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Background Progress in neonatal medicine has dramatically improved the survival rate of preterm births, but the evidence suggests that these low-birth weight infants (LBWIs) go on to develop pervasive development disorders and attention deficit hyperactivity disorder (ADHD) at greater rates than the general population. Children with neurodevelopmental disorders are known to suffer from deficits in visual cognition, such as in face perception and attentional functions, the characteristics of which already manifest in early infancy. Purpose This study aimed to investigate visual cognition in LBWIs during infancy. Subjects 20 LBWIs and 20 normal-birth-weight infants (NBWIs: control) of age 9–10 months (corrected age was used for LBWIs). Method Children were held seated in front of an eye tracking system by a parent, and presented with facial photos as visual stimuli. During the familiarization phase, the child was presented with two images of the same human face (familiarization stimulus) on the left and right side of a display screen (5 × 10 s trials). Next, during the test phase, the child was presented with the same image on one side of the screen, and a photo of a different person’s face (novel stimulus) on the other (2 × 5 s trials). Gaze behavior was assessed in terms of the total time spent looking at either facial stimulus, and specifically at the eyes of the stimuli, as well as the number of attentional shifts between stimuli, and novelty preference. Results/Discussion LBWIs spent significant less time looking at facial stimuli overall, and less time at the eye region, than NBWIs. These findings seem to evidence developmental differences in functions related to visual cognition.

There have been a number of recent reports on the occurrence of autoimmune conditions after autologous hematopoietic stem cell transplantation. We describe a rare case of Evans syndrome (ES) that developed in a 16-year-old patient >1 year after autologous peripheral blood stem cell transplantation for recurrent Hodgkin lymphoma. ES is a rare and frequently refractory condition. No therapy for the condition has been established, and it can often be fatal. In the present case, i.v. cyclosporine A injection was significantly effective against the ES, which has not recurred.

BACKGROUND The Japanese government has established a law encouraging early detection and treatment of developmental disorders in children. Child behavior problems (CBP) tend to be recognized at school as a result of developmental disorders. The aim of this study was to identify factors associated with CBP in Japan. We hypothesized that factors other than developmental disorders are important in explaining CBP. METHODS The study was conducted between February and March 2015. Parents of 3,515 children aged 2-5 years attending one of 34 public nursery schools in Takamatsu, Kagawa, Japan received self-administered questionnaires addressing parental socioeconomic factors, mental health, parenting style (i.e. hostile, overreactive, or lax), developmental disorders in children, and CBP. A multiple regression analysis was applied to explore associations between CBP and possible factors. RESULTS Overall, 1,410 mothers were eligible to participate in the study. Children diagnosed with developmental disorders accounted for 7.8% of the sample, while on the Eyberg Child Behavior Inventory 17% of children had behavior problems needing clinical intervention. After adjustment for confounding factors, as well as for the diagnosis of developmental disorders, poor mental status and all three dysfunctional parenting styles had strong associations with CBP, and hostile, overreactive, and lax parenting had standardized β-values (β) of 0.29, 0.28, and 0.15, respectively (P < 0.01). A problematic relationship between the parents was also significantly associated with CBP (β = -0.29, P < 0.01). CONCLUSION When CBP are identified, parenting skills, mental health status and parental relationships should be considered along with the possibility of developmental disorders in the development of interventions.

Background Hypoxic-ischemic encephalopathy (HIE) usually results in a poor clinical outcome even when treated with hypothermic therapy (HT). Early postnatal changes in cerebral blood oxygenation and hemodynamics may be critical determinants of brain injury and the efficacy of HT. Objectives We measured cerebral hemoglobin oxygen saturation (ScO 2 ) and cerebral blood volume (CBV) by near-infrared time-resolved spectroscopy (TRS) in HT-treated and non-HT-treated neonatal HIE patients to assess the influence of these parameters on clinical outcome. Methods We retrospectively compared ScO 2 , CBV, and clinical outcomes of 11 neonates with HIE: 5 were treated by HT (HT-treated; 33.5 °C ± 0.5 °C for 72 h starting approximately 6 h after delivery) and 6 were not (non-HT-treated). Both CBV and ScO 2 were measured by TRS at 6, 24, 48, and 72 h after birth. Magnetic resonance imaging (MRI) was performed 1–2 weeks after birth to assess brain injury. Results Five neonates had adverse outcomes (3 HT-treated, 2 non-HT-treated). Of these, 1 died within 3 days of birth and 4 had abnormal MRI findings, including basal ganglia, white matter, and/or thalamic lesions. The other 6 neonates had normal MRI findings (favorable outcome). At 6 h after birth, CBV was significantly higher in neonates with adverse outcomes compared with those with a favorable outcome. At 24 h after birth, ScO 2 was significantly higher in neonates with adverse outcomes. Furthermore, we found that combined CBV at 24 h after birth plus ScO 2 had the best predictive ability for neurological outcome: sensitivity, specificity, positive predictive value, and negative predictive value were all 100%. Conclusion Early postnatal CBV and ScO 2 elevations were predictive of a poor outcome in HIE. Therefore, measuring combined CBV plus ScO 2 at 24 h after birth can allow more precise prediction of neurological outcome. Control of postnatal CBV and ScO 2 is critical for effective HIE treatment.

Objective The purpose of the present study was to investigate factors associated with quality of life (QOL) among mothers raising 4 and 18-month-old infants. Methods A cross-sectional study was conducted using self-report questionnaires. Participants included 400 women who took their infants for health checkups at a city in Aichi Prefecture, Japan (4-month-olds: n = 197, 18-month-olds: n = 203). Study variables included the World Health Organization Quality of Life-BREF (WHOQOL-BREF) scale, mother's lifestyle, support from family, and other factors potentially related to QOL. Results For mothers of 4-month-old infants, total QOL was associated with emotional support from families (OR 6.09, 95% CI 2.13-17.43) and having enough sleep (7 h or more; OR 4.18, 95% CI 1.86-9.36). These mothers had shorter sleeping hours than mothers of 18-month-old infants. QOL of mothers of 18-month-old infants was associated with emotional support from families (OR 3.06, 95% CI 1.14-8.22) and using childrearing support facilities (OR 2.02, 95% CI 1.01-4.01). Conclusion Different factors contributed to mothers' QOL as a function of infant age. Emotional support from families was associated with better QOL in both mother groups. Differences were that for mothers of 4-month-old infants, enough sleep was relevant to QOL, while in mothers of 18-month-old infants, childcare services helped improving their QOL.

The purpose of this study was to examine developmental changes in visuocognitive function, particularly face recognition, in early infancy. In this study, we measured eye movement in healthy infants with a preference gaze problem, particularly eye movement between two face stimulations. We used the eye tracker system (Tobii1750, Tobii Technologies, Sweden) to measure eye movement in infants. Subjects were 17 3-month-old infants and 16 4-month-old infants. The subjects looked two types of face stimulation (upright face/scrambled face) at the same time and we measured their visual behavior (preference/looking/eye movement). Our results showed that 4-month-old infants looked at an upright face longer than 3-month infants, and exploratory behavior while comparing two face stimulations significantly increased. In this study, 4-month-old infants showed a preference towards an upright face. The numbers of eye movements between two face stimuli significantly increased in 4-month-old infants. These results suggest that eye movements may be an important index in face cognitive function during early infancy.

The aim of this study was to evaluate the hypothesis that cerebral hemoglobin (Hb) oxygenation is related to phosphorylation potential during primary and secondary cerebral energy failure in newborn infants who have experienced birth asphyxia. We subjected newborn piglets to severe transient cerebral hypoxic-ischemia followed by resuscitation and examined cerebral energy metabolism by 31P-magnetic resonance spectroscopy and evaluated changes in cerebral Hb oxygen saturation (ScO2) using full-spectrum near-infrared spectroscopy before, during, and up to 54 h after the hypoxic-ischemic insult. ScO2 was significantly decreased during the hypoxic-ischemic insult compared with baseline values. During secondary energy failure, piglets were separated based on the relationship between the ratio of phosphocreatine to inorganic phosphate and ScO2; those with a negative correlation were less injured than those with a positive correlation. These results indicate that changes in ScO2 as measured by near-infrared spectroscopy are related to phosphorylation potential during secondary energy failure in asphyxiated infants.

Background: The light-emitting diode is used as one of the new light sources for phototherapy. NeoBLUE (Atom Medical, Tokyo, Japan) incorporates blue light-emitting diodes for the treatment of neonatal hyperbilirubinemia. The authors compared the in vitro efficacy of neoBLUE with conventional phototherapy devices. Methods: The three light devices used included neoBLUE and two conventional phototherapy devices with six blue–white (BW) or six green (GR) fluorescent tubes. A bilirubin/human serum albumin solution (15 mg/dL) in 200 × 300 mm elliptical bag was irradiated with each three light device. The average light intensity of neoBLUE, BW and GR was 22.5, 10.2 and 2.6 μW/cm2 per nm, respectively, for the irradiated area. Bilirubin photoisomers and native bilirubin were measured by high-performance liquid chromatography. Results: In neoBLUE, BW and GR, the respective production rate of cyclobilirubin was 6.0, 3.7 and 3.9 × 10−2 mg/dL/min, and the respective (4Z, 15E)-bilirubin/(4Z, 15Z)-bilirubin ratio after irradiation was 0.44, 0.33 and 0.12; the (4Z, 15Z)-bilirubin reduction rate at 20 min after irradiation was 60, 68 and 82%, respectively. The reduction rate of (4Z, 15Z)-bilirubin correlated with the (4Z, 15E)-bilirubin/(4Z, 15Z)-bilirubin ratio. Conclusion: Phototherapy using the neoBLUE under high level may be clinically more effective than therapy using the conventional light source from the results of the production rate of cyclobilirubin.

Background: The developmental changes of the umbilical cortisol levels in neonates at gestational age of 23–41 weeks were studied and the effect of antenatal steroid administration on the umbilical cortisol levels were examined. Methods: Cortisol levels in the umbilical vein (UV) and the umbilical artery (UA) were studied in 35 neonates at the gestational age (GA) of 23–41 weeks with or without antenatal administration of corticosteroids. Serum cortisol concentrations were measured by the high performance liquid chromatography method. Results: The correlation between cortisol levels in UV and birthweight (BW) was weak and negative in premature infants. UV cortisol levels in the neonates with antenatal corticosteroid were lower than those in the neonates without antenatal corticosteroid, but the relation was not significant. The developmental changes of UV cortisol levels were the same as those in Murphy's study (spontaneous-onset labor). The cortisol levels in UV and UA had a significantly positive correlation and both had almost equal concentrations. There were no correlations between cortisol levels in UV and placental weight, Apgar Score at 1 and 5 min. Conclusions: In the neonates whose birthweight was less than 2000 g without antenatal corticosteroid, there was a negative correlation between cortisol levels in UV and BW but there was no correlation between cortisol levels in UV and GA. That the neonates with antenatal corticosteroid would have a suppressed adrenocortical function after birth could not be proved.

Background: The clinical effect of phototherapy for neonatal hyperbilirubinemia is based on the production and elimination of cyclobilirubin. Generally, the clinical effect of light sources is estimated by the reduction in the total serum bilirubin level. One procedure with less invasiveness than blood collecting is urine collection. Whether the effectiveness of light sources used for phototherapy could be assessed using measurements of bilirubin photoisomers in urine was studied. Methods: This study was a retrospective analysis of 38 term infants with hyperbilirubinemia who underwent phototherapy. Bilirubin fractions in serum and urine before and 24 h after the phototherapy were measured by high-performance liquid chromatography. The light sources used for the phototherapy were blue-white light (n = 11), Biliblanket plus high output (n = 13) or green light (n = 14). The relationships between serum and urine bilirubin photoisomers after phototherapy and whether the levels of urine bilirubin photoisomer are affected by the light sources with different wavelength characteristic were analyzed. Results: There was no correlation between serum (ZE)-bilirubin and urine configurational isomers, but a weak positive correlation between serum (EZ)-cyclobilirubin and urine structural isomers after phototherapy. Although serum (ZE)-bilirubin levels depended on the wavelength characteristic of each light source during phototherapy, the urine configurational isomer levels did not depend on it. The increase in serum (EZ)-cyclobilirubin levels and the urine structural isomer levels were mostly in agreement. Conclusions: The urine bilirubin structural isomers may be used to estimate the serum (EZ)-cyclobilirubin levels and to evaluate the clinical effects of light sources.

Low-frequency oscillations in cerebral blood flow that are suggestive of resting-state brain activity have recently been reported, but no study on the development of resting-state brain activity in preterm infants has been performed. The objective of this study was to measure the cerebral blood flow oscillations, which are assumed to represent brain function in the resting state, in preterm and term infants of the same postconceptional age. The subjects were 9 preterm infants who had reached full term (gestational age (GA): 23-34 weeks, postconceptional age: 37-46 weeks) and 10 term infants (GA: 37-40 weeks, postconceptional age: 37-41 weeks). Their changes in concentration of oxyhemoglobin ([oxyHb]) and deoxyhemoglobin ([deoxyHb]) were measured in the parieto-temporal region during quiet sleep using multi-channel near-infrared spectroscopy, and the power spectral densities (PSD) of the oscillations in the concentrations of these molecules were analyzed and compared. The preterm infants displayed a higher proportion of 0.06-0.10 Hz low frequency oscillations of [oxyHb] and [deoxyHb] than the term infants, and the gestational age and the proportion of low frequency oscillations were inversely correlated. These findings suggest that resting-state cerebral blood flow oscillations differ between preterm and term infants, and that the development of circulatory regulation and nerve activity in preterm infants are influenced by the extrauterine environment.

Adenine phosphoribosyltransferase deficiency is a disorder in which 2,8-dihydroxyadenine (2,8-DHA) crystalluria is caused by a congenital deficiency in the enzyme adenine phosphoribosyltransferase (APRT). In most cases, APRT deficiency is caused by autosomal recessive inheritance of a homozygote of the mutant gene APRT*Q0 or APRT*J, but there are also some cases in which the disorder is caused by the compound heterozygote APRT*Q0 and APRT*J. In the patients described here, brown round crystals were found in their urinary sediment. Crystalluria was the first sign of APRT deficiency, thereafter confirmed by genetic screening for APRT*/Q0 and APRT*. We performed genetic screening for APRT*Q0 and APRT*J in two families and diagnosed three cases of APRT*Q0 /APRT*J compound heterozygote-type APRT deficiency. Genetic screening for APRT*Q0 and APRT*J of family members is effective for early diagnosis and early treatment for family members.

Near-infrared spectroscopy has been used for measurement of changes in cerebral Hb concentrations in infants to study cerebral oxygenation and hemodynamics. In this study, measurements by time-resolved spectroscopy (TRS) were performed in 22 neonates to estimate the values of light absorption coefficient and reduced scattering coefficient (mu'(s)), cerebral Hb oxygen saturation (SCO2), cerebral blood volume (CBV), and differential pathlength factor (DPF), and the relationships between postconceptional age and mu'(s), SCO2, CBV, and DPF were investigated. A portable three-wavelength TRS system with a probe attached to the head of the neonate was used. The mean mu'(s) values at 761, 795, and 835 nm in neonates were estimated to be (mean +/- SD) 6.46 +/- 1.21, 5.90 +/- 1.15 and 6.40 +/- 1.16/cm, respectively. There was a significant positive relationship between postconceptional age and mu'(s) at those three wavelengths. The mean SCO2 value was calculated to be 70.0 +/- 4.6%, and postconceptional age and SCO2 showed a negative linear relationship. The mean value of CBV was 2.31 +/- 0.56 mL/100 g. There was a significant positive relationship between postconceptional age and CBV. The mean DPF values at 761, 795, and 835 nm were estimated to be 4.58 +/- 0.41, 4.64 +/- 0.46, and 4.31 +/- 0.42, respectively. There was no relationship between postconceptional age and DPF at those three wavelengths. The results demonstrated that our near-infrared TRS method can be used to monitor mu'(s), SCO2, CBV, and DPF in the neonatal brain at the bedside in an intensive care unit.

Background Phototherapy has been a standard treatment for neonatal hyperbilirubinemia for more than 40 years, but it has remained sub-optimal. Aims To clarify the developmental changes in parameters of (4 E , 15 Z )-cyclobilirubin (( EZ )-C) elimination in order to obtain basic data for establishing optimal phototherapy. Study design Blood samples were taken at regular intervals after stopping phototherapy, and bilirubin fractions were analyzed by high-performance liquid chromatography. Subjects and methods The subjects were 46 infants with hyperbilirubinemia who underwent phototherapy. The gestational age and birth weight of the subjects ranged from 25.0 to 41.0 weeks and from 656 to 3810 g, respectively, and the age at cessation of phototherapy was a median of 5 days. A kinetic model of ( EZ )-C elimination was established, and the serum half-life of ( EZ )-C was calculated on the basis of the determined model. Relationships of the half-life of ( EZ )-C with birth weight and gestational age were investigated. Results Serum ( EZ )-C elimination followed a first-order kinetic model in 43 infants and a zero-order kinetic model in three extremely low birth weight infants. The half-life of ( EZ )-C calculated on the basis of a first-order elimination model in serum ranged from 68 to 274 min and showed weak negative correlations with birth weight and gestational age. Conclusions Serum ( EZ )-C excretion followed a first-order kinetic model in most of the neonates. The half-life of ( EZ )-C becomes more prolonged in the very low birth weight infant and early gestational age.