Treatment odyssey to epilepsy surgery in children with focal cortical dysplasia: Risk factors for delayed surgical intervention.

OBJECTIVE: To elucidate the patient's journey to epilepsy surgery and identify the risk factors contributing to surgical delay in pediatric patients with drug-resistant epilepsy (DRE) due to focal cortical dysplasia (FCD). METHODS: A retrospective review was conducted of 93 pediatric patients who underwent curative epilepsy surgery for FCD between January 2012 and March 2023 at a tertiary epilepsy center. The Odyssey plot demonstrated the treatment process before epilepsy surgery, including key milestones of epilepsy onset, first hospital visit, epilepsy diagnosis, MRI diagnosis, DRE diagnosis, and surgery. The primary outcome was surgical delay; the duration from DRE to surgery. Multivariate linear regression models were used to examine the association between surgical delay and clinical, investigative, and treatment characteristics. RESULTS: The median age at seizure onset was 1.3 years (interquartile range [IQR] 0.14-3.1), and at the time of surgery, it was 6 years (range 1-11). Notably, 46% experienced surgical delays exceeding two years. The Odyssey plot visually highlighted that surgical delay comprised a significant portion of the patient journey. Although most patients underwent MRI before referral, MRI abnormalities were identified before referral only in 39% of the prolonged group, compared to 70% of the non-prolonged group. Multivariate analyses showed that delayed notification of MRI abnormalities, longer duration from epilepsy onset to DRE, older age at onset, number of antiseizure medications tried, and moderate to severe intellectual disability were significantly associated with prolonged surgical delay. CONCLUSION: Pediatric DRE patients with FCD experienced a long journey until surgery. Early and accurate identification of MRI abnormalities is important to minimize surgical delays.

Effects of brief family psychoeducation on family caregiver burden of people with schizophrenia provided by psychiatric visiting nurses: a cluster randomised controlled trial.

BACKGROUND: The purpose of this study was to examine the effects of a brief family psychoeducation (BFP) programme provided by psychiatric visiting nurses on caregiver burden of family caregivers of people with schizophrenia through a cluster randomised controlled trial (cRCT). METHODS: The study was a two-arm, parallel-group cRCT. Forty-seven psychiatric visiting nurse agencies were randomly allocated to the BFP programme group (intervention group) or treatment as usual group (TAU; control group). Caregivers of people with schizophrenia were recruited by psychiatric visiting nurses using a randomly ordered list. The primary outcome was caregiver burden, measured using the Japanese version of the Zarit Burden Interview. Outcome assessments were conducted at baseline, 1-month follow-up, and 6-month follow-up. Intention-to-treat analysis was conducted to examine the effects of the BFP programme on caregiver burden. RESULTS: Thirty-four psychiatric visiting nurse agencies and 83 family caregivers of people with schizophrenia participated in the study. The participant attrition rate was less than 20%. Adherence to the program was 100%. Compared with TAU group, the BFP programme group had decreased caregiver burden. However, this improvement was not significant at 1-month follow-up (adjusted mean difference [aMD] = 0.27, 95% CI = - 5.48 to 6.03, p = 0.93, d = 0.01) or 6-month follow-up (aMD = - 2.12, 95% CI = - 7.80 to 3.56, p = 0.45, d = 0.11). CONCLUSIONS: The BFP programme provided by psychiatric visiting nurses did not achieve significant decreases in caregiver burden. This result may be attributed to the difficulty in continuing the research due to the COVID-19 pandemic, which prevented us from achieving the targeted sample size necessary to meet the statistical power requirements, as well as to the participation of caregivers with relatively low burden. However, the program had the advantage of high adherence to treatment plan. Further studies should be conducted with a larger sample size and a more diverse sample that includes caregivers with a higher care burden. TRIAL REGISTRATION: The study protocol was registered in the University Hospital Medical Information Network Clinical Trials Registry (UMIN000038044) on 2019/09/18.

A psychometric analysis of the Japanese version of the clinically useful depression outcome scale supplemented with questions for the DSM-5 anxious distress specifier (CUDOS-A).

AIM: The aim of the study was to identify the clinical significance of anxiety in those with depression, the Diagnostic and Statistical Manual of Mental Disorders 5th edition (DSM-5) defined criteria for an anxious distress specifier for major depressive disorder (MDD). The Clinically Useful Depression Outcome Scale (CUDOS) supplemented with questions for the DSM-5 anxious distress specifier (CUDOS-A) is a self-report instrument to assess the clinical significance of anxiety in addition to assess symptoms and the severity of depression. This study aimed to evaluate the psychometric properties of the Japanese version of the CUDOS-A. METHODS: An observational, prospective study was conducted with 131 MDD outpatients and 200 healthy controls. The Japanese version of the CUDOS-A, along with other measures, was administered to assess depressive symptoms, anxiety, social function, and biological rhythm. Reliability and validity analyses were performed, including internal consistency, test-retest reliability, convergent validity, and contrasted-groups validity. RESULTS: The Japanese version of the CUDOS-A demonstrated excellent internal consistency (Cronbach's alpha = 0.96) and test-retest reliability (ICC = 0.78). Significant positive correlations were found between the CUDOS-A and measures of depression, anxiety, social function, and biological rhythm (all, p < 0.001), supporting its convergent validity. The CUDOS-A effectively differentiated between patients with MDD and healthy controls (p < 0.001), indicating good contrasted-groups validity. CONCLUSIONS: The Japanese version of the CUDOS-A is a useful measure for research and for clinical practice, enabling the efficient assessment of anxious distress in individuals with depression.

Association of sugary drinks, carbonated beverages, vegetable and fruit juices, sweetened and black coffee, and green tea with subsequent depression: A five-year cohort study.

BACKGROUND & AIMS: Evidence on the impact of beverage consumption on depression is limited in the Asian population. Specifically, there is little information available on vegetable and fruit juices, while whole vegetables and fruits are reportedly protective against depression. Furthermore, evidence is scarce in differentiating the impacts of sweetened and black coffee. We aimed to examine the association of the consumption of total sugary drinks, carbonated beverages, vegetable and fruit juices, sweetened and black coffee, and green tea with subsequent depression in a general population sample. METHODS: We studied individuals without a history of cancer, myocardial infarction, stroke, diabetes, or depression at baseline in 2011-2016, with a five-year follow-up. We used Poisson regression models and the g-formula, thereby calculating the risk difference (RD) for depression. Multiple sensitivity analyses were conducted. Missing data were handled using random forest imputation. We also examined effect heterogeneity based on sex, age, and body mass index by analyzing the relative excess risk due to interaction and the ratio of risk ratios. RESULTS: In total, 94,873 individuals were evaluated, and 80,497 completed the five-year follow-up survey for depression. Of these, 18,172 showed depression. When comparing the high consumption group with the no consumption group, the fully adjusted RD (95% CI) was 3.6% (2.8% to 4.3%) for total sugary drinks, 3.5% (2.1% to 4.7%) for carbonated beverages, 2.3% (1.3% to 3.4%) for vegetable juice, 2.4% (1.1% to 3.6%) for 100% fruit juice, and 2.6% (1.9% to 3.5%) for sweetened coffee. In contrast, the fully adjusted RD (95% CI) was -1.7% (-2.6% to -0.7%) for black coffee. The fully adjusted RD for green tea did not reach statistical significance. The results were robust in multiple sensitivity analyses. We did not find substantial effect heterogeneity based on sex, age, and body mass index. CONCLUSIONS: Total sugary drinks, carbonated beverages, vegetable and fruit juices, and sweetened coffee may increase the risk of depression, whereas black coffee may decrease it.

Assessing the Quick Inventory of Depressive Symptomatology Self-Report scores to predict continuous employment in mood disorder patients.

Objective: Depression significantly impacts the job performance and attendance of workers, leading to increased absenteeism. Predicting occupational engagement for individuals with depression is of paramount importance. This study aims to determine the cut-off score which predicts continuous employment for patients with mood disorders using the Quick Inventory of Depressive Symptomatology, Self-Report (QIDS-SR). Methods: In a prospective observational trial conducted in Tokyo, 111 outpatients diagnosed with either major depressive disorder or bipolar depression were enrolled. Their employment statuses of these participants were tracked over a six-month period after their QIDS-SR scores were recorded. Based on their employment trajectories, participants were categorized into either continuous or non-continuous employment groups. Binary logistic regression was applied to examine the relationship between the QIDS-SR scores and employment outcomes, with adjustments for age, gender, and psychiatric diagnoses. Receiver operating characteristic curves were utilized to identify the optimal QIDS-SR cut-off values for predicting continuous employment. Findings: Binary logistic regression demonstrated that a lower score on the QIDS-SR was linked to an elevated likelihood of continuous employment (adjusted odds ratio 1.15, 95% CI: 1.06-1.26, p=0.001). The optimal cut-off point, determined by the Youden Index, was 10/11, showcasing a 63% sensitivity and 71% specificity. Conclusion: The results emphasize the potential of the QIDS-SR as a prognostic instrument for predicting employment outcomes among individuals with depressive disorders. These findings further underscore the importance of managing depressive symptoms to mild or lower intensities to ensure ongoing employment.

Requirements for hospitals in Japan to have low operative mortality and failure-to-rescue rates.

Aim: We explored institutional factors in Japan associated with lower operative mortality and failure-to-rescue (FTR) rates for eight major gastrointestinal procedures. Methods: A 22-item online questionnaire was sent to 2119 institutional departments (IDs) to examine the association between institutional factors and operative mortality and FTR rates. IDs were classified according to the number of annual surgeries, board certification status, and locality. In addition, the top 20% and bottom 20% of IDs were identified based on FTR rates and matched with the results of the questionnaire survey. Factors associated with operative mortality were selected by multivariate analysis. Results: Of the 1083 IDs that responded to the questionnaire, 568 (213 382 patients) were included in the analysis. Operative morbidity, operative mortality, and FTR rates in the top 20% and bottom 20% of IDs were 13.1% and 8.4% (p < 0.001), 0.52% and 4.3% (p < 0.001), and 4.0% and 51.2% (p < 0.001), respectively. Based on the patients' background characteristics, the top 20% of IDs handled more advanced cases. No significant difference in locality was seen between better or worse hospital FTR rates, but fewer esophagectomies, hepatectomies, and pancreatoduodenectomies were performed in depopulated areas. Six items were found to be associated with operative mortality by multivariate logistic analysis. Only 50 (8.8%) IDs met all five factors related to better FTR rates. Conclusions: The present findings indicate that several hospital factors surrounding surgical treatment, characterized by abundant human resources, are closely related to better postoperative recovery from severe complications.

The impact of COVID-19 on the diagnosis and treatment of HCC: analysis of a nationwide registry for advanced liver diseases (REAL).

The number of cancer cases diagnosed during the coronavirus disease 2019 (COVID-19) pandemic has decreased. This study investigated the impact of the pandemic on the clinical practice of hepatocellular carcinoma (HCC) using a novel nationwide REgistry for Advanced Liver diseases (REAL) in Japan. We retrieved data of patients initially diagnosed with HCC between January 2018 and December 2021. We adopted tumor size as the primary outcome measure and compared it between the pre-COVID-19 (2018 and 2019) and COVID-19 eras (2020 and 2021). We analyzed 13,777 patients initially diagnosed with HCC (8074 in the pre-COVID-19 era and 5703 in the COVID-19 era). The size of the maximal intrahepatic tumor did not change between the two periods (mean [SD] = 4.3 [3.6] cm and 4.4 [3.6] cm), whereas the proportion of patients with a single tumor increased slightly from 72.0 to 74.3%. HCC was diagnosed at a similar Barcelona Clinic Liver Cancer stage. However, the proportion of patients treated with systemic therapy has increased from 5.4 to 8.9%. The proportion of patients with a non-viral etiology significantly increased from 55.3 to 60.4%. Although the tumor size was significantly different among the etiologies, the subgroup analysis showed that the tumor size did not change after stratification by etiology. In conclusion, the characteristics of initially diagnosed HCC remained unchanged during the COVID-19 pandemic in Japan, regardless of differences in etiology. A robust surveillance system should be established particularly for non-B, non-C etiology to detect HCC in earlier stages.

Impact of the novel coronavirus infection on pediatric surgery: an analysis of data from the National Clinical Database.

PURPOSE: The coronavirus disease 2019 (COVID-19) pandemic limited the delivery of medical resources. Although surgeries are triaged according to disease severity and urgency, a delay in diagnosis and surgery can be detrimental. We conducted this study to analyze data on the impact of the COVID-19 pandemic on pediatric surgery for different diseases or disorders. METHODS: We compiled and compared data on pediatric surgical cases from 2018 to 2020, using the National Clinical Database. The number of diseases, severity, complication rates, mortality rates by disease/disorder, and the COVID-19 pandemic areas were analyzed. RESULTS: The total number of cases of pediatric surgery in 2018, 2019, and 2020 was 50,026, 49,794, and 45,621, respectively, reflecting an 8.8% decrease in 2020 from 2018 and an 8.4% decrease in 2020 from 2019. A decrease was observed when the number of patients with COVID-19 was high and was greater in areas with a low infection rate. There was a marked decrease in the number of inguinal hernia cases. The number of emergency room visits and emergency surgeries decreased, but their relative proportions increased. CONCLUSIONS: The COVID-19 pandemic decreased the number of pediatric surgeries, reflecting the limitations of scheduled surgeries and infection control measures.

A multimodal deep learning approach for the prediction of cognitive decline and its effectiveness in clinical trials for Alzheimer's disease.

Alzheimer's disease is one of the most important health-care challenges in the world. For decades, numerous efforts have been made to develop therapeutics for Alzheimer's disease, but most clinical trials have failed to show significant treatment effects on slowing or halting cognitive decline. Among several challenges in such trials, one recently noticed but unsolved is biased allocation of fast and slow cognitive decliners to treatment and placebo groups during randomization caused by the large individual variation in the speed of cognitive decline. This allocation bias directly results in either over- or underestimation of the treatment effect from the outcome of the trial. In this study, we propose a stratified randomization method using the degree of cognitive decline predicted by an artificial intelligence model as a stratification index to suppress the allocation bias in randomization and evaluate its effectiveness by simulation using ADNI data set.

Feasibility of remote interviews in assessing disease severity in patients with major depressive disorder: A pilot study.

AIM: Interview quality is an important factor in the success of clinical trials for major depressive disorder (MDD). There is a substantial need to establish a reliable, remote clinical assessment interview system that can replace traditional in-person interviews. METHODS: We conducted a multicenter, randomized, unblinded, prospective, cross-sectional study to assess the reliability of remote interviews in patients with MDD (UMIN000041839). Eligible patients with MDD underwent remote and in-person sessions of the Montgomery-Åsberg Depression Rating Scale (MADRS) assessment performed by different raters within 28 days of providing consent. Patients were randomized to a group first assessed using in-person interviews and secondarily using remote interviews (in-person-first group) or a group first assessed by remote interviews and secondarily using in-person interviews (remote-first group). Nineteen trained people (15 clinical psychologists, 3 nurses, and 1 clinical laboratory technologist) performed interviews. RESULTS: Of 59 patients (in-person-first group, n = 32; remote-first group, n = 27) who completed both remote and in-person interviews, 51% (n = 30) were women; the mean age was 41.6 years (range, 21-64 years). There was a strong association between remote and in-person MADRS scores (r = 0.891, kappa = 0.901). An overall intraclass correlation coefficient (ICC) of 0.886 (95% confidence interval, 0.877-0.952) indicated good consistency between MADRS scores in remote and in-person interviews. The ICC decreased as the severity of depression increased. CONCLUSION: Our results suggest remote interviews are a feasible alternative option to in-person interviews in assessing symptom severity in MDD patients and could promote clinical trials in Japan.

A multicenter, single-arm, open-label interventional study of adherence to brexpiprazole during switching from previous antipsychotic drugs in patients with schizophrenia or schizoaffective disorder.

The rate of medication persistence was examined in patients with schizophrenia or schizoaffective disorder during switching from previously administered antipsychotics to brexpiprazole, a new dopamine D2 receptor partial agonist. A multicenter, single-arm, open-label 24-week interventional study was conducted, consisting of two 12-week consecutive periods: an initial switch (by plateau cross-titration) with the subsequent period, followed by a second maintenance period. Prior antipsychotics were olanzapine or risperidone/paliperidone. The primary and secondary outcome measures were medication persistence rates after the first 12 weeks and changes from baseline in the Specific Levels of Functioning Scale (SLOF), Subjective Well-being under Neuroleptic drug treatment Short form (SWNS), and Positive and Negative Syndrome Scale (PANSS) scores, respectively. In total, 79 patients were administered brexpiprazole and the medication persistence rate at 12 weeks was 78.5%, which was significantly higher than the predefined threshold of 65%. Regarding the prior medication, the persistence rate at 12 weeks was 84.6% for olanzapine and 72.5% for risperidone/paliperidone. Significant improvements from baseline were observed in the SLOF, SWNS, and PANSS scores. There were no adverse events of concern. Thus, brexpiprazole appeared to be a suitable antipsychotic on switching from olanzapine, risperidone, or paliperidone.

Distinct Clinical Effects of Two RP1L1 Hotspots in East Asian Patients With Occult Macular Dystrophy (Miyake Disease): EAOMD Report 4.

Purpose: To characterize the clinical effects of two RP1L1 hotspots in patients with East Asian occult macular dystrophy (OMD). Methods: Fifty-one patients diagnosed with OMD harboring monoallelic pathogenic RP1L1 variants (Miyake disease) from Japan, South Korea, and China were enrolled. Patients were classified into two genotype groups: group A, p.R45W, and group B, missense variants located between amino acids (aa) 1196 and 1201. The clinical parameters of the two genotypes were compared, and deep learning based on spectral-domain optical coherence tomographic (SD-OCT) images was used to distinguish the morphologic differences. Results: Groups A and B included 29 and 22 patients, respectively. The median age of onset in groups A and B was 14.0 and 40.0 years, respectively. The median logMAR visual acuity of groups A and B was 0.70 and 0.51, respectively, and the survival curve analysis revealed a 15-year difference in vision loss (logMAR 0.22). A statistically significant difference was observed in the visual field classification, but no significant difference was found in the multifocal electroretinographic classification. High accuracy (75.4%) was achieved in classifying genotype groups based on SD-OCT images using machine learning. Conclusions: Distinct clinical severities and morphologic phenotypes supported by artificial intelligence-based classification were derived from the two investigated RP1L1 hotspots: a more severe phenotype (p.R45W) and a milder phenotype (1196-1201 aa). This newly identified genotype-phenotype association will be valuable for medical care and the design of therapeutic trials.

Influence of surgical volume on the mortality and morbidity of gastrointestinal perforation in children.

PURPOSE: To clarify the influence of surgical volume on the mortality and morbidity of gastrointestinal perforation in children in Japan. METHODS: We collected data on pediatric patients with gastrointestinal perforation between 2017 and 2019, from the National Clinical Database. The surgical volumes of various institutions were classified into three groups: low (average number of surgeries for gastrointestinal perforation/year < 1), medium (≥ 1, < 6), and high (≥ 6). The observed-to-expected (o/e) ratios of 30-day mortality and morbidity were calculated for each group using an existing risk model. RESULTS: Among 1641 patients (median age, 0.0 years), the 30-day mortality and morbidity rates were 5.2% and 37.7%, respectively. The 30-day mortality rates in the low-, medium-, and high-volume institutions were 4.9%, 5.3%, and 5.1% (p = 0.94), and the 30-day morbidity rates in the three groups were 26.8%, 39.7%, and 37.7% (p < 0.01), respectively. The o/e ratios of 30-day mortality were 1.05 (95% confidence interval [CI] 0.83-1.26), 1.08 (95% CI 1.01-1.15), and 1.02 (95% CI 0.91-1.13), and those of 30-day morbidity were 1.72 (95% CI 0.93-2.51), 1.03 (95% CI 0.79-1.28), and 0.95 (95% CI 0.56-1.33), respectively. CONCLUSION: Surgical volume does not have significant impact on the outcomes of pediatric gastrointestinal perforation in Japan.

Intraventricular hemorrhage volume and younger age at surgery may be risk factors for postoperative hydrocephalus after hemispherotomy in children.

OBJECTIVE: Hemispherotomy is an effective treatment for intractable hemispheric epilepsy; however, hydrocephalus remains a common complication of the procedure. The causes of hydrocephalus following hemispherotomy have not been fully elucidated; therefore, the purpose of this study was to identify the risk factors associated with the condition. METHODS: The authors investigated the records of all patients aged < 18 years who underwent hemispherotomy at their institution between 2003 and 2020 and were monitored for hydrocephalus for at least 1 year after the procedure. To identify the risk factors for hydrocephalus, the following information about each patient was collected: sex, corrected age at surgery, body weight at surgery, previous intracranial surgery, etiology of epilepsy, results of PET for hypermetabolism, side of surgery, type of operation (vertical or horizontal approach), operation time, blood loss during surgery, use of intraventricular drainage, occurrence of intraventricular hemorrhage (IVH) on the 1st postoperative day, duration of postoperative fever of > 38°C, and maximum C-reactive protein level after the operation. Multivariate logistic regression analyses were performed. RESULTS: This study included 51 children who underwent hemispherotomies for drug-resistant epilepsy at our hospital. Seven patients (13.7%) experienced hydrocephalus and were treated with ventricular or subdural peritoneal shunts or fenestration. Multivariate logistic analysis using the Bayesian information criterion revealed that 3 factors were associated with the occurrence of hydrocephalus: age at surgery, postoperative IVH volume, and duration of postoperative fever of > 38°C. CONCLUSIONS: This study showed that younger age at surgery, postoperative IVH volume, and duration of postoperative fever of > 38°C might be risk factors for hydrocephalus after hemispherotomy. The risk of hydrocephalus should be considered in cases of early surgical indication in children. Intraoperative hemostasis and postoperative use of anti-inflammatory measures may reduce the risk of hydrocephalus.

Natural history of Becker muscular dystrophy: a multicenter study of 225 patients.

OBJECTIVE: Becker muscular dystrophy (BMD) is a milder variant of Duchenne muscular dystrophy (DMD), a lethal X-linked muscular disorder. Here, we aim to investigat the clinical involvement of skeletal, respiratory, cardiac, and central nervous systems in patients with BMD, as well as genotype-phenotype relationships. METHODS: This nationwide cohort study investigated the clinical manifestations and genotype-phenotype relationships in 225 patients with BMD having in-frame deletion from 22 medical centers. The primary outcome was to elucidate the association of genotype with skeletal muscle, respiratory, cardiac, and central nervous system disorders. Descriptive statistics were used to analyze the data. RESULTS: The average age of the subjects was 31.5 (range, 1-81) years. Initial symptoms of BMD were muscular (60%), followed by asymptomatic hypercreatine kinasemia (32.4%) and central nervous system disorders (5.3%). Gait disturbance was observed in 53.8% of patients and the average age at wheelchair introduction was 36.5 years. The ventilator introduction rate was 6.7% at an average age of 36.6 years. More than 30% of patients had an abnormal electrocardiogram and approximately 15% had heart failure symptoms. Cardiac function on echocardiography varied significantly among the patients. The frequencies of seizures and intellectual/developmental disability were 8.0% and 16.9%, respectively. Exon 45-47deletion (del) was the most common (22.6%), followed by exon 45-48del (13.1%). Patients with exon 45-49del patients demonstrated severe skeletal muscle damage. Patients with exon 45-47del and exon 45-55del patients did not require ventilator use. INTERPRETATION: The study provides important prognostic information for patients and clinicians to establish therapy plans and to implement preventative medicine.

Long-term Observation in Patients with Duchenne Muscular Dystrophy with Early Introduction of a Standing Program Using Knee-ankle-foot Orthoses.

Objectives: This study investigated the outcomes of the early introduction of a standing program for patients with Duchenne muscular dystrophy (DMD). Methods: This was a retrospective observational study of 41 outpatients with DMD aged 15-20 years. We introduced the standing program using knee-ankle-foot orthoses (KAFO) to slow the progression of scoliosis when ankle dorsiflexion became less than 0° in the ambulatory period. Results: Thirty-two patients with DMD were offered the standing program with KAFO; 12 continued the program until the age of 15 years (complete group) and 20 discontinued the program before the age of 15 years (incomplete group). The non-standing program group included 9 patients. The standing program with KAFO was significantly associated with the Cobb angle at the age of 15 years after adjustment for the duration of corticosteroid use and DMD mutation type (P=0.0004). At the age of 15 years, significant correlations were found between the ankle dorsiflexion range of motion (ROM) and non-ambulatory period (P=0.0010), non-ambulatory period and Cobb angle (P<0.0001), Cobb angle and percent predicted forced vital capacity (P=0.0004), and ankle dorsiflexion ROM and Cobb angle (P=0.0066). In the complete group, the age at ambulation loss (log-rank P=0.0015), scoliosis progression (log-rank P=0.0032), and pulmonary dysfunction (log-rank P=0.0006) were significantly higher than in the non-standing program group. Conclusions: The early introduction of a standing program for DMD patients may prolong the ambulation period and slow the progression of scoliosis and pulmonary dysfunction.

Japanese public health nurses classified based on empathy and secondary traumatic stress: variable-centered and person-centered approaches.

BACKGROUND: Healthcare providers frequently help traumatized people and are regularly exposed to indirect trauma from their work, resulting in negative psychological responses, such as secondary traumatic stress. Empathy has been associated with patient's quality of care and secondary traumatic stress among healthcare providers. However, the relationship between dispositional empathy and secondary traumatic stress has not been fully elucidated. This study used person- and variable-centered approaches to explore the nature of this relationship. METHODS: A total of 1,006 Japanese public health nurses working in the Tohoku region and Saitama prefecture completed questionnaires that included scales assessing dispositional empathy, secondary traumatic stress, and burnout. First, we examined predictors of secondary traumatic stress using multiple linear regression analysis. Then, we conducted a latent profile analysis to classify participants into unique groups based on four subscales of dispositional empathy (i.e., empathic concern, perspective taking, personal distress, fantasy) and secondary traumatic stress. Finally, we compared the mean values of the study variables across these groups. RESULTS: The multiple regression indicated that in those working in Saitama prefecture, lifetime traumatic experiences, work-related distress, and personal distress were positively related to secondary traumatic stress, but perceived support was negatively related to secondary traumatic stress. Latent profile analysis extracted four unique subgroups. Group 1 displayed the highest secondary traumatic stress levels. Group 2 was characterized by the highest level of empathic concern, personal distress, and fantasy and the lowest perspective taking. Group 3 had a moderate secondary traumatic stress level. Group 4 had the lowest secondary traumatic stress and personal distress scores. In these four groups, the burnout scale (exhaustion, cynicism, and professional efficacy) showed a pattern similar to the secondary traumatic stress scale. CONCLUSIONS: Our person-centered approach showed that this sample of public health nurses could be classified into four unique groups based on their empathy and secondary traumatic stress scores. Although this group of public health nurses was not large, one group displayed high personal distress levels and high secondary traumatic stress levels. Further research is needed to determine effective interventions for this group.

Severe Disease Activity May Predispose Patients to Post-colectomy Duodenitis Associated with Ulcerative Colitis.

Objective Diffuse mucosal inflammation in the duodenum, distinct from peptic ulcer disease, has been repeatedly reported in patients with ulcerative colitis (UC). The pathogenesis of this complication remains uncertain; however, colectomy for medically refractory UC appears to trigger duodenitis. Cases in which colectomy was performed for UC were analyzed to characterize UC-related duodenitis after colectomy. Methods A retrospective case-control study of UC-related duodenitis that developed after colectomy in medically refractory UC between January 2011 and June 2020 was conducted. UC-related duodenitis was diagnosed based on typical clinical, endoscopic, and histological findings, and no duodenitis was endoscopically defined by the normal duodenal mucosa. Clinical and laboratory data, disease severity, and medications used were collected and compared between the UC-related and non-duodenitis cases. Results Ten UC-related duodenitis and 35 non-duodenitis cases were identified among 45 patients with UC who underwent esophagogastroduodenoscopy after colectomy. Disease severity, defined by the C-reactive protein level and partial Mayo score prior to colectomy, was significantly higher in duodenitis patients than in non-duodenitis patients. In comparison to non-duodenitis patients, duodenitis patients more frequently received rescue therapies with calcineurin inhibitors or anti-TNF-α agents at the time of colectomy (100% vs. 65.7%). Conclusion Patients with UC with higher disease activity, especially those who require rescue therapies with calcineurin inhibitors and anti-TNF-α agents, may be prone to developing UC-related duodenitis after colectomy.

Occult Macular Dysfunction Syndrome: Identification of Multiple Pathologies in a Clinical Spectrum of Macular Dysfunction with Normal Fundus in East Asian Patients: EAOMD Report No. 5.

Occult macular dystrophy (OMD) is the most prevalent form of macular dystrophy in East Asia. Beyond RP1L1, causative genes and mechanisms remain largely uncharacterised. This study aimed to delineate the clinical and genetic characteristics of OMD syndrome (OMDS). Patients clinically diagnosed with OMDS in Japan, South Korea, and China were enrolled. The inclusion criteria were as follows: (1) macular dysfunction and (2) normal fundus appearance. Comprehensive clinical evaluation and genetic assessment were performed to identify the disease-causing variants. Clinical parameters were compared among the genotype groups. Seventy-two patients with OMDS from fifty families were included. The causative genes were RP1L1 in forty-seven patients from thirty families (30/50, 60.0%), CRX in two patients from one family (1/50, 2.0%), GUCY2D in two patients from two families (2/50, 4.0%), and no genes were identified in twenty-one patients from seventeen families (17/50, 34.0%). Different severities were observed in terms of disease onset and the prognosis of visual acuity reduction. This multicentre large cohort study furthers our understanding of the phenotypic and genotypic spectra of patients with macular dystrophy and normal fundus. Evidently, OMDS encompasses multiple Mendelian retinal disorders, each representing unique pathologies that dictate their respective severity and prognostic patterns.

Morbidity after left trisectionectomy for hepato-biliary malignancies: An analysis of the National Clinical Database of Japan.

BACKGROUND: The aim of this study was to analyze the nationwide surgical outcome of a left trisectionectomy (LT) and to identify the perioperative risk factors associated with its morbidity. METHODS: Cases of LT for hepato-biliary malignancies registered at the Japanese National Clinical Database between 2013 and 2019 were retrospectively reviewed. Statistical analyses were performed to identify the perioperative risk factors associated with a morbidity of Clavien-Dindo classification (CD) ≥III. RESULTS: Left trisectionectomy was performed on 473 and 238 cases of biliary and nonbiliary cancers, respectively. Morbidity of CD ≥III and V occurred in 45% and 5% of cases with biliary cancer, respectively, compared with 26% and 2% of cases with nonbiliary cancer, respectively. In multivariable analyses, biliary cancer was significantly associated with a morbidity of CD ≥III (odds ratio, 1.87; p = .018). In subgroup analyses for biliary cancer, classification of American Society of Anesthesiologists physical status (ASA-PS) 2, portal vein resection (PVR), and intraoperative blood loss ≥30 mL/kg were significantly associated with a morbidity of CD ≥III. CONCLUSIONS: Biliary cancer induces severe morbidity after LT. The ASA-PS classification, PVR, and intraoperative blood loss indicate severe morbidity after LT for biliary cancer.