Search

OBJECTIVE To evaluate the outcomes and factors influencing colorectal endoscopic submucosal dissection (ESD) with a long procedure time. MATERIALS AND METHODS In this single-center, retrospective study, we included 1,100 patients with 1,199 lesions who underwent colorectal ESD between April 2016 and December 2020. ESD was performed using an advanced system knife for lesions >20 mm. An S-O clip was used as the traction device. The long-time group (LP; procedure time >120 min) and normal-time group (NP; procedure time

Low birth weight (LBW) infants have higher risk of developing insulin resistance and its comorbidities later in life. The concept of "developmental origins of health and disease" suggests that intrauterine and postnatal environments have an important role in increasing these risks. The risk of such adult-onset diseases in LBW infants might be associated with adipose tissue maldevelopment including altered body composition and increased amount of visceral fat, which is the same mechanism as that in children and adults with metabolic syndrome. However, LBW infants often have different characteristics: they are not always overweight or obese over their life course. The inconsistency might be associated with the thrifty phenotype, which is produced in response to impaired growth potential and decreased lean body mass. LBW infants tend to be obese within the limits of impaired growth potential. Through our previous investigations evaluating longitudinal changes in adiponectin levels at an early stage of life, we speculated that probably, the intrauterine life of term infants or the period up to term-equivalent age in preterm infants might be the key age for the development of adipose tissues including fat cells. Because of that, we hypothesized that the smaller number of adipocytes in LBW infants might be associated with overloading of single adipocytes and impaired adipose tissue expandability. The possible mechanisms are discussed from the perspective of adipose tissue maldevelopment in LBW infants.

Background The importance of breast‐feeding for very low birthweight (VLBW) infants has been pointed out. Some overseas studies suggested that the standardization of enteral nutrition (EN) leads to improved prognosis in VLBW infants. In Japan, however, physicians in charge of infants are responsible for making nutrition management decisions on an individual basis. We conducted an online survey to clarify the course of nutrition management of VLBW infants currently implemented in Japan. Methods We mailed a notice to 300 representative neonatologists throughout Japan requesting their participation in the online survey. On the survey website, neonatologists responded to questions regarding the nutritional strategy for five birthweight groups (less than 500 g, 500–749 g, 750–999 g, 1,000–1,249 g and 1,250–1,499 g). Results Responses were recieved from 137 neonatologists. The first choice for EN up to 1 week after birth was breast milk regardless of birthweight (92.0% for 1,250–1,499 g to 95.6% for 500–999 g). More than 30% of the respondents answered that they fast infants who weigh

Background Donor human milk (DHM) became available in Japan when the first human milk bank was established in 2017. This study investigated the effects of DHM on enteral nutrition (EN) in very low birth weight (VLBW) infants in the single center in Japan. Methods Seventy-six VLBW infants hospitalized between April 2017 and March 2020 at Showa University Hospital were included in the study. We retrospectively evaluated age (hours) at which EN was initiated and age (days) until complete feeding (EN > 100 mL/kg/day) was achieved. We compared the DHM and non-DHM groups, or the early human milk (EHM) and non-EHM groups. The EHM group was defined as those in which EN was initiated with the mother's own milk or DHM within 12 h of birth. Results In 30 extremely low birth weight (ELBW) infants, EN was initiated at significantly earlier postnatal hours in the DHM group compared to those in the non-DHM group. Complete feeding was achieved at significantly earlier ages in the EHM group after adjusting for gastrointestinal complications and gestational age. Additionally, the changes in body weight z-scores from birth to term-equivalent age were significantly greater in the EHM group after adjusting for exclusive breastfeeding and small for gestational age, compared to the non-EHM group. Statistical significance was not noted in 46 subjects (birth weight, 1000-1500 g). Conclusions The use of DHM may contribute to earlier initiation and achievement of EN, resulting in greater early postnatal growth in ELBW infants in Japan.

Distal malignant biliary obstruction is caused by various malignant diseases that require biliary drainage. In patients with operable situations, preoperative biliary drainage is required to control jaundice and cholangitis until surgery. In view of tract seeding, endoscopic biliary drainage is the first choice. Since neoadjuvant therapies are being developed, the time to surgery is increasing, especially in pancreatic cancer cases. Therefore, it requires long stent patency. Recently, preoperative biliary drainage using self-expandable metal stents has been reported as a useful modality to secure long stent patency. In patients with unresectable distal malignant biliary obstruction, self-expandable metal stent is the first choice for maintaining long stent patency. Although there are many comparison studies between a covered and an uncovered self-expandable metal stent, their use is still controversial. Recently, endoscopic ultrasound-guided biliary drainage has been performed as an alternative treatment. The clinical success and stent patency are favorable. We should take into consideration that both endoscopic retrograde cholangiopancreatography-guided biliary drainage and endoscopic ultrasound-guided biliary drainage have advantages and disadvantages and chose the drainage method depending on the patient’s situation or the expertise of the endoscopist. Here, we discuss the current status of endoscopic biliary drainage in patients with distal malignant biliary obstruction.

Aim This study aimed to elucidate the gene and lipid profiles of children clinically diagnosed with familial hypercholesterolemia (FH). Methods A total of 21 dyslipidemia-related Mendelian genes, including FH causative genes (LDLR, APOB, and PCSK9) and LDL-altering genes (APOE, LDLRAP1, and ABCG5/8), were sequenced in 33 Japanese children (mean age, 9.7±4.2 years) with FH from 29 families. Results Fifteen children (45.5%) with pathogenic variants in LDLR (eight different heterozygous variants) and one child (3.0%) with the PCSK9 variant were found. Among 17 patients without FH causative gene variants, 3 children had variants in LDL-altering genes, an APOE variant and two ABCG8 variants. The mean serum total cholesterol (280 vs 246 mg/dL), LDL-cholesterol (LDL-C, 217 vs 177 mg/dL), and non-HDL cholesterol (228 vs 188 mg/dL) levels were significantly higher in the pathogenic variant-positive group than in the variant-negative group. In the variant-positive group, 81.3% of patients had LDL-C levels ≥ 180 mg/dL but 35.3% in the variant-negative group. The mean LDL-C level was significantly lower in children with missense variants, especially with the p.Leu568Val variant, than in children with other variants in LDLR, whereas the LDL-altering variants had similar effects on the increase in serum LDL-C to LDLR p.Leu568Val. Conclusion Approximately half of the children clinically diagnosed with FH had pathogenic variants in FH causative genes. The serum LDL-C levels tend to be high in FH children with pathogenic variations, and the levels are by the types of variants. Genetic analysis is useful; however, further study on FH without any variants is required.

Objective The therapeutic effect of pemafibrate on metabolic dysfunction-associated fatty liver disease (MAFLD) remains unknown. This retrospective, single-arm study investigated the efficacy and safety of pemafibrate in MAFLD patients with hypertriglyceridemia. Methods A total of 10 patients who received pemafibrate (oral, 0.1 mg, twice a day) at Gunma Saiseikai Maebashi Hospital between September 2018 and September 2019 were included. All patients underwent a liver biopsy, and the disease grade and stage were pathologically assessed based on the FLIP algorithm. Results The median age was 66.0 (53.8-74.8) years old, and 5 patients (50.0%) were men. All patients were diagnosed with non-alcoholic steatohepatitis (NASH). The fasting and non-fasting triglyceride (TG) levels were 175 (149-247) mg/dL and 228 (169-335) mg/dL, respectively. The AST and ALT values at 6 months were significantly lower than at baseline [AST: 28.0 (22.0-33.8) U/L vs. 43.5 (24.0-55.0) U/L, p=0.008, ALT: 23.0 (14.8-26.5) U/L vs. 51.5 (23.0-65.3) U/L, p=0.005, respectively], especially in NASH patients with significant activity and advanced fibrosis (p=0.040 and 0.014, respectively). Fasting TG levels were significantly lower and HDL-C levels significantly higher at 6 months than at baseline (p=0.005 and 0.032, respectively). At six months, FIB-4, the aspartate aminotransferase-to-platelet ratio index, and the macrophage galactose-specific lectin-2 binding protein glycosylation isomer level were significantly improved compared with baseline (p=0.041, 0.005 and 0.005, respectively). Treatment-related adverse events were not observed. Conclusion Pemafibrate treatment may be safe and effective for MAFLD patients with hypertriglyceridemia.

Objective Urotensin II (U-II) is a potent vasoconstrictor peptide, and increased U-II levels are associated with atherosclerosis and hypertension in adults. Low birth weight (LBW) infants have higher risks of such diseases in the future. A small number of nephrons is one of possible mechanism underlying these risks in LBW infants, while vascular elasticity and cardiac function might be another important factor. The objective of this study is to evaluate U-II levels in preterm LBW infants at an early stage of life and determine perinatal factors associated with U-II levels. Study Design The study population consisted of 57 preterm LBW infants (26 males and 31 females), including 49 appropriate for gestational age (AGA) and 8 small for gestational age (SGA) infants, born at a gestational age of ≤34 weeks with a mean birth weight of 1,589 g. Serum U-II levels were measured at term-equivalent age to evaluate perinatal factors related to serum U-II levels. Results Preterm SGA infants had significantly higher serum U-II levels than preterm AGA infants at term-equivalent age (p = 0.019). Serum U-II levels in preterm LBW infants at term-equivalent age were inversely correlated with birth weight standard deviation (SD) score in a simple regression analysis (r = − 0.395, p = 0,002) and the correlation was maintained in the multiple regression analysis. Conclusion Our results indicate that birth weight SD score might be associated with serum U-II levels in preterm LBW infants at term-equivalent age. Further studies are required to determine whether U-II levels at an early stage of life might influence the risk of atherosclerosis and hypertension. Key Points

Background Serum adiponectin circulates in three multimeric isoforms: high-molecular-weight (HMW), middle-molecular-weight (MMW), and low-molecular-weight (LMW) isoforms. Potential change in the circulating adiponectin levels in patients with nephrotic syndrome (NS) remain unknown. This study aimed to assess the levels of total adiponectin and the distribution of its isoforms in pediatric patients with NS. Methods We sequentially measured total adiponectin and each adiponectin isoform levels at the onset of NS, initial remission, and during the remission period of the disease in 31 NS patients. We also calculated the ratios of HMW (%HMW), MMW (%MMW), and LMW (%LMW) to total adiponectin incuding 51 control subjects. Results The median of total serum adiponectin levels in patients were 36.7, 36.7, and 20.2 μg/mL at the onset, at initial remission, and during the remission period of NS, respectively. These values were significantly higher than those in control subjects. The median values of %HMW, %MMW, and %LMW values were 56.9/27.0/14.1 at the onset, 62.0/21.8/13.4 at the initial remission, and 58.1/21.7/17.5 at during the remission period of NS, respectively. Compared with control subjects, %HMW at initial remission and %MMW at the onset were high, and the %LMW values at the onset and at initial remission were low. Conclusions In patients with NS, total serum adiponectin levels increase at the onset of the disease, and the ratio of adiponectin isoforms changes during the course of the disease. Further studies are needed to delineate the mechanisms between proteinuria and adiponectin isoforms change.

Prematurity and low birth weight are risk factors for the future development of chronic kidney disease (CKD) and hypertension caused by fewer nephrons with limited filtration surface area. Few reports to date have evaluated their clinical backgrounds and pathological findings, including glomerular hypertension and focal segmental glomerulosclerosis. This report describes two patients, a 15-year-old girl (patient 1), with a birth weight of 618 g and a gestational age of 24 weeks, and a 14-year-old boy (patient 2), with a birth weight of 842 g and a gestational age at 25 weeks. Both had a birth weight appropriate for gestational age. Both were first diagnosed with proteinuria during adolescence, and patient 2 also had hypertension. Pathological findings included glomerulomegaly in both and hypertrophy of the juxtaglomerular apparatus and perihilar glomerulosclerosis in patient 1, suggesting glomerular hypertension. Treatment with lisinopril resulted in the immediate disappearance of proteinuria. Renal dysfunction was observed in both patients, but neither showed evidence of severe aggravation after a follow-up of 5 or 6 years. Proteinuria in both patients was caused by glomerular hypertension with hyperfiltration. Extremely preterm birth itself may be a risk factor for future CKD. Long-term follow-up of patients born prematurely and at low birth weight, including urinalysis and blood pressure measurements, is necessary to diagnose and treat late renal complications.

Aim The aim of this study was to predict the neurological prognosis of very low birthweight (VLBW) infants. We examined the relationship between nutritional status, brain volume measured by magnetic resonance imaging (MRI) and anthropometric measurements of VLBW infants at term-equivalent age (TEA). Methods We evaluated 27 VLBW infants, born at Showa University Hospital in Japan between April 2012 and August 2013, who underwent brain MRI at TEA. Based on their clinical data, we analysed their protein and energy intake. Results Median values for the 27 VLBW infants were as follows: gestational age, 29.7 weeks; birthweight 1117 g; protein intake 2.7 g/kg/day and energy intake 97.9 kcal/kg/day. At TEA, the standard deviation scores (SDSs) of body weight, body length and the occipitofrontal circumference (OFC) were -0.8, -1.4 and 0.7, respectively. Multiple regression analysis revealed that the SDSs of body length and the OFC at TEA were significant determinants of white matter volume, but that the SDS of body weight at TEA was not. Conclusion Our findings suggest that the SDSs of body length and the OFC at TEA may be better indicators than body weight for predicting the development of the central nervous system in VLBW infants receiving nutritional management.

Background:Factors associated with successful provision of mother’s own milk (MOM) for premature infants in a Japanese neonatal intensive care unit (NICU) context are not well known.Objective:We determined the independent risk factors for low milk volume at day 4 postpartum and formula feeding at the time of NICU discharge.Methods:We reviewed the medical records of mothers who delivered at < 32 weeks’ gestation. We determined maternal, premature infant, and milk expression variables predictive of (1) day 4 postpartum milk volume being less than the cohort median and (2) formula feeding at the time of NICU discharge, reported as adjusted odds ratios (95% confidence interval).Results:Among 85 dyads, median (quartile range) milk volume on day 4 postpartum was 153 (34-255) mL. The rate of formula feeding at discharge was 42%. Mothers delivering by cesarean (vs vaginal) delivery had 4.3-fold (1.5-12.4) greater odds of day 4 milk volume < median ( P < .01). Pregnancy-induced hypertension, delayed milk expression initiation, and low pumping frequency were strongly associated with cesarean delivery. Subsequently, mothers with day 4 milk volume < median (vs ≥ median) had 7.1-fold (2.6-19.5) greater odds of formula feeding at discharge ( P < .01).Conclusion:Cesarean delivery is associated with lower milk volume on day 4 but may represent a composite of underlying risk factors for low milk volume in the early postpartum period. Further, low milk volume on day 4 is a strong correlate of lack of exclusive breast milk feeding at NICU discharge.

Background/Aims: This study aimed to investigate total adiponectin (T-Ad) and high-molecular-weight adiponectin (HMW-Ad) levels in preterm infants at term-equivalent age and to assess the relationship between adiponectin levels and early postnatal growth. Methods: The study included 43 term infants and 58 preterm infants born at 34 weeks' gestation or less. T-Ad and HMW-Ad levels were measured in the preterm infants at birth and at term-equivalent age, and in the term infants at birth. Adiponectin levels were statistically compared between preterm and term infants to evaluate the association between postnatal growth and changes in the adiponectin levels in preterm infants. Results: The T-Ad levels were higher and the ratio of HMW-Ad to T-Ad (HMW%) was lower in preterm infants at term-equivalent age than in term infants. Further, body weight SD score changes were positively associated with T-Ad and HMW-Ad increases in preterm infants from birth to term-equivalent age. The HMW% changes had a negative association only with HMW% in cord blood. Conclusion: Preterm infants have altered adiponectin levels at term-equivalent age. Further, postnatal growth may contribute to adiponectin increases from birth to term-equivalent age, although HMW% changes might be regulated before birth at least up to term-equivalent age in preterm infants.

Background: The roles of adiponectin and leptin in the early stages of life are poorly understood. We previously studied longitudinal changes in these adipocytokines from birth to 12 months of age. The aim of this investigation was to evaluate the correlation between cord serum adipocytokine levels and postnatal growth by 3 years of age. Methods: A questionnaire was sent to obtain the general physical measurements of 3-year-olds from 56 healthy newborn infants born at a gestational age of 35 weeks or more; 45 valid responses were obtained. The correlations between variables, including cord serum adipocytokine levels at birth and general physical measurements at 3 years, were investigated. Results: Body mass index (BMI) Z-score gain from birth to 3 years was negatively correlated with birthweight SD scores (β=−0.395, P= 0.019) and gestational age (β=−0.557, P= 0.016), and positively correlated with cord serum adiponectin levels (β= 0.253, P= 0.043). BMI Z-score gain from birth to 6 months was negatively correlated with only birthweight SD score (β=−0.442, P= 0.017). Cord serum leptin levels were not a significant predictor of BMI Z-scores gain in our subjects. BMI Z-scores at 6 months, 12 months, and 3 years of age were not related to cord serum adiponectin or leptin levels. Conclusions: Birthweight SD score, gestational age, and cord serum adiponectin levels are significant predictors of BMI Z-score gain from birth to 3 years of age in Japanese infants.

Background Serum leptin and adiponectin levels are strongly associated with lipid metabolism in adults. These adipocytokines play a role in intrauterine and postnatal growth but the association between cord blood levels of these adipocytokines and cholesterol at birth remains unclear. Methods The study involved 52 appropriate-for-gestational-age (AGA) infants (25 male, 27 female, 37-41 weeks of gestational age) born at Keiai Hospital without any maternal complications. Cord blood concentrations of leptin, total adiponectin, total cholesterol (TC), low-density lipoprotein cholesterol (LDL-C), other neonatal characteristics, and maternal factors were assessed to investigate the association between cord blood leptin or adiponectin levels and cholesterol levels. Results Cord blood leptin levels were significantly higher in girls. Simple regression analysis indicated that cord blood leptin levels were significantly associated with birthweight, gestational age, birthweight SD score, body mass index (BMI), ponderal index, LDL-C, and TC; but total adiponectin levels were not significantly associated with any neonatal characteristics or maternal factors. A multiple regression analysis indicated that cord leptin levels were significantly associated with BMI (beta= 0.407, P= 0.001), LDL-C (beta=-0.345, P= 0.004), and infant's gender (beta=-0.332, P= 0.006); adiponectin levels were significantly associated with only gestational age (beta= 0.279, P= 0.047). Conclusion Cord leptin level is significantly associated with cord cholesterol level at birth, anthropometry, and infant gender. The present study suggests that cord blood leptin may be a key hormone for controlling fetal lipid metabolism.

To determine the factors associated with high molecular weight adiponectin (HMW-ad) levels during the first year of life, the longitudinal changes in serum HMW-ad were investigated from birth to 12 mo of age. The total adiponectin levels, HMW-ad, and leptin were measured longitudinally from birth to 12 mo of age in 56 healthy infants (or=35 wk of gestation). Serum HMW-ad levels were positively correlated with total adiponectin levels (R2 = 0.93, p0.0001). Serum HMW-ad levels at birth, six, and 12 mo were 13.7 +/- 0.9 (mean +/- SE), 13.3 +/- 0.8, and 7.7 +/- 0.5 microg/mL, respectively. The levels at 12 mo were significantly lower than those at birth and 6 mo, respectively. In a multiple regression analysis, the HMW-ad levels at 12 mo were found to be related only to cord blood HMW-ad levels (beta = 0.680, p0.001) and not to any other factors. These results imply that postnatal production of HMW-ad at least during infancy may be regulated before birth. Further study is necessary to determine whether cord blood HMW-ad has an effect and whether it is involved in the development of metabolic syndrome.

Background context A digit/rib-like ectopic bone is a rare congenital anomaly that is most commonly seen in the thorax or pelvis. There is a single report of an adult possessing a phalanx-like bone in the cervical region; however, whether the abnormal bone was congenital or acquired remains elusive. Purpose To elucidate that a phalanx-like bone in the cervical region represents a congenital anomaly. Study design Case report. Methods Report of the imaging findings in a neonate with a palpable bone projection in the posterior neck. Results Plain radiographs demonstrated an ectopic bone posterior to the spinous process of the C5 vertebra. Three-dimensional computed tomography demonstrated that the anomalous bone was attached to the left lamina of the C5 vertebra and that there was abnormal segmentation of the left side of the cervical spine. Conclusions The anomalous bone in the neck is a congenital malformation that is accompanied by the maldevelopment of the cervical spine.

Recently aortic intima-media thickness (IMT) has been used as an earlier marker of preclinical atherosclerosis in high-risk children, such as those with type 1 diabetes mellitus and hypercholesterolemia. Children who were born preterm have an early elevation in insulin resistance, which may be a risk factor for metabolic syndrome in adulthood. However, there is no optimal marker of subsequent cardiovascular disease for children born preterm. In this study, we aimed to evaluate the effect of preterm birth on aortic IMT during the preschool period. Mean aortic IMT was measured by ultrasound in 26 subjects born preterm (gestational age

A male infant born at 32 weeks of gestation and weighing 1.44 kg had penile agenesis, bifid scrotums, an anorectal anomaly, and suspected omphalocele. An operation revealed complete urethral agenesis, duplicated and blind bowels, a vesicointestinal fistula without exstrophy, and a urachal fistula; however, no omphaloceles were detected. To our knowledge, this is the first report on aphallia accompanied with completely separated scrotum and a vesicointestinal fistula without exstrophy; this condition is associated with urorectal septum malformation sequence (URSMS) and covered cloacal exstrophy. The possible embryogenesis is discussed in this study.

179 Cord Blood Adiponectin, High Molecular Weight Form of Adiponectin and Leptin in Full- Term Neonates Are Positively Correlated with Anthropometric Para

We demonstrate 5 Gbps modulation in a 200 μm-length photonic crystal Mach-Zehnder modulator, fabricated using CMOS processes. V л L < 0.029 V. cm was observed under DC operation. RF modulation was performed at 2.2 V peak-to-peak.