Individual Activity Anomaly Estimation in Operating Rooms Based on Time-Sequential Prediction.

Surveillance videos of operating rooms have potential to benefit post-operative analysis and study. However, there is currently no effective method to extract useful information from the long and massive videos. As a step towards tackling this issue, we propose a novel method to recognize and evaluate individual activities using an anomaly estimation model based on time-sequential prediction. We verified the effectiveness of our method by comparing two time-sequential features: individual bounding boxes and body key points. Experiment results using actual surgery videos show that the bounding boxes are suitable for predicting and detecting regional movements, while the anomaly scores using key points can hardly be used to detect activities. As future work, we will be proceeding with extending our activity prediction for detecting unexpected and urgent events.

Acute Cheilitis Associated with Oseltamivir after Influenzae A Infection.

Oseltamivir is a neuraminidase inhibitor used to treat acute influenza A or B in adult and pediatric patients. Adverse reactions are usually mild. Here, we report novel side effects associated with oseltamivir. The patient was an 11-year-old girl who developed lower lip cheilitis and stomatitis, after taking oseltamivir. Her symptoms and signs resolved within 36 h of oseltamivir discontinuation. She has clinically fully recovered and has remained well.

Waterhouse-Friderichsen Syndrome and Central Diabetes Insipidus Due to Escherichia coli Meningitis.

Waterhouse-Friderichsen syndrome and central diabetes insipidus are uncommon but potentially fatal endocrine and metabolic diseases. Waterhouse-Friderichsen syndrome is defined as adrenal insufficiency caused by adrenal hemorrhage, which is typically bilateral and most frequently due to meningococcal infection. It is usually diagnosed by necropsy. Central diabetes insipidus in children is often caused by trauma, intracranial lesions, autoimmune diseases, and infections. In addition, it can be caused by mutations in the AVP-NPII gene, although this occurs typically later in childhood rather than in the neonatal period. This report describes a term infant who developed Escherichia coli meningitis, which resulted in septic shock and disseminated intravascular coagulation. Abdominal ultrasound led to an early diagnosis of bilateral adrenal hemorrhage and appropriate treatment with corticosteroids. Symptomatic central diabetes insipidus developed a few days after the onset of meningitis. Intravenous vasopressin was effective in resolving hemodynamic instability. In conclusion, sepsis and meningitis may have severely affected the endocrine system in this patient. Early diagnosis and appropriate treatment for both diseases may have resulted in better clinical outcomes for this patient.

Olanzapine Overdose in a Two-Year-Old Girl Resulting in Both High Serum and Plasma Levels.

The antipsychotic olanzapine is used increasingly to treat various psychiatric illnesses. Accidental olanzapine overdose is uncommon among children. Here, we report a case of a child presenting with an unexplained coma. Accidental ingestion of olanzapine (20 mg) was confirmed by measurement of drug concentrations in both serum and plasma.

Neonatal onset of Niemann-Pick disease type C in a patient with cholesterol re-accumulation in the transplanted liver and inflammatory bowel disease.

BACKGROUND: Niemann-Pick disease type C (NPC) is an autosomal recessive inherited and neurodegenerative disorder. Approximately 10% of NPC patients have acute liver failure and sometimes need liver transplantation (LT), and 7% reportedly develop inflammatory bowel disease (IBD). We report the case of a girl with NPC who had a re- accumulation of cholesterol in the transplanted liver and NPC-related IBD. CASE REPORT: The patient underwent living donor liver transplantation (LDLT) due to severe acute liver failure caused by an unknown etiology inherited from her father. At 1 year and 6 months (1Y6M), she developed neurological delay, catalepsy, and vertical supranuclear gaze palsy. The foam cells were found in her skin, and fibroblast Filipin staining was positive; hence, she was diagnosed with NPC. It was identified that her father had NPC heterozygous pathogenic variant. At 2 years, she had anal fissure, skin tag and diarrhea. She was diagnosed with NPC-related IBD, using a gastrointestinal endoscopy. Three years after LT, liver biopsy revealed foam cells and numerous fatty droplets. At 8 years, broken hepatocytes and substantial fibrosis were observed. She died from circulation failure due to hypoalbuminemia at 8Y2M. CONCLUSIONS: In NPC, load of cholesterol metabolism is suggested to persist even after LT. LDLT from NPC heterozygous variant donor was insufficient to metabolize cholesterol overload. In NPC patients, the possibility of cholesterol re-accumulation should be considered when LT is performed. NPC-related IBD should be considered when NPC patients have anorectal lesions or diarrhea.

Safety and efficacy of vedolizumab in pediatric patients with ulcerative colitis: multicenter study in Japan.

BACKGROUND: Vedolizumab (VDZ) is a humanized monoclonal antibody that binds to α4ß7 integrin expressed in T-lymphocytes and is gut selective. Few studies have evaluated the safety and efficacy of VDZ in pediatric ulcerative colitis (UC) patients, especially from Asia. METHODS: A longitudinal multicenter retrospective study was conducted at 10 Japanese tertiary medical institutions. Patients aged ≤18 years old who received VDZ for UC between January 2019 and July 2021 were enrolled. Information on the clinical characteristics, prior/concomitant treatment, and safety during the observation period was collected. RESULTS: The data obtained from 48 patients (males, n = 30; females, n = 18) were analyzed. The median age at VDZ induction was 14 (range 4-18) years old. VDZ was indicated in 73% of patients as switching from previous biologics due to primary failure, loss of response, and adverse events (AEs) and was the first biologic in 27%. Remission was achieved or maintained at weeks 14, 30, and 54 in 79.2%, 75.0%, and 65.8% of patients, respectively. There were no significant differences between the number of previous biologics exposures and VDZ effectiveness. The hematocrit, serum albumin concentrations, and erythrocyte sedimentation rate (ESR) at baseline differed significantly by VDZ effectiveness. Nine AEs, including infusion reaction, were noted in seven (14.3%) patients. There were no severe AEs related to VDZ administration. CONCLUSIONS: VDZ was safe and effective in children with UC. The hematocrit, albumin, and ESR at VDZ initiation might be predictors for VDZ effectiveness. VDZ may be an important option for pediatric patients and can be used as an alternative to immunomodulators.

Clinical significance of serum cytokine profiles for differentiating between Kawasaki disease and its mimickers.

OBJECTIVES: To investigate the clinical significance of serum cytokine profiles for differentiating between Kawasaki disease (KD) and its mimickers. METHODS: Patients with KD, including complete KD, KD shock syndrome (KDSS), and KD with macrophage activation syndrome (KD-MAS), and its mimickers, including multisystem inflammatory syndrome in children, toxic shock syndrome, and Yersinia pseudotuberculosis infection, were enrolled. Serum levels of interleukin (IL)-6, soluble tumor necrosis factor receptor type II (sTNF-RII), IL-10, IL-18, and chemokine (C-X-C motif) ligand 9 (CXCL9) were measured using enzyme-linked immunosorbent assay and compared them with clinical manifestations. RESULTS: Serum IL-6, sTNF-RII, and IL-10 levels were significantly elevated in patients with KDSS. Serum IL-18 levels were substantially elevated in patients with KD-MAS. Patients with KD-MAS and KD mimickers had significantly elevated serum CXCL9 levels compared with those with complete KD. Area under the receiver operating characteristic curve analysis showed that serum IL-6 was the most useful for differentiating KDSS from the others, IL-18 and CXCL9 for KD-MAS from complete KD, and CXCL9 for KD mimickers from complete KD and KD-MAS. CONCLUSION: Serum cytokine profiles may be useful for differentiating between KD and its mimickers.

Epidemiology conduction of paediatric rheumatic diseases based on the registry database of the Pediatric Rheumatology Association of Japan.

OBJECTIVES: Although epidemiological surveys of paediatric rheumatic diseases in Japan have been conducted, they were single surveys with no continuity. This is the first report of the Pediatric Rheumatology Association of Japan registry database, which was established to continuously collect data for paediatric rheumatic diseases. METHODS: Pediatric Rheumatology International Collaborate Unit Registry version 2 (PRICUREv2) is a registry database established by the Pediatric Rheumatology Association of Japan. The registry data were analysed for the age of onset, time to diagnosis, sex differences, seasonality, and other factors. RESULTS: Our data showed the same trend regarding rates of paediatric rheumatic diseases reported in Japan and other countries. The age of onset was lower in juvenile idiopathic arthritis (JIA) and juvenile dermatomyositis and higher in systemic lupus erythematosus and Sjögren's syndrome. The time to diagnosis was relatively short in JIA and systemic lupus erythematosus but longer in juvenile dermatomyositis and Sjögren's syndrome. Rheumatoid factor-positive polyarticular JIA showed a seasonality cluster with regard to onset. CONCLUSION: PRICUREv2 aided the retrieval and evaluation of current epidemiological information on patients with paediatric rheumatic diseases. It is expected that the data collection will be continued and will be useful for expanding research in Japan.

Controlled introduction of defects into single-walled carbon nanotubes via a fluorination-defluorination strategy using xenon difluoride and their alkaline oxygen reduction reaction catalytic activity.

The defect edges in carbon nanomaterials have attracted attention as catalytic active sites for the oxygen reduction reaction (ORR) of the cathode in electrolyte fuel cells, and the defect control in carbon nanomaterials is becoming increasingly important. This study evaluates a fluorination-defluorination strategy for the controlled introduction of defects into single-walled carbon nanotubes (SWCNTs) involving the fluorination of SWCNTs using xenon difluoride (XeF2) and their subsequent defluorination through thermal annealing. We synthesized fluorinated SWCNTs with different fluorine contents using gaseous XeF2 and annealed the fluorinated SWCNTs at 1000 °C for 3 h under nitrogen gas flow. Structural analyses revealed that SWCNTs derived from fluorinated SWCNTs with low fluorine contents primarily had single point defects. In contrast, SWCNTs derived from fluorinated SWCNTs with high fluorine contents had vacancy defects with edges. According to the ORR catalyst evaluation in alkaline aqueous solution, SWCNTs with edge defects, rather than point defects, can improve the efficiency of ORR catalytic activity. The proposed fluorination-defluorination strategy using gaseous XeF2 is expected to enable the controlled introduction of defects in different types of carbon materials.

Calcium sparks enhance the tissue fluidity within epithelial layers and promote apical extrusion of transformed cells.

In vertebrates, newly emerging transformed cells are often apically extruded from epithelial layers through cell competition with surrounding normal epithelial cells. However, the underlying molecular mechanism remains elusive. Here, using phospho-SILAC screening, we show that phosphorylation of AHNAK2 is elevated in normal cells neighboring RasV12 cells soon after the induction of RasV12 expression, which is mediated by calcium-dependent protein kinase C. In addition, transient upsurges of intracellular calcium, which we call calcium sparks, frequently occur in normal cells neighboring RasV12 cells, which are mediated by mechanosensitive calcium channel TRPC1 upon membrane stretching. Calcium sparks then enhance cell movements of both normal and RasV12 cells through phosphorylation of AHNAK2 and promote apical extrusion. Moreover, comparable calcium sparks positively regulate apical extrusion of RasV12-transformed cells in zebrafish larvae as well. Hence, calcium sparks play a crucial role in the elimination of transformed cells at the early phase of cell competition.

Longitudinal analysis of electroencephalography pattern changes in an infant with Schaaf-Yang syndrome and a novel mutation in melanoma antigen L2 (MAGEL2).

BACKGROUND: Schaaf-Yang syndrome (SYS) is a rare hereditary disease caused by truncating point mutations of the paternal allele of melanoma antigen L2 (MAGEL2), one of five protein-coding genes within the Prader-Willi syndrome (PWS) critical domain. SYS shares many clinical and molecular characteristics with PWS but has some distinct features, such as joint contractures and autism. Patients with PWS show abnormal electroencephalography (EEG) patterns. However, there are very few reports on EEG findings in patients with SYS. METHODS: A SYS patient was included in this study. Detailed neurological examinations and EEG were performed from neonate to infant ages. Sanger sequencing was performed. RESULTS: Our patient presented abnormal EEG findings and had diffuse brain dysfunction symptoms including a reduced level of consciousness, diminished spontaneous movements, hypotonia, feeding difficulties, and hypoventilation from early after birth. As she grew older and her background activity of EEG normalized, her neurodevelopmental symptoms remained but improved. Sanger sequencing of this patient revealed a novel, heterozygous c.2005C > T, truncating mutation in the MEGAL2 gene. CONCLUSIONS: We described an SYS-associated, time-dependent, EEG pattern in a patient with SYS. Our findings of longitudinal EEG changes in a patient with SYS revealed a specific pattern of how affected individuals develop brain function.

Campylobacter jejuni Bacteremia in the Term Infant A Rare Cause of Neonatal Hematochezia.

Early-onset sepsis caused by Gram-negative spiral organisms is rarely reported, with Campylobacter fetus being a better known causative agent than other Campylobacter species. We report the case of a 2-day-old girl who presented with hematochezia and bacteremia caused by Campylobacter jejuni. She was born full-term. Her family ate undercooked chicken, and Campylobacter enteritis was diagnosed before her birth.

Reduction of acute gastric volvulus in a 3-year-old using a balloon-attached endoscope combined with gel immersion endoscopy.

When performing endoscopic reduction in patients with gastric volvulus, it is important to maintain a low level of intragastric pressure and to fix the endoscope in the duodenum. Gel immersion endoscopy is a new method for securing the visual field by injecting clear gel. The balloon-attached endoscope makes it easier to fix the tip in the duodenum without mucosal damage. We report successful reduction of a mesenteroaxial gastric volvulus using an endoscope with a balloon in combination with gel immersion endoscopy. A 3-year-old Japanese male developed gastric volvulus. Since gastric decompression using a nasogastric tube failed to reduce the volvulus, endoscopic reduction was performed under general anesthesia. After aspiration of intragastric gas, clear gel was injected through the accessory channel which secured the visual field in the stomach even with residue while maintaining low intragastric pressure. After reaching the descending portion of the duodenum, the balloon attached to the tip of the endoscope was inflated and fixed in the duodenum. The volvulus was successfully reduced by pulling back the endoscope with clockwise torque. Acute mesenteroaxial gastric volvulus has the potential to cause ischemia and perforation which can be life-threatening, so most patients are treated with surgical intervention. Gel immersion endoscopy is safe and effective to secure the visual field, even in children. Endoscopic reduction may be a viable treatment option for reducing gastric volvulus in non-emergent patients.

Distribution of Aspergillus section Nigri at shochu fermenting places in Japan.

Koji mold, which belongs to the Aspergillus section Nigri, is used in the production of shochu. The section Nigri is composed of very morphologically similar members that in some cases produce mycotoxins, which rises concerns as to whether the presence of mycotoxin-producing fungi in shochu producing sites can compromise consumer safety. Thus, we examined the presence of mycotoxin-producing sec. Nigri fungi in six shochu factories (named A-F) in Japan. Airborne fungal levels in the factories were determined, and a traditional koji called "kona-koji" made from the mold naturally present in factory C (Aogashima village) was analyzed. Isolates of sec. Nigri fungi were identified morphologically and confirmed via cytochrome b gene analysis. In factory A (Nago city), airborne fungal levels of sec. Nigri were 4,000 and 100 cfu/m3 in the koji-making and fermentation rooms, respectively. In factories B, C, and D, the levels were 40, >104 cfu/m3, and 100 cfu/m3, respectively. In factory F (Iki city), there were high levels of airborne white-koji mold (a white mutant of Asp. luchuensis). The most dominant fungal species of sec. Nigri was isolated and identified as Asp. luchuensis via genetic analysis. This is likely to have originated from the commercial fermentation culture used. Asp. niger and Asp. luchuensis were isolated from kona-koji. Mycotoxin production (ochratoxin and fumonisin B2) by Asp. luchuensis (eight strains) and Asp. niger (three strains) was virtually inexistent; only one strain of Asp. niger was positive for fumonisin B2. This study clearly shows that mycotoxin-producing fungi are not dominant in the fungal flora present in the shochu factories examined and therefore, that the liquor can be safely fermented.Implications: In this study, we examined the presence of mycotoxin-producing Aspergillus sec. Nigri fungi in six shochu (Japanese distilled beverage) factories. The most dominant fungal species of sec. Nigri was isolated and identified as Aspergillus luchuensis (black-koji mold). The proportion of mycotoxin-producing Aspergillus niger and Aspergillus carbonarius was very small. In addition, the Asp. niger isolated from koji mold did not have the ability to produce ochratoxins or fumonisin B. This study clearly shows that shochu can be safely fermented.

Multisystem Imaging Manifestations of Kawasaki Disease.

Kawasaki disease (KD) is a common pediatric vasculitis syndrome involving medium- and small-sized arteries that is especially prevalent in early childhood (ie, age 6 months to 5 years). The diagnosis of KD is made on the basis of clinical features, such as fever, characteristic mucocutaneous changes, and nonsuppurative cervical lymphadenopathy. However, early diagnosis is often challenging because many children with KD present with atypical symptoms. The most serious complication of KD is coronary artery aneurysm caused by coronary arteritis. Prompt intravenous immunoglobulin therapy reduces the risk of cardiac morbidity. In addition, the systemic extension of KD-related vasculitis during the acute phase causes a variety of multisystem manifestations, including encephalopathy, stroke, retropharyngeal edema, pericarditis, myocarditis, KD shock syndrome, pulmonary lesions, intestinal pseudo-obstruction, gallbladder hydrops, arthritis, and myositis. These complications tend to be more common in affected children with atypical presentation. Radiologists can play an important role in the timely identification of diverse KD-associated morbidities and thus may contribute to the early diagnosis of atypical KD. Online supplemental material is available for this article. ©RSNA, 2021.

Case Report: A Domestic Sponge Brush Used to Clean a Milk Feeding Bottle: The Source of Neonatal Meningitis Caused by Pseudomonas aeruginosa.

Pseudomonas aeruginosa is a relatively rare cause of neonatal meningitis, and most patients have serious underlying diseases, prematurity, immunodeficiency, or anatomical abnormalities. We report the case of a 7-day-old girl with meningitis caused by P. aeruginosa. She was born full-term and had no immunodeficiency or anatomical abnormalities as far as our investigation ascertained. Through the use of anti-Pseudomonas antibiotics, she recovered without any complications other than a slight hearing disability revealed by audiology testing. P. aeruginosa was also isolated from a domestic sponge brush used to clean her milk bottle. Physicians should consider P. aeruginosa as a possible pathogen of neonatal meningitis even in full-term infants with no immunodeficiency or anatomical abnormalities. Physicians should give advice concerning appropriate hygiene practices to be applied to the neonate's environment.

Na-ion mobility in P2-type Na0.5MgxNi0.17-xMn0.83O2 (0 ≤ x ≤ 0.07) from electrochemical and muon spin relaxation studies.

Sodium transition metal oxides with a layered structure are one of the most widely studied cathode materials for Na+-ion batteries. Since the mobility of Na+ in such cathode materials is a key factor that governs the performance of material, electrochemical and muon spin rotation and relaxation techniques are here used to reveal the Na+-ion mobility in a P2-type Na0.5MgxNi0.17-xMn0.83O2 (x = 0, 0.02, 0.05 and 0.07) cathode material. Combining electrochemical techniques such as galvanostatic cycling, cyclic voltammetry, and the galvanostatic intermittent titration technique with µ+SR, we have successfully extracted both self-diffusion and chemical-diffusion under a potential gradient, which are essential to understand the electrode material from an atomic-scale viewpoint. The results indicate that a small amount of Mg substitution has strong effects on the cycling performance and the Na+ mobility. Amongst the tested cathode systems, it was found that the composition with a Mg content of x = 0.02 resulted in the best cycling stability and highest Na+ mobility based on electrochemical and µ+SR results. The current study clearly shows that for developing a new generation of sustainable energy-storage devices, it is crucial to study and understand both the structure as well as dynamics of ions in the material on an atomic level.

Juvenile polyposis syndrome-hereditary hemorrhagic telangiectasia associated with a SMAD4 mutation in a girl.

Juvenile polyposis syndrome (JPS) and hereditary hemorrhagic telangiectasia (HHT) are both relatively rare hereditary disorders. Some patients with the SMAD4 gene mutation develop both JPS and HHT, a condition termed JPS-HHT. We herein report a case of childhood-onset JPS-HHT. At nine years old, the patient underwent colonoscopy under suspicion of Crohn's disease, which revealed multiple polyps. A genetic analysis for familial adenomatous polyposis and Peutz-Jeghers syndrome found no mutations. After several years, extraintestinal manifestations, such as repeated epistaxis and several telangiectasias in the upper palate and stomach, were identified, which led to the performance of gene mutation analysis for SMAD4. As a result, a missense mutation in exon 8, codon 361 from arginine to cysteine (c.1081 C>T) was found. Based on this finding, the patient underwent cerebral magnetic resonance angiography, pulmonary perfusion scintigraphy and thoracoabdominal contrast computed tomography. The examination revealed that she had pulmonary arteriovenous fistulas and arteriovenous malformations in both the liver and right mammary gland. Thus, continuous surveillance for vascular lesions and gastrointestinal cancer is scheduled. Making a precise diagnosis of JPS-HHT can lead to the detection of asymptomatic complications and enable appropriate future disease management.