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

The Morbidity and Survival of Very-Low-Birth-Weight Infants in Taiwa

Advances in obstetrical and neonatal care have increased the survival of very-low-birth-weight (VLBW) infants, defined as infants weighing < or = 1 ,500 g at birth, in many populations. To understand the morbidity and survival of VLBW infants in Taiwan, the records of all VLBW admitted to the 12 hospitals with a level II+ or level III neonatal intensive care unit (NICU), at < 7 days of age, from January 1 to December 31, 1996, were collected prospectively. A total of 613 VLBW infants (292 males and 301 females) met the enrollment criteria: 305 cases from the northern region, 181 cases from the central region, and 127 cases from the southern region of Taiwan. The mean birth weight was 1,133 g (range, 368-1,500); the mean gestational age (GA) was 28.9 weeks (range, 21-38). Among the VLBW infants , 25.8% were small-for-gestational-age, 90.2% were born to mothers with high-risk factor(s) for preterm delivery, 55% were born by cesarean section, and 68.1% required resuscitation at birth. The percentage of prenatal use of steroids was 52.9%, and < 20% received more than one dose of antenatal steroids. Thirty-three percent were born after antenatal maternal transfer, and the neonatal transfer rate was 23%. The most common neonatal complication was apnea of prematurity (66.1% ), followed by respiratory distress syndrome (RDS) (60%). Chronic lung disease occurred in 76 cases (16.5%). The overall survival rate of the 613 VLBW infants was 76.2%; for infants weighing < or = 1000 g at birth, it was 49.2%, and for infants weighing 1,001-1,500 g at birth, it was 88.5%. The survival rate for infants with a GA < or = 26 weeks was 35.3%, and for infants with a GA of 27-36 weeks was 87.5%. No infant with a birth weight < or = 600 g or a GA < 23 weeks survived. The most common cause of death was sepsis, followed by extreme prematurity (GA < or = 23 wks) and RDS. Several perinatal and neonatal factors were related to the mortality. Multiple regression analysis of survival showed that GA < or = 26 weeks, birth weight < or = 800 g, delivery room resuscitation and the occurrence of pneumothorax were related to mortality. Therefore, although the survival rate of VLBW infants admitted to level II(+)-III NICUs showed an improvement over the rate for the previous 20 years in Taiwan, perinatal and neonatal care of extremely preterm infants and neonatal resuscitation programs need to be emphasized to improve the outcome of VLBW infants furthermore

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