BackgroundExpanded newborn screening (ENBS) utilizing tandem mass spectrometry (MS/MS) for inborn metabolic diseases (IMDs), such as organic acidemias (OAs), fatty acid oxidation disorders, (FAODs), and amino acid disorders (AAs), is increasingly popular but has not yet been introduced in many Asian countries. This study aimed to determine the incidence rates of OAs, FAODs, and AAs in Asian countries and Germany using selective screening and ENBS records.Materials and methodsSelective screening for IMDs using gas chromatography–mass spectrometry and MS/MS was performed among patients suspected to be afflicted in Asian countries (including Japan, Vietnam, China, and India) between 2000 and 2015, and the results from different countries were compared. Similarly, ENBS results from Japan, South Korea, Taiwan, and Germany were compared. Additionally, the results of selective screening and ENBS in Japan were compared.ResultsAmong 39,270 patients who underwent selective screening, IMDs were detected in 1170. Methylmalonic acidemia was frequently identified in several countries, including Japan (81/377 diagnosed IMDs), China (94/216 IMDs), and India (72/293 IMDs). In Vietnam, however, β-ketothiolase deficiency was particularly frequent (33/250 IMDs). ENBS yielded differences in overall IMD rates by country: 1:8557 in Japan, 1:7030 in Taiwan, 1:13,205 in South Korea, and 1:2200 in Germany. Frequently discovered diseases included propionic acidemia (PPA) and phenylketonuria (PKU) in Japan, 3-methylcrotonyl-CoA carboxylase deficiency (MCCD) and PKU in Taiwan, MCCD and citrullinemia type I in South Korea, and PKU and medium-chain acyl-CoA dehydrogenase deficiency in Germany. Furthermore, in Japan, selective screening and ENBS yielded respective PPA frequencies of 14.7% and 49.4% among all organic acidemias.ConclusionThe incidence rates of IMDs vary by country. Moreover, the disease spectra of IMDs detected via selective screening differ from those detected via ENBS.

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Aim In post‐intensive care syndrome (PICS), long‐term survivors of critical illness present various physical and mental symptoms that can persist for years after discharge. Post‐intensive care syndrome in Japan has not been well described, so this study aims to elucidate its epidemiology. Methods We undertook a single‐center prospective longitudinal cohort study in a mixed intensive care unit (ICU) in a Japanese tertiary hospital. Adult patients emergently admitted to the ICU were eligible for inclusion in the study. To assess activity of daily living (ADL) status and psychiatric symptoms, we posted a questionnaire at 3 and 12 months after discharge from the ICU. We evaluated ADL status, anxiety, depression, and post‐traumatic stress disorder symptoms using the Barthel index, Hospital Anxiety and Depression Scale, and Impact of Event Scale − Revised, respectively. Results Enrolled in this study were 204 patients. We received responses from 117/147 (80%) and 74/98 (76%) patients at 3 and 12 months, respectively. At 3 months, the prevalence of ADL disability, anxiety, depression, and post‐traumatic stress disorder symptoms was 32%, 42%, 48%, and 20%, respectively. At 12 months, the prevalence was 22%, 33%, 39%, and 21%, respectively. The prevalence of any symptoms was 66% at 3 months and 55% at 12 months. Barthel index score at 12 months was improved significantly from that at 3 months. Hospital Anxiety and Depression Scale and Impact of Event Scale − Revised scores at 12 months showed no improvement. Conclusions At 3 and 12 months after ICU discharge, over half of our Japanese patients suffered ADL disability and/or psychiatric symptoms. The ADL disability improved at 1 year, but psychiatric symptoms did not.

Aim: Rikkunshito is a traditional Japanese medicine used for delayed gastric emptying in intensive care units in Japan. This study aimed to investigate whether standard-or high-dose rikkunshito can improve the achievement of enteral calorie target among critically ill adults. Methods: This open-label, single-center, pilot randomized controlled trial was carried out from March 2018 until December 2018 and enrolled critically ill adult patients requiring enteral nutrition by gastric tube for at least 5 days. Patients were randomized into the control group, the standard-dose rikkunshito group (2.5 g three times daily), and the high-dose rikkunshito group (5 g three times daily). Intervention was given for 5 days. The primary outcome measure was the percentage of enteral calorie intake achieved in the target at the fifth day after randomization. Results: The cohort comprised 26 patients; of these, 9, 8, and 9 were included in the control group, the standard-dose group, and the high-dose group, respectively. Twenty-one patients (81%) were included in the primary analysis. The percentage of enteral calorie intake achieved in the target at the fifth day was 59% (interquartile range [IQR], 39-63%), 40% (IQR, 26-61%), and 62% (IQR, 17-83%) in the control, the standard-dose, and the high-dose groups, respectively (P = 0.42). The number of adverse events did not differ significantly between the groups (control group, 4 [44%]; standard-dose group, 3 [38%]; and high-dose group, 4 [44%], P = 1.00). Conclusions: Standard-or high-dose rikkunshito did not improve the achievement of enteral calorie target in critically ill adults.

Aim: The aims of this study were to investigate outcomes of abdominal trauma in patients with hemorrhagic shock requiring emergency laparotomy and clarify the beneficial effects of intra-aortic balloon occlusion (IABO) for intra-abdominal hemorrhage in patients with critically uncontrollable hemorrhagic shock (CUHS).Methods: We reviewed 44 hemorrhagic shock patients who underwent emergency laparotomy for intra-abdominal hemorrhage over a 6-year period. Of these patients, we examined data for 19 subjects who underwent IABO during initial resuscitation to control massive intra-abdominal bleeding leading to CUHS. Results:The average Injury Severity Score and probability of survival (Ps) of the 44 patients were 27.6 AE 15.4 and 0.735 AE 0.304, respectively, and the overall survival rate was 77.3%. The differences in the Glasgow Coma Scale, lactate level, prothrombin time -international normalized ratio, and Ps between the two groups (21 responders and 23 non-responders) were statistically significant (P < 0.05). Intra-aortic balloon occlusion was attempted in 19 of 23 patients (82.6%) with CUHS, and there were no statistically significant differences in presenting Glasgow Coma Scale, body temperature, lactate, prothrombin timeinternational normalized ratio, or Revised Trauma Score between the survivors (n = 12) and non-survivors (n = 7). The only significant differences between these two groups were observed in Injury Severity Score (P = 0.047) and Ps (P = 0.007). In all patients, the balloons were successfully placed in 8.1 AE 3.3 min in the thoracic aorta, and a significant increase in systolic blood pressure was observed immediately after IABO. Conclusion:The IABO procedure can be life-saving in the management of patients with CUHS arising from intra-abdominal hemorrhage, permitting transport to surgery.