Validity and Reproducibility of a Food Frequency Questionnaire for Dietary Assessment in Japanese People.

A 76-item food frequency questionnaire (FFQ) was developed to investigate nutritional epidemiology in urban residents in Japan. The authors prepared two food models-a life-size three-dimensional model and a life-size two-dimensional photograph-to assess the FFQ portion size. The validity of the FFQ was verified using the two food models by comparing them with 16-d weighted dietary records (WDRs). Validation was conducted by comparing the FFQ1 findings with those obtained with the WDR, which is regarded as the gold standard, and reproducibility was verified by comparing the findings from FFQ2 and FFQ1. After completion of the WDR, the participants were randomized into two groups. In one group, the FFQ was conducted using life-size three-dimensional models (3D-FFQ) to estimate the portion size. In the other group, the FFQ was administered using life-size photo collection (2D-FFQ). Regarding validity, the median values (range) of Pearson's correlation coefficients for the energy and nutrient intake of the 32 items by the WDR and FFQ1 were r=0.53 (0.30-0.68) in the 3D-FFQ and r=0.57 (0.33-0.87) in the 2D-FFQ. When FFQs with 2D or 3D food models and two different portion sizes were compared with regard to the intake of certain food groups, energy, and nutrients, both the 2D-FFQ and 3D-FFQ provided good correlation coefficients with the WDR.

[Two Cases of Retroperitoneal Synovial Sarcoma].

We experienced two cases of renal primary synovial sarcoma. Case 1: A 29-year-old man underwent laparoscopic radical nephrectomy and was originally diagnosed with renal cell carcinoma. Case 2: A 25-year-old man was treated by open radical nephrectomy since radiographical findings indicated tumor invasion to the ureter causing hydronephrosis. Both cases were pathologically diagnosed as renal synovial sarcomas, and were followed using computed tomography. Recurrence was observed within a year in both cases.

A Japanese patient with Teebi hypertelorism syndrome and a novel CDH11 EC1 domain variant.

The gene CDH11 encodes cadherin-11, a Type II cadherin superfamily member that contains five extracellular cadherin (EC) domains. Cadherin-11 undergoes trans-dimerization via the EC1 domain to generate cadherin complexes. Compound heterozygous and homozygous loss-of-function CDH11 variants are observed in Elsahy-Waters syndrome (EWS), which shows characteristic craniofacial features, vertebral abnormalities, cutaneous syndactyly in 2-3 digits, genitourinary anomalies, and intellectual disability. Heterozygous CDH11 variants can cause Teebi hypertelorism syndrome (THS), which features widely spaced eyes and hypospadias. We report a THS patient with a novel CDH11 variant involving the EC1 domain. The patient was a 10-month-old male with normal developmental milestones, but had widely spaced eyes, strabismus, hypospadias, shawl scrotum, broad thumbs (right bifid thumb in x-ray), polysyndactyly of the left fourth finger, and cutaneous syndactyly of left third/fourth fingers. Exome sequencing identified a de novo heterozygous CDH11 variant (NM_001797.4:c.229C > T [p.Leu77Phe] NC_000016.9:g.64998856G > A). Clinical features were consistent with previously reported THS patients, but polysyndactyly, broad thumb, and cutaneous syndactyly overlapped phenotypic features of EWS. THS and EWS may represent a spectrum of CDH11-related disorders. Residue Leu77 in this novel CDH11 variant lines a large hydrophobic pocket where side chains of the partner cadherin-11 insert to trans-dimerize, suggesting that the cadherin-11 structure might be altered in this variant.

Quantitative assessment of skin disorders induced by panitumumab: a prospective observational study.

PURPOSE: Acneiform rash is frequently observed in patients undergoing cancer treatment with anti-epidermal growth factor receptor (EGFR) antibody drugs and can often necessitate treatment discontinuation. However, the specific changes in skin parameters resulting from anti-EGFR antibody drug administration are poorly understood. Therefore, this study aimed to longitudinally and quantitatively evaluate the changes in skin parameters (transepidermal water loss [TEWL], hydration level, and sebum level) caused by anti-EGFR antibody drugs and investigate their potential as control markers for skin disorders. METHODS: This prospective study included 12 patients with colorectal cancer who received anti-EGFR antibody drugs for the first time. The assessment items included the grade of acneiform rash and skin parameters (TEWL, hydration level, and sebum level), which were observed for up to 6 weeks after administration of the medication. RESULTS: The enrolled patients were classified into two groups based on the grade of acneiform rash caused by anti-EGFR antibody drugs: "Grade 1 and lower," and "Grade 2 and higher." The skin parameters were compared between these groups. The results showed that in the "Grade 2 and higher" group, TEWL in the face (at week 2 of administration), chest (baseline, weeks 2 and 6 of administration), and back (at week 2 of administration) were significantly higher than those in the "Grade 1 and lower" group. However, the two groups showed no significant differences in hydration or sebum levels at any time point. CONCLUSION: TEWL can serve as a marker for acneiform rashes induced by anti-EGFR antibody drugs during cancer treatment.

Does the Act on the Safety of Regenerative Medicine in Japan ensure "safety"?: Implications of low adverse event reporting.

Public document analysis reveals that the adverse events reported for therapeutic administration under the Act on the Safety of Regenerative Medicine (ASRM) in Japan are substantially fewer than those under the Pharmaceuticals and Medical Devices Act. This study highlights the flawed reporting mechanisms and unmet legislative intentions of the ASRM.

A CAMK2B variant associated with tetralogy of Fallot, developmental delay, and growth retardation.

CAMK2B encodes the beta-subunit of calcium/calmodulin-dependent protein kinase II (CAMKII), which is expressed mainly in the brain. Variants of CAMK2A and CAMK2B cause neurodevelopmental disorders, and CAMK2B alterations have been described in at least 14 patients with intellectual disability and developmental delay. Here, we describe a novel CAMK2B variant in a patient with tetralogy of Fallot (TOF), developmental delay, and growth retardation. The patient was a 2-year-old female. She was delivered at 36 weeks 6 days gestational age by caesarean section due to non-reassuring fetal status, with birth weight of 1680 g (-2.0 SD), birth length of 43.5 cm (-1.5 SD), and occipital-frontal head circumference (OFC) of 29.4 cm (-1.7 SD). Growth retardation, microcephaly, developmental delay, tetralogy of Fallot, and dysmorphic features were present. The patient controlled her head position at four months, rolled at six months, sat at 13 months, crawled at 18 months, and walked with support at 21 months. She began speaking words at 2 years old. Her dysmorphic features included a wide face, broad forehead, puffy eyelids, broad nasal base, broad and prominent philtrum, pointed chin, full cheeks, and prominent ears. A de novo missense CAMK2B variant (NM_172079.2:c.895A > G (p.Lys299Glu) NC_000007.14:g.44241708T > C (hg38)) was identified by proband exome sequencing and confirmed by Sanger sequencing. The variant was located at an autoregulatory segment and highly conserved among species. This patient displayed many of the physical features of CAMK2B-related neurodevelopmental disorder (NDD), but the TOF present in the current case is not a feature of patients with the NDD. Since a de novo CAMK2B (p.Leu443Val) variant has previously been found in a cohort of TOF, we conclude that CAMK2B variants may be associated with this specific cardiac defect.

Enhancing scientific transparency in national CO2 emissions reports via satellite-based a posteriori estimates.

Biennial Update Reports (BURs) are essential requirements from the United Nations Framework Convention on Climate Change (UNFCCC). However, many non-Annex I countries have not submitted these reports due to difficulties in compiling the inventories. We developed a satellite-based method for the top-down inverse estimation of CO2 emissions using partial-column data in the lower troposphere obtained by the Greenhouse Gases Observing Satellite, adopted to validate the Mongolian 2nd BUR (BUR2) for the energy sector in 2018. The estimated CO2 emissions were only 1.5% higher than those reported in the BUR2; these were also very close (4.2% smaller) to estimates from the Emission Database for Global Atmospheric Research. Mongolia is the first country to introduce an independent inverse estimate in its BUR, thereby increasing scientific transparency. Our method could be applied into other countries and could be incorporated into UNFCCC reporting guidelines, significantly improving global CO2 emission estimates.

PHACES-like syndrome with TMEM260 compound heterozygous variants.

PHACES syndrome is a multiple congenital disorder with unknown etiology that is characterized by Posterior fossa anomalies, Hemangioma, Arterial lesions, Cardiac abnormalities/coarctation of the aorta, Eye anomalies, and Sternal cleft. Compound heterozygous or homozygous TMEM260 variants cause structural heart defects and renal anomalies syndrome (SHDRA). We describe a 10-year-old male patient with a PHACES-like syndrome and TMEM260 compound heterozygous variants who demonstrated overlapping phenotypes between the two syndromes. He presented with truncus arteriosus, supraumbilical raphe, ophthalmological abnormality, vertebral abnormality, borderline intellectual disability, and hearing loss. He had normal serum creatinine. In proband exome sequencing, compound heterozygous TMEM260 variants (NM_017799.4 c.1617delG p.(Trp539Cysfs*9)/c.1858C > T p.(Gln620*)) were identified. Twelve patients have been reported with TMEM260-related SHDRA: 10 had truncus arteriosus and 6 had renal failure. One previously reported patient had facial port wine nevus and another patient had supraumbilical raphe, which are the cardinal signs for PHACES syndrome. TMEM260-related SHDRA could share overlapping clinical features with PHACES syndrome. This report expands the phenotypic spectrum of a TMEM260-related disorder.

Long-Term Analysis of Internal Exposure Dose-Reduction Effects by Food Regulation and Food Item Contribution to Dose after the Fukushima Daiichi Nuclear Power Plant Accident.

Over 10 years have passed since the Fukushima Daiichi Nuclear Power Plant accident. This study verifies the efficacy of longitudinal regulation on internal exposure doses and analyzes food group contributions to radiation doses using accumulated monitoring test results. The committed effective doses in 10,000 virtual persons from fiscal year (FY) 2012 to 2021, with and without regulation, were estimated as products of radioactivity concentrations randomly sampled from the test results, food intake, and dose coefficient. The distributed values of food intake rather than a mean value in dose estimation were assumed to reflect food intake variations and avoid underestimation of internal exposure doses for high-intake consumers. Furthermore, the ingestion of radioactive cesium from the calculation was analyzed per food group. The 95th percentile of the internal exposure dose (the dose of a "representative person") was less than 1 mSv/year in both FYs. The regulation effect was substantial in FY 2012, and no noticeable difference in radiation doses was found between the regulation and no regulation conditions after FY 2016. Internal exposure doses decreased until approximately FY 2016 and then remained constant. It was also shown that not only radioactivity concentration but also food intake is a major factor affecting cesium intake. In summary, it was confirmed that Japan had ensured food safety regarding radioactive materials.

Assessing the costs of GHG emissions of multi-product agricultural systems in Vietnam.

Besides a vital sector of the economy, agriculture is a primary source of greenhouse gas (GHG) emissions. The present paper investigates the impact of carbon tax policy on Vietnamese agriculture by focusing on multi-product systems such as rice, livestock, and aquaculture, traditionally called the Vuon (Garden)-Ao (Pond)-Chuong (livestock pen) system (VAC). In it, farmers use garden, pond, and pen by-products as fertilizer and feed. We use shadow prices and Morishima substitution elasticities as greenhouse gas emissions indicators, estimated with directional output distance function. Farmers in the Mekong Delta region are found to be technologically less efficient than in other regions of Vietnam, though the shadow prices of GHG emissions are lower there too. This indicates that farmers in the Mekong Delta, generally concentrating either on livestock or aquaculture, have greater potential for reducing GHG emissions by way of improvements in technical efficiency than do those in other regions. However, Morishima elasticity estimates show that policy impacts diminish more quickly in the Mekong than elswhere. We suggest the Vietnamese government encourage Mekong Delta farmers to employ technologically more efficient methods or shift to more balanced farming to reduce the shadow price of GHG emissions, encouraging more efficient emissions reduction.

Effect of risk factors for acneiform rash induced by anti-epidermal growth factor receptor antibody drugs on survival: a retrospective observational study.

BACKGROUND: We previously reported that high body weight was a risk factor affecting the onset of anti-epidermal growth factor receptor (EGFR) antibody drug-induced acneiform rash. The current study investigated the relationship between risk factors for anti-EGFR antibody drug-induced acneiform rash and survival probability in colorectal cancer patients, as well as effects of drug withdrawal, dose reduction, or treatment discontinuation on treatment continuation. METHODS: This retrospective study included 67 patients with unresectable advanced or recurrent colorectal cancer treated with anti-EGFR antibody drugs for the first time. RESULTS: The survival time and acneiform rash grade of patients with high body weight (≥ 67.2 kg) were significantly longer and higher than those of patients with low body weight (< 67.2 kg). Moreover, the treatment continuation time of patients with drug withdrawal or dose reduction was significantly longer than that of patients without drug withdrawal or dose reduction or with/without treatment discontinuation. Meanwhile, the treatment continuation time of patients with treatment discontinuation was significantly shorter than that of patients with drug withdrawal or dose reduction or those without drug withdrawal, dose reduction, or treatment discontinuation. CONCLUSIONS: High body weight is a novel prognostic factor for patients receiving cancer drugs with anti-EGFR antibody drugs. Hence, the results of this study suggest that patients with high body weight should be carefully monitored for the development of acneiform rash when receiving anti-EGFR antibody drugs as cancer drug therapy.

Higher proportions of a healthy gastric mucosa in healthy Japanese adults with later birth year: analysis of 41 957 participants.

BACKGROUND: In Japan, most gastric cancers are associated with gastric mucosal atrophy caused by chronic infection with Helicobacter pylori (H. pylori). Recognizing the condition of the gastric mucosa and determining the infection status of H. pylori are important for predicting the individual risk of gastric cancer. This study aimed to determine the proportion of Japanese adults with a healthy gastric mucosa (without H. pylori infection) among 12 birth-year groups encompassing 1935-1990 by morphological images. METHODS: The gastric mucosa was classified as healthy or having gastritis based on routine double-contrast upper gastrointestinal barium X-ray radiography. The participants included 41 957 healthy Japanese adults. Serum or urine H. pylori antibody levels were also assessed. RESULTS: In total, 25 424 participants had healthy mucosa without a history of H. pylori eradication. The proportions of participants with a healthy mucosa by birth year were 19.8% (57/288), 27.1% (306/1128), 32.4% (569/1756), 37.6% (1808/4811), 49.2% (3207/6522), 60.1% (3966/6550), 71.2% (5224/7342), 77.2% (5114/6624), 80.6% (3342/4149), 85.0% (1404/1652), 85.3% (302/354) and 94.7% (125/132) in 1935, 1940, 1945, 1950, 1955, 1960, 1965, 1970, 1975, 1980, 1985 and after 1990, respectively (P for trend <0.01). All the participants with a healthy mucosa showed negative results in the H. pylori antibody tests. CONCLUSIONS: The proportion of participants with normal gastric mucosa linearly increased with the birth years. The prevalence of morphologically healthy gastric mucosa could have consistently increased with decreasing prevalence of H. pylori infection.

Longitudinal Verification of Post-Nuclear Accident Food Regulations in Japan Focusing on Wild Vegetables.

Focusing on the importance of wild vegetables for local residents, this study aims to validate the effects of food regulations under the current criteria (e.g., 100 Bq/kg for general foods) established approximately a year after the Fukushima Dai-ichi Nuclear Power Plant accident. Over 2,500,000 monitoring tests were performed under the criteria until fiscal year (FY) 2020. We estimated changes in internal exposure dose using test results. The effective dose was estimated using the radioactive concentration randomly sampled from the results, food intake, and dose conversion factor. As a new attempt, dose estimation reflecting the intake of wild vegetables that may have irreplaceable value for local residents was conducted. The median, 95th, and 99th percentile of the estimated dose without reflecting the wild vegetables' intake were 0.0485, 0.183, and 10.6 mSv/year, respectively, in the estimation with all test results (no regulation) and 0.0431, 0.0786, and 0.236 mSv/year, respectively, in the estimation with results within the standard limits (regulated) in FY2012. These doses decreased with time. Although estimated doses with or without the reflection of wild vegetables' intake were similar, estimation that is more plausible is possible, particularly for a high percentile, by reflecting the wild vegetables' intake. Radiation doses (regulated) were significantly less than 1 mSv/year in different FYs. In Japan, food regulation measures benefit food safety.

[The Method for Estimation of Radiation Dose of Eye Lens Covered by Radiation Protection Glasses Using Personal Dosimeter for Trunk of the Body and X-ray Shielding Material].

PURPOSE: Radiation protection glasses is a useful tool for dose reduction of eye lens. However, the method to evaluate the dose of eye lens, which is covered with radiation protection glasses in actual radiation management situation, has not been established. We invented the easy-to-use procedure to estimate the dose of eye lens inside of radiation protection glasses for occupationally exposed person using an X-ray shielding material and an additional personal dosimeter for trunk of the body. The objective of this study was to evaluate the feasibility of the new method. METHODS: The radiation field in interventional radiology (IR) was reconstructed. A personal dosimeter was set to the eye position and neck position of medical staff phantom. Each dosimeter was covered by radiation protection glasses or X-ray shielding material, and the 1 cm or 70 mm dose equivalent quantity in the neck position was compared to the 3 mm dose equivalent quantity in the eye position. RESULTS: The dose equivalent quantity measured inside of X-ray shielding material in the neck position was similar to that of inside radiation protection glasses. Thus, the X-ray attenuation by radiation protection glasses was able to be simulated using the X-ray shielding material. CONCLUSION: It was suggested that the dose of eye lens inside of radiation protection glasses could be easily estimated by covering the conventional personal dosimeter for trunk of the body with the X-ray shielding material.

Effectiveness of negative pressure wound therapy for the wound of ileostomy closure: a multicenter, phase II randomized controlled trial.

BACKGROUND: The American Society of Surgery and American Society for Surgical Infections issued guidelines for surgical site infections (SSIs) in December 2016. These guidelines recommend a purse-string suture (PSS) for stoma closure as it facilitates granulation and enables open wound drainage. This study investigated the effect of using negative pressure wound therapy (NPWT) along with standard PSS and aimed to determine the optimal period of NPWT use. METHODS: The patients were divided into three groups as follows: Group A, postoperative wound management alone with gauze exchange as the representative of conventional PSS; Group B, the performed management was similar to that of Group A plus NPWT for 1 week; and Group C, the performed management was similar to that of Group A plus NPWT for 2 weeks. Regarding objective measures, the wound reduction rate was the primary outcome, and the incidence of SSIs, length of hospital stay, and wound healing duration were the secondary outcomes. RESULTS: In total, 30 patients (male: 18, female: 12) were enrolled. The average age was 63 (range: 43-84) years. The wound reduction rate was significantly higher in Group B than in Group A on postoperative days (PODs) 7 (66.1 vs. 48.4%, p = 0.049) and 10 (78.6 vs. 58.2%, p = 0.011), whereas no significant difference was observed on POD 14. Compared with Group A, Group C (POD 7: 65.9%, POD 10: 69.2%) showed an increase in the wound reduction rate on POD 7, although the difference was not significant (p = 0.075). SSIs were observed in Groups B (n = 2) and C (n = 2) (20%) but not in Group A (0%). CONCLUSIONS: The most effective duration of NPWT use for ileostomy closure with PSS in terms of the maximum wound reduction rate was from PODs 3 to 10. However, NPWT did not shorten the wound healing duration. NPWT may reduce the wound size but should be used with precautions for SSIs. The small sample size (30 cases), the use of only one type of NPWT system, and the fact that wound assessment was subjective and not blinded were the limitations of this study. Further studies are needed to confirm our findings. TRIAL REGISTRATION: UMIN Clinical Trials Registry; UMIN000032174 (10/04/2018).

Adrenal suppression and anthropometric data at two years of age was not influenced by the initial hydrocortisone dose in patients with 21-hydroxylase deficiency.

In contrast to the glucocorticoid maintenance therapy employed in patients with 21 hydroxylase deficiency (21OHD), the initial therapy remains to be optimized. The Japanese Society for Pediatric Endocrinology recommends a hydrocortisone (HC) dose of 25-100 mg/m2, which is higher than that employed in Western countries. Herein, we aimed to retrospectively verify the impact of initial HC treatment during infancy and early childhood. Between 2010 and 2018, 15 classical patients with 21OHD were enrolled and divided into the following groups based on initial HC therapy: high dose group (HDG, n = 6), medium dose group (MDG, n = 5), and low dose group (LDG, n = 4). In the HDG and MDG, HC was initiated at 100 mg/m2 and reduced to maintenance doses over 4-6 mo and 2-3 wk, respectively. In the LDG, HC was initiated with a maintenance dose of 7 mg/d, accompanied by fludrocortisone and oral NaCl. During the second year, 17α-hydroxyprogesterone was sufficiently suppressed in all three groups. At two years of age, no significant differences in anthropometric data were observed. Our retrospective study did not reveal any apparent advantages or disadvantages of high-dose initial HC therapy for 21OHD, and a lower dose would be preferable for the initial 21OHD treatment.

Different population size change and migration histories created genetic diversity of three oaks in Tokai region, central Japan.

To understand genetic diversity in focal species, it is important to consider the possibility of speciation with gene flow, especially in species with porous genomes such as oaks. We studied genetic diversity and structure in three oaks, Quercus mongolica var. mongolicoides (QM), Q. mongolica var. crispula (QC) and Q. serrata (QS), growing in the Tokai region, central Japan. QM is semi-endemic to the region while the others are common taxa. We also conducted demographic modeling to infer their population size change and migration histories using an approximate Bayesian computation (ABC) approach. The three taxa showed distinct genetic structures but there was genetic admixture among the taxa, especially between QM and QC. ABC analysis of population size change revealed that the population size of QM was stable during and after the last glacial period, while QC and QS showed population expansion after the last glacial maximum. ABC analysis of population divergence and migration revealed that continuous gene flow between QM and QC after their divergence was supported, while between QM and QS, and between QC and QS, secondary contact after sufficient isolation was supported. These historical migration patterns among the three taxa indicate that QM and QC are currently in the early stage or gray zone of speciation, whereas speciation of the other two taxon pairs is considered to have almost been established. Observed gene flow patterns and strength between QM and QC, and between QM and QS, were explained by both flowering patterns and historical distributions, but those between QC and QS were not.