Extracorporeal membrane oxygenation for neonatal congenital diaphragmatic hernia: The initial single-center experience in Taiwan

Background/Purpose Extracorporeal membrane oxygenation (ECMO) is a treatment option for stabilizing neonates with congenital diaphragmatic hernia (CDH) in a critical condition when standard therapy fails. However, the use of this approach in Taiwan has not been previously reported. Methods The charts of all neonates with CDH treated in our institute during the period 2007–2014 were reviewed. After 2010, patients who could not be stabilized with conventional treatment were candidates for ECMO. We compared the demographic data of patients with and without ECMO support. The clinical course and complications of ECMO were also reviewed. Results We identified 39 neonates with CDH with a median birth weight of 2696 g (range, 1526–3280 g). Seven (18%) of these patients required ECMO support. The APGAR score at 5 minutes differed significantly between the ECMO and non-ECMO groups. The survival rate was 84.6% (33/39) for all CDH patients and 57.1% (4/7) for the ECMO group. The total ECMO bypass times in the survivors was in the range of 5–36 days, whereas all nonsurvivors received ECMO for at least 36 days (mean duration, 68 days). Surgical bleeding occurred in four of seven patients in the ECMO group. Conclusion The introduction of ECMO rescued some CDH patients who could not have survived by conventional management. Prolonged (i.e., > 36 days) ECMO support had no benefit for survival

Newborn Genetic Screening for Hearing Impairment: A Preliminary Study at a Tertiary Center

Universal newborn hearing screening (UNHS) is of paramount importance for early identification and management of hearing impairment in children. However, infants with slight/mild, progressive, or late-onset hearing impairment might be missed in conventional UNHS. To investigate whether genetic screening for common deafness-associated mutations could assist in identifying these infants, 1017 consecutive newborns in a tertiary hospital were subjected to both newborn hearing screening using a two-step distortion-product otoacoustic emissions (DPOAE) screening and newborn genetic screening (NGS) for deafness. The NGS targeted 4 deafness-associated mutations commonly found in the Taiwanese population, including p.V37I (c.109G>A) and c.235delC of the GJB2 gene, c.919-2A>G of the SLC26A4 gene, and mitochondrial m.1555A>G of the 12S rRNA gene. The results of the NGS were then correlated to the results of the NHS. Of the 1017 newborns, 16 (1.6%) had unilateral DPOAE screening failure, and 22 (2.2%) had bilateral DPOAE screening failure. A total of 199 (19.6%) babies were found to have at least 1 mutated allele on the NGS for deafness, 11 (1.1%) of whom were homozygous for GJB2 p.V37I, 6 (0.6%) compound heterozygous for GJB2 p.V37I and c.235delC, and 1 (0.1%) homoplasmic for m.1555A>G, who may potentially have hearing loss. Among them, 3 babies, 5 babies, and 1 baby, respectively, passed the NHS at birth. Comprehensive audiological assessments in the 9 babies at 3 months identified 1 with slight hearing loss and 2 with mild hearing loss. NGS for common deafness-associated mutations may identify infants with slight/mild or potentially progressive hearing impairment, thus compensating for the inherent limitations of the conventional UNHS

促進血管生成因子及其接受器在出生後小鼠發育中肺臟的表現

Background and Purpose: Several lines of evidence suggest that angiogenesis is necessary for alveolarization and that inhibition of vascular growth during a critical period of early growth may impair alveolarization. However, little is known about the role of angiogeuic factors during alveolarization. This study investigated the expression patterns of the Ang-Tie-2 family of endothelium-specific receptor tyrosine kinases and vascular endothelial growth factors and their receptors (VEGF -VE, GFR system) during postnatal mouse lung development. Methods: The lungs from 3 or 4 mice from groups aged 3, 7, 10 and 14 days and adults were removed and dissected from the main bronchi for reverse transcriptase -polymerase chain reaction (RT-PCR) analysis. Semi-quantitative RT-PCR of mouse lung total RNA was used to measure the expression levels of these angiogenic factors and their receptors. Results: During alveolarization, NTEGF/ Flk-1, Ang-1, Ang-2, and Tie-2 were up-regulated and PlGF/ Flt-1 was kept at a relatively constant level. After alveolarization was completed, PIGF was down-regulated, Flt- 1 was up-regulated, and VEGF/Flk- 1, Ang-1 Ang-2, and Tie-2 were maintained at relatively high levels. Conclusions: During alveolarization, in mice, VEGF/Flk-1, Ang-1, Ang-2, and Tie-2 are up-regulated and PlGF/Flt-1 is kept at relatively constant level to promote pulmonary microvascular development. In the adult mouse lung, when most of the vascular network is complete, PlGF is down- regulated and Flt -1 is up-regulated to stop angiogenesis and VEGF/Flk-1, Ang- 1, Ang-2 and Tie-2 are kept at relatively high levels to maintain mature pulmonary microvasculature

Longitudinal Follow up of Lymphocyte Subsets during the First Year of Life

A longitudinal study of lymphocyte subsets during infancy was evaluated by using the flow cytometric immunophenotyping method. Two hundred and thirteen blood samples were obtained from 92 healthy, full-term infants of the following ages: 1-7 days old (n = 43), 3 months old (n = 55), 6 months old (n = 57) and 11 months old (n = 58). The absolute numbers of CD 3(+) and CD3(+)/CD4(+) T lymphocytes increased from birth to 3 months of age, and remained stable thereafter. The absolute number of CD3(+)/CD8(+) T lymphocytes increased from birth to 11 months of age. The absolute number of CD19(+) B lymphocytes and NK cells increased rapidly (3 months) after birth and continued to increase throughout the study period. However , the changes in the relative counts of lymphocyte subsets did not always correspond with the changes in their absolute numbers. These results demonstrate the age-related changes in lymphocyte subpopulations and provide reference ranges for lymphocyte subsets during infancy

Severe Echovirus 30 Infection in Twin Neonates

Althoughenteroviruses can cause overwhelmong and fatal systematic infections in neonates, such severe neonatal infections remain uncommon and rarely involve both of twin neonates at the same time. We report the cases of twin neonates who developed fever initially, and then progressed to disseminated systematic disease with marked thrombocytopenia, coagulopathy, and hepatic failure. One of the neonates died and the other survived. Both neonates were treated with intravenous immunoglobulin and material fresh frozen plasma was also given to the neonate who survived. Virus cultures from the nasopharynx, rectum and cerebral spinal fluid of both neonates yielded enterovirus, later typed as echovirus 30. The surviving noenate had normal development without obvious sequelae during a follow-up period of 1 year.The major determinant of the survival from severe neonatal enterovirus infection might have been the pre-existing severity of the disease before treatment, and complete recovery could be expected if the infant survived the acute stage of illness

The Thrombopoietin Level in the Cord Blood in Premature Infants Born to Mothers with Pregnancy-Induced Hypertension

Objectives: To investigate the level of thrombopoietin in the cord blood of preterm infants, and its relationship with neonatal platelet count and pregnancy-induced hypertension. Study Method: Thrombopoietin levels in the cord blood of preterm neonates, with or without maternal pregnancy- induced hypertension, were measured by enzmye-linked immunosorbent assay. Results: The platelet count was significantly lower in very low birth weight infants, infants with maternal pregnancy-induced hypertension, and infants with maternal thrombocytopenia. Neonatal thrombocytopenia was associated with maternal pregnancy-induced hypertension and very low birth weight. The neonatal platelet count was correlated significantly with the birth weight and the maternal platelet count. There was no difference in the cord blood level of thrombopoietin between infants born to mothers with pregnancy-induced hypertension and those without. No correlation was found between the thrombopoietin level and the neonatal platelet count. A positive correlation between the cord blood thrombopoietin and the maternal platelet count was identified. Conclusion: Maternal pregnancy- induced hypertension and very low birth weight were significantly associated with thrombocytopenia in premature infants, which cannot be explained by decreased thrombopoietin level. Copyright (C) 2002 S. Karger AG, Basel