Rare type of tracheal agenesis: Unexpected presentation and immediate consideration of emergent esophageal intubation in neonatal resuscitation program. Case reports and review of the literature.

BACKGROUND: Tracheal agenesis, or tracheal atresia, is a rare congenital anomaly. The presence of a tracheoesophageal fistula (TEF) can help with breathing for newborns with tracheal agenesis. In this article, we presented three unique cases and outcomes of neonates with tracheal agenesis along with a review of the literature. METHODS: This study consisted of a single center case series followed by a review of literature. Case reports were generated using both written and electronic medical records from a single hospital. We summarized three unique cases and outcomes of neonates with tracheal agenesis and performed a review of the literature. RESULTS: We identified three cases of tracheal agenesis presented with severe cyanosis without spontaneous crying upon birth. Experienced pediatricians attempted to intubate the babies but were unsuccessful. Endotracheal tubes were subsequently either accidentally or purposely placed into the esophagus, and oxygen saturation levels improved. This suggested tracheal agenesis with TEF. Two cases underwent surgical intervention after resuscitation with esophageal intubation. CONCLUSION: Esophageal intubation may be a life-sustaining ventilation support for patients with tracheal agenesis and TEF at initial resuscitation. Clinicians should suspect tracheal agenesis when a newborn presents with severe cyanosis and voiceless crying upon birth, and esophageal intubation should be immediately attempted.

Deciduoid mesothelioma of the pleura in an adolescent boy.

Deciduoid mesothelioma is an extremely rare variant of malignant epithelioid mesothelioma. This report presents the case of a 13-year-old boy with this type of tumor in his pleura, whose initial main symptoms were chest pains and progressive scoliosis. Ensuing chemotherapy, which comprised pemetrexed and cisplatin, yielded good response after 5 cycles. Subsequent radical surgery was carried out and another 3 cycles of chemotherapy were given. The patient has been doing well 2 years after completion of this regime of treatment.

Impact of urine osmolality/urine sodium on the timing of diuretic phase and non-invasive ventilation support: Differences from late preterm to term neonates.

BACKGROUND: Extracellular fluid retained in the lungs lead to respiratory distress in both late preterm (LP) and term neonates. The relationship between urine parameters toward the diuresis and the duration of ventilation postnatally is unknown. To find the correlation between the diuretic phase with urine parameters in the first 4 days after birth and the duration of non-invasive ventilation (NIV). METHODS: Serial measurements of urine osmolality (Uosm), urine sodium (UNa), and urine output (U/O) in neonates were collected at 5 time periods (T1:0-12 postnatal hours, T2:12-24 postnatal hours, T3:24-48 postnatal hours, T4:48-72 postnatal hours, T5:72-96 postnatal hours) were recorded. The correlations were analyzed in late preterm and term neonates. RESULTS: Ninety-seven neonates were included. Negative correlation between Uosm and U/O were observed. LP neonates (n=26) and term neonates (n=71) had differences with Uosm at T2, UNa at T4, T5, and U/O at T2, T3. Factors of U/O < 1 ml/kg/hr at T1 (odds ratio (OR) = 20.0; 95% confidence interval (CI) 1.796-222.776; p = 0.015) or Uosm > 273 mOsm/L at T1 (OR = 9.0; 95% CI 1.031-78.574; p = 0.047) in LP neonates and UNa > 26.5 mEq/L at T5 (OR = 23.625; 95% CI 2.683-79.276; p < 0.01) in term neonates were associated with prolonged NIV use (> 120 hours). CONCLUSION: We speculate the significant correlation between Uosm/UNa and the diuretic phase. The LP neonates acquire earlier diuretic phase than the term neonates. The Uosm/UNa in the first few postnatal days had the correlation with the duration of NIV support.

Iron Status of Infants in the First Year of Life in Northern Taiwan.

Iron deficiency (ID) and iron deficiency anemia (IDA) typically occur in developing countries. Notably, ID and IDA can affect an infant's emotion, cognition, and development. Breast milk is considered the best food for infants. However, recent studies have indicated that breastfeeding for more than six months increases the risk of ID. This study investigated the prevalence of ID and IDA, as well as the association between feeding type and iron nutritional status in northern Taiwan. A cross-sectional study was conducted on infants who returned to the well-baby clinic for routine examination from October 2012 to January 2014. Overall, 509 infants aged 1-12 months completed the iron nutritional status analysis, anthropometric measurement, and dietary intake assessment, including milk and complementary foods. The results revealed that 49 (10%) and 21 (4%) infants in their first year of life had ID and IDA, respectively, based on the World Health Organization criteria. Breastfed infants had a higher prevalence rate of ID and IDA than mixed-fed and formula-fed infants (p < 0.001). Regarding biomarkers of iron status, plasma hemoglobin (Hb), ferritin, and transferrin saturation (%) levels were significantly lower in ID and IDA groups. The prevalence of ID and IDA were 3.7% and 2.7%, respectively, in infants under six months of age, but increased to 20.4% and 6.6%, respectively, in infants above six months of age. The healthy group had a higher total iron intake than ID and IDA groups, mainly derived from infant formula. The total dietary iron intake was positively correlated with infants' Hb levels. Compared with formula-fed infants, the logistic regression revealed that the odds ratio for ID was 2.157 (95% confidence interval [CI]: 1.369-3.399) and that for IDA was 4.196 (95% CI: 1.780-9.887) among breastfed infants (p < 0.001) after adjusted for all confounding factors (including gestational week, birthweight, sex, body weight percentile, body length percentile, age of infants, mothers' BMI, gestational weight gain, education level, and hemoglobin level before delivery). In conclusion, our results determined that breastfeeding was associated with an increased the prevalence of ID and/or IDA, especially in infants above six months. This suggests that mothers who prolonged breastfeed after six months could provide high-quality iron-rich foods to reduce the prevalence of ID and IDA.

Infants' Vitamin D Nutritional Status in the First Year of Life in Northern Taiwan.

Vitamin D deficiency (VDD) and insufficiency (VDI) are common among exclusively breastfeeding infants. However, epidemiological evidence for the prevalence of VDD in infants during their first year of life in Taiwan has never been found. This trial determined the prevalence of VDD and VDI and the association between dietary vitamin D and vitamin D nutritional status in Northern Taiwan. A cross-sectional study was conducted on infants who returned to well-baby examinations from October 2012 to January 2014 in three hospitals: Shin Kong Wu Ho-Su Memorial Hospital, Taipei Medical University Hospital, and Shuang Ho Hospital. The specific vitamin D cut-off concentrations for VDD, VDI, and VDS are 25(OH)D3 levels ≤ 20, 21-29, and ≥ 30 (ng/mL). Overall, 481 infants' parents completed a questionnaire comprising questions related to vitamin D nutritional status, including weekly time outdoors, breastfeeding status, anthropometric measurement, and assessment of dietary intake, including milk and complementary food. The results revealed that 197 (41%) and 212 (44%) of infants in their first year of life had VDI and VDD, respectively, by the Endocrine Society guidelines. Breastfed infants had a higher prevalence of VDI (86.1%) than did mixed-fed (51.9%) and formula-fed (38.5%) infants (p < 0.001). The prevalence of VDD was 55.4% in infants aged under six months but increased to 61.6% in infants aged over six months. Infants in the VDI and VDD groups had the same anthropometrics as those in the vitamin D sufficiency (VDS) group. Our results revealed that 25(OH)D3 had a negative correlation with the intact parathyroid hormone (iPTH) when the serum 25(OH)D3 level ≤ 20 ng/mL (r = -0.21, p = 0.001). The VDS group had a higher total vitamin D intake than did the VDI and VDD groups, which was mainly obtained from infant formula. Our data revealed that dietary vitamin D intake and birth season were major indicators in predicting VDD. Lower dietary vitamin D intake and born in winter and spring significantly increased the odds ratio (OR) for VDI by 1.15 (95% CI 1.09-1.20) and 2.02 (95% CI 1.10-3.70), respectively, and that for VDD by 1.23 (95% CI 1.16-1.31) and 2.37 (95% CI 1.35-4.17) without covariates adjustment, respectively. Furthermore, ORs for VDI and VDD significantly differed after adjustment for covariates. In conclusion, the prevalence of VDI and VDD were high in infants during the first year of life. Breastfeeding infants had difficulty in obtaining sufficient vitamin D from diet. In cases where the amount of sun exposure that is safe and sufficient to improve vitamin D status is unclear, breastfed infants aged below one year old are recommended to be supplemented with vitamin D.

The Role of Heated Humidified High-flow Nasal Cannula as Noninvasive Respiratory Support in Neonates.

Recently, heated humidified high-flow nasal cannula (HHHFNC) has been introduced and applied as a noninvasive respiratory support in neonates. Although HHHFNC is widely used in neonates presenting with respiratory distress, the efficiency and safety when compared with nasal continuous positive airway pressure or noninvasive positive pressure ventilation are still controversial. This review aims to evaluate the performance and applications of HHHFNC in neonates.

Prolonged Hyperbilirubinemia in a Neonate with a Novel Mutation in the UDP-glucuronosyltransferase 1A1 Gene.

The total bilirubin value of a male infant was 385 µmol/l on day 5. Liver function test results were normal and there was no evidence of sepsis and no hemolysis reaction. Phototherapy was administered and on day 8 the patient's total bilirubin level was 255 µmol/l. Intermittent episodes of hyperbilirubinemia occurred without phototherapy, with the total bilirubin level reaching 335 µmol/l on day 19. A 3-day regimen of phenobarbital was administered and on day 24 his total bilirubin level was 180 µmol/l. The patient was discharged. At the age of 2 months, the total bilirubin value was 27 µmol/l. His direct bilirubin value was <15% of total bilirubin in every determination. A family study of the UDP-glucuronosyltransferase(UGT)1A1 gene showed that the infant carries a homozygous mutation at nucleotide -3279 plus compound heterozygous mutations at nucleotides 782 and 1091. The mutation at nucleotide 782 is a novel finding. Gilbert's syndrome was diagnosed.

The impact of small-for-gestational-age on neonatal outcome among very-low-birth-weight infants.

BACKGROUND: This study aimed to evaluate the impact of small-for-gestational-age (SGA) on mortality and morbidity in very-low-birth-weight (VLBW) infants. METHODS: We conducted a retrospective cohort study on VLBW infants registered at the Premature Baby Foundation of Taiwan between 2007 and 2011. All 21 neonatal departments in Taiwan participated in the data collection, and a total of 4636 VLBW infants were registered during the study period. The SGA group (n = 560) was selected from the database on the basis of birth weight below the 10(th) percentile for gestational age, whereas the appropriate-weight-for-gestational-age (AGA) group (n = 1120) included infants randomly selected via incidence density sampling with a 2:1 match for each SGA case. The association of SGA with individual outcome variables including mortality, respiratory distress syndrome, necrotizing enterocolitis, retinopathy of prematurity (ROP), intraventricular hemorrhage, periventricular leukomalacia, and bronchopulmonary dysplasia (BPD) was evaluated after adjustment for potential confounders. RESULTS: The SGA group was associated with increased risks of mortality [odds ratio (OR) 1.89; 95% confidence interval (CI) 1.39‒2.58], severe ROP (OR 1.56; 95% CI 1.13-2.14), and BPD (OR 2.08; 95% CI 1.58-2.75) compared to the AGA group. Further subgroup analysis showed that SGA had significant effects on mortality in the VLBW infants with a gestational age of 24-29 weeks, as well as on BPD in those with a gestational age of 27-32 weeks. By contrast, the association of SGA with severe ROP was only significant in the VLBW infants with a gestational age of 27-29 weeks. CONCLUSION: Our data provide evidence that SGA may be associated with increased risks of neonatal mortality, ROP, and BPD in VLBW infants.

Parental smoking during pregnancy and its association with low birth weight, small for gestational age, and preterm birth offspring: a birth cohort study.

BACKGROUND: Intrauterine exposure to tobacco smoke has been discerned as an important risk factor for low birth weight (LBW), small for gestational age (SGA), and preterm birth infants. In this cohort study, we investigated the association of the amount of parental smoking during the different pregnancy stages with birth weight and the incidence of preterm delivery. METHODS: Our study population was acquired from the Taiwan Birth Cohort Study. Between June 2005 and July 2006, 21,248 postpartum women were interviewed 6 months after their deliveries by a structured questionnaire. The parents were divided into four groups according to the amount of smoking during preconception, the first trimester, and the second and third trimesters. The relationships of parental smoking with gestational age and birth weight during the different pregnancy stages were assessed using multivariate linear regression. Multiple logistic regression analyses were performed to estimate the odds ratios and 95% confidence intervals of preterm delivery, LBW, and SGA infants during the different parental smoking status and the different pregnancy stages. RESULTS: After adjusting for the physical and socioeconomic status of the parents and for paternal smoking during the same period, we found that maternal smoking decreased birth weight. Compared with the nonsmoking groups, all the maternal smoking groups had higher incidences of LBW, SGA, and preterm birth infants, especially when the mothers smoked >20 cigarettes/day. The association of paternal smoking with LBW, SGA, and preterm birth infants was insignificant. CONCLUSION: Maternal smoking is responsible for increased incidences of LBW and preterm delivery of babies, and therefore, smoking cessation/reduction should be advised to pregnant women to reduce morbidities in their neonates. Further studies are needed to clarify the correlation of fetal health with passive smoking, including exposure to environmental tobacco smoke and to other smokers in the family.

Distinct clinical characteristics of patients with congenital duodenal obstruction in a medical center in Taiwan.

BACKGROUND: Patients with congenital duodenal obstruction (CDO) predominantly have a postampullary lesion site and a high association with Down syndrome, according to previous reports from Western countries. This study aimed to determine whether there are racial differences in the clinical characteristics of congenital duodenal obstruction, based on the experience from a Taiwanese medical center. METHODS: The charts were retrospectively reviewed among neonates with CDO who received surgery at National Taiwan University Hospital during the period 1985- 2008. Patients were grouped into a preampullary or postampullary group according to the obstruction in relation to the ampulla of Vater. Other characteristics, including sex, time of diagnosis, operative findings, as well as associated anomalies, were recorded for further analysis. RESULTS: A total of 30 patients with CDO including 16 with atresia, 10 with mucosal webs, and 4 with stenosis, were recruited; among them, 16 were boys and 14 were girls. In 15 patients (50%), the diagnosis of duodenal obstruction was made prenatally. A total of 11 of the 30 patients (37%) were in the preampullary group and 19 (63%) were in the postampullary group. Seventeen patients (56.7%) had at least one additional anomaly, including four (13%) who had trisomy 21. The preampullary group had significantly fewer associated congenital anomalies than in the postampullary group (27%vs. 74%, p = 0.012). CONCLUSION: Our cohort showed a relatively lower incidence of postampullary lesions and associated Down syndrome in patients with CDO compared with Western countries. Additionally, patients with preampullary lesions had significantly less association with other anomalies.

Neonatal listeriosis in Taiwan, 1990-2007.

OBJECTIVES: Listeria monocytogenes is an important pathogen in neonates in Western countries, with a fatality rate of 20-30%. There is limited information on neonatal listeriosis in Eastern countries. The purpose of this study was to delineate the occurrence and clinical picture of neonatal listeriosis in Taiwan. METHODS: A questionnaire-based survey of all of the 17 medical centers in Taiwan was performed, and a literature review of neonatal listeriosis as reported in Taiwan from 1990 to 2007 was made. RESULTS: A total of 14 cases (10 male, four female) of neonatal listeriosis were identified, including 11 found from the survey of four medical centers and another three collected from the literature review. Three were found to have occurred prior to 2000 and 11 were found to have occurred after 2000. The age of onset was less than 3 days in all cases. L. monocytogenes was identified from blood in 13, cerebrospinal fluid in four, and gastric aspirate in two. Half of the cases (7/14) had involvement of the central nervous system with pleocytosis and hypoglycorrhachia in cerebrospinal fluid, and three of them even developed hydrocephalus. The mortality rate was 29%. CONCLUSIONS: Our findings suggest that listeriosis may emerge as an important health threat among newborn infants in Taiwan.