Takashi Muguruma

Severe form of encephalopathy associated with 2009 pandemic influenza A (H1N1) in Japan.

BACKGROUND Every year, an estimated 200-500 children in Japan develop influenza-associated encephalopathy (IAE), and 10-30% of these children die. OBJECTIVE To clarify the clinical features of a severe form of acute encephalopathy seen with 2009 pandemic influenza A (H1N1). STUDY DESIGN This retrospective survey examined 20 children with acute encephalopathy associated with the 2009 pandemic influenza A (H1N1) who died or were in a prolonged deep coma with a flat electroencephalogram tracing and loss of spontaneous respiration. We obtained demographic, clinical, laboratory, and neuroimaging data through interviews with the attending physicians and chart reviews. RESULTS Subjects were 13 boys and seven girls. Their median age was 45 (range 11-200) months. Five patients had one or more pre-existing conditions. Acute encephalopathy developed within 2 days after influenza onset in 16 patients. As the initial neurological symptom, delirious behavior was seen in six children, and brief seizures in six. Eighteen patients were comatose within 6h of the onset of encephalopathy. Marked brain edema on computed tomography (CT) was seen in all but one patient. Brainstem lesions on CT were recognized in 12 patients. Sixteen patients died 0-45 (median 2.5) days after the onset of acute encephalopathy, and the others remained in deep comas without spontaneous respiration. CONCLUSIONS The clinical course of the patients was characterized by an onset with mild neurological symptoms and rapid deterioration of consciousness into coma. Head CT revealed marked cerebral edema, often associated with brainstem lesions.

Liver transplantation in a patient with propionic acidemia requiring extra corporeal membrane oxygenation during severe metabolic decompensation

Abstract:  LDLT is an effective treatment modality in patients with congenial metabolic liver disease. PA is a rare autosomal recessive disorder caused by deficiency in propionyl‐CoA carboxylase. The present study demonstrates a two‐yr‐old girl with PA who was admitted for metabolic decompensation and immediately treated with CHD and protein intake restriction at 46 days of age. Two yr later, the patient was readmitted for severe metabolic decompensation with complete atrioventricular block and ventricular fibrillation. CHDF and ECMO were indicated because of progressive metabolic and cardiac deterioration. After full recovery of the ejection fraction, planned LDLT was performed to prevent further metabolic decompensation and fatal cardiac insufficiency. No significant events occurred after the operation and the condition of the patient is stable with continued protein restriction and carnitine supplementation.

Case of congenital lobar emphysema in an 18-month-old boy and review of earlier cases.

An 18-month-old boy developed recurrent episodes of coughing and wheezing and was repeatedly admitted to the local hospital under the diagnosis of bronchial asthma. On his most recent admission, he required continuous treatment for over 1 month, and his symptoms were more serious than they had previously been. He was suspected of having foreign body aspiration because he often put small toys in his mouth. Chest X-ray showed remarkable hyperlucency in the right lobe (Fig. 1). A bronchoscopic study and a computed tomography (CT) scan were performed, however, they did not show a foreign body. Hyperinflation of the right middle lung was revealed by the chest CT (Fig. 2). One week after admission, he was referred to Okayama National Hospital. On admission he was 82 cm tall (+0.5 SD) and weighed 9.8 kg (–0.5 SD), his condition was generally good except for coughing, wheezing and fever. Although he was treated with aminophiline and antibiotics intravenously for 3 weeks, these symptoms did not improve. On the third week a bronchoscopy showed that the bronchus was relatively narrow and easily collapsed during expiration, which was suspected to be the cause of lobar emphysema, and foreign body was ruled out as the cause of obstruction. A bronchography also showed relative narrowing of the middle bronchus (Fig. 3). On the fourth week, lobectomy of the right middle lobe was performed because of persistent serious symptoms. We did not perform any pulmonary function tests before the operation. His postoperative course was so stable that he was discharged 13 days after the operation. Histological examination of the resected middle lobe showed widely distended and overinflated alveoli without any destruction of the lung tissue (Fig. 4).

Continuous Veno-Venous Hemodiafiltration and Plasma Exchange in Infantile Acute Liver Failure.

Objectives: The purpose of the current study was to assess our multidisciplinary approach consisting of early application of neurology-oriented intensive care, aggressive artificial liver support and liver transplantation at the appropriate time for infants with acute liver failure. Design: Retrospective cohort study. Setting: A tertiary pediatric medical center in Japan. Patients: Seventeen infants younger than 12 months with acute liver failure who subsequently underwent liver transplantation between February 2006 and June 2011. Interventions: None. Measurements and Main Results: The patients varied from 1 to 11 months, with a median of 6 months. The median body weight was 8.0 kg (range, 2.7–10 kg). With respect to the encephalopathy grading before liver transplantation, four cases were categorized as grade II, seven cases were categorized as grade III, and five cases were categorized as grade IV. Continuous veno-venous hemodiafiltration and plasma exchange were applied to all the infants until liver transplantation. Bilirubin, ammonia, prothrombin time/international normalized ratio and creatinine decreased significantly after continuous veno-venous hemodiafiltration + plasma exchange (p < 0.001). The median value of catecholamine index changed from 10 to 0 (range, 0–20.6). Notably, among the 16 infants who underwent electroencephalography assessment, five did not show slow waves throughout their stay, and one who did so before treatment ceased to show any after treatment. The all patients underwent living-donor liver transplantation and were subsequently discharged from the PICU. The overall survival rate was 88% (15/17) with a median follow-up period of 28 months (range, 2–64 mo). Regarding the neurological outcomes of the survivors, 73% (11/15) had no neurological morbidities and 20% (3/15) had mild disabilities. Conclusions: Our multidisciplinary approach for infants with acute liver failure achieved favorable outcomes. Further investigations are needed to examine the efficacy of the artificial liver support.

Current experience and limitations of extracorporeal cardiopulmonary resuscitation for cardiac arrest in children: a single-center retrospective study

BackgroundThere are few reports detailing the importance of extracorporeal membrane oxygenation (ECMO) for pediatric cardiac arrest in Japan. We investigated the status and issues surrounding extracorporeal cardiopulmonary resuscitation (ECPR) at our institution.MethodsPatients aged <15 years who underwent ECPR between April 1, 2003 and March 31, 2012 were eligible. The characteristics, cannulation site, durations of cardiopulmonary resuscitation (CPR), cannulation procedure, and ECMO, and neurologic outcomes were retrospectively reviewed. A favorable neurologic outcome was defined as Pediatric Cerebral Performance Categories 1 and 2.ResultsA total of 21 ECPR events were identified. The median CPR and cannulation durations were 60 and 25 min, respectively. Central and peripheral access sites were employed in 15 and six cases, respectively. Five of the 21 patients (24%) were successfully weaned from ECMO and three of the 21 (14%) survived. Two of the three survivors had a favorable neurologic outcome.ConclusionsThe mortality of patients undergoing ECPR at our institution was low. However, about 10% of all patients had a favorable neurologic outcome, which suggests that ECPR may be effective in pediatric cardiac arrest patients.

Unexpected cardiopulmonary arrest associated with influenza: our experience during the 2009 pandemic in Japan.

influenza: our experience during the 2009 pandemic in Japan Akihisa Okumura, Satoshi Nakagawa, Hisashi Kawashima, Takashi Muguruma, Osamu Saito, Jun-ichi Fujimoto, Chiaki Toida, Shuji Kuga, Toshihiro Imamura, Toshiaki Shimizu, Naomi Kondo, Tsuneo Morishima Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan. Division of Critical Care Medicine, National Center for Child Health and Development, Tokyo, Japan. Department of Pediatrics, Tokyo Medical University School of Medicine, Tokyo, Japan. Department of Pediatrics, Gifu University Graduate School of Medicine, Gifu, Japan. Department of Pediatrics, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan. Correspondence: Akihisa Okumura, Department of Pediatrics, Juntendo University Faculty of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo 113-8421, Japan. E-mail: okumura@juntendo.ac.jp

Development of a Japanese scale for assessment of paediatric normal weight.

The Broselow tape (BT) system has helped health care profesionals succeed in avoiding errors made in paediatric acute care.1 lthough the BT has been used worldwide, it is not surprising that his American-based scale has limited applicability to paediatric opulations outside of the United States.2,3 We therefore aimed o develop a length-based weight estimation scale for Japanese hildren, the Japanese Paediatric Assessment of Normal weight JAPAN) scale. We used data of participants in a large, ongoing ongitudinal survey conducted in Japan (the Longitudinal Survey f Babies in the 21st Century4), to achieve better performance of he JAPAN scale. We also described our method of developing the cale. We obtained 370,980 measurements of body weight and height rom 39,547 participants 12–155 months old. We created the APAN scale using 75% of measurements, randomly selected from he total using the Stata random-number function binomial. We alculated and listed means of weight (kg) and the number of amples according to height, ranging from 45 cm to 179 cm, in n integral format. We then linked weight with height, begining with a height of 72 cm, with more than 100 measurements. p to 19 kg, we rounded means of weight to whole numbers and hecked the corresponding heights. For weights between 19 kg nd 36 kg, we considered the means of weights 19.5–20.9 kg to e 20 kg, those of 21.0–22.9 kg to be 22 kg, and so on up to 6 kg, and checked the corresponding heights. To check validity f the scale compared with the BT (2007 and 2011 versions), we pplied the three scales to the remaining 25% of measurements nd calculated mean absolute error as well as root mean square rror (RMSE) for each scale.5 Stata Statistical Software Release 13 StataCorp LP, College Station, TX, USA) was used for all analyes. The relationship between weight and ranges of height for the APAN scale is shown in Table 1. The newly developed JAPAN scale erformed better than the 2007 and 2011 versions of the BT. For the APAN scale vs. the BT 2007 vs. the BT 2011, mean absolute error kg) was 1.79 vs. 1.85 vs. 1.91, and RMSE (kg) was 2.77 vs. 2.92 vs. .80, respectively. Considering that the JAPAN scale was developed based on a apanese paediatric population and performed best for Japanese hildren, each country should develop a similar weight estimation cale for its own paediatric population, using available anthroometric data. Because of changing paediatric growth patterns ithin each country, these weight estimation methods should be pdated or re-evaluated regularly. Use of an appropriate scale in an 4

Targeted age, device deployment, and problems associated with pediatric defibrillation in pediatric prehospital emergency medical care settings in Japan

The use of automated external defibrillators was expanded to include infants according to the 2010 cardiopulmonary resuscitation guidelines in Japan. However, deployment has been slower for pediatric patients in Japan, because there are fewer appropriate pediatric patients for automated external defibrillators than adults. This study aimed to investigate the targeted age range for pediatric defibrillation and device deployment of defibrillators for pediatric patients in prehospital emergency medical care settings in Japan, and present the issues associated with automated external defibrillators.

Medical equipment deployment in pediatric emergency prehospital medical units in Japan

The deployment status of pediatric emergency equipment in ambulances in Japan is unknown. To investigate the status of and issues associated with prehospital emergency medical care for pediatric patients, we conducted a descriptive epidemiological study. We carried out a Web‐based survey of 767 fire defense headquarters in Japan, of which 671 responded (valid response rate, 88%). Most of the fire defense headquarters equipped all of their ambulances with oxygen masks (82%), bag‐valve masks (for neonates, 83%; for children, 84%), straight laryngoscope blades (for neonates, 47%; for children 68%), blood pressure cuffs for children (91%), oximeter probes (78%), and stiff neck collars (91%); but despite the need for other equipment such as nasopharyngeal and oropharyngeal airways, and Magill forceps, they were insufficiently deployed. In Japan, prehospital emergency medical equipment deployment does not meet the needs of pediatric patients. Minimum equipment standards need to be established for pediatric prehospital care.

Hospitals’ Preparedness to Treat Pediatric Patients During Mass Casualty Incidents

OBJECTIVES Little is known about the preparedness of hospitals to care for pediatric patients during a major incident in Japan. This study assessed the disaster preparedness of a childrens hospital in Japan by using a disaster drill.Materials and MethodsWe performed a triage drill with all hospitalized patients. The triage tags and medical records were reviewed retrospectively. We determined the efficacy of triage education, the validity of the Simple Triage and Rapid Treatment (START) method for children, and the potential need for evacuation through the disaster drill. RESULTS This study highlights 3 important issues about the hospitals preparedness. First, it is difficult to promote disaster education for staff who are not well trained on handling disasters. Second, the START method is suitable for children older than 5 years, but it has a high rate of over-triaging among younger children. Third, approximately 40% of patients who are coded as immediate may require transportation resources in a hospital evacuation. CONCLUSIONS Our findings suggest that disaster preparedness, such as educating hospital staff regarding disasters and establishing evacuation systems for a number of pediatric patients when a disaster happens, is essential for caring for hospitalized children during a mass casualty incident. (Disaster Med Public Health Preparedness. 2018;page 1 of 4).