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Orthotopic liver transplantation (OLT) is an established therapeutic option for pediatric end-stage liver disease (PELD). The postoperative respiratory conditions of OLT recipients may be associated with subsequent clinical outcomes including length of stay (LOS) in the pediatric intensive care unit (PICU). This study aimed to characterize the postoperative respiratory conditions, associated factors, and outcomes after pediatric OLT.Clinical data of children receiving OLT from July 2014 to July 2020 were retrospectively collected. Postoperative respiratory conditions were defined as time to extubation, significant pleural effusion, and initial postoperative PaO2/FiO2 ratio. Logistic and multiple regressions were applied to analyze the associations among clinical factors, postoperative respiratory conditions, and clinical outcomes.Twenty-two patients with median age of 1.4-year-old (range: 25 days to 12 years old) were analyzed. Mortality within 28 days was 4.5% and median LOS in the PICU was 18 days. Of 22 patients, 11 patients (50.0%) were extubated over 24 hours after surgery, and 8 patients (36.4%) required drainage for pleural effusions. Longer LOS in the PICU were noted in patients extubated over 24 hours (p = 0.008), complicated with significant pleural effusions (p = 0.02) after surgery, and having low initial postoperative PaO2/FiO2 (300 mmHg) (p = 0.001). Among clinical factors, massive intraoperative blood transfusion (40 mL/kg) was significantly associated with prolonged intubations, significant pleural effusions, low initial postoperative PaO2/FiO2, and prolonged LOS in the PICU (14 days). The initial postoperative PaO2/FiO2 significantly depended on age, disease severity (PELD score), and whether the patient received massive intraoperative blood transfusion.Pediatric patients of OLT with poor postoperative respiratory conditions including low initial PaO2/FiO2 ratio, extubation over 24 hours or significant pleural effusions have longer LOS in the PICU, and the requirement of massive intraoperative transfusion was a risk factor for both poor postoperative respiratory conditions and prolonged LOS in the PICU.

Background The advancements in neonatal critical care have not only improved the outcomes of extreme prematurity but also prolonged the process of death in terminally ill neonates. This study analyzed the characteristics of neonates who died at a single tertiary center in Taiwan. The utilization of neonatal hospice care before and after the legalization of life-sustaining treatment (LST) withdrawal in Taiwan in 2013 was also compared. Methods This study enrolled the neonatal mortality cases in the Taipei Veterans General Hospital during January 2008 to December 2017 through chart review. Data on birth history, primary diagnosis, complications, and death circumstances were recorded and analyzed. Results In total, 105 neonatal deaths were analyzed. The circumstances of death were as follows: 22 (21%) cases of full LST and cardiopulmonary resuscitation (CPR) performed until death; 63 (60%) cases of LST initiated but no more CPR after do-not-resuscitate (DNR) consents signed; 8 (7.6%) cases of LST withdrawn; 4 (3.8%) cases of DNR signed without LST initiation; 3 (2.9%) cases of CPR not performed, although no DNR signed; and 5 (4.8%) cases of discharge against medical advice under critical condition. The incidence of written DNR consents (57.9% in 2008-2009 vs 93.8% in 2016-2017; p = 0.02) showed an increasing trend. Regarding the incidence of comorbidities, renal failure rate was higher in the DNR group than in the non-DNR group (p = 0.002). Conclusion There was an increasing trend for written DNR consent and the utilization of neonatal hospice care. Renal failure, as a comorbidity, was significantly associated with the written DNR consent in the neonates. Further studies to evaluate the factors associated with neonatal hospice care utilization are suggested.

Background: Extubation failure (EF) in acute pediatric cases causes high morbidity and prolonged hospitalization, some of which might encounter EF repeatedly. This study aims to investigate flexible bronchoscopic findings of airway problems associated with repeated EF (REF) in children. Methods: We retrospectively reviewed the medical records of intubated children from 2005 to 2013 and enrolled those with EF (reintubated within 48 h after extubation) and receiving flexible bronchoscopy (FB) examinations. We divided all subjects into two groups, the REF group (reintubated within 48 h after FB examination) and control group (no need of reintubation), and compared the related clinical conditions and outcomes. Results: We assessed 30 children (REF group, 17 cases; control group, 13 cases). Among them, no significant difference was observed in age, weight, and underlying diseases. In the REF group, the outpatient ratio, tracheostomy rate, intubation days, respiratory or oxygen supported days, and EF episodes were significantly higher than the control group (p

Background: Standing height (SH) is the most reliable parameter used to predict spirometric values in children, but measurement of this parameter may be difficult in children with thoracic or spinal abnormalities. This study was designed to establish reference equations to estimate SHs of children using their arm span length (ASL) or forearm ulnar length (UL) as an index. Methods: Children aged 1–17 years were enrolled to measure their SH, body weight, ASL, and UL. Sex and age were also recorded. The relationship between SH and children's weight, age, ASL, and UL were analyzed. Regression equations using different indexes for SH of enrolled cases were used, and adults aged 18–64 years were also enrolled for comparison. Results: A total of 512 children and 144 adults were enrolled. There was a strong linear relationship between SH and both ASL and UL in children and adults. Pearson's correlation coefficients of SH for ASL and UL were 0.989 and 0.968 (p

Air leak syndrome includes pulmonary interstitial emphysema, pneumothorax, pneumomediastinum, pneumopericardium, pneumoperitoneum, subcutaneous emphysema, and systemic air embolism. The most common cause of air leak syndrome in neonates is inadequate mechanical ventilation of the fragile and immature lungs. The incidence of air leaks in newborns is inversely related to the birth weight of the infants, especially in very-low-birth-weight and meconium-aspirated infants. When the air leak is asymptomatic and the infant is not mechanically ventilated, there is usually no specific treatment. Emergent needle aspiration and/or tube drainage are necessary in managing tension pneumothorax or pneumopericardium with cardiac tamponade. To prevent air leak syndrome, gentle ventilation with low pressure, low tidal volume, low inspiratory time, high rate, and judicious use of positive end expiratory pressure are the keys to caring for mechanically ventilated infants. Both high-frequency oscillatory ventilation (HFOV) and high-frequency jet ventilation (HFJV) can provide adequate gas exchange using extremely low tidal volume and supraphysiologic rate in neonates with acute pulmonary dysfunction, and they are considered to have the potential to reduce the risks of air leak syndrome in neonates. However, there is still no conclusive evidence that HFOV or HFJV can help to reduce new air leaks in published neonatal clinical trials. In conclusion, neonatal air leaks may present as a thoracic emergency requiring emergent intervention. To prevent air leak syndrome, gentle ventilations are key to caring for ventilated infants. There is insufficient evidence showing the role of HFOV and HFJV in the prevention or reduction of new air leaks in newborn infants, so further investigation will be necessary for future applications.

Background To evaluate the influence of hyperoxia on neurotransmitters in the developing brain of newborn piglets. Methods Ten newborn piglets were randomly assigned to hyperoxia (inhaled 100% oxygen) or control (inhaled room air) groups and ventilated for 4 hours. Blood samples were obtained at 0, 15, 30, and then every 30 minutes for 4 hours. Extracts of whole brain tissue were assayed for dopamine, serotonin, and their metabolites using high-performance liquid chromatography. Results In comparison with the control group, there was a general trend of lower neurotransmitter content in the brains of the hyperoxia group. In addition, the levels of dopamine and 3,4-dihydroxyphenylacetic acid in the left frontal lobe, and serotonin in the right occipital lobe and left frontal lobe, of the hyperoxia group were significantly lower compared with the control group ( p Conclusion The results indicate that hyperoxia may alter the production or metabolism of dopamine and serotonin in some cortical areas of the neonatal central nervous system, and it tended to have some inhibitory effects. Therefore, pediatricians should be very judicious in using high oxygen on the developing brain.

Background To investigate the incidence, clinical presentation and possible etiologies or risk factors of early onset of sudden and unexpected death or near-miss. Methods From 2001 to 2005, a retrospective analysis of observational database of neonates who were younger than one week old without any risk factors at five tertiary medical centers. The demographic data, clinical manifestations, laboratory data and possible etiologies were retrospectively collected and analyzed. Results Seventeen neonates presumed to be healthy at birth encountered early near death in five medical centers in Taipei city. The mean gestation age (GA) was 38.5 ± 1.2 weeks, mean birth body weight (BBW) was 2948.2 ± 327.8 gm. The median age at event was 26 hours old. Eleven patients were rooming-in babies with exclusive breast feeding. Seven patients (41.2%) died; seven patients (41.2%) survived with neurological sequelas, and the remaining three patients (17.6%) survived without complication. Possible causative factors included infection in two cases, urea cycle disorder in one case, hypertrophic cardiomyopathy in one case, hypocalcemia only in one case, hypocalcemia plus airway obstruction in one case, dehydration-related diseases in seven cases and unknown in 4 cases; there was no autopsy case. Conclusion More effort on promotion of autopsy to discover the underlying disease is necessary and helpful. To build up an alarm system or protocol for education and early detection is the basis to prevent this tragedy.

Background: Supplemental oxygen (O2) is mandatory during flexible bronchoscopy (FB) in infants, but there are limited studies that deal with the efficacy of different O2 delivery methods. This study aims to compare the oxyhemoglobin saturation in infants during FB among three different O2 delivery methods, as measured by pulse oximeter (SpO2). Methods: A prospective study enrolled infants with two criteria: (1) less than 2 years old; and (2) needing FB examination. All infants received intravenous sedation and topical anesthesia. They were randomly placed into the following three groups: (1) nasal cannula (NC; 0.5 L/kg/min); (2) nasal prongs with continuous positive airway pressure (NP-CPAP; 5–10 L/min, pressure 5 cmH2O); and (3) nasopharyngeal catheter (NPC; 0.3–0.5 L/kg/min). SpO2, heart rate, blood pressure and respiratory rate were measured and compared at different stages: (1) prior to the FB (baseline); the FB tip at (2) the nose tip; (3) the pharynx; (4) the carina and (5) 30 minutes after the FB. Results: A total of 75 infants, with 25 infants per group, were enrolled during a 2-year period. There were no significant differences in basic characteristics and baseline SpO2. After the designated O2 was delivered, SpO2 decreased significantly (p

BackgroundThe objective of this retrospective cohort study was to compare the patent ductus arteriosus (PDA) closure rate with different routes (intravenous and enteral) of indomethacin treatment and neonatal outcomes.MethodsInfants with a birthweight < 1,000 g born between July 1997 and June 2007 at Taipei Veterans General Hospital and who received indomethacin treatment for PDA were included in the study. Outcome measures were ductal closure rate and neonatal outcomes.ResultsOf 41 extremely-low-birth-weight infants with PDA, 3 infants had spontaneous closure and 3 died before treatment. Of the remaining 35 infants, 13 received enteral ethanol solution of indomethacin and 22 received the intravenous (IV) form. The total closure rates of the IV and enteral groups were 81.8% and 76.9%, respectively. There were no significant differences in the incidence of impaired renal function, necrotizing enterocolitis, chronic lung disease or severe retinopathy of prematurity between the 2 groups.ConclusionOur results suggest that ethanol-based indomethacin is an effective alternative to IV indomethacin for the pharmacological closure of PDA in extremely-low-birth-weight infants.

Background Spirometry is a valuable technique for evaluating pulmonary function, but there were few normative reference values for young children in Taiwan, and none for the last 10 years. The objective of our study was to establish updated reference values and equations for children aged 6–11 years in northern Taiwan. Methods A total of 309 healthy children (153 boys and 156 girls) were enrolled in the present study. The data of at least 3 trials for each child were collected, and the highest values analyzed. The analyzed pulmonary function parameters were focused on forced vital capacity (FVC), forced expiratory volume in 1 second (FEV 1 ), peak expiratory flow rate (PEF), forced expiratory flow between 25% and 75% expired volume (FEF 25–75 ), and the ratio of FEV 1 /FVC. Results The results revealed that there were mostly no significant differences between boys and girls, and the standing height (H, cm) was the factor with the highest correlation with the pulmonary function parameters. Regression equations of the major pulmonary function parameters for both boys and girls were obtained: FVC=−2.690 + 0.0330H; FEV 1 = −2.559 + 0.0311H; PEF=−300.231 + 3.938H; FEF 25–75 = −3.218 + 0.0425H ( p Conclusion Our study determined the updated normative values and reference equations for Chinese children aged 6–11 years living in northern Taiwan. These values can be used as normative reference values to evaluate pulmonary function in diseased children with the same ethnicity and lifestyle.

Background Esophageal atresia (EA), tracheoesophageal fistula (TEF), or both is a complicated problem. The purpose of this study was to evaluate the outcomes and postoperative complications in patients with EA/TEF who were admitted to our hospital. Methods In total, 15 patients were enrolled from 1994 to 2003, including 8 males and 7 females. Patient demographics, associated anomalies, and outcomes were analyzed. Results The most common variant was EA with a distal TEF (type C), which occurred in 12 patients (80%). The latter had associated congenital anomalies, and cardiac anomalies were the most frequent, occurring in 8 patients (53.3%). Of the 6 cases who had life-threatening anomalies, 4 (66.7%) died, and of the 9 cases who had no life-threatening anomalies, 2 (22.2%) died. Tracheomalacia and/or stenosis were diagnosed in 8 patients (66.7%) postoperatively. Though 3 of the 4 cases who suffered from dying spell received intratracheal stent implantation, 2 cases still died. Conclusion The survival rate of the patients with EA/TEF is influenced mainly by associated life-threatening anomalies. TMS combined with a history of dying spell may be the major fatal complication.

Background Ultrasonography is a non-invasive diagnostic technique, and it has been used to detect intracranial lesions in neonates for a long time. Correspondingly, screening tests using cranial ultrasonography have been applied for early detection of intracranial lesions in full-term neonates during the past decade. Methods We retrospectively reviewed the findings of cranial ultrasonographic screening tests in healthy full-term neonates between September 2004 and August 2009. The ultrasonographic findings were divided into the following categories: (a) nonsignificant (NS) group, including normal and normal variations, (b) minor anomaly group, including tiny cystic lesions, mild hemorrhage, or mild ventricular anomaly, and (c) major anomaly group, including significant anomaly of any intracranial pathology. The participants with major anomalies were further reviewed, and the following medical records of all enrolled patients were reviewed until they were 24 months of age. Results There were a total of 3186 neonates who received cranial ultrasonographic screening examination during the 5-year period, and most of them (2982 cases, 93.6%) were assigned to the NS group. The most common normal variation was the presence of cavum septum pellucidum (1979 cases, 62.1%). Minor anomalies were found in 202 (6.3%) neonates, including 119 (3.7%) neonates with tiny cysts, and 59 (1.9%) neonates with mild intraventricular hemorrhage. Major anomalies were found in two (0.06%) neonates, including obstructive hydrocephalus and agenesis of the corpus callosum. Two other infants (0.06%) initially presented with minor anomaly or normal variation, but they were diagnosed as Moyamoya disease and neonatal seizure some months later. Conclusion The incidence of minor and major anomalies detected by cranial ultrasonographic screening examinations in healthy full-term neonates is 6.3% and 0.06%, respectively. Thus, cranial ultrasonographic screening testing may play a role in the early diagnosis of intracranial anomalies of otherwise healthy neonates. However, this examination cannot exclude or detect all cranial abnormalities, including many potential neurologic diseases of neonates, so continuing clinical diligence is still important for all infants.

A 40-year-old, primigravid woman presented at 23 weeks of gestation for evaluation of an extra-abdominal echogenic cystic mass of the fetus. Amniocentesis revealed a karyotype of 46,XX. Prenatal ultrasound showed a two-vessel umbilical cord, hydrocolpos, and distended bladder, urethra, and colon, and a perineal cystic mass. The kidneys and amniotic fluid amount were normal. Fetal magnetic resonance imaging revealed ascites, hydrocolpos, distended urinary bladder and colon, high rectum, and a perineal cyst. The fetus postnatally manifested persistent cloaca. The perineum was distended and smooth, without patent anal, vaginal, and urethral openings. The external genitalia were ambiguous with no labia majora, labia minora, or clitoris. The perineal cyst had a very small single orifice. We suggest that cloacal anomalies be considered in any female fetus with hydrocolpos, distended bladder and colon, ascites, and a perineal cyst.