysis showed that six-symptom status was related to the risk of being a non-responder (odds ratio [OR], 5.3; 95% confidence interval [95%CI]: 1.6-17.4). This association was still significant after adjustment for the effect of age, neutrophil proportion, and total bilirubin and sodium (OR, 4.4; 95%CI: 1.4-17.3). Conclusions: The number of principal symptoms before treatment is a useful guide to KD disease severity. Six-symptom patients have a higher risk of being a non-responder than five-symptom patients. [ABSTRACT FROM AUTHOR]

The article presents a case study of an infant girl diagnosed with Interrupted aortic arch underwent the Norwood procedure with the RV-PA conduit (8 mm GORETEX) at 3 months of age. It mentions that She had left hemiplegia after surgery; computed tomography confirmed cerebral infarction in the right middle cerebral artery region.