We investigated the incidence of bronchopulmonary dysplasia (BPD) in very-low-birth-weight (VLBW) infants in Korea using the Korean Neonatal Network (KNN) data. In total, 2,386 VLBW infants born from January 2013 to June 2014 were prospectively registered. BPD was defined as supplemental oxygen or positive pressure support at 36 weeks postmenstrual age (PMA). The overall incidence of BPD was 28.9%, and the overall mortality rate in the neonatal intensive care units (NICUs) was 11.9%. To investigate recent changes in the incidence of BPD among VLBW infants, we compared the BPD rate in the present study with the latest nationwide retrospective survey conducted between 2007 and 2008. For comparison, we selected infants (23-31 weeks of gestation) (n=1,990) to adjust for the same conditions with the previous survey in 2007-2008 (n=3,841). Among the limited data on VLBW infants (23-31 weeks of gestation), the incidence of BPD increased by 85% (from 17.8% to 33.0%) and the mortality rate in the NICU decreased by 31.4% (from 18.8% to 12.9%) compared to those in the study conducted in 2007-2008. The current trend of increase in the incidence of BPD among infants can be attributed to the increase in the survival rate of VLBW infants.

Background and ObjectivesThe prevalence of incomplete Kawasaki disease (iKD) is progressively increasing. We aimed to retrospectively investigate the predictors of intravenous immunoglobulin (IVIG) resistance in iKD patients and compare them with those of IVIG resistance in complete Kawasaki disease (cKD) patients. We also compared the prognosis of coronary artery lesions (CALs) between the IVIG non-responders and responders in both iKD and cKD groups.Subjects and MethodsA total of 234 cKD and 77 iKD patients were treated with IVIG between February 2009 and April 2012. Among these 311 patients, we reviewed the data of 77 iKD patients and 75 age-matched cKD patients.ResultsPatients with iKD having an elevated neutrophil count {percentage of segmented neutrophils (SEG%) ≥79.0} were at risk of IVIG resistance, while patients with cKD having SEG% ≥79.25 and serum total bilirubin (TB) ≥0.56 mg/dL were at risk of IVIG resistance as shown by multivariable logistic regression analysis. Fractional changes of laboratory data before and after IVIG treatment showed that Creactive protein (CRP) and N-terminal B type natriuretic peptide (NT-proBNP) levels were significantly elevated in IVIG non-responders of the iKD group, whereas erythrocyte sedimentation rate was significantly elevated in IVIG non-responders of the cKD group. Among the patients who had CALs at 10 months after the start of illness, the z scores of coronary arteries were higher in IVIG non-responders of the iKD group, when compared with IVIG non-responders of the cKD group.ConclusionElevated SEG%, changes in CRP and NT-proBNP levels may help in early detection of IVIG resistance in patients of the iKD group, which may aid in predicting the prognosis of CALs in these patients. Further studies with a larger number of patients are warranted.

Neonatal hypocalcemia and congenital heart defects has been known as the first clinical manifestation of the chromosome 22q11.2 deletion syndrome (22q11DS). However, because of its wide clinical spectrum, diagnosis of 22q11DS can be delayed in children without classic symptoms. We report the case of a girl with the history of imperforate anus but without neonatal hypocalcemia or major cardiac anomaly, who was diagnosed for 22q11DS at the age of 11 after the onset of overt hypocalcemia. She was born uneventfully from phenotypically normal Korean parents. Imperforate anus and partial cleft palate were found at birth, which were surgically repaired thereafter. There was no history of neonatal hypocalcemia, and karyotyping by GTG banding was normal. At the age of 11, hypocalcemia (serum calcium, 5.0 mg/dL) and decreased parathyroid hormone level (10.8 pg/mL) was noted when she visited our Emergency Department for fever and vomiting. The 22q11DS was suspected because of her mild mental retardation and velopharyngeal insufficiency, and a microdeletion on chromosome 22q11.2 was confirmed by fluorescence in situ hybridization. The 22q11DS should be considered in the differential diagnosis of hypocalcemia at any age because of its wide clinical spectrum.

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Most of the congenital diaphragmatic hernia (CDH) cases are diagnosed at prenatal period or immediately after birth with severe respiratory symptom. The classic triad, which is respiratory distress, apparent dextrocardia and a scaphoid abdomen, is usually seen in this period. Several case reports have described older infants and children with a wide spectrum of symptoms of CDH, whereas extremely few cases were reported in neonatal period except classic triad such as straungulation of the bowel. These atypical manifestations can lead physician to delayed diagnosis. We report two cases of CDH newborns. First case was diagnosed with pneumope ritoneum following tension pneumothorax, transient diaphragm eventration on 5 days after birth. The other case was diagnosed with failure to thrive and mediastinal mass on 30 days after birth. These cases suggest phy sicians to consider CDH in late newborn period with pneumoperitoneum following tension pneumothorax, transient diaphragm eventration, failure to thrive, and mediastinal mass.

Anterior fontanel (AF) sizes in newborns are influenced by ethnicity, gender, genetics, nutrition, and other pathological conditions. However, AF sizes in recent Korean newborns have not been reported yet. Thus, this study aimed to understand variation in AF size and clinical implications of large AF sizes in newborns. This cohort study was conducted on nursery newborns born at a University Hospital between September 2019 and August 2020. AF size was measured at 24 to 48 hours after birth. Newborns with a large AF (> 3.6cm) were examined for other pathological reasons using radiological and laboratory tests. Demographic data were analyzed in relation to AF size. A total of 573 newborns were investigated. Their mean gestational age was 38.5 ± 1.2 weeks at birth and their mean birth weight was 3140 ± 450 g. Their mean AF size was 1.85 ± 0.83 cm (90 and 97-percentile of AF size were 2.96 and 3.65 cm, respectively). Among demographic factors, small for gestational age (SGA) weight, SGA head circumference, and multiple births were correlated with larger AF size. Among 18 infants with a large AF, 2 had intracranial abnormalities and 11 had vitamin D deficiency. This is the first recent study on AF sizes of Korean newborn infants. Their mean AF size was the smallest so far. For the largest AF size, a cutoff of 3.65 cm met the 97-percentile of the cohort. Both SGA weight and head circumference were risk factors for large AF size. For the largest AF size, intracranial lesions and vitamin D deficiency as well as SGA presented clinical implications.

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Objective: Intrauterine growth retardation of lung has been suggested as being one of the risk factors in the development of bronchopulmonary dysplasia (BPD). The aim of this study was to clarify birth weight percentile among Korean infants using the contemporary Korean reference curve for birth weight by gestational age published in 2014, and on the basis of that clarification, identify the association between birth weight percentile and BPD development. Methods: All newborn infants born at <32 weeks of gestation admitted to the neonatal intensive care unit of CHA Bundang Medical Center from January 2010 through December 2015 were review ed. Infants were divided into BPD group and non-BPD group. BPD was defined as a need of oxygen or positive airway pressure at 36 weeks postmenstrual age. All prenatal and neonatal data were collected via retrospective chart review and analyzed by multivariate logistic regression analysis. Results: During the study period, 198 singleton infants were born at <32 weeks' gestation. Among these, 179 infants were included, 54 infants in BPD group and 125 infants in non-BPD group. According to logistic regression analysis, lower gestational age, lower birth weight percentile, occurrence of late onset sepsis showed increased risk of BPD (odds ratio [OR] 0.77, P=0.027; OR 0.98, P=0.021; OR 7.98, P<0.0001). Conclusion: In this single center study, lower birth weight percentile in preterm infants seems to increase the risk of BPD. In these infants, anticipate the high risk of BPD and cautious treatment strategies are needed.