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Introduction The p75 neurotrophin receptor (p75NTR) is known as an efficient marker for the prospective isolation of mesenchymal stem cells (MSCs) and neural crest-derived stem cells (NCSCs). To date, there is quite limited information concerning p75NTR-expressing cells in umbilical cord (UC), although UC is known as a rich source of MSCs. We show for the first time the localization, phenotype, and functional properties of p75NTR+ cells in UC. Methods Human UC tissue sections were subjected to immunohistochemistry for MSC markers including p75NTR. Enzymatically isolated umbilical artery (UA) cells containing p75NTR+ cells were assessed for immunophenotype, clonogenic capacity, and differentiation potential. To identify the presence of neural crest-derived cells in the UA, P0-Cre/Floxed-EGFP reporter mouse embryos were used, and immunohistochemical analysis of UC tissue was performed. Results Immunohistochemical analysis revealed that p75NTR+ cells were specifically localized to the subendothelial area of the UA and umbilical vein. The p75NTR+ cells co-expressed PDGFRβ, CD90, CD146, and NG2, phenotypic markers of MSCs and pericytes. Isolated UA cells possessed the potential to form neurospheres that further differentiated into neuronal and glial cell lineages. Genetic lineage tracing analysis showed that EGFP+ neural crest-derived cells were detected in the subendothelial area of UA with p75NTR immunoreactivity. Conclusions These results show that UA tissue harbors p75NTR+ pericyte-like cells in the subendothelial area that have the capacity to form neurospheres and the potential for neurogenic differentiation. The lineage tracing data suggests the p75NTR+ cells are putatively derived from the neural crest.

Background: The pathogenesis of nephrotic syndrome is thought to be due to a decrease in protein sieving function in the glomerular capillary wall. However, no research has been conducted on the renal sieving coefficient of serum protein in nephrotic patients. We investigated the renal protein sieving coefficient in pediatric patients with severe nephrotic syndrome.Methods: Thirty pediatric patients (age: 3−20, mean: 9.3, years; 18 male and 12 female) with severe nephrotic syndrome: hypoproteinemia (TP (Total Protein) ≤ 5.0 g/dL), heavy proteinuria (UTP/Cr (Creatinine) >2 g/gCr), and a normal GFR(glomerular filtration rate) (creatinine clearance ≥ 90 mL/min), were studied. The sieving coefficient of the serum total protein (SCTP) was calculated using a simple formula: Total protein clearance/creatinine clearance (%). The correlation with a twenty-four-hour (24hr) urinary protein test corrected by body surface area (24hrUPT/BSA) was analyzed to assess the accuracy of SCTP. For reference, the correlation between 24hrUPT/BSA and UTP/Cr with SCTP was also analyzed, and the differences between these correlation coefficients were statistically examined.Results: The mean ± standard error of SCTP was 0.13 ± 0.017%. The correlation coefficients of SCTP and U-TP/Cr with 24hrUPT/BSA were 0.90 and 0.78, respectively. SCTP had a significantly stronger correlation with 24hrUPT/BSA than U-TP/Cr (p < 0.05).Conclusion: We found that only 0.13% of sieving coefficient dysfunction causes severe nephrotic syndrome. SCTP has a stronger correlation with 24hrUPT/BSA than with UTP/Cr. If nephrotic syndrome is defined as a protein-sieving dysfunction of the glomerular capillary wall, SCTP can be considered a conceptually correct indicator.

Limited data are available on the survival and causes of death in pediatric patients with chronic kidney disease (CKD) stage 5 receiving kidney replacement therapy (KRT) in Asia.Data were obtained from the Japanese nationwide cross-sectional CKD stage 5 survey on pediatric patients (20 years of age) who started KRT from 2006 to 2013. The cohort was divided into three groups according to age at the start of KRT:1, 1-5, and 6-19 years.Among the 701 children who were included, 59.3% were boys. Peritoneal dialysis was the most common initial modality of KRT (60.3%). Median age at KRT initiation was 10.2 years. Infants (1 year old) accounted for 16.0% of the total cohort. Overall survival at 1 and 5 years was 97.2% and 92.5%, respectively. Infants had significantly lower survival rates than the other groups (hazard ratio, 5.35; 95% CI, 2.60-11.03; P0.001). In contrast, after the age of 1 year, the survival rate improved and did not differ from that of other age groups. The most common causes of death were infection (35.9%) and sudden death (15.4%).The overall survival rate of pediatric patients with CKD stage 5 in Japan is like that in other high-income countries. Age at initiation of KRT is an important factor affecting survival since the poorest survival rate was observed in infants. Further improvement in infant dialysis therapy is still needed to improve survival of the youngest children. A higher resolution version of the Graphical abstract is available as Supplementary information.

Background Screening using dipstick urinalysis has long been performed in 3-year-old children; however, it is ineffective in detecting congenital anomalies of the kidney and urinary tract (CAKUT). Measurement of the urinary β2-microglobulin (B2MG)/creatinine (Cr) ratio may be more effective for this purpose. Analysis of dried urine spots (DUS) on filter paper is suitable for mass screening since operational costs are low and samples are easy to collect and transport. We examined the accuracy of measuring the urinary B2MG/Cr ratio in DUS on filter paper. Methods We collected 2,623 urine samples from 3-year-old children. B2MG and Cr levels were measured in DUS on filter paper. We examined the correlation between the B2MG/Cr ratios measured in DUS and using the conventional method (CM) in 640 samples using the coefficient of determination test. Children with high B2MG/Cr ratios (>0.6 µg/mg Cr) in DUS samples were examined further to establish a definitive diagnosis. Results There was strong correlation between the two methods for determination of B2MG levels (r2 =0.68; P 0.6. Thirty-five children were subsequently examined; resulting in findings of renal hypodysplasia (n=2, 0.08%), horseshoe kidney (n=1, 0.04%), renal tubular dysfunction with hepatoblastoma (n=1, 0.04%), data abnormality (high urine B2MG level, n=6, 0.23%; high serum Cr level, n=1, 0.04%), and normal values (n=24, 0.91%). Conclusions We evaluated a practical method for measuring B2MG/Cr ratios in DUS as a screening method to detect CAKUT in 3-year-old children.

Background and Aims Urinary total protein/creatinine ratio (U-TP/Cr) is a simple and useful indicator of proteinuria. And it had been shown to correlate well with 24-hour urinary protein test (24hr UPT). However, U-TP/Cr is not always accurately reflecting the degree of proteinuria, especially in cases with heavy proteinuria. Proteinuria in nephrotic syndrome could be defined as the result of changed sieving coefficient (SC) of plasma total protein through the glomerular capillary wall. Therefore, we studied fractional excretion of total protein (FETP; total protein clearance/creatinine clearance %) instead of directly unmeasurable SC. The accuracy of the FETP was verified by 24hr UPT in comparison with U-TP/Cr. Method Sixty-three serum and urine samples were collected from 35 pediatric patients with various age (age: 3 − 20 y; mean: 10.3 y, 22 male, 13 female) with hypoproteinemia (TP≦ 5.0 mg/dL), heavy proteinuria (FETP≧ 0.01%), and normal GFR (creatinine clearance ≧ 100 ml/min). The correlations between 24hrUPT and U-P/Cr and FETP were studied using samples obtained the same time and the superiority was statistically examined. Results Mean±SE of 24hUPT, U-TP/Cr and FETP (%) were 5.53±0.71 (g/day), 10.23±1.26 (g/gCr), and 0.12±0.016 (%) respectively. R values for the correlation between 24hrUPT and U-TP/Cr and 24hr UPT and FETP were 0.64 and 0.86 respectively. FETP showed a statistically better correlation with 24hrUPT than with U-TP/Cr (P: 0.0001). Conclusion FETP is an accurate indicator of 24hr UPT for nephrotic patients under conditions of heavy proteinuria and hypoproteinemia in comparison with UTP/CR.

Extended-spectrum beta-lactamase (ESBL) producing bacteria spread worldwide and became major concern for antibiotic treatment. Although surveillance reports in general hospitals and long-term care facilities are increasing, their frequencies in individuals with severe motor and intellectual disabilities (SMID) are so far unknown. In this study, we examined the frequency of ESBL in stool samples collected from 146 asymptomatic SMID subjects hospitalized in a single institution. With their clinical information, we evaluated possible risk factors for ESBL colonization. From 146 fecal samples, ESBL-producing bacteria were isolated in 45 cases (31%). Drug sensitivity testing showed that 82% of the isolates were resistant to levofloxacin but were sensitive to tazobactam/piperacillin and cefmetazole. The most frequent genotype was CTX-M-9 detected in 36/45 (80%). A high degree of disability, antibiotic use within three months before sampling and post-tracheostomy were statistically significant risk factors. Tube feeding was also strongly correlated with ESBL colonization (p 0.001) and associated with lower micro-organismic diversities. Our findings are the first to reveal a high prevalence of ESBL in the fecal samples of SMID individuals and suggest possible relationships between high degree disability, tube feeding and latest histories of antibiotic use.

Carnitine is a water-soluble amino acid derivative required for β-oxidation of long-chain fatty acids. In carnitine cycle abnormalities and low carnitine states, fatty acid β-oxidation is inhibited during fasting, resulting in hypoglycemia. Pivalic acid is a substance used in prodrugs to increase absorption of parent drugs, and antibiotics containing pivalic acid are frequently used as wide spectrum antibiotics for pediatric patients in Japan. Pivalic acid released after absorption is conjugated with free carnitine to form pivaloylcarnitine, which is then excreted in urine. As a consequence, long-term administration of pivalic acid containing antibiotics has been associated with depletion of free carnitine, inhibition of energy production and subsequent hypoglycemia. Here we report a case of a 23-month-old boy treated with an antibiotic containing pivalic acid for 3 days for upper respiratory tract infection. Laboratory data at referral indicated hypoglycemia, decreased free carnitine and elevated five-carbon acylcarnitine. Isomer separation confirmed the major component of increased five-carbon acylcarnitine to be pivaloylcarnitine, thereby excluding the possibility of a genetic metabolic disorder detected with similar acylcarnitine profile. The level of carnitine was normal when the antibiotic was not administered. Our case shows that the use of antibiotics containing pivalic acid in young children requires consideration of hypocarnitinemia, even with short-term administration.

Background During the neonatal resuscitation, careful oxygenation is needed. The use of pulse oximetry is recommended to evaluate the need for oxygenation, but it is not clear whether adequate peripheral perfusion is established to allow the evaluation of arterial oxygen saturation (SpO2). Additionally, there is no study of the changes in SpO2 immediately after birth in Japan, despite the indispensable need for definitive oxygenation criteria. Methods A prospective observational study was performed in neonates with gestational ages of 35-41 weeks. A SpO2 measurement probe was attached to the neonates immediately after birth at right palm or wrist, and the perfusion index (PI), pulse rate, and SpO2 were measured until 10 minutes after birth. Results Sixty neonates were examined. Stable PI values were obtained soon after birth, preceding SpO2 measurement. The median PI (%) was constant at approximately 1.3, and the median SpO2 values (%) at 2-10 minutes were 70, 81, 82, 87, 89, 92, 92, 94, and 95, respectively. The current target value for SpO2 in Neonatal Cardio Pulmonary Resuscitation (NCPR) guideline in Japan was at approximately the 25th percentile. Conclusion The finding of stable and sufficient PI in the early postnatal period proved that peripheral perfusion was adequate to allow the measurement of SpO2. The current target SpO2 used in NCPR guideline was at approximately the 25th percentile and was thought to be beneficial for oxygenation criteria. This article is protected by copyright. All rights reserved.

Acute focal bacterial nephritis (AFBN) is a localized bacterial infection of the kidney presenting as an inflammatory mass without frank abscess formation. In children, most patients with AFBN present with nonspecific conditions, such as fever, vomiting, and abdominal pain. A small number of reported cases are accompanied by neurological symptoms, including meningeal irritation, unconsciousness, and seizures. We experienced 2 rare cases of AFBN associated with central nervous system lesions. The first case was a 3-year-old girl who had neurological symptoms, including unconsciousness and seizures, with AFBN associated with acute reversible encephalopathy. The second case was a 5-year-old girl who had neurological symptoms, including unconsciousness, with AFBN accompanied by clinically mild encephalitis/encephalopathy with a reversible splenial lesion.

Leigh syndrome (LS) is a progressive neurodegenerative disorder of infancy and early childhood. It is clinically diagnosed by typical manifestations and characteristic computed tomography (CT) or magnetic resonance imaging (MRI) studies. Unravelling mitochondrial respiratory chain (MRC) dysfunction behind LS is essential for deeper understanding of the disease, which may lead to the development of new therapies and cure. The aim of this study was to evaluate the clinical validity of various diagnostic tools in confirming MRC disorder in LS and Leigh-like syndrome (LL). The results of enzyme assays, molecular analysis, and cellular oxygen consumption rate (OCR) measurements were examined. Of 106 patients, 41 were biochemically and genetically verified, and 34 had reduced MRC activity but no causative mutations. Seven patients with normal MRC complex activities had mutations in the MT-ATP6 gene. Five further patients with normal activity in MRC were identified with causative mutations. Conversely, 12 out of 60 enzyme assays performed for genetically verified patients returned normal results. No biochemical or genetic background was confirmed for 19 patients. OCR was reduced in ten out of 19 patients with negative enzyme assay results. Inconsistent enzyme assay results between fibroblast and skeletal muscle biopsy samples were observed in 33% of 37 simultaneously analyzed cases. These data suggest that highest diagnostic rate is reached using a combined enzymatic and genetic approach, analyzing more than one type of biological materials where suitable. Microscale oxygraphy detected MRC impairment in 50% cases with no defect in MRC complex activities. Electronic supplementary material The online version of this article (doi:10.1007/s10545-017-0042-6) contains supplementary material, which is available to authorized users.

Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is often associated with ovarian teratomas in young women. However, children are less likely to develop this condition. We herein report a very rare case of anti-NMDAR encephalitis and an ovarian teratoma in a young girl. A previously healthy 9-year-old girl presented with convulsions and unconsciousness. Her cerebral spinal fluid was positive for anti-NMDAR antibody. She underwent immunotherapy, and although her clinical course improved, she did not fully recover. A right ovarian teratoma was then found and removed, and she became negative for anti-NMDAR antibody. She exhibited almost full recovery and no relapse. Tumor resection is recommended in the treatment of anti-NMDAR encephalitis. A tumor search should be actively carried out in patients aged < 12 years. Int J Clin Pediatr. 2017;6(1-2):20-23 doi: https://doi.org/10.14740/ijcp260w

Although there is debate regarding the timing of initiation of renal replacement therapy (RRT) in adults with end-stage renal disease, there is a paucity of reliable epidemiological data on pediatric patients. The present study was performed to investigate current practice in Japan with regard to the timing of initiation of RRT in children based on estimated glomerular filtration rate (eGFR). A total of 649 pediatric patients 15 mL/min/1.73 m2) at the initiation of RRT. Initiation of RRT was more likely in those undergoing preemptive transplantation (PEKT) with high eGFR [odds ratio (OR) 4.16; 95% confidence interval (CI) 1.95–8.90, P

Glutaric acidemia type 1 is a rare autosomal recessive disease caused by a deficiency of glutaryl-CoA dehydrogenase. Previous studies have reported subdural hemorrhage in untreated patients with glutaric acidemia type 1. However, there is only one report of severe acute subdural hemorrhage after minor head trauma in a patient with glutaric acidemia type 1 under guideline-recommended treatment. We report a second case of life-threatening severe acute subdural hemorrhage after a minor head trauma in a patient with glutaric acidemia type 1. This patient was previously diagnosed by newborn screening, and treatment began at 25 days of age. Early diagnosis and guideline-recommended treatment produce better outcomes for patients with glutaric acidemia type 1, although the risk of subdural hemorrhage remains.

BACKGROUND Rectal temperature is commonly used as the core temperature during therapeutic hypothermia therapy in neonates with hypoxic-ischemic encephalopathy (HIE). The purpose of this study was to examine whether nasopharyngeal temperature could serve as a substitute for rectal temperature. METHODS We prospectively investigated 40 neonates with HIE who underwent therapeutic hypothermia by selective head cooling, which involved cooling the body to 34°C for 72 h. During this period, nasopharyngeal temperature was measured and compared with rectal temperature every hour. RESULTS For 40 neonates included in this study, the mean rectal and nasopharyngeal temperatures were 34.3 ± 0.4°C (n = 2920) and 34.3 ± 0.4°C (n = 2920), respectively. Nasopharyngeal temperature strongly correlated with rectal temperature (R2 = 0.623, P < 0.0001) and magnitude of the mean difference between nasopharyngeal and rectal temperature varied little during the 72 h of therapeutic hypothermia. CONCLUSIONS Nasopharyngeal temperature in neonates with perinatal HIE undergoing therapeutic hypothermia may be a suitable substitute for rectal temperature.

Extrapontine myelinolysis (EPM) is an uncommon disorder in children, with few pediatric cases reported to date. We report the first case of an infant with EPM without central pontine myelinolysis (CPM) presenting with severe hypernatremia. On admission, the infant had impaired consciousness, mild dehydration, and severe hypernatremia (190 mmol/L). The following day, the patient developed abnormal involuntary movements. Brain magnetic resonance imaging (MRI) confirmed EPM without CPM. He recovered without sequelae, and clinical examinations were within normal limits approximately 6 months after discharge. Brain MRI at 1 year after onset showed complete disappearance of the previous EPM regions. To the best of our knowledge, this represents the youngest patient with EPM without CPM presenting with severe hypernatremia. Given that treatment for osmotic demyelination syndrome (ODS) is yet to be established, preventing the development of ODS is crucial.

Background The aim of this study was to investigate the clinical significance and factors that affect N-terminal pro-brain natriuretic peptide (NT-proBNP) elevation in the acute phase of Kawasaki disease (KD) despite the absence of apparent cardiac complications. Methods The laboratory and echocardiography results of 44 KD patients in the acute and subacute phases were reviewed. Results With preserved cardiac function, median NT-proBNP was significantly elevated in the acute phase compared with the subacute phase (343 pg/mL, IQR, 162–1182 pg/mL vs 98 pg/mL, IQR, 61–205 pg/mL, respectively; P < 0.0001). The respective levels of tumor necrosis factor (TNF)-α, soluble TNF receptor (sTNFR)1, and sTNFR2 were also significantly elevated in the acute phase compared with the subacute phase: TNF-α, 3.3 pg/mL (IQR, 2.6–4.8 pg/mL) versus 2.4 pg/mL (IQR 1.9–4.0 pg/mL; P < 0.01), sTNFR1, 2741 pg/mL (IQR, 2080–3183 pg/mL) versus 976 pg/mL (IQR, 814–1247 pg/mL; P < 0.0001), sTNFR2, 5644 pg/mL (IQR, 4693–7520 pg/mL) versus 3169 pg/mL (IQR, 2132–3878 pg/mL; P < 0.0001). Log-transformed NT-proBNP was correlated with TNF-α (r = 0.29, P = 0.056), sTNFR1 (r = 0.60, P < 0.0001), and sTNFR2 (r = 0.65, P < 0.0001). TNF-α was correlated with sTNFR1 (r = 0.35, P = 0.02) and sTNFR2 (r = 0.51, P < 0.001). Conclusion Tumor necrosis factor-α may cause NT-proBNP elevation in the acute phase of KD, and NT-proBNP level may be an indicator of TNF-α activity.

A 7-month-old girl was brought to hospital due to vomiting. Upon admission, she was in a convulsive state and stupor with extremely low blood glucose. Head computed tomography showed brain edema, and comprehensive treatment for acute encephalopathy was initiated immediately. Severe hypoglycemia, metabolic acidosis, elevation of ammonia and serum transaminases and creatine kinase suggested metabolic decompensation. Infusion of a high-glucose solution containing vitamins, biotin, and l-carnitine resolved the metabolic crisis quickly, but brain damage was irreversible. She was found to have been fed exclusively on a hypoallergenic formula (HF) for 7 months, although she was found later to be non-allergic. Evidence of inborn metabolic diseases was absent, therefore biotin deficiency and carnitine deficiency were concluded to be a consequence of reliance on a HF for a prolonged period. Health-care professionals should warn parents of the consequences of using HF.

t(17;19)(q22;p13) translocation, which generates an E2A-HLF chimeric transcription product, is rarely found in childhood precursor B-cell acute lymphoblastic leukemia (ALL). This type of ALL is cha...

Electronic supplementary material The online version of this article (doi: 10.1007/s00277-017-3015-9 ) contains supplementary material, which is available to authorized users.

Background Sarcoidosis is a multi-organ disease of unknown etiology characterised by the presence of epithelioid granulomas, without caseous necrosis. Systemic sarcoidosis is rare among children, while neurosarcoidosis in children is even rarer whether it is systemic or not. Case presentation We described the case of a 12-year-old boy who presented with monocular vision loss accompanied by unusual MRI features of an extensive meningeal infiltrating mass lesion. The patient underwent surgical resection (biopsy) via a frontotemporal craniotomy to establish a definitive diagnosis based on the histopathology, since neurosarcoidosis remains a very difficult diagnosis to establish from neuroradiogenic imagings. Based on the histopathology of the resected mass lesion, neurosarcoidosis was diagnosed. On follow-up after 3 months of steroid therapy, the patient displayed a good response on the imaging studies. MRI revealed that the preexisting mass lesion had regressed extremely. We also conducted a small literature review on imaging studies, manifestations, appropriate treatments, etc., in particular neurosarcoidosis including children. Conclusion Although extremely rare, neurosarcoidosis, even in children, should be considered in the differential diagnosis of skull base mass lesions to avoid unnecessary aggressive surgery and delay in treatment, since surgery may have little role in the treatment of sarcoidosis.

Kawasaki disease (KD) is a systemic vasculitis with a predilection for damage to the coronary arteries. In the acute phase, clinical decision making for KD relies on the measurements of the coronary z-score obtained by 2-dimensional echocardiography (2DE). In the convalescent phase, KD patients with coronary artery abnormalities (CAAs) eventually show arteriosclerotic vascular remodeling characterized by marked intimal proliferation and neoangiogenesis after KD vasculitis, which often induces myocardial ischemia. To date, several well-established surrogate markers including dobutamine stress echocardiography (DSE), the carotid intima-media thickness (CIMT) and flow-mediated dilatation (FMD), have been made available for risk assessment and the prediction of cardiovascular disease (CVD) in KD patients. Additionally, the use of carotid contrast-enhanced ultrasonography (CEUS), has enabled the visualization and quantification of the adventitial vasa vasorum (VV) network, assessing active vascular remodeling at remote arterial sites in KD patients with CAAs. However, there was no evidence of major vascular structural changes in KD patients in whom CAAs had never been detected. Thus, assessment of multiple modalities using 2DE may provide direct information not only on the vascular health but also on the stratification of the risk of CVD in KD patients with CAAs.

Background The aim of this study was to investigate cholesterol and triglyceride levels in the chylomicron fraction of preterm infants at birth and during the early postnatal period. Methods The subjects consisted of 133 infants (81 boys and 52 girls): 74 were term infants born at 37-41 weeks of gestation and 59 were preterm infants born at 29-36 weeks of gestation. Cholesterol and triglyceride in the chylomicron fraction were measured using high-performance liquid chromatography. Results Compared with term infants, preterm infants had higher cholesterol and lower triglyceride in the chylomicron fraction, both in cord blood and at 1 month after birth. Thus, the chylomicron triglyceride/cholesterol ratio was significantly lower in preterm infants than in term infants in cord blood and at 1 month of age. On single regression analysis the chylomicron triglyceride/cholesterol ratio correlated positively with gestational age at birth (r = 0.331, P = 0.0003) and at 1 month (r = 0.221, P = 0.0119). Conclusions Preterm infants have a less-lipidated chylomicron composition at birth and at 1 month of age. Some prenatal factors may persist to influence chylomicron lipidation during the early postnatal period.

Background Neurological manifestations caused by hypoglycemia range from reversible focal deficits and transient encephalopathy to irreversible coma or death. Recently, high signal intensity lesions in the splenium of the corpus callosum on diffusion-weighted magnetic resonance imaging were reported in adults experiencing hypoglycemia. However, patients presenting with agraphia are rare. Subject and methods We examined a 17-year-old left-handed female patient with type 1 diabetes who exhibited transient left agraphia with a reversible splenium lesion of the corpus callosum on diffusion-weighted imaging caused by hypoglycemia, which was improved with blood glucose management alone. Conclusion This rare case indicates that agraphia, a sign of callosal disconnection syndrome, can result from a reversible splenial lesion of the corpus callosum caused by hypoglycemia.

We report the case of a 9-year-old girl with Down syndrome (DS) diagnosed with idiopathic pulmonary hemosiderosis (IPH). Although acute pneumonia complicated by hemolytic anemia was suspected, IPH was finally diagnosed on bronchoscopy. Treatment with prednisolone achieved good clinical response. An association between IPH and DS was not able to be identified, but immunological issues in DS may contribute to the onset of IPH. Recurrent and intractable respiratory symptoms with marked infiltrative shadows in the bilateral lungs and complicated by severe anemia in patients with DS should suggest IPH.

Fetal organs require much lipid for growth, but the cord blood had low TG concentrations, compared to adult serum. We investigated the association between the concentration of apolipoprotein A-V (apoA-V) and lipid profile in cord blood and neonatal serum.ApoA-V was identified as an important determinant of plasma triglyceride concentrations. We sought to determine the association between serum apoA-V concentrations and lipoprotein profile in preterm infants and its early postnatal change.Sixty-three neonates (35 males and 28 females; 15 term and 48 preterm) were included. Serum lipoprotein profile and apoA-V concentrations were determined at birth and 1 month.Cord blood apoA-V concentrations in appropriate-for-gestational age infants were extremely low (13.1 ± 3.4 ng/mL in term infants, 4.4 ± 0.9 ng/mL in preterm infants) compared with adult values, and those of small-for-gestational age infants were further low (6.4 ± 4.2 ng/mL, 2.2 ± 1.3 ng/mL, respectively). During the first month, serum apoA-V concentration markedly increased, and the concentration of preterm appropriate-for-gestational age infants caught up, whereas that of preterm small-for-gestational age infants did not. At birth, apoA-V concentration positively correlated with gestational age (r = 0.354, P = .0069) but not with birth weight Z-score. ApoA-V concentration had a positive association with very low-density lipoprotein triglyceride concentrations (r = 0.646, P.0001), and the relationships still remained at 1 month (r = 0.283, P = .0348).ApoA-V in neonates was unique in its serum concentration and in the association with lipoprotein profile.

In Japan, urinary screening for preschool children has been obligatory since 1961. The system was reconsidered and has been under review since 2012, because many problems in the system had been identified, and its usefulness was uncertain. In the process, the following were analyzed: (i) frequency of urinary abnormalities identified on screening; (ii) diseases identified from urinary abnormalities; (iii) clinical course of children found to have urinary abnormalities; and (iv) screening for asymptomatic urinary tract infection (UTI) as a way of screening for congenital anomalies of the kidney and urinary tract. A computerized literature search was conducted, and study reports issued by the Ministry of Health, Labour and Welfare study group, and data of Akita City and Chiba City were reviewed. The prevalence of abnormal results at the first urinalysis was high, but at the second urinalysis the prevalence decreased in the range 1/6-1/20. The prevalence of tentative diagnosis at the third urinalysis was similar to the school urinary screening results. Serious illness was not found in children who had hematuria alone. In contrast, diseases requiring immediate attention were found in children with proteinuria, although the prevalence of proteinuria was not high. The dipstick method for leukocyturia was inefficient. The importance of two consecutive urinalyses before detailed examination, the lack of usefulness of screening for hematuria in 3-year-old children, and the importance of proteinuria were confirmed. Screening for asymptomatic UTI using urinary leukocytes was very inefficient.

Yuriko Abe1), Mamoru Ayusawa1), Masataka Kato1), Hirofumi Watanabe1), Ami Cho1), Akiko Komori1), Hiromi Okuma1), Rie Ichikawa1), Hiroshi Kamiyama1), Naokata Sumitomo2), Sango Ito1), and Shori Takahashi1) 1) Department of Pediatrics and Child Health, Nihon University School of Medicine, Tokyo, Japan 2) Department of Pediatric Cardiology, Saitama Medical University International Medical Center, Saitama, Japan

Objectives Head trauma is one of the main causes of death in childhood and often leaves severe disability with serious neurological damage. Appropriate treatment must be provided immediately to improve outcomes. This study was performed to identify factors associated with a poor prognosis at an early stage of severe head injury in children. Methods The subjects were registered in the Japan Neurotrauma Data Bank. They were 119 children (mean age, 8 years; male, 67.2%) with severe head injury registered during a period of 4 years (from July 1, 2004 to June 30, 2006 and from July 1, 2009 to June 30, 2011). Univariate and multivariate analyses were performed to examine relationships among factors and outcome 6 months after discharge. Logistic regression analysis was performed to develop models for poor prognosis and death. Results Outcome was evaluated based on the Glasgow Outcome Scale: 73 children (61.3%) had good recovery, 11 (9.2%) had moderate disability, 8 (6.7%) had severe disability, 4 (3.3%) were in a vegetative state, and 23 (19.3%) had died. Four factors were identified as predictors of a poor prognosis: serum glucose level greater than or equal to 200 mg/dL, Glasgow Coma Scale score on admission less than or equal to 5, presence of mydriasis, and presence of traumatic subarachnoid hemorrhage. Three factors were identified as predictors of death: serum glucose level greater than or equal to 200 mg/dL, Glasgow Coma Scale score on admission less than or equal to 5, and presence of mydriasis. Conclusions Using these predictors, subsequent exacerbation may be predicted just after arrival at the hospital and appropriate treatment can be provided immediately.

BACKGROUND Serum N-terminal pro-brain natriuretic peptide (NT-proBNP) tends to rise in acute phase Kawasaki disease (KD), but the cause of NT-proBNP elevation has not been clarified. In a previous study, cardiac function evaluated on 2-D echocardiography (2D-E) such as ejection fraction was normal, but this does not reflect subtle changes in cardiac dysfunction, and hence the association between cardiac function and NT-proBNP elevation is still controversial. The aim of this study was therefore to elucidate the influence of cardiac function on NT-proBNP elevation, by evaluating cardiac function via strain on 3-D speckle tracking imaging (3D-STI), in acute and subacute KD patients. Given that cytokines are also thought to induce NT-proBNP in acute phase KD, serum cytokines and cytokine receptors were measured at the same time. METHODS Laboratory data and echocardiography in 52 KD patients in the acute and subacute phases were reviewed. RESULTS Median NT-proBNP was significantly elevated in the acute phase compared with the subacute phase (356.5 pg/mL; IQR, 145-904 pg/mL vs 103.5 pg/mL; IQR, 59-150 pg/mL, P < 0.01). All cytokines were also significantly elevated in the acute phase compared with the subacute phase. Tumor necrosis factor (TNF)-α, soluble TNF receptor (sTNFR)1, and sTNFR2 concentration were all significantly higher in the acute phase. Indices of cardiac function were not significant different between phases. NT-proBNP in the acute and subacute phases correlated with sTNFR1 (r = 0.63/0.43, P < 0.01), sTNFR2 (r = 0.50/0.31, P < 0.05), and interleukin-6 (r = 0.58/0.43, P < 0.01). NT-proBNP did not correlate with global longitudinal strain (GLS) on 3D-STI. CONCLUSION Although no correlation was seen between NT-proBNP and GLS on 3D-STI, correlations between NT-proBNP and cytokines were clear. NT-proBNP might be a marker of inflammation in KD, but is not a marker of cardiac function.

Background The aim of this study was to investigate residual blood volume in the umbilical cord of extremely premature infants. Methods Twenty extremely premature infants were held at or below the placenta while the umbilical cord was clamped and cut at approximately 2–3 cm from the umbilicus within 30 s after birth. The umbilical cord was then clamped near the placenta to obtain a length of approximately 30 cm and cut. The residual blood volume in the segment of cord was drained and measured in milliliters. Results Mean birthweight was 846 ± 172 g (range, 587–1180 g). The average length of the clamped segment of umbilical cord was 29.8 ± 1.5 cm (range, 27–32 cm). Total residual blood volume and residual blood volume per cm were 15.5 ± 6.7 mL (range, 6–25 mL) and 0.5 ± 0.2 mL/cm (range, 0.2–0.8 mL/cm), respectively. The residual cord blood volume per kilogram of infant weight per 30 cm was 17.7 ± 5.5 mL/kg/30 cm (range, 8.9–29.0 mL/kg/30 cm). Conclusion Infants could receive approximately 18 mL/kg of whole blood by one-time milking of 30 cm umbilical cord. With an average hematocrit of 40%, this volume is equivalent to approximately 13 mL of packed red blood cells (hematocrit 55%).

We investigated the efficacy and safety of switching to insulin glulisine (GLU) from other rapid-acting insulin analogs (Ra) in children with type 1 diabetes treated with multiple daily injections of insulin or continuous subcutaneous insulin infusion. A total of 26 children with type 1 diabetes were included. Ra in all of these patients was changed to GLU, and they were observed for a 6-month period after having previously finished treatment with other Ra. The mean glycated hemoglobin value decreased from 7.6 ± 1.0 to 7.4 ± 0.9% (P = 0.0034), and mean plasma glucose values after breakfast and supper also improved from 183 ± 50 to 153 ± 32 mg/dL (P = 0.0035), and from 203 ± 29 to 164 ± 23 mg/dL (P