Plasma B-type natriuretic peptide (BNP) is a useful marker for diagnosis of hemodynamically significant pDA (hspDA) and serial Bnp measurement is also valuable for monitoring treatment response. this retrospective study was performed to evaluate whether plasma Bnp level can predict treatment response to ibuprofen in preterm infants born at <30 weeks of gestation with hsPDA. Plasma BNP was measured before (baseline) and 12 to 24 h after (post-treatment) completion of the first (IBU1) and second (IBU2) course of ibuprofen. We compared the BNP levels of responders (closed or insignificant PDA) with those of non-responders (hspDA requiring further pharmacologic or surgical closure) to each course of ibuprofen. The treatment response rates for IBU1 (n = 92) and IBU2 (n = 19) were 74% and 26%, respectively. In IBU1, non-responders had lower gestational age and birth weight than responders (both, P = 0.004), while in IBU2, non-responders had lower birth weight (P = 0.014) and platelet counts (P = 0.005) than responders; however, baseline BNP levels did not differ significantly between responders and non-responders in either IBU1 (median 1,434 vs. 1,750 pg/mL) or IBU2 (415 vs. 596 pg/mL). Post-treatment BNP was a useful marker for monitoring treatment efficacy of IBU1 and IBU2 for hsPDA with a cutoff value of 331 pg/mL (P < 0.001) and 423 pg/mL(P < 0.010), respectively. We did not identify a cutoff baseline BNP level that could predict treatment response to ibuprofen in preterm infants with hspDA.
The purpose of this study was to investigate the impact of surgical interventions on the survival rate of the trisomy 18 and to provide the clinical information of the developmental status of the neonatal intensive care unit (NICU) survivors. Methods: We retrospectively reviewed the medical records of the patients who were confirmed genetically as trisomy 18 in a tertiary hospital. Patients were divided into those who were treated with active surgical interventions and those who were not (the control group). Data on mortality and longterm developmental outcomes were obtained and compared between the study groups. Results: A total of 24 patients were included. All of them had major congenital anomalies with a 100% prevalence of congenital heart diseases. The overall survival rate was 75%, and 35% at one month, and one year of age, and the median survival time was 170 days. The one-year survival rate of the group of active surgical intervention (n=14) was 56% with a median survival of 398 days. Meanwhile, none in the control group (n=7) survived for more than three months. The NICU survivors were unable to stand up even after two years of age. Their cognitive and social developmental stages generally consistent with those under six months of age. Conclusions: Despite the potential survival benefits of active surgical intervention, NICU survivors with trisomy 18 had severe developmental delays. To guide early decision-making for newborns with trisomy 18, further research is needed regarding the quality of life of trisomy 18 survivors and their families.
Smith-Lemli-Opitz syndrome (SLOS) is a rare autosomal recessive disorder caused by 7-dehydrocholesterol reductase deficiency. The characteristic clinical features are syndactyly of the second and third toes, facial dysmorphism, multiple malformations, and intellectual disability. Few cases of SLOS have been reported in Korea. We observed a male patient with SLOS who presented with typical facial features, undescended testes, microcephaly, bilateral syndactyly of the second and third toes, and cardiac defects, including patent ductus arteriosus and atrial septal defect. Mutation analysis of the DHCR7 gene identified compound heterozygous mutations of c.907G>A (p.Gly303Arg) and c.1055G>A (p.Arg352Gln). In a review of the literature, c.1054C>T (p.Arg352Trp) was the most common mutation reported in Far East Asian countries. This report describes the clinical features, biochemical data, molecular characteristics, and clinical outcome of a Korean patient with SLOS.
Purpose: To determine the efficacy of inhaled nitric oxide (iNO) in very low birth weight (VLBW) infants with early pulmonary hypertension (PH). Methods: We reviewed the medical records of 22 preterm infants who were born <30 weeks of gestational age with birth weight <1,500 g, diagnosed with early PH, and treated with iNO within the first 72 hours after birth. Responders were defined by a reduction in FiO 2 >20% and/or oxygenation index (OI) >20% from the baseline values at 1 hour after beginning iNO therapy. Cardiorespiratory support indices including OI, oxygen saturation index, and vasoactive-inotropic score (VIS) were serially obtained for 96 hours following iNO therapy. Results: The mean gestational age of the patients was 26.1±2.0 weeks and the mean birth weight was 842±298 g. The mean OI at the start of iNO was 63.8±61.0. Improvement in oxygenation indicated by prompt decrease in FiO 2 and OI from the baseline values were observed 1 hour after beginning iNO therapy and lasted up to 96 hours. After iNO therapy, VIS increased until 24 hours and decreased thereafter. At 1 hour after iNO, 16 patients (73%) were classified as responders and six (27%) as nonresponders. Compared with nonresponders, responders did not demonstrate the beneficial effect of iNO in terms of short-term survival and neonatal complications. The 1-year mortality rate did not differ between responders (56%) and nonresponders (67%). Conclusion: Although iNO treatment immediately improved oxygenation in most VLBW infants with early severe PH, the long-term mortality rate was high. A largescale study is needed to determine whether the initial response to iNO can predict patients' survival.
The primary aim of this study was to investigate the compositional differences of the first passed meconium microbiome in preterm and term infants, and the secondary aim was to compare the meconium microbiomes of preterm and term infants that later developed necrotizing enterocolitis (NEC)/Feeding intolerance (FI) compared to those that did not develop NEC/FI. During the study period, a total of 108 preterm and term newborns' first passed meconium occurring within 72 hours of birth were collected and microbiome analyzed. Meconium microbiomes showed a disruption in the percentages of the core microbiome constituents in both the phylum and genus levels in infants born < 30 weeks of gestational age (GA) compared to those born ≥ 30 weeks of GA. In the phylum level, Bacteroidetes and Firmicutes, and in the genus level, Prevotella and Bacteroides, were predominant, with Prevotella accounting for 20–30% of the relative abundance. As GA increased, a significant increase in the relative abundance of Bacteroidetes (P for trend < 0.001) and decrease in Proteobacteria (P for trend = 0.049) was observed in the phylum level. In the genus level, as GA increased, Prevotella (P for trend < 0.001) and Bacteroides (P for trend = 0.002) increased significantly, whereas Enterococcus (P for trend = 0.020) decreased. Compared to the control group, the meconium of infants that later developed NEC/FI had significantly lower alpha diversities but similar beta-diversities. Furthermore, the NEC/FI group showed a significantly lower abundance of Bacteroidetes (P < 0.001), and higher abundance of Firmicutes (P = 0.034). To conclude, differences were observed in the composition of the first passed meconium in preterm and term infants that later develop NEC/FI compared to those that did not.
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