Hayato Go

Serum KL‐6 levels as a biomarker of lung injury in respiratory syncytial virus bronchiolitis

To evaluate whether KL‐6 concentration is a useful biomarker of the severity of respiratory syncytial virus (RSV) bronchiolitis, we determined KL‐6 concentrations in patients with RSV bronchiolitis with or without chronic heart disease (CHD). We enrolled 52 patients who had been diagnosed with RSV bronchiolitis and required admission to the hospital at the Department of Pediatrics of Fukushima Medical University School of Medicine from 2004 to 2005. These patients were divided into two groups: Group 1 consisted of patients without any underlying disease, and Group 2 consisted of patients with CHD. These patients were assigned to three categories. Stage A consisted of patients without oxygen dosage, stage B of patients who required oxygen dosage, and stage C of patients required artificial respiration. We evaluated baseline characteristics, clinical features, and serum KL‐6 concentration in Group 1, Group 2, and a control group (healthy infants without infection). Mean serum KL‐6 concentrations in patients with RSV bronchiolitis were higher than those in the control group (471.8 ± 236.9 and 127.1 ± 69.1 U/ml, respectively). Mean serum KL‐6 concentration was higher in Group 2 than in Group 1 (692.8 ± 313.1 and 390.4 ± 132.7 U/ml, respectively). Mean serum KL‐6 concentrations were higher in stage C than in stages A and B, and mean serum KL‐6 concentrations were higher in stage B than in stage A. These findings suggest that serum KL‐6 is associated with the severity of RSV bronchiolitis and that it may be a useful biomarker for the severity of RSV bronchiolitis. J. Med. Virol. 81:2104–2108, 2009.

Five Cases of Congenital Chylothorax Treated by Intrapleural Minocycline

Minocycline pleurodesis was performed on five infants with congenital chylothorax in our institutions. They could not achieve sufficient efficacy though they had received other conservative therapies. Four of the five cases obtained reduction of pleural effusion using the minocycline pleurodesis. We concluded that minocycline pleurodesis is a safe and an effective technique for congenital chylothorax.

Prenatal findings and epimutations for paternal uniparental disomy for chromosome 14 syndrome

The phenotypes associated with paternal uniparental disomy for chromosome 14 (UPD(14)pat) are clinically distinctive and caused by genetic alterations at the 14q32.2 imprinted region. Here we describe prenatal and neonatal findings in a case of epimutation associated with UPD(14)pat‐like phenotype. A 25‐year‐old Japanese woman was referred to hospital at 32 weeks of gestation for management of threatened premature delivery. Fetal ultrasound and magnetic resonance imaging showed a narrow thorax and polyhydramnios. At 35 weeks of gestation, emergency cesarean section was performed and placentomegaly was identified. Physical examination of the neonate indicated a small narrow thorax, diastasis recti, and dysmorphic facial features that included hirsute forehead, broad flat nasal bridge, micrognathia, small ears, and a long protruding philtrum. Genetic analysis identified epimutation at the intergenic differentially methylated region (IG‐DMR) and at MEG3‐DMR.

The Microbiome of the Lower Respiratory Tract in Premature Infants with and without Severe Bronchopulmonary Dysplasia.

Aim We determined whether the bacteria in the lower respiratory tract (LRT) in extremely premature infants with severe bronchopulmonary dysplasia (BPD) are different from those with nonsevere BPD. Study Design We conducted a retrospective study of extremely premature infants who were admitted to the neonatal intensive care unit of Fukushima Medical University Hospital, Japan between April 2005 and March 2014. We screened for the bacterial colonization of the LRT using tracheobronchial aspirate fluid. Results A total of 169 extremely premature infants were included. Overall, 102 did not experience severe BPD, whereas the remaining 67 experienced severe BPD. Corynebacterium species (Cs) were more frequently detected in the severe BPD than nonsevere BPD infants (p = 0.03). There were significant differences between infants with and without severe BPD in the duration of endotracheal ventilation (p = 0.00, odds ratio [OR], 1.03; 95% confidence interval [CI], 1.01‐1.06), the duration of supplemental oxygen (p = 0.00, OR, 1.02; 95% CI, 1.01‐1.03) before 36 weeks of postmenstrual age, and the frequency of sepsis after 7 postnatal days (p = 0.01, OR, 1.73; 95% CI, 1.18‐2.54). Conclusion Cs are more likely to be present in the severe BPD infants with longer duration of endotracheal ventilation.

An extremely low body weight infant born to a mother with measles

The incidence of measles epidemics has decreased recently owing to the development and widespread use of measles vaccine in the United States of America and Europe. However, repeated measles epidemics have been reported in Japan. Here, the authors report a case of an extremely low body weight infant (ELBWI) whose mother had a measles virus infection. Real-time PCR was performed on the infants blood and urine samples and skin, nasal secretion, and anal swabs, as well as on the mothers breast milk, blood samples and throat swabs. The infant was found to be positive for measles virus by PCR, but not immunoglobulin M positive. An earlier report showed that there were no such cases in which the patient was found to be positive for measles virus by real-time PCR but was not infected by the measles virus.

Glucocorticoid receptor expression and cortisol level in cord blood of term infants

Objective. To examine the expression levels of glucocorticoid receptor (GR) isoforms in peripheral blood mononuclear cells (PBMCs) and serum cortisol levels in cord blood from term infants. Methods. The study population consisted of 172 term infants who were delivered from healthy pregnant women. GRalpha and GRbeta expression levels, and serum cortisol level in cord blood were determined by real-time PCR and ELISA, respectively. Results. Detection rates of GRalpha, GRbeta, and GAPDH were 100%, 63.4%, and 100%, respectively. The expression level of GRalpha was about 200 times that of GRbeta. There were no associations between GR expression level and clinical variables. There were significant associations of low UmApH, maternal gravidity or parity, and vaginal delivery with a high cortisol level; however, there were no correlations between GR expression levels and cortisol level. Conclusions. It is considered that glucocorticoid effects could be expected from the fetal period to the neonatal period, because GRalpha expression level was not related to perinatal factors, GRbeta expression level, and cortisol level in term infants. Further studies of larger populations including very preterm and small for gestational age infants are necessary to determine the balance of expression between GRalpha and GRbeta, and cortisol level.

Rare case of isolated levocardia with polysplenia including normally structured lung without cardiac anomaly

Isolated levocardia (IL) is a rare type of situs inversus in which the heart is in the normal left‐side position, but the abdominal viscera are in the dextroposition. Polysplenia is a congenital disorder affecting the asymmetric organs, including the heart, lungs, bronchi, liver, stomach, intestines, and spleen. In this report, we present a rare type satisfying the characteristics of both IL and polysplenia, confirmed by several imaging investigations, which revealed normally structured lungs and heart and the existence of the inferior vena cava (IVC), which runs on the left side, returns to the anterior‐right side at the liver, and returns to the right atrium directly. Anatomical investigation and careful observation will make it possible to improve the prognosis of IL.

Continuous hemodialysis therapy for an extremely low‐birthweight infant with hyperammonemia

Hyperammonemia of newborns should be treated promptly, and the outcome depends on the rapid elimination of excessive plasma ammonia. We encountered a case of transient hyperammonemia in an extremely low‐birthweight infant whose plasma ammonia decreased sufficiently after continuous hemodialysis therapy. It seems that continuous hemodialysis therapy using the peripheral artery and umbilical vein is useful for hyperammonemia of extremely low‐birthweight infants; however, there are several problems to consider due to the immaturity of these infants.

Successful prospective management of neonatal citrullinemia.

Abstract Classical citrullinemia generally involves hyperammonemic coma in the first few days of life and leads to neurological sequelae in survivors. We report a case of an elder sister who fell into a hyperammonemic coma on the fifth day after birth. She was successfully treated with intravenous benzoate and hemodialysis, and was subsequently diagnosed with citrullinemia on the basis of biochemical analysis. Two years later, a younger sister was born without prenatal diagnosis. We monitored plasma ammonia and citrulline levels after birth, and again diagnosed her with CTLN1 on the basis of biochemical and DNA analyses. There have been few reports of the prospective treatment of citrullinemia; however, our experience indicates the need for the prospective management and the rapid reduction of ammonia levels to avoid neonatal hyperammonemic coma and subsequent sequelae.

Glucocorticoid receptor expression in whole blood with preterm infants.

Abstract Background: Although glucocorticoid hormones play important roles in fetal development, the expression of their receptors in the whole blood of preterm infants remains unknown. Objectives: The aim of this study was to investigate the levels of glucocorticoid receptor (GR) α and β in the whole blood of preterm and term infants. Study design: The study group consisted of 131 infants, of which 54 (41%) were preterm. Whole blood from preterm and term infants was analyzed by real-time PCR to monitor the levels of each receptor mRNA. Results: GRβ mRNA were detected in 96.6% and GRα mRNA in 100% of participants. The GRα and GRβ isoforms were detected at a ratio of 1:0.0002. GRβ mRNA/GAPDH expression in preterm infants was significantly higher than that in term infants (p=0.002). There was significant correlation between GRα/GRβ ratio and birth weight in preterm infants (rs=0.317, p=0.019), as well as between GRβ/GAPDH expression and birth weight (rs=–0.296, p=0.030). Furthermore, in preterm infants, GRβ/GAPDH expression was higher in those with SGA than in those without SGA (p=0.022). Conclusion: Importantly, in preterm infants, both the expression of GRβ and the GRα/GRβ ratio were associated with birth weight. Further studies with larger populations are necessary to determine the relation between the expression of GR and the clinical relevance of preterm infants.