Study protocol for a type-II hybrid effectiveness-implementation trial to reach teenagers using mobile money shops to reduce unintended pregnancies in Uganda.

INTRODUCTION: Unintended teenage pregnancies have become a global public health challenge, particularly in sub-Saharan Africa. There is a notably high prevalence of unintended pregnancies among unmarried teenagers in Uganda. This study will develop an intervention programme using mobile money shops (vendors) as a platform to deliver sexual and reproductive health and rights (SRHR) services to teenagers and assess its effectiveness and scalability in Uganda. METHODS AND ANALYSES: This hybrid study comprises two integral components: an intervention study to assess the effectiveness of vendor-mediated intervention and implementation research to evaluate the implementation process. 30 vendors will be recruited for both intervention and control arms in 2 municipalities in Eastern Uganda, which have a high unintended pregnancy prevalence rate among unmarried teens aged 15-19 years. A preintervention and postintervention repeated survey involving 600 participants for each arm will be conducted over 4 months. The primary outcome is the rate of condom users among teenage vendor users. The secondary outcomes include the rate of preference for receiving SRHR services at vendors and knowledge regarding SRHR. A difference-in-differences analysis will be used to determine the effectiveness of the intervention. The Bowen model will be employed to evaluate the implementation design. ETHICS AND DISSEMINATION: Ethical approval was obtained from the Ethics Review Committee of Uganda Christen University and JICA Ogata Sadako Research Institute for Peace and Development in Japan. The findings will be widely disseminated. This study was registered with the University Hospital Medical Information Network in Japan (UMIN000053332) on 12 January 2024. TRIAL REGISTRATION NUMBER: UMIN000053332.

Anxiety and its risk factors among non-Japanese residents living in Japan undergoing COVID-19 situation: A cross-sectional survey.

INTRODUCTION: In the context of collective efforts taken in Japan to control the spread of COVID-19, the state of emergency and social distancing have caused a negative impact on the mental health of all residents, including foreign communities in Japan. This study aimed to evaluate the level of anxiety and its associated factors among non-Japanese residents residing in Japan during the COVID-19 pandemic. METHODS: A web-based survey in 13 languages was conducted among non-Japanese residents living in Japan during the COVID-19 situation. The State-Trait Anxiety Inventory assessed the level of anxiety-State (STAI-S) scores prorated from its six-item version. The multivariable logistic regression using the Akaike Information Criterion (AIC) method was performed to identify the associated factors of anxiety among participants. RESULTS: From January to March 2021, we collected 392 responses. A total of 357 valid responses were analyzed. 54.6% of participants suffered from clinically significant anxiety (CSA). In multivariable logistic model analysis, the CSA status or the high level of anxiety was associated with three factors, including having troubles/difficulties in learning or working, decreased sleep duration, and decreased overall physical health (p<0.05). CONCLUSION: Our study suggests several possible risk factors of anxiety among non-Japanese residents living in Japan undergoing the COVID-19 pandemic, including the troubles or difficulties in learning or working, the decrease in sleep duration, and the decrease in overall physical health.

Molecular phylogenetic analysis of Echinococcus multilocularis from horses raised in Canada or Japan, using mitochondrial cytochrome b gene-targeted PCR.

Alveolar echinococcosis is a zoonotic disease caused by a larval-stage Echinococcus multilocularis infection. Geographical haplotyping targeting the parasite's mitochondrial cytochrome b (cob) gene has been reported for isolates from definitive and intermediate hosts (wild canids and rodents); however, there are limited reports on strain typing for the dead-end host, the horse, which could act as a sentinel for E. multilocularis. Accordingly, we investigated the diversity of E. multilocularis in isolates obtained from slaughtered Japanese and Canadian horses originating from the Iburi and Hidaka regions in Hokkaido and from Alberta, respectively, with PCR and haplogroup analyses targeting cob gene sequences obtained. Seventy horses were diagnosed with alveolar echinococcosis based on histopathology and cob-gene PCR testing. The E. multilocularis detected in these horses was classified as either an Asian (for Hokkaido-raised horses) or a European (for Alberta-raised horses) haplogroup, based on the obtained cob-gene sequence analysis. In addition, haplotype network analysis revealed that E. multilocularis isolated from Hokkaido-raised horses is highly homologous to Kazakhstan isolates, and E. multilocularis isolated from Alberta-raised horses is highly homologous to Austrian isolates. The results of this study suggest that cob-gene-targeted PCR analysis could be useful for the geographical genetic characterization of E. multilocularis isolated from horses.

Intraocular pressure changes at different gaze positions after superior rectus muscle-lateral rectus muscle loop myopexy in highly myopic strabismus.

PURPOSE: To evaluate changes in intraocular pressure (IOP) at different gaze positions before and after superior rectus muscle-lateral rectus muscle (SR-LR) loop myopexy in highly myopic strabismus (HMS). STUDY DESIGN: Nonrandomized clinical, prospective, interventional trial. METHODS: Fourteen patients with HMS (18 eyes) who underwent SR-LR loop myopexy were divided into 3 groups: < 100 prism diopters (PD) (mild esotropia [ET] group), > 100 PD (large ET group), and > 100 PD, and simultaneous recession of the medial rectus (MR) muscle was performed (large ET + MR group). Intraocular pressure was measured preoperatively and postoperatively at the primary, abduction, and adduction positions in each group. RESULTS: Intraocular pressure did not change after surgery in the mild ET group. Intraocular pressure significantly decreased in the abduction position (from 20.0 ± 2.1 to 16.0 ± 1.9 mmHg, P = 0.043) in the large ET group and in the abduction (from 22.2 ± 5.9 to 15.6 ± 4.3 mmHg, P = 0.048) and primary positions (from 15.8 ± 5.0 to 10.2 ± 2.8 mmHg, P = 0.043) in the large ET + MR group. The preoperative significant differences in IOP between the abduction and adduction positions in the large ET group (7.4 ± 3.4 mmHg) and the large ET + MR group (10.0 ± 5.5 mmHg) disappeared postoperatively (3.2 ± 2.8 mmHg and 3.6 ± 1.7 mmHg, respectively). The differences in IOP between abduction and adduction were similar in all the groups. CONCLUSION: SR-LR loop myopexy decreased IOP in patients with HMS in the abduction and primary positions.

Extragonadal recurrence of benign mature teratoma in the pouch of Douglas.

Laparoscopic cystectomy for mature teratomas is associated with a high incidence of intraperitoneal spillage and tumor spread; however, extragonadal recurrence of this benign tumor is rare. We hereby present an additional case of extragonadal mature teratoma that recurred in the pouch of Douglas after ovarian cystectomy. A 43-year-old Japanese woman presented with atypical genital bleeding. A 7 cm mature teratoma was detected using transvaginal ultrasonography and magnetic resonance imaging. At 26 years old, she underwent bilateral cystectomy for bilateral mature teratoma of the ovary. During laparoscopic surgery, a cystic tumor appeared in the pouch of Douglas and was firmly adhered to the surrounding tissues. Both ovaries were normal. The resected tumor was diagnosed as extragonadal, benign, mature teratoma. To avoid the extragonadal recurrence of mature teratoma, removal of tumor contents from intraperitoneal spillage by lavage should be performed at the end of surgery.

Barriers to the hospital treatment among Bede snake charmers in Bangladesh with special reference to venomous snakebite.

Snakebite envenoming is a potentially life-threatening global public health issue with Bangladesh having one of the highest rates of snakebite cases. The Bede, a nomadic ethnic group in Bangladesh, traditionally engages in snake-related business such as snake charming. The Bede relies on their own ethnomedicinal practitioners for snakebite treatment while there is a lack of concrete evidence on the effectiveness of such ethnomedicinal treatment. To identify the barriers to the utilization of biomedical treatment for snakebite we conducted interviews with 38 Bede snake charmers, who have experienced snakebite, and six family members of those who died of snakebite. Our results show that four critical barriers, Accessibility, Affordability, Availability, and Acceptability (4As), prevented some of the Bede from seeking biomedical treatment. Moreover, we found that a few Bede died of a snakebite every year. There are survivors of snakebite who were able to receive biomedical treatment by overcoming all of the 4As. Our results provide insights into the current state of snakebite treatment in Bangladesh and can inform the development of more effective and accessible treatment options for those affected. Partnership between the public sector and the Bede community has the potential to make a significant impact in reducing snakebite morbidity and mortality in Bangladesh.

The Structural Abnormalities Are Deeply Involved in the Cause of RPGRIP1-Related Retinal Dystrophy in Japanese Patients.

Leber congenital amaurosis (LCA) is the most severe form of inherited retinal dystrophy. RPGRIP1-related LCA accounts for 5-6% of LCA. We performed whole-exome sequencing and whole-genome sequencing (WGS) on 29 patients with clinically suspected LCA and examined ophthalmic findings in patients with biallelic pathogenic variants of RPGRIP1. In addition to five previously reported cases, we identified five cases from four families with compound heterozygous RPGRIP1 variants using WGS. Five patients had null variants comprising frameshift variants, an Alu insertion, and microdeletions. A previously reported 1339 bp deletion involving exon 18 was found in four cases, and the deletion was relatively prevalent in the Japanese population (allele frequency: 0.002). Microdeletions involving exon 1 were detected in four cases. In patients with RPGRIP1 variants, visual acuity remained low, ranging from light perception to 0.2, and showed no correlation with age. In optical coherence tomography images, the ellipsoid zone (EZ) length decreased with age in all but one case of unimpaired EZ. The retinal structure was relatively preserved in all cases; however, there were cases with great differences in visual function compared to their siblings and a 56-year-old patient who still had a faint EZ line. Structural abnormalities may be important genetic causes of RPGRIP1-related retinal dystrophy in Japanese patients, and WGS was useful for detecting them.

Clinical presentations of acquired comitant esotropia in 5-35 years old Japanese and digital device usage: a multicenter registry data analysis study.

PURPOSE: To describe clinical presentations of acquired comitant esotropia and digital device use in children, adolescents, and young adults without neurological problems. STUDY DESIGN: Multicenter prospective observational study. METHODS: Patients with acquired comitant esotropia, without intracranial diseases aged 5-35 years at the time of visit, who were seen at pre-registered facilities within 1 year of onset were enrolled. The duration from the onset of symptoms and the time of digital device usage approximately 1 month before onset and their lifestyles were surveyed. Visual acuity, cycloplegic refraction, and strabismus angles were measured. Data were analyzed in three age groups (Child: 5-12 years, Adolescent: 13-18 years, and Young adult: 19-35 years). RESULTS: Between November 2019 and December 2021, 218 patients were enrolled from 55 facilities, and 194 patients (including 62 children, 69 adolescents, and 63 young adults) were analyzed. The child group spent the least amount of time using digital devices (children: 159; adolescents: 210; young adults: 267 min/work day, p < 0.05; (mean time in the same order below) 229, 338, 314 min/holiday, p < 0.05) and had the largest strabismus angle (mean strabismus angle at near: 30, 22, 18 PD, p < 0.01; at far: 28, 26, 21 PD, p<0.05). CONCLUSION: The clinical features of acquired comitant esotropia and hand-held digital device usage differed between children aged ≤ 12 years and older patients. This report gives the current clinical characteristics of young patients with acquired esotropia and digital device usage.

Factors that influence modern contraceptive use among women aged 35 to 49 years and their male partners in Gomoa West District, Ghana: a qualitative study.

BACKGROUND: Fertility declines with age, but it remains important to protect women from unplanned pregnancies throughout their reproductive lives. The objective of this study was to describe factors that influence modern contraceptive use among women aged 35 to 49 years and their male partners in Gomoa West District of Ghana. METHODS: In-depth interviews were conducted remotely for 22 women, 15 male partners of the women interviewed and seven family planning (FP) providers. In all, a total of 44 participants took part in the study. Seven refusals were recorded, four females and three males. Four focus group discussions were organized for 21 participants who took part in the in-depth interviews. Data collected were transcribed and coded after exporting to Nvivo12 qualitative analysis software. Thematic analysis was undertaken using an abductive approach. RESULTS: Factors that influenced the use of modern contraceptives included: achieved desired family size, providing for the family, counselling by health professionals, influence of the male partner, and health reasons. Barriers cited included: religious or socio-cultural reasons, experience or fear of side effects, rumors or misconceptions, declining fertility, and the belief that contraceptive use is a matter for women. Within the study group, roughly half of women used modern contraceptives, while the majority of male partners were non-users. CONCLUSION: Contraception among women aged 35 to 49 years and their male partners is influenced by several factors such as achieved desired family size, influence of the male partner, rumors or misconceptions, and declining fertility. Strengthening male involvement in family planning activities and health educational activities could alleviate fear and reduce misconceptions about using modern contraceptives.

Recovery of stereoacuity after Yokoyama procedure in patients with highly myopic strabismus with good vision.

Purpose: To report stereopsis after bilateral Yokoyama procedure in patients with highly myopic strabismus and good visual acuity. Observations: Five patients aged between 34 and 81 years with best-corrected visual acuity of 20/25 or better were operated on. The preoperative strabismus angle ranged from esotropia of 35-113 prism diopters (PD) at distance and esotropia of 40-113 PD at near. One patient had left hypotropia of 4 PD and excyclotorsion of 15°, and the other had left hypotropia of 10 PD. Their axial lengths were 27.65-33.07 mm, and the posterior globe dislocation angles were between 123 and 148° on coronal magnetic resonance imaging (MRI). Limitations of abduction were between -1 and -2. All patients complained of diplopia, and none of them showed stereopsis. The Yokoyama procedure was performed on both eyes. Postoperative alternate cover testing showed from esotropia of 2 PD to exophoria of 8 PD at distance and from exotropia of 12 PD to esophoria of 10 PD at near.Three patients recovered stereopsis of 100, 50, and 140 sec, respectively. Two cases required unilateral inferior rectus muscle recessions, and their postoperative stereopsis was 25 and 50 sec. Conclusions and Importance: The Yokoyama procedure is effective not only in cases of heavy eye syndrome but also in cases of myopic esotropia associated with a globe dislocation angle of 120° or with little abduction restriction. If visual acuity is good, stereopsis may be restored with improvement in eye deviation.

Steroid-induced ocular hypertensive response in pediatric patients with acute lymphoblastic leukemia.

PURPOSE: To report the characteristics of the steroid-induced ocular hypertensive response in pediatric patients with acute lymphoblastic leukemia (ALL) treated with prednisolone (PSL) during induction therapy and with dexamethasone (DEX) during reinduction therapy. STUDY DESIGN: Retrospective. PATIENTS AND METHODS: This study included pediatric patients diagnosed with B-cell precursor ALL and treated with systemic corticosteroids sometime during the period from 2016 to 2018 at Shizuoka Children's Hospital. Data were extracted from the hematology/oncology records related to the type, dose, and duration of systemic corticosteroids as well as to the ophthalmologic examination findings, intraocular pressure (IOP) data, symptoms of high IOP, and antiglaucoma medications obtained during corticosteroid administration. The maximal IOPs of the PSL and DEX groups were compared. RESULTS: Twenty-eight patients (18 boys and 10 girls; mean age 5.5 years) were treated with systemic corticosteroids. Twelve of the 22 courses of PSL and 33 of the 44 courses of DEX were found to be associated with high IOP. The maximal IOP was higher with the use of DEX than with the use of PSL, including in those who received prophylactic therapy (PSL 25.2 mmHg, DEX 33.6 mmHg; P = 0.02). Antiglaucoma medication was given to 21 patients; 6 patients had symptoms of ocular hypertension. The maximal IOPs were 52.8 mmHg and 70.8 mmHg in the PSL and DEX groups, respectively. Both groups of patients reported severe headache. CONCLUSION: Increased IOP was frequently observed during systemic corticosteroid therapy in pediatric patients with ALL. Although most patients were asymptomatic, they occasionally presented with severe systemic symptoms. Regular ophthalmologic examinations should be included in the treatment guidelines for ALL.

Effects of size at birth on health, growth and developmental outcomes in children up to age 18: an umbrella review.

BACKGROUND: Size at birth, an indicator of intrauterine growth, has been studied extensively in relation to subsequent health, growth and developmental outcomes. Our umbrella review synthesises evidence from systematic reviews and meta-analyses on the effects of size at birth on subsequent health, growth and development in children and adolescents up to age 18, and identifies gaps. METHODS: We searched five databases from inception to mid-July 2021 to identify eligible systematic reviews and meta-analyses. For each meta-analysis, we extracted data on the exposures and outcomes measured and the strength of the association. FINDINGS: We screened 16 641 articles and identified 302 systematic reviews. The literature operationalised size at birth (birth weight and/or gestation) in 12 ways. There were 1041 meta-analyses of associations between size at birth and 67 outcomes. Thirteen outcomes had no meta-analysis.Small size at birth was examined for 50 outcomes and was associated with over half of these (32 of 50); continuous/post-term/large size at birth was examined for 35 outcomes and was consistently associated with 11 of the 35 outcomes. Seventy-three meta-analyses (in 11 reviews) compared risks by size for gestational age (GA), stratified by preterm and term. Prematurity mechanisms were the key aetiologies linked to mortality and cognitive development, while intrauterine growth restriction (IUGR), manifesting as small for GA, was primarily linked to underweight and stunting. INTERPRETATION: Future reviews should use methodologically sound comparators to further understand aetiological mechanisms linking IUGR and prematurity to subsequent outcomes. Future research should focus on understudied exposures (large size at birth and size at birth stratified by gestation), gaps in outcomes (specifically those without reviews or meta-analysis and stratified by age group of children) and neglected populations. PROSPERO REGISTRATION NUMBER: CRD42021268843.

The clinical characteristics, etiologic pathogens and the risk factors associated with dehydration status among under-five children hospitalized with acute diarrhea in Savannakhet Province, Lao PDR.

BACKGROUND: Acute diarrhea is a common health problem in children, especially those under five years of age (U5). The mortality rate due to acute diarrhea among U5 children accounted for 11% in Lao PDR in 2016. No study has been done to investigate the etiologic pathogens of acute diarrhea and the risk factors associated with dehydration status among U5 children hospitalized with acute diarrhea in this region. OBJECTIVES: The study aimed to evaluate the clinical characteristics, etiological agents and associated factors of dehydration status of acute diarrhea among hospitalized U5 children in Savannakhet Province, Lao PDR. METHODS: This retrospective study reviewed paper-based medical records with available stool examination results of 33 U5 children hospitalized with acute diarrhea in Savannakhet Provincial Hospital, Lao PDR between Jan. 2018 and Dec. 2019. Descriptive statistics were used to describe clinical characteristics and etiologic agents of acute diarrhea of the children. Nonparametric test, Pearson's Chi-square test and Fisher exact test were used to determine the risk factors associated with level of dehydration of the participants. RESULTS: Vomiting was the most common symptom (66.6%), followed by fever (60.6%). Dehydration was found in 48.4% of subjects. Rotavirus was the most common identified pathogen with a prevalence of 55.5%. Bacterial enteric infection was identified in 15.1% of patients. There is a significantly higher prevalence of dehydration among children with acute diarrhea caused by rotavirus compared to those with negative rotavirus testing (70.0% vs. 12.5%, p = 0.02). CONCLUSIONS: Rotavirus was the most prevalent pathogen of acute diarrhea among U5 children. Pediatric patients with acute diarrhea caused by rotavirus had a higher prevalence of dehydration compared to those with negative rotavirus testing.

Rapid detection of alveolar echinococcosis in hepatic nodules of horses by recombinase polymerase amplification assay.

Alveolar echinococcosis in slaughtered horses remains a public health issue. This study aimed to develop a Recombinase polymerase amplification (RPA) assay targeting the mitochondrial NADH dehydrogenase subunit 5 (Nad5) gene of Echinococcus multilocularis for the rapid detection of equine alveolar echinococcosis. Thirty-six hepatic solid nodules obtained from each horse (n = 36) were evaluated based on histopathological examination and Nad5-targeted PCR and then submitted to the RPA assay. The results of the developed RPA assay were 94.4% consistent with those of Nad5 PCR and Cohen's kappa coefficient value was 0.89 statistically, indicating high agreement. In addition, the RPA assay using the plasmid samples was one hundredfold more sensitive than PCR testing. Consequently, these results suggest that the performance of the RPA assay developed in this study is equal to that of conventional PCR testing.

A conditional Smg6 mutant mouse model reveals circadian clock regulation through the nonsense-mediated mRNA decay pathway.

Nonsense-mediated messenger RNA (mRNA) decay (NMD) has been intensively studied as a surveillance pathway that degrades erroneous transcripts arising from mutations or RNA processing errors. While additional roles in physiological control of mRNA stability have emerged, possible functions in mammalian physiology in vivo remain unclear. Here, we created a conditional mouse allele that allows converting the NMD effector nuclease SMG6 from wild-type to nuclease domain-mutant protein. We find that NMD down-regulation affects the function of the circadian clock, a system known to require rapid mRNA turnover. Specifically, we uncover strong lengthening of free-running circadian periods for liver and fibroblast clocks and direct NMD regulation of Cry2 mRNA, encoding a key transcriptional repressor within the rhythm-generating feedback loop. Transcriptome-wide changes in daily mRNA accumulation patterns in the entrained liver, as well as an altered response to food entrainment, expand the known scope of NMD regulation in mammalian gene expression and physiology.

The impact of the first peak of the COVID-19 pandemic on childhood myopia control practice patterns among ophthalmologists-an international pediatric ophthalmology and strabismus council global perspective.

BACKGROUND: The prevalence of myopia keeps increasing during the COVID-19 pandemic. We aimed to map the worldwide treatment preferences of ophthalmologists managing myopia control during the first wave of the pandemic. METHODS: An online questionnaire inquiring about pharmacological and optical treatment patterns during the first half of 2020 was sent to pediatric ophthalmology as well as general ophthalmology memberships worldwide. The results among pediatric ophthalmologists were compared to a previous study we performed before the pandemic. RESULTS: A total of 2269 respondents from 94 countries were included. Most respondents were pediatric ophthalmologists (64.6%), followed by ophthalmologists from other subspecialties (32.3%). The preferred modality for all geographical regions was a combination therapy of pharmacological and optical treatments. When evaluated independently, the pharmacological treatment was more popular than the optical treatment in most regions other than East Asia (P < 0.001). Compared to a pre-pandemic questionnaire, the participation of pediatric ophthalmologists affiliated with non-university hospitals increased. Additionally, the prevalence of respondents utilizing either any type of pharmacological treatment and those that using only evidence-based treatments increased globally. Although a decline in the use of optical treatment was evident worldwide, the use of evidence-based optical treatments increased. CONCLUSION: Ophthalmologists around the world preferred a combination therapy of pharmacological and optical treatments. More pediatric ophthalmologists treated myopia progression and preferred a better evidence-based approach to control myopia. These trends reflect a positive response and more awareness of the rising prevalence of myopia due to the increased burden of myopia imposed by the COVID-19 pandemic.

Prophylactic oophorectomy and aromatase inhibitors for premenopausal deep angiomyxoma: A case report and literature review.

Deep angiomyxoma is a rare, infiltrative, hormone-dependent, benign-mesenchymal neoplasm that occurs in the deep soft tissues of the perineal regions. In total, 33% females with newly diagnosed deep angiomyxoma will typically relapse within 5 years after the standard treatment of radical resection. Postoperative hormone therapy is frequently administered to prevent recurrence, but the role of prophylactic oophorectomy in premenopausal women remain to be fully elucidated. In the present report, a 42-year-old Japanese woman was referred for a refractory Bartholin's cyst that is 14 cm in diameter. Based on the results of imaging (unenhanced CT and MRI) and histopathology, deep angiomyxoma was suspected, but no definitive diagnosis was possible. Tumor resection and bilateral salpingo-oophorectomy were performed before the postoperative diagnosis was confirmed to be deep angiomyxoma. The patient received an aromatase inhibitor (2.5 mg letrozole daily) as adjuvant hormonal therapy. There was no evidence of recurrence at the 1-year postoperative follow-up. In conclusion, prophylactic oophorectomy and postoperative adjuvant therapy with aromatase inhibitors may be a promising treatment option for deep angiomyxoma to optimize the outcome of surgical treatment. Long-term follow-up is required to monitor for the late and/or local recurrence of deep angiomyxoma and possible adverse effects of adjuvant hormonal therapy.

A case of siblings with juvenile retinitis pigmentosa associated with NEK1 gene variants.

BACKGROUND: Axial spondylometaphyseal dysplasia(axial SMD) is associated with early-onset retinal dystrophy and various skeletal dysplasias of varying severity. NEK1 is the causative gene for short rib polydactyly syndrome and axial SMD. Here, we report a case of siblings with juvenile retinitis pigmentosa (RP) and NEK1 variants not associated with systemic disorders. MATERIALS AND METHODS: The patients were a 7-year-old-girl and a 9-year-old boy with RP, who were followed for 9 years. Whole exome sequencing (WES) was performed on the siblings and their parents, who were not consanguineous. RESULTS: The corrected visual acuity of the girl and the boy at first visit was binocular 20/63 and 20/100 OD and 20/63 OS, respectively. The siblings had narrowing of retinal blood vessels and retinal pigment epithelium atrophy in the fundus and showed an extinguished pattern in electroretinogram. On optical coherence tomography, there was a mottled ellipsoid band with progressive loss in the outer macular, the edges of which corresponded to the ring of hyperautofluorescence on fundus autofluorescence imaging. The siblings showed progressive visual field constriction. Radiological examination did not reveal any skeletal abnormalities. We identified two rare heterozygous NEK1 variants in the patients: c.240 G>A; p.(M80I) and c.634_639dup;p.(V212_L213dup). Heterozygous variants were recognized in the father and mother, respectively. According to the guidelines of the American College of Medical Genetics and Genomics, both variants were classified as likely pathogenic. CONCLUSION: This is the first report of RP patients with NEK1 variants not associated with skeletal abnormalities.

Validity and reliability of the Japanese version of the Nursing Performance Instrument.

AIM: Work performance of nurses is a critical aspect that concerns the safety of both nurses and patients, but also secondarily influences nurses' turnover due to serious fatigue and burnout. Monitoring nursing performance as well as fatigue is important for appropriate safety management of patients and nurses, and the development of a monitoring tool is essential. The Nursing Performance Instrument (NPI) can provide comprehensive and brief monitoring of nursing performance. This study aimed to verify the validity and reliability of a Japanese version of the NPI (NPI-J). METHODS: A cross-sectional study was conducted on nurses who work in inpatient care across five Japanese hospitals. We considered appropriate factor numbers with parallel analysis and the Velicer's minimum average partial test. The construct validity was evaluated using exploratory factor analysis, confirmatory factor analysis (CFA), and Pearson's correlation analysis. Cronbach's alpha was calculated for the internal consistency. RESULTS: A three-factor model eventually showed acceptable fit indices in the CFA. Moreover, the correlation results among the three factors indicated discriminant validity comparable to the original NPI. The correlation results with acute fatigue, chronic fatigue, and burnout verified convergent validity. Internal consistency was insufficient. CONCLUSION: The NPI-J demonstrated acceptable construct validity. This instrument can assess nurses' perceptions of their performance by measuring each item. Future work on the reliability of the sub-concepts will require additional items or reliability testing based on generalizability theory and item response theory.

Impact of the fundamental use of surgical energy certification on surgeons' behavior and awareness of safe use of energy devices: a cross-sectional survey research.

BACKGROUND: The Fundamental Use of Surgical Energy (FUSE) program was established to educate surgeons and trainees to promote awareness and behaviors for the safe use of surgical energy devices. Despite its implementation, the impact of FUSE certification on surgeons' behavior and safety awareness regarding practice of energy devices remains unclear. This study aimed to identify the perceived impact of FUSE certification on surgeons' behavior and awareness regarding the safe use of surgical energy devices. METHODS: We performed a descriptive cross-sectional survey study, using non probabilistic purposive sampling, and distributed 22-item web-based questionnaires among all 59 FUSE-certified surgeons in Japan, excluding operating room nurses and medical students. The questionnaire items covered demographics, surgical techniques using various energy devices, changes in behavior and safety awareness, communication with colleagues about surgical energy devices, and educational activities related to energy devices. RESULTS: Fifty-seven participants completed the questionnaire (response rate 96.6%). Most surgeons (91.3%) could apply material learned from the FUSE program in practice, especially material related to monopolar electrosurgery. Fifty-six surgeons (98.3%) reported increased awareness of surgical safety, and 35 (61.5%) reported increased communication with operating room personnel about the safe use of energy devices. Moreover, 56 participants (98.3%) indicated a need for systematic education in surgical energy, with participants recommending fellows (94.7% of participants specified that fellows should participate in further education), residents (75.4%), and attending surgeons (63.2%) as the target recipients of this training. Conclusions After FUSE certification, not only did surgeons' knowledge increase, but their energy-related surgical techniques in practice also improved. Furthermore, FUSE-certified surgeons felt that they were more aware of surgical-energy safety and were dedicated to its promotion.