Clinical significance of assessing cerebral blood volume by time-domain near-infrared spectroscopy in children with congenital heart disease.

BACKGROUND: Despite providing cerebral tissue oxygen saturation (StO2 ), the lack of quantitative information for continuous wave near-infrared spectroscopy (CW-NIRS) is an obstacle in evaluating cerebral hemodynamic conditions. Time-domain NIRS (TD-NIRS) provides both StO2 and cerebral blood volume and has recently become clinically available. AIM: To investigate if the additional monitoring of cerebral blood volume by TD-NIRS facilitates the understanding of cerebral hemodynamic conditions in patients with congenital heart disease. METHODS: Preoperative TD-NIRS values were retrospectively reviewed in patients who underwent a cardiac surgery or catheter examination. We compared the values between patients with single and two ventricles. Moreover, we investigated the association of these values with the demographic and clinical variables. RESULTS: There was no significant difference in StO2 between single ventricle and two ventricles groups (median: 59.9 vs. 54.4, median difference [95% CI]: -4.06 [-9.90 to 2.90], p = .37). However, cerebral blood volume was significantly higher in the single ventricle group (median: 4.68 vs. 2.84, median difference [95% CI]: -2.01 [-2.88 to -1.06], p < .001). Spearman's rank correlation analysis demonstrated an association between StO2 and postmenstrual age (r = 0.35, p = .03). In contrast, cerebral blood volume was correlated with single ventricle physiology (r = 0.62, p < .001), postmenstrual age (r = 0.74, p < .001), central venous pressure (r = 0.38, p = .02), and SaO2 (r = -0.38, p = .02). The multivariable regression analysis identified the postmenstrual age, single ventricle physiology, and SaO2 as independent factors associated with cerebral blood volume. In the logistic analysis, cerebral blood volume was identified as a significant predictor of unfavorable conditions. CONCLUSION: Cerebral blood volume monitoring detected differences in cerebral hemodynamic conditions, related to the age and the type of ventricle physiologies. However, the differences were not apparent in StO2 . The additional monitoring of cerebral blood volume by TD-NIRS would facilitate a better understanding of cerebral hemodynamic conditions in patients with congenital heart disease.

The frequency of postoperative BNP measurement and intervention threshold of BNP concentration in pediatric cardiac intensive care unit: a prospective multicenter observational study.

PURPOSE: The purpose of this study is to investigate the current status of postoperative BNP measurement in the pediatric cardiac intensive care unit (PCICU). METHODS: This was a prospective multicenter observational study. Children under 15 years old who underwent pediatric cardiac surgery were included. Postoperatively, all BNP measurement was collected in PCICU. We checked whether each BNP measurement was used for the decision-making of intervention or not. We divided the BNP measurements into 4 groups: group A 0-299 pg/ml (reference), group B 300-999 pg/ml, group C 1000-1999 pg/ml, group D ≧ 2000 pg/ml. We performed logistic regression analysis to compare the intervention ratio between group A and B, C, D. We also did multiple comparison analyses to compare the intervention ratio in each group. RESULTS: Thirty-nine (15.8%) measurements were used as a criterion to intervene in all BNP measurements. There was no protocol for the measurement of BNP in all institutions. The number of BNP measurements in each group is as follows: group A 113 (45.9%), group B 81 (32.9%), group C 45 (18.3%), group D 7 (2.8%). The intervention ratio in each group was 6.2% (group A), 8.6% (group B), 44.4% (group C), and 71.4% (group D). The intervention ratio of group C and D were significantly higher than group A: (Odds ratio (95%CI): 12.1(4.8-33.9), p < 0.0001, 25.2(5.2-146.2), p < 0.0001). The result of multiple comparisons is similar to logistic regression analysis. CONCLUSION: High BNP concentration, especially more than 1000 pg/ml, was more often intervened upon compared to that of less than 1000 pg/ml.

Perioperative management of Fontan operation for the child with panhypopituitarism: a case report.

With the surgical improvement of congenital heart disease, Fontan operation has been applied to many complicated patients in recent years. This is the first report of a child with panhypopituitarism who underwent Fontan operation. A 5-year-old boy was scheduled for Fontan operation. He previously underwent Blalock-Taussig shunt and bidirectional Glenn operations for univentricular heart with double-outlet right ventricle and pulmonary atresia. He was receiving hydrocortisone and 1-desamino-8-D-arginine vasopressin (DDAVP) for panhypopituitarism secondary to removal of craniopharyngioma performed at the age of three years. Although urine output and serum sodium concentration were adequately controlled by adjustment of vasopressin infusion rate during surgery, massive pleural effusions and ascites developed postoperatively, which required several days for control by adjusting the dose of oral DDAVP and normalize the serum sodium level. Intraoperative management of Fontan operation for a patient with panhypopituitarism was controllable by appropriate hormone replacement. However, postoperative fluid management was complicated by the clinical features of panhypopituitarism and Fontan physiology.

Gene therapy improves motor and mental function of aromatic l-amino acid decarboxylase deficiency.

In patients with aromatic l-amino acid decarboxylase (AADC) deficiency, a decrease in catecholamines and serotonin levels in the brain leads to developmental delay and movement disorders. The beneficial effects of gene therapy in patients from 1 to 8 years of age with homogeneous severity of disease have been reported from Taiwan. We conducted an open-label phase 1/2 study of population including adolescent patients with different degrees of severity. Six patients were enrolled: four males (ages 4, 10, 15 and 19 years) and one female (age 12 years) with a severe phenotype who were not capable of voluntary movement or speech, and one female (age 5 years) with a moderate phenotype who could walk with support. The patients received a total of 2 × 1011 vector genomes of adeno-associated virus vector harbouring DDC via bilateral intraputaminal infusions. At up to 2 years after gene therapy, the motor function was remarkably improved in all patients. Three patients with the severe phenotype were able to stand with support, and one patient could walk with a walker, while the patient with the moderate phenotype could run and ride a bicycle. This moderate-phenotype patient also showed improvement in her mental function, being able to converse fluently and perform simple arithmetic. Dystonia disappeared and oculogyric crisis was markedly decreased in all patients. The patients exhibited transient choreic dyskinesia for a couple of months, but no adverse events caused by vector were observed. PET with 6-[18F]fluoro-l-m-tyrosine, a specific tracer for AADC, showed a persistently increased uptake in the broad areas of the putamen. In our study, older patients (>8 years of age) also showed improvement, although treatment was more effective in younger patients. The genetic background of our patients was heterogeneous, and some patients suspected of having remnant enzyme activity showed better improvement than the Taiwanese patients. In addition to the alleviation of motor symptoms, the cognitive and verbal functions were improved in a patient with the moderate phenotype. The restoration of dopamine synthesis in the putamen via gene transfer provides transformative medical benefit across all patient ages, genotypes, and disease severities included in this study, with the most pronounced improvements noted in moderate patients.10.1093/brain/awy331_video1awy331media15991361892001.

Influence of the aldehyde dehydrogenase 2 polymorphism on the vasodilatory effect of nitroglycerin in infants with congenital heart disease and pulmonary arterial hypertension.

PURPOSE: The influence of the aldehyde dehydrogenase 2 (ALDH2) gene polymorphism on the pharmacokinetics and haemodynamics of nitroglycerin (GTN) was determined in human subjects. METHODS: Eighteen infants (nine each with and without ALDH2 gene polymorphism) with congenital heart disease and pulmonary arterial hypertension participated in this study. GTN treatment started at a dose of 2 µg/kg/min, and the dose was escalated by 1-2 µg/kg/min until pulmonary vascular resistance (PVR) was reduced by more than 30%. The plasma GTN concentration and PVR were measured at the end of each infusion period. RESULTS: Plasma GTN concentrations were significantly higher in patients with the ALDH2 gene polymorphism than in those without the polymorphism. Conversely, the reduction in PVR was smaller in patients with the ALDH2 gene polymorphism than in those without. CONCLUSIONS: These data suggest that the ALDH2 gene polymorphism influences the pharmacokinetics and haemodynamics of GTN in human subjects.

The first case of hemorrhagic shock and encephalopathy syndrome with fulminant hypercytokinemia associated with pediatric COVID-19.

BACKGROUND: Infection by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) can lead not only to respiratory symptoms but also to neurologic symptoms with various levels of severity. After the worldwide prevalence of Omicron variant, severe neurological manifestations of coronavirus disease 2019 (COVID-19) such as febrile seizure, demyelinating disease, and cerebrovascular disease, have been reported. However, reports of acute encephalopathy in patients with COVID-19 are quite limited. Especially in terms of cytokine storm-inducing hemorrhagic shock and encephalopathy syndrome (HSES), there is no case reported related to COVID-19. CASE PRESENTATION: We describe the case of an 8-year-old girl who presented with fatal HSES associated with pediatric SARS-CoV-2 infection. Status epilepticus occurs after the onset of fever and diarrhea and lasted for at least an hour. Unconsciousness was followed by circulatory failure and ultimately leading to death within 2 days after the fever onset. Analysis of forty-eight cytokines and chemokines measured in three consecutive serum samples revealed that interferon (IFN)-γ, interleukin (IL)-6, IL-10, IL-17A, tumor necrosis factor (TNF)-a, IL-8, Interferon gamma inducible protein (IP)-10, and Monocyte chemoattractant protein (MCP)-1, were increased within an hour after the onset of impaired consciousness. CONCLUSION: Here, we describe a case of fatal fulminant encephalopathy with rapid progression because of HSES associated with COVID-19. High levels of cytokines and chemokines observed in this case may be because of the SARS-CoV-2-associated cytokine storm. This study is the first COVID-19-associated case of HSES.

[Use of RRa sensor in a pediatric patient with post-adenotonsillectomy].

Rad-87 and RRa are new acoustic monitoring devices which can monitor the respiratory rate. To our knowledge, no studies have reported the RRa sensor used in pediatric patients after surgery. We succeeded in measuring the respiratory rate with the RRa sensor in the Pediatric Intensive Care Unit(PICU). A 10-year-old boy, 14.5 kg in weight and 119.6 cm in height, with cerebral palsy, mental retardation, epilepsy, and obstructive sleep apnea due to adenoidal and tonsillar hypertrophy, was scheduled for adenotonsillectomy under general anesthesia. Anesthesia was maintained with oxygen, air, sevoflurane (1.5-2.0%), remifentanil (0.1 to 0.5 microg . kg-1. min-1), and fentanyl (4 microg . kg-1). The operating time was 55 minutes, and the duration of anesthesia was 133 minutes. After finishing the surgery, we attached the RRa sensor to his anterior neck and monitored his respiratory rate. Furthermore, RRa could count his respiratory rate, during transfer from the operating room to PICU. The patient was sedated with dexmedetomidine (0.28 microg . kg-1 . min-1) at PICU, and his respiratory rate was accurately measured with the RRa sensor. We hope that Rad-87 and RRa sensors will become useful for measuring the respiratory rate in pediatric patients in the future.

[General anesthesia for pediatric cardiac catheterization--effects of anesthetics on blood pressure, heart rate, oxygen saturation, and carbon dioxide].

BACKGROUND: There are limited data about the correlations between amount of anesthetics and variations in vital signs during pediatric cardiac catheterization. METHODS: Data in 80 children with congenital heart disease undergoing cardiac catheterization with/without interventional cardiology in 2004 were examined in this retrospective cohort study. Data on blood pressure, heart rate, oxygen saturation, partial tension in end tidal carbon dioxide (PETCO2), and total amount of anesthetics given during general anesthesia were obtained from anesthetic charts. The correlations between amount of anesthetics and those vital signs were analyzed. RESULTS: Median age of the patients was 14 months and median body weight was 8.8 kg. Median rates of variation in heart rate, blood pressure, oxygen saturation and PET(CO2) were 22.8%, 29.3%, 5.9% and 10.8%, respectively. Although there were no statistical correlations between those vital signs and amounts of anesthetics such as fentanyl, vecuronium and sevoflurane, rates of variation in heart rate was smaller in patients for whom the amount of fentanyl given was more than 4 microg x kg(-1) x hr(-1). CONCLUSIONS: There were no associations between amount of anesthetics and variations in vital signs in pediatric cardiac catheterization.

[Case report of endotracheal intubation using AirWay Scope (Pentax-AWS) in a Forestier's disease patient].

A Forestier's disease patient was scheduled for endoscopic mucosal resection under general anesthesia, because of his hypoxic episode during gastric endoscopy. Endotracheal intubation was planned while awake, because he was suspected as a case of difficult airway. By using AWS, we could easily confirm his larynx and aditus of trachea in spite of his narrow pharynx caused by Forestier's disease. The procedure was successful with no complications. AWS seems to be a useful device for endotracheal intubation in Forestier's disease.

[Airtraq optical laryngoscope: clinical assessment of its performance in 100 children].

BACKGROUND: The aim of the present study was to evaluate the suitability of Airtraq optical laryngoscope for the tracheal intubation in children. METHODS: Endotracheal intubation was performed using the Airtraq optical laryngoscope in 100 pediatric patients undergoing general anesthesia. The time to complete tracheal intubation and optimizing procedures were recorded. RESULTS: The Airtraq optical laryngoscope allowed visualization of the glottis and successful intubation in the 100 patients, including three patients with difficult airway. Utilization of a gum elastic bougie was helpful to introduce a tube tip to the trachea. CONCLUSIONS: The Airtraq optical laryngoscope might be an alternative apparatus for endotracheal intubation in pediatric patients.

Bispectral Index™ variation during living donor liver transplantation in a child with hepatic encephalopathy: a case report.

BACKGROUND: It is unclear whether perioperative Bispectral Index™ (BIS) monitoring in pediatric cases with acute liver failure (ALF) is effective for evaluation of neurological function. We describe a pediatric patient with hepatic encephalopathy (HE) in whom the BIS value increased from low levels to the normal range during liver transplantation (LT). CASE PRESENTATION: Electroencephalography in a 6-year-old comatose girl diagnosed with ALF and HE who was unresponsive to pain and auditory stimuli revealed continuous slow waves, and hence, emergency LT was performed. Intraoperatively, BIS values remained low until reperfusion. However, BIS value variability increased after reperfusion. She was subsequently discharged without any neurological sequelae. CONCLUSIONS: Low BIS values were considered to reflect the severity of HE. It is possible that improvement of the BIS value and waveform was a reflection of graft function. BIS monitoring might be a good indicator of neurological recovery after LT.

Dopaminergic restoration of prefrontal cortico-putaminal network in gene therapy for aromatic l-amino acid decarboxylase deficiency.

Aromatic l-amino acid decarboxylase (AADC) is an essential dopamine-synthesizing enzyme. In children with AADC deficiency, the gene delivery of AADC into the putamen, which functionally interacts with cortical regions, was found to improve motor function and ameliorate dystonia. However, how the restoration of dopamine in the putamen in association with cortico-putaminal networks leads to therapeutic effects remains unclear. Here, we examined neuroimaging data of eight patients with AADC deficiency (five males and three females, age range 4-19 years) who received the AADC gene therapy of the bilateral putamen in an open-label phase 1/2 study. Using high-resolution positron emission tomography with a specific AADC tracer, 6-[18F]fluoro-l-m-tyrosine (FMT), we showed that FMT uptake increased in the broad area of the putamen over the years. Then, with the structural connectivity-based parcellation of the putaminal area, we found that motor improvement is associated with dopaminergic restoration of the putaminal area that belongs to the prefrontal cortico-putaminal network. The prefrontal area dominantly belongs to the frontoparietal control network, which contributes to cognitive-motor control function, including motor initiation and planning. The results suggest that putaminal dopamine promotes the development of an immature motor control system, particularly in the human prefrontal cortex that is primarily affected by AADC deficiency.

[Work load of anesthesiologists at the Central Surgical Unit of the Jichi Medical University Hospital].

BACKGROUND: Since the diagnosis procedure combination (DPC) for health insurance plans in Japan was started in medical practice, the number of surgical procedures is increasing at teaching hospitals. METHODS: We retrospectively surveyed 8,672 surgical procedures performed at the central surgical unit of the Jichi Medical University Hospital from April 1, 2007 to March 31, 2008. RESULTS: Of the 8,672 surgical procedures, 6,922 operations were performed under the management of anesthesia staffs, and 1,904 procedures (27.5%) were done in emergency situation. Central surgical unit has 14 operating rooms and an estimated maximum number of surgical procedures is 7,700. CONCLUSIONS: This survey revealed that the present status of manpower of anesthesiologists at our hospital was insufficient for the work. In particular, the demands for anesthesiologists have increased in emergency operations including major cardiovascular surgery, neurosurgery and liver transplantations.

[Use of the GlideScope video laryngoscope in 50 pediatric patients].

BACKGROUND: The GlideScope video laryngoscope (Verathon Inc. Bothell, Washington, USA) is a relatively new device for tracheal intubation, which provides a excellent glottic visualization. We here report the clinical experience of the GlideScope (small) in 50 pediatric patients. METHODS: Tracheal intubation with GlideScope (small) was performed in 50 consecutive pediatric patients requiring orotracheal intubation for surgery. The view of glottic opening was scored according to the classification of Cormack-Lehane. The time required to intubate and the number of intubation attempts were recorded. RESULTS: In all, 50 children included 4 neonates, 8 infants under 1 year and 38 children between 1 year and 9 years. Cormack-Lehane classification 1 or 2 was obtained in 74% and 22%, respectively, and successful intubation was achieved in 48 of 50 children (96%). In remaining two babies, GlideScope failed to intubate the trachea. The mean +/- SD time for instrumentation in successful intubation at first attempt was 56.6 +/- 34.2 seconds. CONCLUSIONS: GlideScope seemed to be a novel device in pediatric patients. Further studies are required to evaluate the usefulness in neonates, small infants and children with a difficult airway.

[A case report of successful treatment of a child with respiratory distress using non-invasive continuous positive airway pressure via helmet].

Helmet is a new device of non-invasive continuous positive airway pressure (CPAP). Few cases have been described about usage of the helmet in children. We describe successful treatment of a child with respiratory distress using the helmet-delivered non-invasive CPAP. A 2-month-old male infant (3.1 kg) with multiple anomalies (cardiovascular, facial, and vertebral) developed respiratory distress after extubation. The helmet was well tolerated regardless of facial anomaly. Helmet CPAP started at initial settings of CPAP 8 cm H2O and FI(O2) 0.7, improved oxygenation. Pa(O2)/FI(O2) ratio increased from 106 to 316, and chest X-rays showed a marked improvement (15 hour after NPPV initiation). The helmet offers important advantage: the possibility of fitting to any children, regardless of any facial or external anomalies.

[Crisis management in pediatric anesthesia].

We describe the risk management of pediatric anesthesia. The most important risk management of pediatric anesthesia is airway and temperature management. Neonates and infants easily become hypoxic due to their insufficient functional residual capacity. Therefore airway management is most important not only during induction of anesthesia but also during maintenance of anesthesia and extubation. The management of patients' temperature, including control of room temperature should be taken into consideration. In addition, careful attention should be paid not to introduce air bubbles in any lines, especially in patients with congenital heart diseases.

[Study on the working conditions of rotating residents in the department of anesthesia under the new residency programs].

BACKGROUND: Jichi Medical University Hospital established a new residency program in 2004, which restricted the working time of junior residents to less than 80 hours for one week involving conferences and lectures. METHODS: We evaluated the working conditions of non-anesthesia residents in the department of anesthesia from April 2005 to March 2006. RESULTS: Non-anesthesia residents were engaged in anesthesia for 112 +/- 37 (mean +/-SD) hours for one month. CONCLUSIONS: Non-anesthesia residents worked in the anesthesia department under the regulation of the new residency program. However, a significant increase in the number of anesthesia cases, observed in recent years, is a potential imperilment of the new residency program.

[Use of the airway scope for difficult airway].

The Airway Scope (AWS) is a new videolaryngoscope which consists of an optical system and a single-use blade. The blade of the device is designed according with the pharyngeal anatomy, and thus the AWS is expected to have a role in the management of difficult airway. We here report 15 patients with difficult airway in whom the AWS provided in successful intubation. The AWS provides a view of the glottis with Cormack-Lehane grade 1 and resulted in successful intubation in the 15 patients. The AWS is useful for the management of difficult airway.

[GlideScope videolaryngoscope: a clinical assessment of its performance in 200 consecutive patients].

BACKGROUND: The GlideScope videolaryngoscope is a new device for endotracheal intubation, which provides a view of the glottis without alignment of the oral pharyngeal and tracheal axes. The purpose of this study was to evaluate the performance of the GlideScope in patients. METHODS: We evaluated the performance of the GlideScope" in 200 consecutive patients requiring orotracheal intubation for surgery. RESULTS: In the 200 patients, excellent (Cormack-Lehane [C-L] view 1) or good (C-L 2) laryngeal exposure was obtained in 68% and 31% respectively and successful orotracheal intubation was achieved in all the patients. The mean +/- SD time to intubate was 51 +/- 20 seconds in trainees, 52 +/- 25 seconds in junior anesthesia residents, 48 +/- 18 seconds in senior anesthesia residents, and 50 +/- 18 seconds in staff anesthetists. CONCLUSIONS: The GlideScope was easily handled not only by experienced anesthetists but also by novice personnel. The GlideScope seems to be a novel device in routine and difficult airway management.