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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.

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.

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.

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.