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To translate the Perceived Maternal Parenting Self-Efficacy (PMP S-E) scale to Japanese and test its psychometric properties among Japanese mothers of low-birth-weight (LBW) infants.Descriptive psychometric study.NICUs in two regional perinatal care centers in the Kinki region of Japan.Seventy-nine Japanese mothers of LBW infants hospitalized in NICUs.First, we translated the PMP S-E scale into Japanese and assessed its face and content validity. Second, we assessed the internal consistency, test-retest reliability, and criterion-related and construct validity of the Japanese version of the PMP S-E (JPMP S-E) with data from Japanese mothers of LBW infants.The JPMP S-E mean score was 53.0 (SD = 7.1); the total scores ranged from 38 to 68. The intraclass correlation coefficient of test-retest reliability was .75, and Cronbach's alpha coefficient was .90. With regard to criterion-related validity, we found a significant positive correlation between the JPMP S-E and the Maternal Attachment Inventory Japanese version (r = .45, p .001), but no correlation existed between the JPMP S-E and the General Self-Efficacy Scale (r = .21, p = .06). We evaluated construct validity using confirmatory factor analysis to assess whether the model fit the previously determined structure of the PMP S-E. The model fit was moderate in the confirmatory factor analysis (comparative fit index = .63, goodness-of-fit index = .63, root mean square error of approximation = .15).We found adequate estimates of reliability and moderate indicators of validity for the JPMP S-E, which support the use of the tool for clinical and research purposes.

In children with a first Escherichia coli-induced febrile urinary tract infection (fUTI), routine voiding cytourethrography (VCUG) is not recommended for detecting vesicoureteral reflux (VUR). Meanwhile, the sensitivity of renal and bladder ultrasound (RBUS) for detecting VUR is insufficient. Aiming to implement VCUG properly for children with a first E. coli-induced fUTI, we attempted to construct a predictive scoring system for the early screening of VUR.This study enrolled patients aged2 years of age hospitalized for their first E. coli-induced fUTI during the period when VCUG was implemented for all patients (2007-14, non-selective group [n = 111]) and only for those with VUR-suspected RBUS findings, bacteremia or acute focal bacterial nephritis (2016-19, selective group [n = 102]). We evaluated the accuracy of the current criteria and the VUR predictive score constructed using data from the non-selective group.In the non-selective group, 32 patients had VUR (29%). In the selective group, 20 of 45 VCUG-tested patients had VUR (44%). Among 57 patients not undergoing VCUG in the selective group, 8 had a recurrence of fUTI, 3 of whom were diagnosed with VUR. In the non-selective group, 9 patients with VUR did not fulfill the current criteria and the VUR predictive score consisting of young age, female sex, prolonged fever, hypoproteinemia, hyponatremia and hyperglycemia, showed higher sensitivity, specificity than the current criteria.The current imaging/bacteriological criteria were ineffective in screening for VUR in patients with their first E. coli-induced fUTI. The VUR predictive score can be an accurate indicator for implementing VCUG.

Acute kidney injury (AKI) often occurs in pediatric patients who received allogeneic hematopoietic cell transplantation (HCT). We evaluated the risk and effect of HCT-related AKI in pediatric patients. We retrospectively studied the survival and renal outcome of 69 children 100 days and 1-year posttransplant in our institution in 2004–2016. Stage-3 AKI developed in 34 patients (49%) until 100 days posttransplant. The 100-day overall survival (OS) rates of patients with stage-3 AKI were lower than those without it (76.5% vs. 94.3%, P = 0.035). The 1-year OS rates did not differ markedly between 21 post-100-day survivors with stage-3 AKI and 29 without it (80.8% vs. 87.9%, P = 0.444). The causes of 19 deaths included the relapse of underlying disease or graft failure (n = 11), treatment-related events (4), and second HCT-related events (4). Underlying disease of malignancy (crude hazard ratio (HR) 5.7; 95% confidence interval (CI), 2.20 to 14.96), > 1000 ng/mL ferritinemia (crude HR 4.29; 95% CI, 2.11 to 8.71), stem cell source of peripheral (crude HR 2.96; 95% CI, 1.22 to 7.20) or cord blood (crude HR 2.29; 95% CI, 1.03 to 5.06), and myeloablative regimen (crude HR 2.56; 95% CI, 1.24 to 5.26), were identified as risk factors for stage-3 AKI until 100 days posttransplant. Hyperferritinemia alone was significant (adjusted HR 5.52; 95% CI, 2.21 to 13.76) on multivariable analyses. Hyperferritinemia was associated with stage-3 AKI and early mortality posttransplant. Pretransplant iron control may protect the kidney of pediatric HCT survivors.

Henoch-Schönlein purpura nephritis often resolves spontaneously, without treatment, making decisions regarding therapeutic interventions difficult.Fifty-four patients who were diagnosed as having Henoch-Schönlein purpura nephritis between April 2004 and March 2018, and developed hematuria and/or proteinuria, were studied retrospectively. The observation period ended at the disappearance of hematuria or proteinuria, or the last observation date before December 2019 for each patient. Twenty-four of the patients received no treatment (Group A), 19 underwent renin-angiotensin-aldosterone system inhibitors only (B), 4 experienced steroid pulse therapy and combination therapy only (C) and the remaining 7 received steroid pulse therapy and combination therapy following renin-angiotensin-aldosterone system inhibitors (C). Clinical characteristics were examined according to the treatment method. Survival analysis for persistent urinary abnormalities was performed according to treatment modality, with multiple treatment records created per subject, if necessary.The highest urine protein/creatinine levels were significantly higher in groups B and C than in group A. The lowest estimated glomerular filtration rate was not significantly different among the three groups. In groups A and B, proteinuria resolved in90% of patients. Survival analysis showed that steroid pulse therapy and combination therapy was not related to the better resolution of hematuria or proteinuria than renin-angiotensin-aldosterone system inhibitors.Several patients with Henoch-Schönlein purpura nephritis went into remission either without treatment or with renin-angiotensin-aldosterone system inhibitors alone. The treatment plan for patients with Henoch-Schönlein purpura nephritis needs to be determined carefully.

Several maternal chronic diseases have been reported as risk factors for congenital anomalies of the kidney and urinary tract (CAKUT) in offspring. However, these investigations used case-control designs, and cases with isolated genitourinary CAKUT were not distinguished from cases in which CAKUT were present with extrarenal congenital anomalies (complicated CAKUT). We examined the association of maternal diseases with isolated and complicated CAKUT in offspring using data from a prospective cohort study.A nationwide prospective birth cohort study.100,239 children enrolled in the Japan Environment and Children's Study between January 2011 and March 2014 at 15 research centers. Physicians' diagnoses in mothers and children were collected from medical record transcripts and questionnaires.Medical histories of maternal noncommunicable diseases, including obesity, hypertension, diabetes mellitus, kidney disease, hyperthyroidism, hypothyroidism, psychiatric disease, epilepsy, cancer, and autoimmune disease.CAKUT diagnosed during the first 3 years of life, classified as isolated or complicated.Multivariable Poisson regression with generalized estimating equations accounting for clustering by clinical center.Among the 100,239 children, 560 (0.6%) had CAKUT, comprising 454 (81%) isolated and 106 (19%) complicated forms. The risk of isolated CAKUT was increased in children of mothers who experienced kidney disease (adjusted risk ratio [RR], 1.80 [95% CI, 1.12-2.91]) or cancer (RR, 2.11 [95% CI, 1.15-3.86]). Furthermore, the risk of complicated CAKUT was increased in children of mothers with diabetes mellitus (RR, 3.04 [95% CI, 1.64-5.61]).Lack of standardization or prespecification of clinical definitions, diagnostic criteria, measurements, and testing. Genetic testing was not performed.Isolated CAKUTs and complicated CAKUTs were associated with different maternal diseases. The results may inform clinical management of pregnancy and highlight potential differences in the genesis of isolated and complicated forms of CAKUT.

BackgroundPlastic bronchitis (PB) combined with nephrotic syndrome (NS) is rare, and the pathophysiological relationship between PB and NS has not been elucidated. We report a case of an 8-year-old boy with life-threatening PB caused by an influenza infection during a relapse of NS. Case presentationThe patient was on immunosuppressive drugs for NS. He developed fever due to an influenza A virus infection, followed by respiratory distress and frequent vomiting. Prednisolone was administered for possible bronchial asthma and NS. His respiratory status deteriorated rapidly, which required ventilator management. A large mucus plug was aspirated using bronchoscopy. He was then diagnosed with PB caused by the influenza A virus. Increased lower airway secretion and fluid leakage into the airways by relapse of NS were considered the causes of mucus plug formation. Besides, the decreased circulating blood volume might have made the bronchial secretions viscous with cast formation.ConclusionsPediatric patients with NS may be at a higher risk of developing PB. As PB is a life-threatening condition, patients with NS should be closely monitored when simultaneously infected with influenza virus.

Background Carbapenem-resistant gram-negative bacilli (CRGNB) are increasingly reported around the world as a cause of serious infections. However, the epidemiology and clinical course of patients with CRGNB in Japan is not well understood. Methods We prospectively collected CR cases from 4/2019 to 9/2020 in Multi-Drug Resistant organisms clinical research network (MDRnet) consisting of 5 tertiary care facilities in Japan. We looked for all CRGNB, and all unique patients with CR Enterobacterales, CR nonfermenting gram-negative bacilli (NFGNB) and CR Aeromonas sp. isolation were included. Carbapenem resistance was tested by agar dilution method and defined based on the CLSI criteria for each species. Infections were determined by NHSN protocols. Results In total, 156 patients (30 Enterobacterales, 119 NFGNB, 7 Aeromonas spp.) were included (11 Enterobacter spp., 11 Klebsiella spp., 86 Pseudomonas aeruginosa, 29 Stenotrophomonas maltophilia, 7 Aeromonas spp.). Acinetobacter sp. was not detected. Isolation sites were sputum (n = 12) and urine (n = 7) in Enterobacterales, sputum (n = 62) and blood (n = 18) in NFGNB, and blood (n = 6) in Aeromonas spp. The median age and male ratio of the patients were 68 years [IQR: 53-74] and 19 (63.3%) in Enterobacterales, 72 years [IQR: 60-79] and 70 (58.8%) in NFGNB and 78 years [IQR: 54-83] and 2 (28.6%) in Aeromonas spp. Ten (33.3%) patients with Enterobacterales, 55 (46.2%) patients with NFGNB, and 6 (85.7%) patients with Aeromonas spp. were infected cases. The others were considered as colonized. There were no patients with ICU stay or intubation in Enterobacterales, while 5 (4.2%) and 4 (3.4%) patients were in ICU and intubated in NFGNB, and 2 patients were in ICU and intubated in Aeromonas spp., respectively. All-cause 30-day mortality rates were 10% in Enterobacterales, 16.8 % in NFGNB and 28.6% in Aeromonas spp. In the infected patients, 3 patients (30%) with Enterobacterales, 12 patients (21.8%) with NFGNB and 1 patient (16.7%) with Aeromonas spp. died within 30 days after isolation. Flow diagram outlining the characteristics of the patients and species in this study. Kaplan-Meier survival curves of patients with carbapenem resistant Enterobacterales Kaplan-Meier survival curves of patients with carbapenem resistant nonfermenting gram-negative bacilli Conclusion Mortality rates were high in infected cases of CR Enterobacterales, CR NFGNB and CR Aeromonas spp. Carbapenem-resistant Acinetobacter spp. was not detected, which differed from the CR epidemiology in Europe, the United States, and other Asian countries. Disclosures Sho Saito, n/a, Shionogi (Grant/Research Support) David van Duin, MD, PhD, Entasis (Advisor or Review Panel member)genentech (Advisor or Review Panel member)Karius (Advisor or Review Panel member)Merck (Grant/Research Support, Advisor or Review Panel member)Pfizer (Consultant, Advisor or Review Panel member)Qpex (Advisor or Review Panel member)Shionogi (Grant/Research Support, Scientific Research Study Investigator, Advisor or Review Panel member)Utility (Advisor or Review Panel member) Yohei Doi, MD, PhD, AstraZeneca (Speaker's Bureau)bioMerieux (Consultant)FujiFilm (Advisor or Review Panel member, Speaker's Bureau)Gilead (Consultant)GSK (Consultant)Meiji (Consultant)MSD (Consultant)Shionogi (Consultant) Yohei Doi, MD, PhD, Astellas (Individual(s) Involved: Self): Grant/Research Support; AstraZeneca (Individual(s) Involved: Self): Speakers' bureau; bioMerieux (Individual(s) Involved: Self): Consultant, Speakers' bureau; Chugai (Individual(s) Involved: Self): Consultant; Entasis (Individual(s) Involved: Self): Consultant; FujiFilm (Individual(s) Involved: Self): Advisor or Review Panel member; Gilead (Individual(s) Involved: Self): Consultant; GSK (Individual(s) Involved: Self): Consultant; Kanto Chemical (Individual(s) Involved: Self): Grant/Research Support; MSD (Individual(s) Involved: Self): Speaking Fee; Pfizer (Individual(s) Involved: Self): Grant/Research Support; Shionogi (Individual(s) Involved: Self): Grant/Research Support, Speakers' bureau; Teijin Healthcare (Individual(s) Involved: Self): Speakers' bureau; VenatoRx (Individual(s) Involved: Self): Consultant

Background Pierson syndrome (PS) is a rare autosomal recessive disorder, characterized by congenital nephrotic syndrome and microcoria. Advances in renal replacement therapies have extended the lifespan of patients, whereas the full clinical spectrum of PS in infancy and beyond remains elusive. Case presentation We present the case of a 12-month-old boy with PS, manifesting as the bilateral microcoria and congenital nephrotic syndrome. He was born without asphyxia, and was neurologically intact from birth through the neonatal period. Generalized muscle weakness and hypotonia were recognized from 3 months of age. The infant showed recurrent vomiting at age 5 months of age, and was diagnosed with gastroesophageal reflux and intestinal malrotation. Despite the successful surgical treatment, vomiting persisted and led to severely impaired growth. Tulobuterol treatment was effective in reducing the frequency of vomiting. Targeted sequencing confirmed that he had a compound heterozygous mutation in LAMB2 (NM_002292.3: p.Arg550X and p.Glu1507X). A search of the relevant literature identified 19 patients with severe neuro-muscular phenotypes. Among these, only 8 survived the first 12 months of life, and one had feeding difficulty with similar gastrointestinal problems. Conclusions This report demonstrated that severe neurological deficits and gastrointestinal dysfunction may emerge in PS patients after the first few months of life.

Background Hypokalemic periodic paralysis (HypoPP) is an autosomal dominant disease characterized by the episodic weakness of skeletal muscles and hypokalemia. More than half patients with HypoPP carry mutations in CACNA1S, encoding alpha‐1 subunit of calcium channel. Few reports have documented the non‐neuromuscular phenotypes of HypoPP. Methods The proband is a Japanese woman who developed HypoPP at 6 years of age. An excessive insulin secretion with the oral glucose tolerance test rationalized that she had experienced frequent attacks of paralysis on high‐carbohydrate diets. Results Voglibose and acetazolamide effectively controlled her paralytic episodes. Her 8‐year‐old son and 2‐year‐old daughter started showing the paralytic symptoms from 4 and 2 years of age, respectively. Laboratory tests revealed high concentrations of creatinine kinase in serum and elevated renin activities in plasma of these children. The targeted sequencing confirmed that these three patients had an identical heterozygous mutation (p.V876E) in CACNA1S. Conclusion Our data indicate that the p.V876E mutation in CACNA1S contributes to the early onset of neuromuscular symptoms and unusual clinical phenotypes of HypoPP., Our study confirmed that the Japanese family in this report had a heterozygous mutation of p.V876E in CACNA1S. As the third family carrying the rare variant, these findings provide evidence that p.V876E in CACNA1S is linked to the early onset of neuromuscular symptoms and unusual clinical phenotypes of HypoPP.

Microorganisms that activate plant immune responses have attracted considerable attention as potential biocontrol agents in agriculture because they could reduce agrochemical use. However, conventional methods to screen for such microorganisms using whole plants and pathogens are generally laborious and time consuming. Here, we describe a general strategy using cultured plant cells to identify microorganisms that activate plant defense responses based on plant–microbe interactions. Microbial cells were incubated with tobacco BY-2 cells, followed by treatment with cryptogein, a proteinaceous elicitor of tobacco immune responses secreted by an oomycete. Cryptogein-induced production of reactive oxygen species (ROS) in BY-2 cells served as a marker to evaluate the potential of microorganisms to activate plant defense responses. Twenty-nine bacterial strains isolated from the interior of Brassica rapa var. perviridis plants were screened, and 8 strains that enhanced cryptogein-induced ROS production in BY-2 cells were selected. Following application of these strains to the root tip of Arabidopsis seedlings, two strains, Delftia sp. BR1R-2 and Arthrobacter sp. BR2S-6, were found to induce whole-plant resistance to bacterial pathogens (Pseudomonas syringae pv. tomato DC3000 and Pectobacterium carotovora subsp. carotovora NBRC 14082). Pathogen-induced expression of plant defense-related genes (PR-1, PR-5, and PDF1.2) was enhanced by the pretreatment with strain BR1R-2. This cell–cell interaction-based platform is readily applicable to large-scale screening for microorganisms that enhance plant defense responses under various environmental conditions.