Application of regulation of reactive oxygen species and lipid peroxidation to disease treatment.

Although many diseases in which reactive oxygen species (ROS) and free radicals are involved in their pathogenesis are known, and antioxidants that effectively capture ROS have been identified and developed, there are only a few diseases for which antioxidants have been used for treatment. Here, we discuss on the following four concepts regarding the development of applications for disease treatment by regulating ROS, free radicals, and lipid oxidation with the findings of our research and previous reports. Concept 1) Utilization of antioxidants for disease treatment. In particular, the importance of the timing of starting antioxidant will be discussed. Concept 2) Therapeutic strategies using ROS and free radicals. Methods of inducing ferroptosis, which has been advocated as an iron-dependent cell death, are mentioned. Concept 3) Treatment with drugs that inhibit the synthesis of lipid mediators. In addition to the reduction of inflammatory lipid mediators by inhibiting cyclooxygenase and leukotriene synthesis, we will introduce the possibility of disease treatment with lipoxygenase inhibitors. Concept 4) Disease treatment by inducing the production of useful lipid mediators for disease control. We describe the treatment of inflammatory diseases utilizing pro-resolving mediators and propose potential compounds that activate lipoxygenase to produce these beneficial mediators.

Evaluation of Spinal Alignment and Clinical Findings for the Efficacy of One-Stage Surgery in Tandem Spinal Stenosis.

Introduction We compared preoperative skeletal muscle, pre-and post-operative spinal alignment or clinical symptoms between tandem spinal stenosis (TSS) patients who underwent simultaneous cervical and lumbar decompression and lumbar spinal stenosis (LSS) patients who underwent only lumbar decompression and the efficacy of one-stage surgery was examined. Methods This study included 82 patients, identifying 13 patients for the TSS group (mean age 77.2 years) and 69 patients for the LSS group (mean age 72.2 years). One-stage decompression surgery was performed on both groups. The spinal alignments were evaluated using Lumbar scoliosis (LS), Sagittal vertical axis (SVA), Lumbar lordosis (LL), Pelvic tilt (PT), Pelvic incidence (PI), and Sacral slope (SS). The clinical symptoms were evaluated using the visual analogue scale (VAS) score for low back pain (LBP), the Japanese Orthopedic Association scoring system (JOA score), the Roland-Morris Disability Questionnaire (RDQ), the Japanese Orthopedic Association Back Pain Evaluation Questionnaire (JOABPEQ) and Oswestry Disability Index (ODI). Results The amount of bleeding was not significantly different between the two groups (p > .05). SVA, LL, PT, and SS were significantly improved in the LSS group (p < 0.05). In the TSS group, SVA, LL, PT, and SS tended to improve, but without significant differences. The proportion of JOABPEQ gait dysfunction that was difficult to climb stairs was 83% in the TSS group, and social life disturbance that was difficult to engage in ordinary activities was 67% in the TSS group, which was significantly higher than that in the LSS group (p < .05). Although clinical symptoms improved by surgery in both groups (p < .05), there was no significant difference in the degree of clinical symptom improvement before and after surgery (p > .05). Conclusions One-stage surgery for TSS is effective because it has the same intraoperative bleeding volume as LSS alone and is minimally invasive. It also improves forward-leaning posture and clinical symptoms equivalent to LSS alone.

N-Acetylcysteine prevents amyloid-ß secretion in neurons derived from human pluripotent stem cells with trisomy 21.

Down syndrome (DS) is caused by the trisomy of chromosome 21. Among the many disabilities found in individuals with DS is an increased risk of early-onset Alzheimer's disease (AD). Although higher oxidative stress and an upregulation of amyloid ß (Aß) peptides from an extra copy of the APP gene are attributed to the AD susceptibility, the relationship between the two factors is unclear. To address this issue, we established an in vitro cellular model using neurons differentiated from DS patient-derived induced pluripotent stem cells (iPSCs) and isogenic euploid iPSCs. Neurons differentiated from DS patient-derived iPSCs secreted more Aß compared to those differentiated from the euploid iPSCs. Treatment of the neurons with an antioxidant, N-acetylcysteine, significantly suppressed the Aß secretion. These findings suggest that oxidative stress has an important role in controlling the Aß level in neurons differentiated from DS patient-derived iPSCs and that N-acetylcysteine can be a potential therapeutic option to ameliorate the Aß secretion.

Treatment outcomes for infantile spasms in Japanese children with Down syndrome.

BACKGROUND: The aim of this study was to assess the treatment response to conventional antiepileptic drugs and low-dose adrenocorticotropic hormone therapy for infantile spasms in children with Down syndrome. METHODS: We retrospectively investigated the response and relapse rates, electroencephalography findings, patient characteristics during drug withdrawal, and developmental outcome in 10 children with Down syndrome treated for infantile spasms in our hospital. RESULTS: All patients showed cessation of infantile spasms and achieved electroencephalographic normalization. Spasm relapse occurred in one of 10 patients (10%). Antiepileptic drugs have been withdrawn for seven of 10 patients (70%), none of whom have experienced seizure relapse since drug withdrawal. The median developmental quotient (n = 8) was 20.5, which shows that the developmental outcome was unfavorable. Low-dose adrenocorticotropic hormone therapy achieved a low seizure remission rate of 28.6%. CONCLUSIONS: Elucidation of the optimal treatment for infantile spasms in children with Down syndrome is needed to reduce the duration of infantile spasms and improve the developmental outcome.

A nation-wide survey of Japanese pediatric MOG antibody-associated diseases.

OBJECTIVE: To elucidate the clinical characteristics of Japanese pediatric patients with acquired demyelinating diseases (ADS), positive for myelin oligodendrocyte glycoprotein antibody (MOG-IgG), we conducted a nation-wide survey. METHODS: Information about pediatric patients under 18 years old with ADS was solicited with surveys sent to 323 facilities. In an initial survey, we asked whether the center had any patients with ADS, and the MOG-IgG serostatus of the patients. In a follow-up survey, we requested more precise information on patients with ADS. RESULTS: Initial survey: 263 replies providing information on 175 patients were received. MOG-IgG were examined in 78 patients and 54 of those (69%) were positive for MOG-IgG. Follow-up survey: The characteristic involvement was optic neuritis, with visual disturbance and optic pain as characteristic symptoms. The relapse rate was 44% in patients positive for MOG-IgG, which was higher than that in seronegative patients (38%). For acute phase treatments, corticosteroid (CS), plasma exchange, and intravenous immunoglobulin (IVIG) were useful. To prevent relapse, CS, intermittent IVIG, immunosuppressants, and monoclonal antibodies were useful, but the efficacies of disease modifying drugs were uncertain. Sequelae such as visual disturbance, cognitive impairment, motor dysfunction, and epilepsy were observed in 11% of patients with MOG-IgG. CONCLUSIONS: MOG antibody-associated diseases were found to be common among pediatric ADS patients. Since a variety of sequelae were observed in these patients, it is important to identify the appropriate treatment to ensure the best outcome. The presence of the MOG autoantibody should be taken into consideration as part of the diagnostic criteria for pediatric ADS.

Secular trends in longevity among people with Down syndrome in Japan, 1995-2016.

BACKGROUND: Life expectancy in Japan has increased dramatically and is one of the longest in the world. However, the changes in lifespan in Japanese individuals with congenital diseases remain unknown. We investigated secular changes in the lifespan of people with Down syndrome over the last 20 years. METHODS: We observed secular trends in the number of stillbirths, deaths and the mortality rates at ages 20, 40, and 60 among all deaths registered with Down syndrome as the cause of death (ICD10 code: Q90) in the Japan national death registry database between 1995 and 2016. Changes in the median age at death between 1995-2005 and 2006-2016 were investigated based on sex and history of surgery. RESULTS: We identified 240 stillbirths and 1,099 deaths in this period. The annual number of stillbirths and deaths above the age of 1 year increased, whereas the number of deaths below 1 year did not change. The proportional mortality indicator at ages 20, 40, and 60 increased from 21.7%, 11.7%, and 1.7% in 1995 to 69.9%, 66.7%, and 36.6% in 2016, respectively. The median age at death was higher in females, individuals without a surgical history, and deaths occurring in 2006-2016. The median age at death increased over the period in those without a surgical history. CONCLUSIONS: The age at death among people with Down syndrome has increased over the last 20 years, with currently 1 in 3 persons living over 60 years, necessitating adequate social welfare services in this aging population.

Relationship between Skeletal Muscle Mass, Bone Mineral Density, and Trabecular Bone Score in Osteoporotic Vertebral Compression Fractures.

STUDY DESIGN: A retrospective observational study was performed. PURPOSE: We investigated the relationships between skeletal muscle mass, bone mineral density (BMD), and trabecular bone score (TBS) in patients with osteoporotic vertebral compression fractures (VCFs). OVERVIEW OF LITERATURE: The TBS has attracted attention as a measurement of trabecular bone microarchitecture. It is derived from data obtained using dual-energy X-ray absorptiometry (DXA) and is a reported indicator of VCFs, and its addition to the Fracture Risk Assessment Tool increases the accuracy of fracture prediction. METHODS: BMD, skeletal muscle mass, and TBS were measured in 142 patients who visited Shimoshizu National Hospital from April to August 2019. Patients were divided into a VCF group and a non-VCF group. Whole-body DXA scans were performed to analyze body composition, including appendicular skeletal muscle mass index (SMI; lean mass [kg]/height [m2]) and BMD. The diagnostic criteria for sarcopenia was an appendicular SMI <5.46 kg/m2. A logistic regression analysis was conducted to identify the risk factors for VCFs. RESULTS: The significant (p<0.05) findings (VCF group vs. non-VCF group, respectively) included age (79 vs. 70 years), femoral BMD (0.50 vs. 0.58 g/cm2), TBS (1.25 vs. 1.29), and lower limb muscle mass (8.6 vs. 9.9 kg). The VCF group was significantly older and had a lower femur BMD and decreased leg muscle mass than the non-VCF group. Based on the multiple logistic regression analysis, lower femoral BMD and decreased leg muscle mass were identified as risk factors for vertebral fracture independent of age, but the TBS was not. CONCLUSIONS: Patients with VCFs had low BMD, a low TBS, and low skeletal muscle mass. Lower femoral BMD and decreased leg muscle mass were identified as risk factors for VCFs independent of age, whereas the TBS was not identified as a risk factor for VCFs.

Myovascular Preserving Open-Door Laminoplasty for Cervical Spondylotic Myelopathy With Miniplate Fixation.

OBJECTIVE: Laminoplasty is an effective procedure for treating cervical spondylotic myelopathy. We conduct myovascular preserving open-door laminoplasty (MPLP) in combination with a laminoplasty plate to improve the stability of the enlarged lamina. We compare the details of the MPLP technique with conventional open-door laminoplasty. METHODS: We compared 25 cases of MPLP (mean age = 70.5, mean follow-up period = 19 months) with 15 controls who received conventional open-door laminoplasty using hydroxyapatite spacers (mean age = 74, mean follow-up period = 53 months). Regarding surgical outcomes, blood loss, operative time, Japanese Orthopaedic Association score, and postoperative visual analog score for neck pain were measured. Regarding image analysis, preoperative and postoperative range of motion (ROM), C2-7 angle, implant back out, hinge bone fusion time, presence or absence of hinge bone union failure, and posterior neck fat infiltration rate were evaluated. RESULTS: Operative time was significantly shorter for MPLP, and postoperative neck pain was significantly decreased. In image evaluation, %ROM was significantly increased in MPLP, but no difference in C2-7 angle existed between the 2 groups. Implant back out was not recognized in either group. In MPLP, the hinge union period was significantly shortened, and the postoperative fat infiltration rate was significantly decreased. CONCLUSIONS: We were able to reduce neck pain after surgery by an approach entailing longitudinal splitting of the spinous processes. We were able to ensure shorter operation times due to cervical plates and better hinge bone fusion times due to initial stability. LEVEL OF EVIDENCE: 4.

Helicobacter pylori test-and-treat strategy for second-year junior high school students aimed at the prevention of gastric cancer in Takatsuki City.

BACKGROUND: More than 90% of gastric cancer cases are caused by Helicobacter pylori infections. To prevent gastric cancer, an H pylori test-and-treat strategy targeting young people has been implemented in various places in Japan. In this study, we evaluated the effectiveness of an H pylori test-and-treat strategy for second-year junior high school students in Takatsuki City. MATERIALS AND METHODS: In 2014-2017, a urine-based H pylori test was used for initial screening. The final infection status was determined by a 13 C-urea breath test (13 C-UBT). Successful H pylori eradication was confirmed by 13 C-UBT 3 months after treatment. First-line eradication therapy was changed from 10 mg of rabeprazole, 750 mg of amoxicillin, and 200 mg of clarithromycin twice daily for 7 days in 2014 to 20 mg of vonoprazan, 750 mg of amoxicillin, and 200 mg of clarithromycin twice daily for 7 days in 2015-2017. Second-line eradication therapy included 10 mg of rabeprazole, 750 mg of amoxicillin, and 250 mg of metronidazole twice daily for 7 days. RESULTS: In total, 8067 of 13 055 students participated this project and 206 students were diagnosed with H pylori infection. The success rate of first-line therapy was 45.9% in 2014 and 83.8% after the revised first-line therapy was administered. The final eradication rate was 98.5%. There were no severe side effects. CONCLUSION: Our results support the use of the H pylori test-and-treat strategy for junior high school students as a safe approach for the prevention of gastric cancer. H pylori eradication therapy with vonoprazan could be a standard therapy in children.

Pilot study of oblique lumbar interbody fusion using mobile percutaneous pedicle screw and validation by a three-dimensional finite element assessment.

The purpose of this study was to try oblique lateral interbody fusion (OLIF) using percutaneous pedicle screws (PPS) with mobility. Twelve patients who underwent single-level OLIF were observed for at least one year. These included 6 patients with conventional PPS (rigid group), and 6 with movable PPS (semi-rigid group). Mobile PPS used cosmicMIA, which is a load sharing system. The anterior and posterior disc height, screw loosening and bone healing period, and implant failure were evaluated at final observation by CT. Moreover, the stress on the vertebral body-cage, on the vertebral body-screw/rod and on the bone around the screw was estimated using a three-dimensional finite element assessment in both groups. There was no significant difference in surgical time, amount of bleeding, JOA score, or low back pain VAS between groups. There were no differences between groups in anterior and posterior disc height, screw loosening, and implant failure at final observation. The bone healing period was significantly shorter in the semi-rigid screw group (18.3 months vs 4.8 months, p = 0.01). The finite element analysis showed that the lower stress on the rod/screw would contribute to fewer implant fractures and that lower stress on the bone around the screw would reduce screw loosening, and that higher compressive force on the cage would promotes bone healing. OLIF combined with a movable screw accelerated bone healing by nearly 75%. We conclude that mobile PPS in combination with OLIF promotes bone healing and can be a better vertebral fusion technique.

Validity of the Wide-range Assessment of Vision-related Essential Skills in Japanese Children with Learning Problems.

SIGNIFICANCE: Our study revealed that the validity of a new Japanese visual-perceptual test was acceptable. Visual-perceptual abilities are important to activities of daily living; thus, accurate assessment of visual perception is especially important for children with neurodevelopmental disorders and particularly so for those with learning problems. PURPOSE: Visual perception refers to the process by which one receives visual information through sensory impulses and then translates those impulses into meaning based on a previously developed view of the environment. A problem in Japan is the paucity of visual perception tests that use normative data from children who are native Japanese. The Wide-range Assessment of Vision-related Essential Skills (WAVES), which measures visual perception and eye-hand coordination skills and is based on Japanese normative data, was recently published in Japan. The validity of this test has not been comprehensively established. METHODS: To investigate the validity of the WAVES, we used the Pearson correlation coefficient to calculate the degrees of association among WAVES scaled and index scores compared with Developmental Test of Visual Perception, Third Edition, scores from 108 elementary school children with symptoms of learning problems. Participants were recruited at Osaka Medical College Learning Disability Center and Sakai Seikeikai Learning Disability Center. RESULTS: The concurrent validity of the WAVES was supported by moderate correlation (r = 0.67, P < .01) between the total scores for visual perception and eye-hand coordination index from the WAVES and general visual perception index from the Developmental Test of Visual Perception, Third Edition, even though a correlation analysis of subtests found differences between the two tests. CONCLUSIONS: Our results showed that the indices from the two tests measured nearly the same underlying visual-perceptual constructs and indicated that the WAVES had acceptable levels of concurrent validity.

Clinical findings in patients with febrile seizure after 5 years of age: A retrospective study.

OBJECTIVE: Febrile seizures (FSs) typically occur in infants and children between 6 and 60 months of age. Rarely, FS can occur in late childhood (late FS [LFS]; >5 years of age); however, the clinical features of LFS remain unclear. We aimed to clarify the clinical features of LFS. METHODS: We retrospectively analyzed data from patients with LFS who visited Hirakata City Hospital between January 2004 and December 2014. We defined LFS as a seizure accompanied by fever (temperature ≥38 °C) occurring after 5 years of age, without a central nervous system infection. RESULTS: A total of 505 patients (349 boys, 156 girls: 5-14 years old) were included. A history of FS before 60 months of age was observed in 319 of 460 patients (69.3%) with sufficient information about previous FS history among the 505 patients enrolled. LFS was more likely to occur in males (69.1%). Seizure duration was ≤15 min in 87.4% of cases. A family history of FS in first-degree relatives was observed in 103/327 cases (31.5%). Among LFS cases, 45% occurred at 5 years of age, and 92.1% experienced only one seizure after 5 years of age. The number of seizure episodes gradually lessened with age, decreasing drastically to 5.6% of cases older than 9 years. CONCLUSIONS: Our findings suggest that sex differences, seizure duration, and family history were similar for LFS and FS. Over 90% patients with LFS experienced no recurrence after 5 years of age. Further study is needed to verify the recurrence rate of LFS.

Simultaneous MR neurography and apparent T2 mapping of cervical nerve roots before microendoscopic surgery to treat patient with radiculopathy due to cervical disc herniation: Preliminary results.

There is no imaging modality to quantitatively evaluate compressed cervical nerve roots in cervical radiculopathy. Here we sought to evaluate the usefulness of simultaneous apparent T2 mapping and neurography with nerve-sheath signal increased with inked rest-tissue rapid acquisition of relaxation-enhancement imaging (SHINKEI-Quant) to evaluate compressed nerves quantitatively in patients with cervical radiculopathy due to cervical disc hernia before microendoscopic surgery. One patient with cervical radiculopathy due to cervical disc hernia before microendoscopic surgery and 5 healthy subjects underwent simultaneous apparent T2 mapping and neurography with SHINKEI-Quant. The patient was a 49-year-old man with severe right upper arm pain and numbness. Based on MRI images, we suspected right C7 radiculopathy due to C6-7 cervical disc hernia. The T2 relaxation times of the cervical dorsal root ganglia of the brachial plexus bilaterally at C5-C8 were measured. We observed no significant differences in T2 relaxation times between the nerve roots on the left and right at each spinal level with values in healthy subjects. In our patient, neurography revealed swelling of the right C7 nerve, and a prolonged T2 relaxation time compared with that of the contralateral, unaffected C7 nerve. We performed microendoscopic surgery and the symptoms improved. We were able to evaluate the injured nerve root quantitatively in a patient with cervical radiculopathy using the SHINKEI-Quant technique, being the first study to our knowledge to show the usefulness of this technique to evaluate cervical radiculopathy quantitatively before microendoscopic surgery.

Post-Operative Spinal Epidural Hematoma after Thoracic and Lumbar Spinous Process-Splitting Laminectomy for Thoracic and Lumbar Spinal Stenosis.

INTRODUCTION: To investigate the risk of epidural hematoma after spinous process-splitting laminectomy (SPSL). METHODS: A total of 137 cases (mean age, 72.4 years; 68 men) of SPSL were included. Of these, there were instances (3.7%; mean age, 70.5 years; all male) of postoperative development of new neurologic deficit due to epidural hematoma requiring reoperation. The 133 subjects (72.5 years; 64 men) with normal postoperative course were used as controls, and comparisons were made between both groups using chi-squared and Student's t-tests. Regarding our investigation of risk factors for epidural hematoma, logistic regression was conducted with presence or absence of hematoma as our primary outcome variable, and age, gender, disease duration, number of laminectomies, which levels were decompressed, blood loss, length of case, drain output, coagulopathy, and whether or not there was an intraoperative dural tear were our explanatory variables. RESULTS: All cases of hematoma were single-level laminectomies; there was one case of T9-10 and 3 cases of L2-3. In our direct comparison of both groups (hematoma versus control), the proportion of men was significantly higher in the hematoma group (100% versus 48%, p < 0.05); levels decompressed were also significantly higher (p < 0.05) in the hematoma group, and drain outputs were significantly lower (113 mL versus 234 mL, p < 0.05). From our logistic regression analysis, the levels were significantly higher (χ2 = 15, p = 0.0001) and the drain outputs were smaller (χ2 = 4.6, p = 0.03) in the hematoma group. CONCLUSIONS: Single-level decompression higher than the L2-3 level and reduced drain output were risk factors for spinal epidural hematoma. With this method of spinous process suturing and reconstruction there is less decompression compared with more conventional methods; therefore, the effect of hematoma may be more pronounced at higher vertebral levels with reduced canal width, and drain failure may also occur with this limited space.

Correlation of the endoscopic findings for small and large bowels in pediatric patients with established Crohn's disease.

Small bowel capsule endoscopy can detect subtle mucosal lesions in pediatric patients with Crohn's disease, and our aim was to evaluate its application in established ileocolonic Crohn's disease. Colonic inflammation was evaluated with the colonic Simple Endoscopic Score for Crohn's Disease (SES-CD) (excluding the score of the terminal ileum). Small bowel inflammation was evaluated with the Lewis score and/or Capsule Endoscopy Crohn's Disease Activity Index (CECDAI). A Lewis score <135 was defined as small bowel inactive. A colonic SES-CD of 0 (colonic inactive group) was observed in 22/42 procedures (52.4%), and active small bowel lesions were observed in 11/22 procedures (50.0%). The Lewis score was lower in the colonic inactive group compared to the colonic active group. Correlations between the colonic SES-CD, the Lewis score and CECDAI were weak. The Lewis score and CECDAI in the colonic inactive group had significant correlation with fecal calprotectin levels. We suggest that Crohn's disease patients without both colonic active lesions and elevation of fecal calprotectin levels may not need to receive small bowel capsule endoscopy due to low incidence of lesions in small bowel.

Diffusion tensor tractography of the lumbar nerves before a direct lateral transpsoas approach to treat degenerative lumbar scoliosis.

OBJECTIVEThe purpose of this study was to determine the relationship between vertebral bodies, psoas major morphology, and the course of lumbar nerve tracts using diffusion tensor imaging (DTI) before lateral interbody fusion (LIF) to treat spinal deformities.METHODSDTI findings in a group of 12 patients (all women, mean age 74.3 years) with degenerative lumbar scoliosis (DLS) were compared with those obtained in a matched control group of 10 patients (all women, mean age 69.8 years) with low-back pain but without scoliosis. A T2-weighted sagittal view was fused to tractography from L3 to L5 and separated into 6 zones (zone A, zones 1-4, and zone P) comprising equal quarters of the anteroposterior diameters, and anterior and posterior to the vertebral body, to determine the distribution of nerves at various intervertebral levels (L3-4, L4-5, and L5-S1). To determine psoas morphology, the authors examined images for a rising psoas sign at the level of L4-5, and the ratio of the anteroposterior diameter (AP) to the lateral diameter (lat), or AP/lat ratio, was calculated. They assessed the relationship between apical vertebrae, psoas major morphology, and the course of nerve tracts.RESULTSAlthough only 30% of patients in the control group showed a rising psoas sign, it was present in 100% of those in the DLS group. The psoas major was significantly extended on the concave side (AP/lat ratio: 2.1 concave side, 1.2 convex side). In 75% of patients in the DLS group, the apex of the curve was at L2 or higher (upper apex) and the psoas major was extended on the concave side. In the remaining 25%, the apex was at L3 or lower (lower apex) and the psoas major was extended on the convex side. Significant anterior shifts of lumbar nerves compared with controls were noted at each intervertebral level in patients with DLS. Nerves on the extended side of the psoas major were significantly shifted anteriorly. Nerve pathways on the convex side of the scoliotic curve were shifted posteriorly.CONCLUSIONSA significant anterior shift of lumbar nerves was noted at all intervertebral levels in patients with DLS in comparison with findings in controls. On the convex side, the nerves showed a posterior shift. In LIF, a convex approach is relatively safer than an approach from the concave side. Lumbar nerve course tracking with DTI is useful for assessing patients with DLS before LIF.

The findings of musculoskeletal ultrasonography on primary Sjögren's syndrome patients in childhood with articular manifestations and the impact of anti-cyclic citrullinated peptide antibody.

Objective: We researched the findings of musculoskeletal ultrasound sonography (MSUS) on primary Sjogren's syndrome in childhood (pSS-C) with articular manifestations. The correlation of rheumatoid factor (RF) and anti-cyclic citrullinated peptide antibody (ACPA) were investigated to evaluate the usefulness of MSUS on their articular prognosis. Method: The objective patients are pSS-C cases who visited our hospital complaining joint pain and/or joint swelling and for whom MSUS was performed. Result: Eight patients included 6 female and 2 male, 5 RF-positive patients and 3 ACPA- positive patients. The mean age of onset was 11.1 ± 3.0 years (352 physical joint findings and 284 MSUS findings. The number of joints found clinical articular manifestations was 58/352 joints, and arthritis detected by MSUS was 30/284 joints). In multivariate analysis, the odds ratio of clinical articular manifestations was significant high in RF-positivity (2.9, 95%CI 1.5-6.2). The odds ratio of arthritis detected by MSUS in ACPA-positivity was significant high (3.7, 95%CI 1.5-11.6), although odds ratio in RF-positivity had no statistical significance and a similar trend was seen in odds ratios of subclinical arthritis (4.9, 95%CI 1.6-18.0). Conclusion: It was indicated that MSUS is useful for pSS-C. ACPA-positive pSS-C patients have arthritis and subclinical arthritis more frequently than ACPA-negative patients.

Acetaminophen and Febrile Seizure Recurrences During the Same Fever Episode.

OBJECTIVES: To confirm the safety of using acetaminophen for febrile seizures (FSs) and to assess its efficacy in preventing FS recurrence during the same fever episode. METHODS: In this single-center, prospective, open, randomized controlled study, we included children and infants (age range: 6-60 months) with FSs who visited our hospital between May 1, 2015, and April 30, 2017. The effectiveness of acetaminophen was examined by comparing the recurrence rates of patients in whom rectal acetaminophen (10 mg/kg) was administered every 6 hours until 24 hours after the first convulsion (if the fever remained >38.0°C) to the rates of patients in whom no antipyretics were administered. No placebo was administered to controls. The primary outcome measure was FS recurrence during the same fever episode. RESULTS: We evaluated 423 patients; of these, 219 were in the rectal acetaminophen group, and 204 were in the no antipyretics group. In the univariate analysis, the FS recurrence rate was significantly lower in the rectal acetaminophen group (9.1%) than in the no antipyretics group (23.5%; P < .001). Among the variables in the final multiple logistic regression analysis, rectal acetaminophen use was the largest contributor to the prevention of FS recurrence during the same fever episode (odds ratio: 5.6; 95% confidence interval: 2.3-13.3). CONCLUSIONS: Acetaminophen is a safe antipyretic against FSs and has the potential to prevent FS recurrence during the same fever episode.

Altered Expression of Retinol Metabolism-Related Genes in an ANIT-Induced Cholestasis Rat Model.

Cholestasis is defined as a reduction of bile secretion caused by a dysfunction of bile formation. Insufficient bile secretion into the intestine undermines the formation of micelles, which may result in the reduced absorption of lipids and fat-soluble vitamins. Here, we investigated the retinol homeostasis and the alterations of retinol metabolism-related genes, including ß-carotene 15,15' monooxygenase (BCMO), lecithin:retinol acyltransferase (LRAT), aldehyde dehydrogenase (ALDH), cytochrome P450 26A1 (CYP26A1), and retinoic acid receptors (RAR) ß, in a α-naphthyl isothiocyanate (ANIT)-induced cholestasis rat model. Moreover, we examined the expression of the farnesoid X receptor (FXR) target genes. Our results showed that plasma retinol levels were decreased in ANIT rats compared to control rats. On the contrary, hepatic retinol levels were not different between the two groups. The expression of FXR target genes in the liver and intestine of cholestasis model rats was repressed. The BCMO expression was decreased in the liver and increased in the intestine of ANIT rats compared to control rats. Finally, the hepatic expression of LRAT, RARß, and ALDH1A1 in cholestatic rats was decreased compared to the control rats, while the CYP26A1 expression of the liver was not altered. The increased expression of intestinal BCMO in cholestasis model rats might compensate for decreased circulatory retinol levels. The BCMO expression might be regulated in a tissue-specific manner to maintain the homeostasis of retinol.