Intellectual development of patients with biliary atresia who underwent living donor liver transplantation in infancy.

BACKGROUND: The impact of pediatric liver transplantation on intellectual development has yet to be determined. We investigated the intellectual outcomes of school-aged patients after living donor liver transplantation for biliary atresia in infancy. METHODS: The Wechsler Intelligence Scale for Children-fourth edition test was administered to 20 patients who survived [Formula: see text] 5 years after living donor liver transplantation. Borderline full scale intelligence quotient was defined as ≤ 85. Pre-, peri-, and postoperative data were compared between patients with > 85 and ≤ 85 to identify predictive factors of borderline performance. RESULTS: The one-sample t test demonstrated that the mean full scale intelligence quotient of patients after transplantation for biliary atresia was significantly lower than that of the general population (91.8 vs. 100.0, p = 0.026) and 7 (35%) were classified as intellectual borderline functioning. Multivariable logistic regression models were unable to identify any factors predictive of full scale intelligence quotients of ≤ 85. CONCLUSION: This is the first study to indicate that the mean full scale intelligence quotient among school-aged patients who underwent living donor liver transplantation for biliary atresia in infancy is significantly lower than that of the general population.

The duodenal tube test is more specific than hepatobiliary scintigraphy for identifying bile excretion in the differential diagnosis of biliary atresia.

PURPOSE: Confirmation of bile excretion into the gastrointestinal tract is important to exclude biliary atresia (BA). We compared the duodenal tube test (DTT) with hepatobiliary scintigraphy (HS) for their efficiency in detecting bile secretion. METHODS: The subjects of this retrospective study were 47 infants who underwent both DTT and HS to diagnose or exclude BA between January 2000 and March 2018. RESULTS: BA was diagnosed in 32 of the 47 patients, and 7 of the remaining 15 non-BA patients underwent intraoperative cholangiography. Among the various DTT parameters, the total bile acid in duodenal fluid (DF-TBA)/serum (S) gamma-glutamyl transferase (γGTP) ratio was found to be the most specific for BA, with sensitivity and specificity of 98.0-100%, respectively. One BA patient in whom cut off values were not met was a premature infant. The sensitivity and specificity of HS were 100-56.3%, respectively. The diagnostic accuracy of the DF-TBA/S-γGTP parameter was higher than that of HS (98.6% vs. 85.1%, respectively). CONCLUSIONS: The DTT could be more a specific method than HS to detect bile excretion. Thus, the DTT should be incorporated into the multidisciplinary diagnostic approach for the differential diagnosis of BA to prevent unnecessary intraoperative cholangiography in patients who do not have BA.

Pretransplant trends in α-fetoprotein levels as a predictor of recurrence after living donor liver transplantation for unresectable hepatoblastoma: A single-institution experience.

LT is a practical therapeutic alternative for unresectable hepatoblastoma; however, deciding when to perform LT is difficult. The aim of this study was to optimize the timing of LT for hepatoblastoma using pretransplant trends in AFP levels. Trends in pretransplant AFP levels and their influence on post-transplant outcomes were retrospectively evaluated. All patients who underwent living donor LT for hepatoblastoma in our institution since 2002 were included. Variables analyzed included history of prior tumor resection, pretransplant AFP responses to chemotherapy, metastatic disease at diagnosis, and post-transplant chemotherapy. Eight patients (seven boys and one girl; median age, 35 months; range, 15 months-12 years) were transplanted. The overall post-transplant recurrence-free survival rate was 62.5% (5/8) with a mean follow-up of 77 months. Patients with post-transplant recurrence showed a 0.573 log increase in AFP levels after the last chemotherapy session before LT. This was significantly higher than the 0.279 log decrease observed in patients without post-transplant recurrence (P = .024). Because the AFP response cannot be accurately predicted before each cycle of chemotherapy, it may be appropriate to perform LT when AFP levels do not decrease after the last cycle and before they are found to be elevated again.

[Two Separate Instances of Anesthetic Management in a Patient Initially Diagnosed with Stiff-person Syndrome].

In this case report, we describe separate instances of general anesthetic management administered to a patient during treatment for two unrelated conditions. The patient, a 57-year-old woman who had been experiencing walking difficulties for about four years, fell down because of muscle rigidity and spasms and fractured her humerus. She was subsequently diagnosed with stiff-person syndrome. The fracture was treated conservatively, but three weeks later the alignment of the humerus became worse and the patient was scheduled to undergo an open reduction and internal fixation under general anesthesia (GA). Anesthesia was induced using propofol and fentanyl, and rocuronium was given for the tracheal intubation. The operation was completed successfully while anesthesia was maintained using sevoflurane and remifentanil without incident Four months later, the patient developed paraneoplastic syndrome stemming from breast cancer, and underwent a resection of the cancer under GA. The patient received propofol for the laryngeal mask insertion, and sevoflurane and pentazocine for maintenance of anesthesia. She was discharged from the hospital without any respiratory or airway problems. We used sevoflurane for maintenance of anesthesia on both occasions, and rocuronium for the first tracheal intubation. The patient's hemodynamics were stable during the operation. No prolonged effect of the muscle relaxant or any autonomic reactions were observed. The patient was fully conscious and in good respiratory condition after both surgeries, and was extubated in the operating room. There were no perioperative complications.

[Anesthesia for electroconvulsive therapy -- comparison of propofol with sevoflurane].

BACKGROUND: Electroconvulsive therapy (ECT) is performed under general anesthesia (GA). GA could reduce the vigorous hemodynamic response including hypertension and tachycardia during ECT. This may be beneficial in patients with cardiovascular diseases. On the other hand, however, many intravenous and inhalational anesthetic agents potentially suppress the seizure and shorten the duration. The aim of this study is to elucidate whether propofol is superior to sevoflurane in maintaining the duration of electroencephalographic (EEG) seizure evoked by Thymatron System while stabilizing hemodynamic responses. METHODS: Thirteen patients (male:female = 3:10) were included in this cross-over study. The patients underwent 54 ECT sessions in total. The sessions were randomized into two groups. In the propofol group (P group), the patients received intravenous propofol 1 mg x kg(-1) on induction of anesthesia, whereas those in the sevoflurane group (S group) were induced with sevoflurane at 5% inspired concentration. In either group, after loss of consciousness, the patients received 1 mg x kg(-1) of suxamethonium. When muscle fasciculation faded away, the electrostimulus was delivered. The duration of EEG seizure, heart rate (HR) and noninvasive mean arterial blood pressure (MAP) were recorded. RESULTS: The duration of EEG seizure was longer in P group (34.6 +/- 15.5 sec versus 23.6 +/- 12.0 sec in S group, P = 0.006). There was no significant difference in hemodynamic parameters (MAP, HR) between the two groups. CONCLUSIONS: The duration of EEG seizure in ECT was significantly longer with propofol anesthesia than sevoflurane anesthesia. Propofol may provide more benefits than sevoflurane as the anesthetic for ECT.

Japanese anesthesiologists' knowledge of the cost of medicines and their attitudes toward cost containment: a cross-sectional survey.

Objectives: This study surveyed Japanese anesthesiologists' knowledge of the cost of medicine and their attitudes toward cost containment to determine how these factors may affect their choice of medication and provide insight into reducing healthcare expenditures. Materials and Methods: In this cross-sectional study, Japanese anesthesiologists' knowledge of medicine prices and their attitudes toward cost containment were surveyed to identify barriers in lowering the cost of anesthesia. The proportion of participants who correctly guessed the cost of the five most frequently used types of drugs within 25% of the actual price was determined, and their attitudes regarding drug prices and barriers to achieving cost containment were analyzed. Results: In total, responses to 60 questionnaires were analyzed. The proportion of participants correctly guessing the price within 25% of the actual price for each of the five drug categories, including neuromuscular blocking agents, inhaled anesthetics, intravenous anesthetics, opioids, and neuromuscular blockade reversal agents, was 30% (n=18), 18.3% (n=11), 6.67% (n=4), 30.0% (n=18), and 63.3% (n=38), respectively. Participants believed they had adequate access to information on prices, that the cost of a product influenced their decisions regarding product use, and that more information about cost might change their use of the drugs but did not believe that they had adequate knowledge about product prices. Conclusion: The proportion of participants with acceptable price estimates is small. Educating anesthesiologists about the cost of anesthetic agents may be required to encourage cost-containing behaviors. This study is the first survey to assess Japanese anesthologists' knowledge of the cost of medicines.

Practicality Assessment of the Improved ICT-Based Dementia Care Mapping Support System.

OBJECTIVES: Authors have developed an ICT (Information-Communication Technology)-based Dementia Care Mapping (DCM™) support system and assessed its usefulness previously using videos. The aim of this study is to evaluate practicality of the improved support system by actual mapping. METHODS: We obtained highly reliable mapping data by a team of 3 mappers (M1, M2, and M3), who mapped 5 elderly people with dementia continuously over 4 hours in 2 sites. M1 mapped by conventional paper-based DCM™, while M2 and M3 mapped using the support system. Collected mapping data (n = 8, total of 232.5 hours = 2790 time frames) were compared. RESULTS: The concordance rates between M1 and M2/M3 were over 70%; the required level for DCM™ evaluation purposes, with 1 exception. We also obtained users' positive ratings regarding the system usability. CONCLUSION: Practicality of the ICT-based DCM™ support system was confirmed for evaluation purposes, demonstrating the system is ready for practical use.

Hepatoblastoma with multiple tumors in a school-aged child.

Clinicians need to consider hepatoblastoma in the differential even in school-aged children or adolescents presenting with multiple liver tumors.

Multicenter, retrospective, comparative study of laparoscopic and open Kasai portoenterostomy in children with biliary atresia from Japanese high-volume centers.

BACKGROUND: Multicenter study was undertaken to analyze the results of laparoscopic and open Kasai portoenterostomy. METHODS: Subjects were infants with type III biliary atresia who underwent open operation (n = 106) or laparoscopic operation (n = 21) between January 2012 and December 2015. Clinical data were compared between open and laparoscopic operations (2016-0534). Propensity score matching was performed to reduce the effect of treatment selection bias. Multivariate analyses were used to estimate the effect of the surgical approach on the jaundice clearance rate and the native liver survival rate. RESULTS: The postoperative jaundice clearance rate and the 1-year native liver survival rate were not significantly different between open and laparoscopic operations. Rates of cholangitis and major complications of laparoscopic operation were comparable to those of open operation. Blood loss, time to resume oral intake, time to drain removal, and duration of analgesic usage of laparoscopic operation were significantly superior to those of open operation. Similar results were observed when analysis was adjusted based on propensity score. Multivariate analyses demonstrated that only age at operation was a poor prognostic factor. CONCLUSION: Laparoscopic Kasai portoenterostomy was associated with several favorable perioperative outcomes compared with open Kasai portoenterostomy. The difference of surgical approach was not a significant independent predictor.

Long-term Outcomes of ABO-incompatible Pediatric Living Donor Liver Transplantation.

BACKGROUND: ABO-incompatible (ABOi) living donor liver transplantation (LDLT) has been performed to compensate for donor shortage. To date, few studies have reported detailed B-cell desensitization protocols and long-term outcomes of ABOi pediatric LDLT. METHODS: Twenty-nine pediatric ABOi LDLT recipients were retrospectively analyzed. We compared the clinical outcomes between ABOi (n = 29) and non-ABOi (n = 131) pediatric LDLT recipients. Furthermore, we evaluated the safety and efficacy of our rituximab-based regimen for ABOi pediatric LDLT (2 ≤ age < 18; n = 10). RESULTS: There were no significant differences in the incidence of infection, vascular complications, biliary complications, and acute cellular rejection between ABOi and non-ABOi groups. The cumulative graft survival rate at 1, 3, and 5 years for non-ABOi group were 92.1%, 87.0%, and 86.1%, and those for ABOi group were 82.8%, 82.8%, and 78.2%, respectively. Rituximab-based desensitization protocol could be performed safely, and reduced CD19+ lymphocyte counts effectively. Although rituximab-treated ABOi group showed comparable clinical outcomes and graft survival rate, 2 patients developed antibody-mediated rejection. CONCLUSIONS: ABOi LDLT is a feasible option for pediatric end-stage liver disease patients. However, it should be noted that current desensitization protocol does not completely prevent the onset of antibody-mediated rejection in several cases.

[High dose L-dopa infusion during general anesthesia for gastrectomy in a patient with parkinsonism].

A 68-year-old man with parkinsonism was scheduled for gastrectomy. Levodopa 1400 mg, droxidopa 300 mg and bromocriptine-mesylate 7.5 mg had been administered orally per day to control the symptom before surgery. On the day before surgery, oral medication was stopped and intravenous infusion of levodopa 100 mg.h-1 was started. Without any premedication but with levodopa infusion, anesthesia was induced with thiopental 175 mg and fentanyl 0.05 mg. Tracheal intubation was facilitated with vecuronium 6 mg and an epidural catheter was inserted. Anesthesia was maintained with O2, N2O and sevoflurane, combined with epidural block using mepivacaine. When blood pressure decreased, phenylephrine but not ephedrine was effective to increase blood pressure. Intravenous infusion of levodopa was continued for 19 days with decreasing doses from 8th postoperative day when injection of levodopa into the intestinal tube was started. On the 53rd day, he left the hospital without any complications. Serum concentrations of levodopa during and after surgery were 50 to 100 times higher than the therapeutic levels. However, he developed no complications, which suggests a wide safety range of levodopa. In conclusion, high dose levodopa infusion was effective in controlling the symptoms of Parkinsonism during general anesthesia.

[The effect of intrathecal fentanyl added to hyperbaric bupivacaine for caesarean section].

BACKGROUND: Management of cesarean section with spinal anesthesia is often accompanied with intraoperative nausea and pain. In a randomized controlled study, we explored the effect of intrathecal fentanyl on the characteristics of subarachnoid block in patients undergoing cesarean section. METHODS: Twenty-four healthy parturients scheduled for elective Cesarean section were allocated to receive either fentanyl 0.3 ml (15 micrograms) or 0.9% saline 0.3 ml added to 0.5% hyperbaric bupivacaine 2.0 ml given intrathecally in the right decubitus position (n = 12 in each group). Level of sensory blockade was evaluated with cold test and intraoperative use of antiemetics and analgesics was recorded. RESULTS: The maximum level of sensory blockade was significantly higher in the fentanyl group as compared with the control group (P = 0.019). Use of intraoperative antiemetics was significantly less often in the fentanyl group (P = 0.007). The required amount of intraoperative analgesics was smaller in the fentanyl group, although the difference was not significant (P = 0.11). No remarkable side effects, such as respiratory depression and hypoxia were observed. Apgar scores in the newborn were similar. CONCLUSION: Addition of intrathecal fentanyl to hyperbaric bupivacaine in parturients undergoing cesarean section improved quality of anesthesia without producing significant side effects.

Efficacy and safety of inhaled carbon monoxide during pulmonary inflammation in mice.

BACKGROUND: Pulmonary inflammation is a major contributor to morbidity in a variety of respiratory disorders, but treatment options are limited. Here we investigate the efficacy, safety and mechanism of action of low dose inhaled carbon monoxide (CO) using a mouse model of lipopolysaccharide (LPS)-induced pulmonary inflammation. METHODOLOGY: Mice were exposed to 0-500 ppm inhaled CO for periods of up to 24 hours prior to and following intratracheal instillation of 10 ng LPS. Animals were sacrificed and assessed for intraalveolar neutrophil influx and cytokine levels, flow cytometric determination of neutrophil number and activation in blood, lung and lavage fluid samples, or neutrophil mobilisation from bone marrow. PRINCIPAL FINDINGS: When administered for 24 hours both before and after LPS, inhaled CO of 100 ppm or more reduced intraalveolar neutrophil infiltration by 40-50%, although doses above 100 ppm were associated with either high carboxyhemoglobin, weight loss or reduced physical activity. This anti-inflammatory effect of CO did not require pre-exposure before induction of injury. 100 ppm CO exposure attenuated neutrophil sequestration within the pulmonary vasculature as well as LPS-induced neutrophilia at 6 hours after LPS, likely due to abrogation of neutrophil mobilisation from bone marrow. In contrast to such apparently beneficial effects, 100 ppm inhaled CO induced an increase in pulmonary barrier permeability as determined by lavage fluid protein content and translocation of labelled albumin from blood to the alveolar space. CONCLUSIONS: Overall, these data confirm some protective role for inhaled CO during pulmonary inflammation, although this required a dose that produced carboxyhemoglobin values close to potentially toxic levels for humans, and increased lung permeability.

Effects of inhaled carbon monoxide on acute lung injury in mice.

Acute lung injury (ALI) and acute respiratory distress syndrome (ARDS) are major causes of morbidity and mortality in the intensive care unit, but despite continuing research few effective therapies have been identified. In recent years, inhaled carbon monoxide (CO) has been reported to have cytoprotective effects in several animal models of tissue injury. We therefore evaluated the effects of inhaled CO in three different in vivo mouse models of ALI. Anesthetized C57BL/6 mice were ventilated with oxygen in the presence or absence of CO (500 parts per million) for 1 h before lung injury was induced by lipopolysaccharide (LPS) or oleic acid (OA) administration. Ventilation was then continued with the same gases for a further 2-3 h, with hemodynamic and respiratory parameters monitored throughout. Intratracheal LPS administration induced lung injury with alveolar inflammation (increased lavage fluid neutrophils, total protein, and cytokines). In contrast, intravenous LPS induced a predominantly vascular lung injury, with increased plasma TNF and increased neutrophil activation (surface Mac-1 upregulation and L-selectin shedding) and sequestration within the pulmonary vasculature. Intravenous OA produced deteriorations in lung function, reflected by changes in respiratory mechanics and blood gases and lavage fluid neutrophil accumulation. However, addition of CO to the inspired gas did not produce significant changes in the measured physiological or immunological parameters in the mouse models used in this study. Thus the results do not support the hypothesis that use of inhaled CO is beneficial in the treatment of ALI and ARDS.

Lung-marginated monocytes modulate pulmonary microvascular injury during early endotoxemia.

RATIONALE: The role of monocytes in acute endotoxemia has been ascribed to systemic release of mediators within the central circulation. Little is known about the potential role of "marginated" monocytes in regulating microvascular inflammatory signaling. OBJECTIVES: To investigate whether lung-marginated monocytes can locally activate pulmonary endothelial cells through cell contact-dependent interactions in early endotoxemia. METHODS: Mice were challenged with LPS to produce acute endotoxemia and pulmonary vascular injury. Adoptive transfer of ex vivo LPS-stimulated donor leukocytes to recipient mice was also performed to evaluate cell-associated inflammatory signaling between monocytes and endothelial cells within the lung. Cell suspensions from excised lungs were analyzed by flow cytometry for expression of tumor necrosis factor alpha (TNF-alpha) on monocytes and cell adhesion molecules on endothelial cells. RESULTS: Substantial numbers of monocytes rapidly marginated to the lungs after endotoxin challenge in mice, and lung-marginated monocytes expressed significantly higher levels of membrane TNF than circulating monocytes, due to higher TNF production by the marginated cells. Injection of activated wild-type donor leukocytes to wild-type or TNF receptor double knockout recipients demonstrated that lung-marginated monocytes can induce TNF-dependent upregulation of adhesion molecules on pulmonary endothelial cells. Injection of activated donor leukocytes from TNF knock-in mice that express uncleavable mutant membrane TNF also induced adhesion molecule upregulation in wild-type recipients without a systemic soluble TNF release. CONCLUSIONS: These results reveal a previously unacknowledged role for lung-marginated monocytes in early endotoxemia, exerting local, cell-associated TNF signaling within the pulmonary microcirculation, contributing to the evolution of pulmonary vascular injury.

Drainage of subcutaneous lymphatic fluid for the management of respiratory distress in a case of generalized lymphangiectasia in an infant.

A 10-month-old girl was referred to our hospital because of congenital and persistent bilateral chylothorax and generalized lymphedema as well as long-standing respiratory disturbance. Radiological studies showed a diffuse network of superficial lymphatic vessels without major trunks throughout her entire body as well as the lung. She was diagnosed with systemic lymphangiomatosis complicated with pulmonary lymphangiectasia. Percutaneous puncture in the lower leg was performed to discharge the lymphatic fluid and proved to be effective for the respiratory disturbance. This procedure is safe and easy and effectively improves the quality of life of the patient and the family in case of such a persistent disease.

No incidence of port-site recurrence after endosurgical procedure for pediatric malignancies.

Port-site recurrence (PSR) following laparoscopic procedures has been an unpredictable complication in adult cancer patients; however, no data exist about this phenomenon in the pediatric field. The aim of this study was to determine whether PSR, following endosurgical procedure for malignancies, is a typical complication or a rare event in the pediatric population. Eighty-one questionnaires were mailed to members of The Japanese Society of Pediatric Endosurgeons. They were asked to provide a list of their institutions that had experience with PSR after endosurgical procedures for pediatric malignancies. Among 29 institutions, a total of 129 endosurgical procedures for pediatric malignancies were reported; these included 85 laparoscopic and 44 thoracoscopic procedures, performed on 104 neuroblastomas, 8 hepatoblastomas, 7 nephroblastomas, and 10 other tumors. Of the 104 neuroblastomas, 83 were found by mass screening using high levels of urinary vanillylmandelic acid and homovanillic acid. Sixty-five of the 83 patients had their tumor excised, and 18 had their tumor biopsied by endosurgical procedures. Additionally, 47 of these patients did not require any postoperative chemotherapy. No incidence of PSR was reported in any of the patients that underwent endosurgical procedures. The PSR following endosurgical procedure is a rare phenomenon in the pediatric population. Both, laparoscopic and thoracoscopic procedures, are safe and recommended for treating pediatric malignancies, especially mass-screened neuroblastomas.