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There has been a remarkable increase in the number of pediatric ventricular assist device (VAD) implanted over the past decade. Asian pediatric heart centers had not participated in the multicenter registries among the Western countries. This article aimed to report the outcomes of pediatric VAD in our hospital.The study enrolled all patients aged18 years at the time of VAD implantation in our institution between 2008 and 2021.There were 33 patients with diagnosis of acute fulminant myocarditis (n = 9), congenital heart disease (n = 5), dilated cardiomyopathy (n = 16), and others. Paracorporeal continuous-flow pump was the most frequently implanted (n = 27). Most of the devices were implanted in patients with INTERMACS profile 1 (n = 24). The median duration on VAD was 22 days (range 2-254). The proportion of patients attaining positive outcomes (alive on device, bridge to transplantation or recovery) was 72.7% at 1 month, 67.7% at 3 months, and 67.7% at 6 months. Most of the deaths on device occurred within the first month post-implant (n = 9), with neurological complications being the most frequent cause of death. All recovered cases were successfully weaned off the device within the first month of implantation.We demonstrated a favorable outcome in pediatric patients supported with VAD at our institution.

Coronavirus disease 2019 (COVID-19) pandemic challenges pediatric health globally by limited medical accessibility. In response to COVID-19 epidemic in Taiwan, public restrictions were applied and the Level 3 alert was announced from May to July in 2021 for local outbreak. This study aims to analyze patients' clinical features and outcomes in the pediatric intensive care unit (PICU) during the COVID-19 epidemic with the Level 3 alert in Taiwan.Medical records were retrospectively collected in patients admitted to the PICU of National Taiwan University Children's Hospital from May to July 2021 (Level 3 alert) and May to July 2019 and 2020 (control periods). Clinical characteristics and outcomes were compared between patients in the period with the Level 3 alert and control periods.During the study period, PICU monthly admissions significantly decreased in the Level 3 alert period and were negatively correlated with monthly newly confirmed COVID-19 cases. Patients admitted during the Level 3 alert were older, had higher disease severity, lower proportion of cardiovascular disease, and higher proportion of hematology-oncology diseases than those in the control group. After adjusting for the above factors, admission during Level 3 alert was an independent factor for higher mortality rate and prolonged length of stay (14 days) in the PICU.During the COVID-19 epidemic with strict public restrictions, critically ill patients admitted to the PICU decreased but had increased disease severity, prolonged length of stay in the PICU, and higher mortality, reflecting the impact of quarantine and limited medical access.

Community-acquired pneumonia (CAP) is a serious clinical concern. A lack of accurate diagnosis could hinder pathogen-directed therapeutic strategies. To solve this problem, we evaluated clinical application of nested multiplex polymerase chain reaction (PCR) in children with severe CAP. We prospectively enrolled 60 children with severe CAP requiring intensive care between December 2019 and November 2021 at a tertiary medical center. Nested multiplex PCR respiratory panel (RP) and pneumonia panel (PP) were performed on upper and lower respiratory tract specimens. We integrated standard-of-care tests and quantitative PCR for validation. The combination of RP, PP, and standard-of-care tests could detect at least one pathogen in 98% of cases and the mixed viral-bacterial detection rate was 65%. The positive percent agreement (PPA), and negative percent agreement (NPA) for RP were 94% and 99%; the PPA and NPA for PP were 89% and 98%. The distribution of pathogens was similar in the upper and lower respiratory tracts, and the DNA or RNA copies of pathogens in the lower respiratory tract were equal to or higher than those in the upper respiratory tract. PP detected bacterial pathogens in 40 (67%) cases, and clinicians tended to increase bacterial diagnosis and escalate antimicrobial therapy for them. RP and PP had satisfactory performance to help pediatricians make pathogenic diagnoses and establish therapy earlier. The pathogens in the upper respiratory tract had predictive diagnostic values for lower respiratory tract infections in children with severe CAP.

Background: Acute kidney injury (AKI) is the most frequent complication in critically ill neonatal and pediatric patients receiving extracorporeal membrane oxygenation (ECMO) support. This study analyzed risk factors for in-hospital mortality and the incidence of AKI in neonatal and pediatric patients received ECMO support. Methods: We reviewed the medical records of 105 neonatal and 171 pediatric patients who received ECMO support at the intensive care unit (ICU) of a tertiary care university hospital between January 2008 and December 2015. Demographic, clinical, and laboratory data were retrospectively collected as survival and AKI predictors, utilizing the Kidney Disease Improving Global Outcome (KDIGO) consensus definition for AKI. Results: In the 105 neonatal and 171 pediatric patients, the overall in-hospital mortality rate were 58% and 55% respectively. The incidence of AKI at post-ECMO 24 h were 64.8% and 61.4%. A greater KDIGO24-h severity was associated with a higher in-hospital mortality rate (chi-square test; p

Pulmonary arterial hypertension (PAH) is a fatal or life-threatening disorder characterized by elevated pulmonary arterial pressure and pulmonary vascular resistance. Abnormal vascular remodeling, including the proliferation and phenotypic modulation of pulmonary artery smooth muscle cells (PASMCs), represents the most critical pathological change during PAH development. Previous studies showed that miR-486 could reduce apoptosis in different cells; however, the role of miR-486 in PAH development or HPASMC proliferation and migration remains unclear. After 6 h of hypoxia treatment, miR-486-5p was significantly upregulated in HPASMCs. We found that miR-486-5p could upregulate the expression and secretion of ET-1. Furthermore, transfection with a miR-486-5p mimic could induce HPASMC proliferation and migration. We also found that miRNA-486-5p could downregulate the expression of SMAD2 and the phosphorylation of SMAD3. According to previous studies, the loss of SMAD3 may play an important role in miRNA-486-5p-induced HPASMC proliferation. Although the role of miRNA-486-5p in PAH in in vivo models still requires further investigation and confirmation, our findings show the potential roles and effects of miR-486-5p during PAH development.

Hoyeraal-Hreidarsson syndrome (HHS) is the most severe form of dyskeratosis congenita (DC) and is caused by genes involved in telomere maintenance. Here, we identified male siblings from a family with HHS carrying a hemizygous mutation (c.1345C>G, p.R449G), located in the C-terminal nuclear localization signal (NLS) of the DKC1 gene. These patients exhibit progressive cerebellar hypoplasia, recurrent infections, pancytopenia due to bone marrow failure, and short leukocyte telomere lengths. Single-cell RNA sequencing analysis suggested defects in the NLRP3 inflammasome in monocytes and the activation and maturation of NK cells and B cells. In experiments using induced pluripotent stem cells (iPSCs) from patients, DKC1_R449G iPSCs had short telomere lengths due to reduced levels of human telomerase RNA (hTR) and increased cytosolic proportions of DKC1. Treatment with dihydroquinolizinone RG7834 and cordycepin rescued telomere length in patient-derived iPSCs. Together, our findings not only provide new insights into immunodeficiency in DC patients but also provide treatment options for telomerase insufficiency disorders.

In heterotaxy syndrome, bronchopulmonary situs usually reflects atrial situs, resulting in either right (RAI) or left atrial isomerism (LAI). This study determines airway anomalies and its implications in patients with heterotaxy. This retrospective study included 223 patients with heterotaxy syndrome who received an integrated cardiac computed tomography evaluation. Patient database from 1995 to 2020 was reviewed. The patients were examined by a congenital heart disease team comprising pediatric cardiologists, radiologists, pulmonologists, and cardiovascular surgeons. Among the 223 patients, 189 (84.8%, M/F=1.66) had RAI and 29 had LAI (13.0%, M/F=0.71). Five patients had indeterminate isomerism (2.2%, M/F=1.5). Discordant bronchopulmonary and atrial situs occurred in 4% patients, while discordant bronchopulmonary, atrial, and splenic situs occurred in 23.2% patients. Lower airway stenosis was observed in 61 patients (27.4%), including 27.5%, 20.7%, and 60% RAI, LAI, and indeterminate isomerism patients, respectively (p=0.189). One patient had an intrinsic long segment lower tracheal stenosis and received slide tracheoplasty. Initial cardiac operation was performed in 213 patients. Higher surgical mortality occurred in patients with RAI (19.5% vs. none for LAI and indeterminate isomerism, p=0.038). In patients with RAI, lower airway anomaly/stenosis increased the duration of ventilator usage (p=0.030) but did not affect surgical mortality. Total anomalous pulmonary venous return to systemic veins and pulmonary venous stenosis were major surgical risk factors. Bronchopulmonary isomerism shares a similar isomeric pattern to cardiac atrial appendage. Lower airway anomalies/stenosis was common in patients with heterotaxy, resulting in prolonged ventilator therapy in patients with RAI.

The cardiac pumping mechanics can be characterized by both the maximal systolic elastance (Emax) and theoretical maximum flow (Qmax), which are generated using an elastance–resistance model. The signals required to fit the elastance–resistance model are the simultaneously recorded left ventricular (LV) pressure and aortic flow (Qm), followed by the isovolumic LV pressure. In this study, we evaluated a single-beat estimation technique for determining the Emax and Qmax by using the elastance–resistance model based solely on the measured LV pressure and cardiac output. The isovolumic LV pressure was estimated from the measured LV pressure by using a non-linear least-squares approximation technique. The measured Qm was approximated by an unknown triangular flow (Qtri), which was generated by using a fourth-order derivative of the LV pressure. The Qtri scale was calibrated using the cardiac output. Values of EmaxtriQ and QmaxtriQ obtained using Qtri were compared with those of EmaxmQ and QmaxmQ obtained from the measured Qm. Healthy rats and rats with chronic kidney disease or diabetes mellitus were examined. We found that the LV Emax and Qmax can be approximately calculated using the assumed Qtri, and they strongly correlated with the corresponding values derived from Qm (P

OBJECTIVES Medical advances and the National Health Insurance coverage in Taiwan mean that mortality in the PICU is low. This study describes change in modes of death and end-of-life care in a single center, 2011-2017. SETTING Multidisciplinary PICU in a tertiary referral Children's Hospital in Taiwan. PATIENTS There were 316 deaths in PICU patients. INTERVENTIONS Palliative care consultation in the PICU service occurred after the 2013 "Hospice Palliative Care Act" revision. MEASUREMENTS AND MAIN RESULTS In the whole cohort, 22 of 316 patients (7%) were determined as "death by neurologic criteria". There were 94 of 316 patients (30%) who had an event needing cardiopulmonary resuscitation within 24 hours of death: 17 of these patients (17/94; 18%) died after failed cardiopulmonary resuscitation without a do-not-resuscitate order, and the other 77 of 94 patients (82%) had a do-not-resuscitate order after cardiopulmonary resuscitation. Overall, there were 200 of 316 patients (63%) who had a do-not-resuscitate order and were entered into the palliative program: 169 of 200 (85%) died after life-sustaining treatment was limited, and the other 31 of 200 (15%) died after life-sustaining treatment was withdrawn. From 2011 to 2017, the time-trend in end-of-life care showed the following associations: 1) a decrease in PICU mortality utilization rate, from 22% to 7% (p < 0.001); 2) a decrease in use of catecholamine infusions after do-not-resuscitate consent, from 87% to 47% (p = 0.001), in patients having limitation in life-sustaining treatment; and 3) an increase in withdrawal of life-sustaining treatment, from 4% to 31% (p < 0.001). CONCLUSIONS In our practice in a single PICU-center in Taiwan, we have seen that the integration of a palliative care consultation service, developed after the revision of a national "Palliative Care Act," was associated with increased willingness to accept withdrawal of life-sustaining treatment and a lowered PICU care intensity at the end-of-life.

Normal ECG values in newborns, infants, and children have been collected and published. ECG in the adolescent, however, remains, to be collected and studied. The present study was designed and carried out to establish the normal ECG standards in male and female adolescents. A total of 898 school children and adolescents screened and examined as healthy were divided by age and sex into 6-9, 9-13, and 13-18 years age-groups. A 12 lead conventional ECG was recorded in 10 mm/mV and 25 mm/s, utilizing an automated Fukuda Denshi FCP-4301, MS-DOS/IBM-AT ECG machine. Lead V3R was not taken. Analog-to-digital conversion was performed by Fukuda signal acquisition module at a sampling rate of 500 Hz. The data on 69 ECG parameters were analyzed for the mean, standard deviation, 2nd to 98th percentiles, 95% confidence intervals, and sex difference. Normal values on 69 ECG parameters, sex-specific heart rate, P-QRS-T interval, duration, axis, wave amplitude, and calculated R/S amplitude ratio and ventricular activation time by age-group and sex were established. Male and female difference was noted in 49 (71.0%) parameters, of which 3 (6.1%) began in 6-9 years age-group, 30 (61.2%) began in 9-13 years age-group, and 16 (32.7%) in 13-18 years age-group. No sex difference occurred in 20 (29.0%) parameters. Normal male and female ECG standards on 69 ECG parameters in the adolescent were established. ECG sex difference began to appear the earliest at ages 6-9 years, and it occurred mostly at ages 9-13 years and 13-18 years, reflecting the anatomical and physiological consequences of puberty.

OBJECTIVES Acute myocarditis can be lethal, but the incidence remains unclear because of its wide manifestation spectrum. We investigated the postnatal incidence of acute myocarditis and risk factors for morbidity and mortality. DESIGN Retrospective derived birth cohort study. SETTING Taiwan National Health Insurance Database for the period 2000-2014. PATIENTS Children born between 2000 and 2009 with complete postnatal medical care data for at least 5 years. INTERVENTIONS None. MEASUREMENTS AND MAIN RESULTS From among 2,150,590 live births, we identified 965 patients (54.8% male) admitted with the diagnosis of acute myocarditis, accounting for an overall incidence of 0.45/1,000. The cumulative incidence rates were 0.19/1,000, 0.38/1,000, 0.42/1,000, and 0.48/1,000 by ages 1, 5, 10, and 15 years, respectively. Male predominance was noted in infants and school age children (age group 6-14 yr). Arrhythmias, including tachyarrhythmia (4.8%) and bradyarrhythmia (1.1%), occurred in 56 patients. Extracorporeal membrane oxygenation support was provided to 62 patients (6.4%) and additional left ventricular assist devices in six of them. The mortality at discharge was 6.3%. The presence of ventricular tachyarrhythmia, bradyarrhythmia, and an onset at school age (6-14 yr) were associated with increased odds for the need for extracorporeal membrane oxygenation, which was the only predictor for mortality at discharge (hazard ratio, 7.85; 95% CI, 3.74-9.29). In patients who survived the acute myocarditis, late mortality was relatively low (36/904 = 4.0%). The overall survival of children with acute myocarditis were 90.9%, 90.3%, and 89.8% by the intervals of 1, 5, and 10 years after the myocarditis, respectively. CONCLUSIONS This birth cohort study determined the cumulative incidence of acute myocarditis for neonates by 15 years old to be one in 2,105. In an era of extracorporeal membrane oxygenation, the need of extracorporeal membrane oxygenation may reflect the severity of acute myocarditis and predict its outcome.

Background Infective endocarditis (IE) is uncommon in childhood. Its associated epidemiological characteristics in patients with congenital heart disease (CHD) remain unclear. Methods The study population included children born in Taiwan during the years 1997 to 2005 who were diagnosed as having CHD before 3years of age. All children were followed up until the end year of 2010, the diagnosis of infective endocarditis, or death. The demographic characteristics of patients with and without IE, the invasive procedures performed during 6months before the index date, the prophylactic antibiotics related to dental procedures, and in-hospital mortality were collected. Results Information of 24,729 children with CHD were retrieved for our analysis and 237 patients with newly diagnosed IE were identified. The incidence rate of IE in all CHD lesions was 11.13 per 10,000person-years. Taking ASD for reference, the following CHD lesions were at risk for IE: cyanotic CHD (adjusted OR, 9.58; 95% confidence interval, 5.38–17.05), endocardial cushion defect (ECD) (8.01; 2.73–23.50), Left-sided lesions (4.36; 1.90–10.01) and VSD (2.93; 1.64–5.23). Patients who underwent procedures have a higher risk of acquiring IE which include central venous catheter (CVC) insertion (3.17; 2.36–4.27), cardiac catheterization (3.74; 2.67–5.22), open-heart surgery (2.47; 1.61–3.77), valve surgery (3.20; 1.70–6.02), and shunt surgery (7.43; 2.36–23.41). However, dental procedures did not increase the risk of IE, irrespective of antibiotic usage. Conclusions The risk of IE varies markedly among CHD lesions in our study. Invasive heart procedures but not dental procedures, are more significantly associated with IE among children with CHD.

Extracorporeal life support (ECLS) is a world-famous life-saving method. Until now, changes in arterial wave properties due to ECLS have remained unexamined. In this study, we determined the effects of ECLS on arterial wave properties and ventricular/arterial coupling in male Wistar rats with the measured aortic pressure alone. Ascending aortic pressure signals were measured before ECLS and at 30, 60, and 90 min after weaned off. The aortic pressure signal then calculated by fourth-order derivative to obtain an assumed triangular flow wave. The ratio of mean systolic pressure to mean diastolic pressure (Pms/Pmd), a parameter for evaluating the matching condition between myocardial oxygen demand and supply, was significantly higher after ECLS. The magnitude of forward pressure (|Pf|) augmented by ECLS prevailed over the backward pressure (|Pb|), leading to a decline in wave reflection factor. Pms/Pmd was positively linearly correlated with |Pf| (Pms/Pmd = 0.9177 + 0.0078 × |Pf|, r = 0.8677; P Pf| was a predominant factor responsible for the mismatch between the myocardial oxygen demand and supply in rats after ECLS phase of experiment.

Critical illnesses caused by undiagnosed genetic conditions are challenging in PICUs. Whole-exome sequencing is a powerful diagnostic tool but usually costly and often fail to arrive at a final diagnosis in a short period. We assessed the feasibility of our whole-exome sequencing as a tool to improve the efficacy of rare diseases diagnosis for pediatric patients with severe illness.Observational analysis.We employed a fast but standard whole-exome sequencing platform together with text mining-assisted variant prioritization in PICU setting over a 1-year period.A tertiary referral Children's Hospital in Taiwan.Critically ill PICU patients suspected of having a genetic disease and newborns who were suspected of having a serious genetic disease after newborn screening were enrolled.None.Around 50,000 to 100,000 variants were obtained for each of the 40 patients in 5 days after blood sampling. Eleven patients were immediately found be affected by previously reported mutations after searching mutation databases. Another seven patients had a diagnosis among the top five in a list ranked by text mining. As a whole, 21 patients (52.5%) obtained a diagnosis in 6.2 ± 1.1 working days (range, 4.3-9 d). Most of the diagnoses were first recognized in Taiwan. Specific medications were recommended for 10 patients (10/21, 47.6%), transplantation was advised for five, and hospice care was suggested for two patients. Overall, clinical management was altered in time for 81.0% of patients who had a molecular diagnosis.The current whole-exome sequencing algorithm, balanced in cost and speed, uncovers genetic conditions in infants and children in PICU, which helps their managements in time and promotes better utilization of PICU resources.

Bronchopulmonary dysplasia (BPD), a disease affecting extremely premature infants, results from the disruption of normal pulmonary vascular and alveolar growth. Currently, there is no specific effective treatment. We report a case of a 10-mo-old female infant with BPD, who was admitted because of adenovirus pneumonia and acute respiratory distress syndrome (ARDS) with prolonged venovenous and arteriovenous extracorporeal membrane oxygenation (ECMO) support (total 125 d). The respiratory condition dramatically improved, and ECMO was removed 25 d after intratracheal delivery of maternal bone marrow-derived mesenchymal stem cells (BM-MSCs). Short tandem repeat examinations revealed that there was no maternal cells in the bronchial wash fluid. To our knowledge, this is the first human report of BM-MSC therapy reversal of the course of BPD superimposed with ARDS. We also suggest that BM-MSC therapy may not only be effective in the newborn stage but also works in infants and children with BPD.

Objective To explore acute and late coronary outcomes and their risk/modifiers in patients with Kawasaki disease (KD). Design Retrospective study. Setting and patients 1073 patients with KD identified from a tertiary care medical centre (1980–2012; 8677 patient-years). Main outcome measures The acute coronary severities and late outcomes (survival free of coronary aneurysm persistence and ischaemia) were assessed. Results Coronary arterial lesions occurred in 40.6% of cases at their acute febrile stages, and persisted beyond 1 month in 196 (18.3%, M/F=138/58) patients: 125 (11.6%) had small aneurysms, 44 (4.1%) had medium aneurysms, and 27 (2.5%) had giant aneurysms. At follow-up (1–46 years), coronary aneurysms persisted in all with giant aneurysms, in 55% of those with medium aneurysms (18% with stenosis), and in 9% of those with small aneurysms. Ischaemia events occurred in 14 patients (M/F=13/1) and caused four deaths. Among the patients with KD with coronary aneurysms, 10-year ischaemia event-free and aneurysm persistence probability was 87.5% and 20.6%, respectively. The only independent risk for aneurysm persistence was the aneurysm severity 1 month after KD onset (χ 2 =80.73, p −3 ). Male patients and intravenous γ-immunoglobulin (IVIG) therapy were independent risk factors of initial coronary severity but were not associated with the late coronary outcomes, even in severity stratified subgroups. Conclusions The coronary severity 1 month after KD onset is most crucial to the late coronary outcomes. Although IVIG use improves the initial severity of coronary lesions, it does not further modify the long-term fate of coronary aneurysms.

Extracorporeal membrane oxygenation (ECMO) was used in neonates with severe cardiopulmonary failure who failed to respond to conventional therapy. We started to apply neck venoarterial ECMO (VA-ECMO) in neonatal patients from 2000. In this study, we have focused on neonates who received ECMO support and described the current status of ECMO in neonates for both cardiac and pulmonary support and the risk factors associated with their outcomes. Methods: Data were retrieved from our ECMO database for the neonates (age < 28 days) who received neck VA-ECMO support from January 2005 to June 2015. Results: In total, 27 neonates, including 21 with respiratory support and six with cardiac support, were enrolled in this study. Sixteen (59.2%) patients survived to hospital discharge, and only one patient had a poor neurological outcome. The survival rate for respiratory support was 61.9% in which meconium aspiration syndrome with persistent pulmonary hypertension of a newborn had a superior outcome (11/13, 84.6%) and congenital diaphragmatic hernia had the worst outcome (4/7, 57.1%). The survival rate in the cardiac support group was only 50%. The median ECMO duration and hospital stay were 6 (1∼35.8) days and 37 (23∼232) days, respectively, for survivors. Furthermore, 11 (52.3%) neonates of 21 outborn patients were put on ECMO in other hospitals by our mobile ECMO team for respiratory support, and their survival (81.8%) was significantly better than those from in-house ECMO institution (40%). Conclusion: This is the first report for ECMO in neonatal disease in Taiwan. We achieved an overall survival rate of 59.2% with good neurological outcomes in this 10-year experience. ECMO could be a useful transportation tool for critical neonates who have a poor response to ventilator support.

New Findings What is the central question of this study? This study aimed to investigate the hypothesis that pyridoxamine, one of the three natural forms of vitamin B6, can protect against myocardial relaxation of senescent animals by targeting arterial stiffening and contractile dysfunction of the left ventricle. What is the main finding and its importance? We found that treating the senescent rats with pyridoxamine for 5 months might improve myocardial relaxation rate, at least partly through its ability to enhance myocardial contractile performance, increase wave transit time and decrease wave reflection factor. Our team demonstrated in the past that pyridoxamine attenuated arterial stiffening by targeting the pathogenic formation of glycated collagen cross-links in aged rats. Herein, we examined whether pyridoxamine therapy can protect against mechanical defects in myocardial relaxation by improving arterial wave properties and cardiac contractile performance in senescent animals. Fifteen-month-old male Fisher 344 rats were treated daily with pyridoxamine (1 g l−1 in drinking water) for 5 months and compared with age-matched untreated control animals (20 months old). Arterial wave properties were characterized by wave transit time (τw) and wave reflection factor (Rf). We measured the contractile status of the myocardium in an intact heart as the left ventricular (LV) end-systolic elastance (Ees). Myocardial relaxation was described according to the time constant of the LV isovolumic pressure decay (τe). Pyridoxamine therapy prevented the age-associated prolongation in LV τe and the diminished Ees in senescent rats. The drug also attenuated the age-related augmentation in afterload imposed on the heart, as evidenced by the increased τw and decreased Rf. We found that the LV τe was significantly influenced by both the arterial τw and Rf (τe = 16.3902 + 8.3123 × Rf − 0.4739 × τw; r = 0.7048, P

The association of congenital tracheal stenosis and tracheoesophageal (TE) fistula is rare. Here, we report 2 patients with tracheobronchial stenosis (complete cartilage ring) involving the lower trachea and right bronchus. Both patients had associated VACTERL (vertebral anomalies, anal atresia, cardiovascular anomalies, TE, renal, and limb defects) congenital cardiac defects and tracheal diverticula after repair of the TE fistula in type C esophageal atresia. The stenotic segment began at the orifice of the TE fistula, which became diverticula after the TE fistula was repaired. Concomitant repair of congenital cardiac defects and a slide tracheoplasty with elimination of the diverticula were performed successfully.

Intermittent hemodialysis (IHD) is the most efficient form of renal replacement therapy (RRT) for removing toxic substances from patients’ bodies. However, the efficacy and safety of IHD in infants and young children with inborn errors of metabolism are still not clear. This retrospective study included patients with urea cycle disorders, maple syrup urine disease, and methylmalonic acidemia who received IHD or non-IHD RRT at our hospital between 2001 and 2012 to remove ammonia, leucine, or methylmalonic acid. Both the efficacy and safety of the RRT were evaluated. Thirty-five courses of RRT, including 25 courses of IHD and ten courses of non-IHD RRT, for 15 patients were included in the analysis. Before 2006, non-IHD RRT procedures, including peritoneal dialysis (PD) and continuous venous-venous hemofiltration (CVVH), were the most often used; from 2006 onwards IHD was used. There was one procedure-unrelated death. Catheter penetration occurred in one course of IHD. The efficacy data revealed that both the median duration of dialysis and the median 50 % toxin reduction time were shorter in IHD than in non-IHD RRT. In infants and young children with inborn errors of metabolism, IHD is safe and more efficient than non-IHD RRT at removing toxins.