Targeting Risk Factors for the Control of Central Line-Associated Bloodstream Infection in the Neonatal Intensive Care Unit: A Single Tertiary Center Experience

Purpose@#The aim of this study was to estimate the effect of targeting risk factors for the control of central line-associated bloodstream infection (CLABSI) among high-risk infants in a tertiary neonatal intensive care unit (NICU). @*Methods@#Infants admitted to the NICU and diagnosed with CLABSI from January to December 2013 were eligible for inclusion to the study. The CLABSI group (n=47) was matched in a 1:2 ratio to the control group (n=94) based on gestational age, birth weight, and Score for Neonatal Acute Physiology-II. Risk factors for CLABSI were identified using the Cox proportional hazard model, and analysis of the effect of these risk factors targeting infection control was performed. @*Results@#The risk factors associated with CLABSI were prolonged central line dwell days (adjusted hazard ratio [HR], 1.028; 95% confidence interval [CI], 1.011 to 1.045; P=0.001), use of a silicone catheter (adjusted HR, 5.895; 95% CI, 1.893 to 18.355; P=0.002), surgical treatment (adjusted HR, 3.793; 95% CI, 1.467 to 9.805; P=0.006), and less probiotic supplementation (adjusted HR, 0.254; 95% CI, 0.068 to 0.949; P=0.042). By targeting these risk factors with a quality improvement initiative, the mean CLABSI incidence rate per 1,000 catheter-days decreased from 6.6 to 3.1 (P=0.004). @*Conclusion@#Targeting risk factors for infection control significantly reduced the rate of CLABSI among high-risk infants in the NICU.

Survival and Morbidities in Infants with Birth Weight Less than 500 g:a Nationwide Cohort Study

Background@#This study aimed to investigate the survival and morbidities of infants in the Korean Neonatal Network (KNN) with birth weight (BW) < 500 g. @*Methods@#The demographic and clinical data of 208 live-born infants with a BW < 500 g at a gestational age of ≥ 22 weeks who were treated in the neonatal intensive care units of the KNN between 2013 and 2017 were reviewed. @*Results@#The survival rate of the infants was 28%, with a median gestational age and BW of 24 3/7 weeks (range, 22 0/7 –33 6/7 ) and 440 g (range, 220–499), respectively. Multivariable Cox proportional hazards analysis demonstrated that survival to discharge was associated with longer gestation, higher BW, female sex, singleton gestation, use of any antenatal corticosteroids, and higher Apgar scores at 5 minutes. The overall survival rates were significantly different between the BW categories of < 400 g and 400–499 g. However, there was no significant difference in the incidence of any morbidity between the BW groups.Half of the deaths of infants with BW < 500 g occurred within a week of life, mainly due to cardiopulmonary and neurologic causes. The major causes of death in infants after 1 week of age were infection and gastrointestinal disease. Among the surviving infants, 79% had moderate to severe bronchopulmonary dysplasia, 21% underwent surgical ligation of patent ductus arteriosus, 12% had severe intraventricular hemorrhage (grade III–IV), 38% had sepsis, 9% had necrotizing enterocolitis (stage ≥ 2), and 47% underwent laser treatment for retinopathy of prematurity. The median length of hospital stay was 132 days (range, 69–291), and 53% required assistive devices at discharge. @*Conclusion@#Despite recent advances in neonatal intensive care, the survival and morbidity rates of infants with BW < 500 g need further improvement.

Targeting Risk Factors for the Control of Central Line-Associated Bloodstream Infection in the Neonatal Intensive Care Unit: A Single Tertiary Center Experience

Purpose@#The aim of this study was to estimate the effect of targeting risk factors for the control of central line-associated bloodstream infection (CLABSI) among high-risk infants in a tertiary neonatal intensive care unit (NICU). @*Methods@#Infants admitted to the NICU and diagnosed with CLABSI from January to December 2013 were eligible for inclusion to the study. The CLABSI group (n=47) was matched in a 1:2 ratio to the control group (n=94) based on gestational age, birth weight, and Score for Neonatal Acute Physiology-II. Risk factors for CLABSI were identified using the Cox proportional hazard model, and analysis of the effect of these risk factors targeting infection control was performed. @*Results@#The risk factors associated with CLABSI were prolonged central line dwell days (adjusted hazard ratio [HR], 1.028; 95% confidence interval [CI], 1.011 to 1.045; P=0.001), use of a silicone catheter (adjusted HR, 5.895; 95% CI, 1.893 to 18.355; P=0.002), surgical treatment (adjusted HR, 3.793; 95% CI, 1.467 to 9.805; P=0.006), and less probiotic supplementation (adjusted HR, 0.254; 95% CI, 0.068 to 0.949; P=0.042). By targeting these risk factors with a quality improvement initiative, the mean CLABSI incidence rate per 1,000 catheter-days decreased from 6.6 to 3.1 (P=0.004). @*Conclusion@#Targeting risk factors for infection control significantly reduced the rate of CLABSI among high-risk infants in the NICU.

Survival and Morbidities in Infants with Birth Weight Less than 500 g:a Nationwide Cohort Study

Background@#This study aimed to investigate the survival and morbidities of infants in the Korean Neonatal Network (KNN) with birth weight (BW) < 500 g. @*Methods@#The demographic and clinical data of 208 live-born infants with a BW < 500 g at a gestational age of ≥ 22 weeks who were treated in the neonatal intensive care units of the KNN between 2013 and 2017 were reviewed. @*Results@#The survival rate of the infants was 28%, with a median gestational age and BW of 24 3/7 weeks (range, 22 0/7 –33 6/7 ) and 440 g (range, 220–499), respectively. Multivariable Cox proportional hazards analysis demonstrated that survival to discharge was associated with longer gestation, higher BW, female sex, singleton gestation, use of any antenatal corticosteroids, and higher Apgar scores at 5 minutes. The overall survival rates were significantly different between the BW categories of < 400 g and 400–499 g. However, there was no significant difference in the incidence of any morbidity between the BW groups.Half of the deaths of infants with BW < 500 g occurred within a week of life, mainly due to cardiopulmonary and neurologic causes. The major causes of death in infants after 1 week of age were infection and gastrointestinal disease. Among the surviving infants, 79% had moderate to severe bronchopulmonary dysplasia, 21% underwent surgical ligation of patent ductus arteriosus, 12% had severe intraventricular hemorrhage (grade III–IV), 38% had sepsis, 9% had necrotizing enterocolitis (stage ≥ 2), and 47% underwent laser treatment for retinopathy of prematurity. The median length of hospital stay was 132 days (range, 69–291), and 53% required assistive devices at discharge. @*Conclusion@#Despite recent advances in neonatal intensive care, the survival and morbidity rates of infants with BW < 500 g need further improvement.

Campomelic Dysplasia with Sex Reversal Harboring a Novel Frameshift Mutation

Campomelic dysplasia (CD) is a rare genetic disease characterized by skeletal dysplasia that also affects several other organ systems. CD is caused by a SOX9 mutation. We here report a case of CD with a 46, XY karyotype and female external genitalia. This child was born with a weight of 3.12 kg after 37 weeks of gestation. She exhibited a number of characteristic features including a small thoracic cage, bowing of both femurs, clubbed feet, hypoplastic scapula, 11 pairs of ribs, a bell-shaped narrow thorax, micrognathia, macroglossia, a cleft palate, a flattened nasal bridge, and low set ears. She experienced additional distress because of the presence of a tracheal ring and because she had tracheomalacia. CD was diagnosed through nucleotide sequence analysis. A frameshift mutation, c.235delC (p.Gln79Argfs*31), was identified in the SOX9 gene that has not previously been reported.

Current Status of Neonatologist Staffing and Workload in Korean Neonatal Intensive Care Units

Purpose@#To identify the recent status of the neonatologist and their workload in neonatal intensive care unit (NICU) in Korea. @*Methods@#On October 2018, a survey was conducted on the statistics of the workforce including the census of certified neonatologists, NICU beds, nursing staffing ratings, bed occupancy rate, annual admission of very low birth weight infant (VLBWI), infant acuity score of nursing, and the proportion of out-born patients. The level of neonatal care was self-rated. @*Results@#A total of 68 centers responded to the survey. An average number of cer tified neonatologists and the number of NICU beds per center was 1.9 (range, 0 to 5) and 23.1 (range, 0 to 30), respectively. Thirty-eight percent (n=26) of NICUs were being operated with only one (n=24) or no (n=2) certified neonatologist and only 19% (n=13) of NICUs had ≥3 neonatologists. The average ratio of NICU beds to neonatologists rated 13.4±6.2. The higher the level of neonatal care, the higher the number of tertiary referral hospitals, neonatologists, NICU volume, infant acuity scores of nursing, and annual VLBWI admissions. However, there was no difference in the beds to neonatologist ratio between level 2 (n=9, 9.5±3.1), level 3 (n=44, 14.0± 6.9), and level 4 (n=14, 13.7±4.2). The infant acuity score was proportional to the NICU volumes, but not related to the beds to neonatologist ratio. @*Conclusion@#Compared with the international standards, most Korean NICUs were understaffed in terms of the certified neonatologist and were unable to provide ‘continuity of care’ for high-risk newborns.

A Novel COL4A1 Mutation in a Neonate with Intrauterine Intraventricular Hemorrhage and Porencephaly

Collagen type IV alpha 1 (COL4A1) plays an important role in construction of the basement membranes of all human tissues, especially vessels. Mutations in COL4A1 lead to various multisystemic dysfunctions, including hereditary porencephaly, hemorrhagic stroke, hemiplegia, cerebral small vessel disease, and nephropathy. In this study, we describe a neonatal case featuring a novel de novo COL4A1 mutation, manifesting as fetal intraventricular hemorrhage and porencephaly. This patient is one of the youngest to have been diagnosed with the most severe phenotype. Our experience may assist clinicians in the diagnosis and management of this extremely rare genetic condition.

Response to Inhaled Nitric Oxide and Clinical Outcome in Very Low Birth Weight Infants with Early Pulmonary Hypertension

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 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. Impro vement in oxygenation indicated by prompt decrease in FiO 2 and OI from the base line 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.

Morbidity and Mortality of Very Low Birth Weight Infants with Congenital Heart Disease

Background and Objectives@#This study aimed to provide morbidity and mortality information on very low birth weight (VLBW) infants with congenital heart disease (CHD-VLBWs). @*Methods@#The study used a 10-year cohort of VLBW infants from a single institution. CHD was classified according to International Classification of Diseases, Version 9, Clinical Modification. Mortality and neonatal outcomes were assessed by comparing the CHD-VLBWs with gestational age- and birth weight-matched controls. @*Results@#The prevalence of CHD-VLBWs was 7.5% (79/1,050), mean gestational age was 31.1±3.2 weeks, and mean birth weight was 1,126.2±268.3 g; 50.6% of the infants were small for the gestational age. The CHD-VLBWs more commonly had bronchopulmonary dysplasia (BPD), and the longer they were exposed to oxygen, the more frequently they developed BPD. Those with cyanotic heart disease developed severe BPD more frequently. Necrotizing enterocolitis (NEC) occurred frequently in the CHD-VLBWs and was not associated with their feeding patterns. CHD-VLBWs had a higher mortality rate; prematurity-related diseases were the leading cause of death before surgery, while heart-related problems were the leading cause of death after surgery. We found no significant difference in mortality from prematurity-related disease between the CHD-VLBWs and controls. In the subgroup analysis of CHD, the cyanotic CHD group had a higher incidence of BPD and higher mortality rate than the acyanotic CHD group. @*Conclusions@#CHD-VLBWs showed higher BPD, NEC, and mortality rates than those without CHD. There was also a higher incidence of BPD and mortality in VLBW infants with cyanotic CHD than in those with acyanotic CHD.

Campomelic Dysplasia with Sex Reversal Harboring a Novel Frameshift Mutation

Campomelic dysplasia (CD) is a rare genetic disease characterized by skeletal dysplasia that also affects several other organ systems. CD is caused by a SOX9 mutation. We here report a case of CD with a 46, XY karyotype and female external genitalia. This child was born with a weight of 3.12 kg after 37 weeks of gestation. She exhibited a number of characteristic features including a small thoracic cage, bowing of both femurs, clubbed feet, hypoplastic scapula, 11 pairs of ribs, a bell-shaped narrow thorax, micrognathia, macroglossia, a cleft palate, a flattened nasal bridge, and low set ears. She experienced additional distress because of the presence of a tracheal ring and because she had tracheomalacia. CD was diagnosed through nucleotide sequence analysis. A frameshift mutation, c.235delC (p.Gln79Argfs*31), was identified in the SOX9 gene that has not previously been reported.

Validity of the Korean Developmental Screening Test for very-low-birth-weight infants / 소아과

PURPOSE: The importance of the neurodevelopmental outcomes of very-low-birth-weight (VLBW) infants has been emphasized as their mortality rate has markedly improved. This study aimed to assess the validity of the Korean Developmental Screening Test (K-DST), a developmental screening tool approved by the Korean Society of Pediatrics, for the timely diagnosis of neurodevelopmental delay in VLBW infants. METHODS: Subjects included VLBW infants enrolled in the Korean Neonatal Network database between January 2012 and December 2014. The collected data were analyzed for sensitivity, specificity, positive predictive value, and negative predictive value (NPV) in the K-DST compared to those in the Bayley Scales of Infant Development-II for VLBW infants. RESULTS: A total of 173 patients were enrolled. Their mean gestational age and mean birth weight were 27.5±2.8 weeks and 980.5±272.1 g, respectively. The frequency of failed psychomotor developmental index (PDI) <85 was similar to that in at least one domain of K-DST <1 standard deviation. Failure in more than one K-DST domain compared with a mental developmental index (MDI) <85 showed a sensitivity and NPV of 73.2% and 75.0%, respectively. Failure in more than one K-DST domain compared with PDI <85 showed a sensitivity and NPV of 60.3% and 71.6%, respectively. Each K-DST domain had a stronger correlation with predicting a failing MDI <85 than a failing PDI <85 (P<0.05). CONCLUSION: K-DST could be a useful screening tool for predicting mental developmental delay in VLBW infants and referring them for neurodevelopmental assessments.

Case of mucinous adenocarcinoma of the lung associated with congenital pulmonary airway malformation in a neonate / 소아과

Congenital pulmonary airway malformation (CPAM), previously known as congenital cystic adenomatoid malformation, is a rare developmental lung abnormality associated with rhabdomyosarcoma, pleuropulmonary blastoma, and mucinous adenocarcinoma of the lung. We report an unusual case of a 10-day-old male newborn with a left lower lobe pulmonary cyst who underwent lobectomy, which revealed type II CPAM complicated by multifocal mucinous adenocarcinoma. KRAS sequencing revealed a somatic mutation in Codon12 (GGT → GAT), suggesting the development of a mucinous adenocarcinoma in the background of mucinous metaplasia. Mucinous adenocarcinoma is the most common lung tumor associated with CPAM, but it generally occurs in older children and adults. Further, all cases in the literature are of type I CPAM. This case in a neonate indicates that malignant transformation can occur very early in type II CPAM.

Chronic Placental Inflammation as a Risk Factor of Severe Retinopathy of Prematurity

BACKGROUND: Chronic placental inflammation (CPI) has been implicated in the pathogenesis of diseases in premature infants, whereas retinopathy of prematurity (ROP) is a major complication primarily affecting preterm and very low-birth-weight (VLBW) infants. This study aims to investigate the association between CPI and ROP in VLBW infants. METHODS: We performed a retrospective review of clinical records of VLBW infants born between 2013 and 2016. Placental pathology findings including CPI cases were analyzed using logistic regression to study infants’ morbidities and other clinical characteristics. RESULTS: A total of 402 infants with a mean (standard deviation) gestational age of 28.5 (2.8) weeks and birth weight of 1,027.2 (304.4) g were included. The incidence of ROP was 24.1%. CPI was found in 90 infants (22.4%), among which 28.9% (26 of 90) developed ROP, and 21.1% (19 of 90) underwent laser photocoagulation. Lower gestational age, lower birth weight, longer duration of oxygen supply, and presence of CPI were associated with the development of ROP. After adjustment for gestational age, birth weight, sex, duration of oxygen supply, and other overlapping placental pathology, CPI was associated with the odds for type 1 ROP that required laser photocoagulation (adjusted odds ratio, 2.739; 95% confidence interval, 1.112 to 6.749; p = .029). CONCLUSIONS: CPI was associated with severe ROP requiring treatment with laser photocoagulation in VLBW infants.

Prenatal diagnosis of isolated coronary arteriovenous fistula

Coronary arteriovenous fistula (CAVF) is a rare condition defined as an anomalous termination of the coronary arteries. The etiology of CAVF is either congenital or acquired, and iatrogenic CAVF is most commonly caused by cardiovascular surgery or percutaneous intervention. Most of the prenatally diagnosed CAVFs were related to complex heart disease, and only few cases of an isolated CAVF have been reported to date. We successfully diagnosed an isolated CAVF by fetal echocardiography at 25.3 weeks of gestation. Accurate prenatal diagnosis resulted in the prompt decision for postnatal surgical correction, and the neonate thrived well without any complications.

Ex utero intrapartum treatment procedure in two fetuses with airway obstruction

The ex utero intrapartum treatment (EXIT) procedure was introduced to reduce fetal hypoxic damage while establishing an airway in fetuses with upper and lower airway obstruction. Delivery of the fetal head and shoulders while maintaining the uteroplacental circulation offers time to secure the fetal airway. Here, we report two cases of EXIT procedure for fetal airway obstruction, which were successfully managed with extensive preoperative planning by a professional multidisciplinary team.

First reported case of fetal aortic valvuloplasty in Asia

Prenatal intervention of severe fetal aortic valve stenosis by ultrasound-guided percutaneous balloon valvuloplasty has been performed to prevent the progression to hypoplastic left heart syndrome, and achieve biventricular circulation in neonates. Here we report a case of fetal aortic valvuloplasty prenatally diagnosed with aortic stenosis at 24 weeks of gestation and showed worsening features on a follow-up echocardiography. Prenatal aortic valvuloplasty was performed at 29 weeks of gestation, and was a technical success. However, fetal bradycardia sustained, and an emergency cesarean delivery was performed. To the best of our knowledge, this is the first reported case of fetal aortic valvuloplasty which was performed in Asia.

Serrated Adenoma with Adenocarcinoma of Stomach Treated by Endoscopic Submucosal Dissection

Since the first case of gastric serrated adenoma found in 2001, 35 additional cases have been reported. Among these cases, 26 cases were associated with invasive adenocarcinoma within the serrated adenoma. Gastric serrated adenoma when compared with traditional adenoma has close correlation with invasive carcinoma. Serrated colorectal polyps are classified as hyperplastic polyps, sessile serrated adenoma/polyps, and tranditional serrated adenoma (TSA) depending on histological features. Two distinct phenotypes of TSA in the colon and rectum are reported. Those are unlocked serrated crypts (US-TSA) and ectopic crypt formations (ECFs). All gastric serrated adenoma are TSA in historical aspect and ECFs on phenotype. Whereas gastric adenomas are reported with high frequency in the antrum, gastric serrated adenomas are founded in the body and cardia. We report a case of a 60-year-old woman receiving endoscopic submucosal dissection for gastric serrated adenoma with adenocarcinoma discovered during routine screening.

The Impact of Introduction of Fetoscopic Laser Surgery on Twin-to-Twin Transfusion Syndrome

PURPOSE: Fetoscopic laser surgery (FLS) is considered an optimal therapeutic strategy for twin-to-twin transfusion syndrome (TTTS). We aimed to determine the clinical outcomes of TTTS patients since the introduction of FLS. METHODS: A retrospective study of TTTS patients born between January 2005 and December 2015 was conducted. Mortality and morbidity were compared in the FLS and non-FLS groups. The FLS group was divided into selective FLS and Solomon technique group, and subgroup analysis was performed. RESULTS: Of 70 pregnancies diagnosed with TTTS during the study period, FLS was performed for 35 (50%). Survival of at least one infant at discharge was achieved in 100% (35/35) of pregnancies in the FLS group and 91.4% (32/35) in the non-FLS group (P=0.028). Subgroup analysis revealed that the Solomon technique group had a higher dual survival rate than the selective FLS group (81.3% vs. 57.9%, P=0.036). Neonatal mortality was significantly lower in the FLS group than in the non-FLS group (1.7% vs. 16.4%, P=0.005). Severe intraventricular hemorrhage more than grade 3 was less frequent in the FLS group than in the non-FLS group (0% vs. 18.2%, P= 0.001). The FLS group showed smaller inter-twin differences in birth weight (24.4% vs. 33.7%, P= 0.032) and lower incidence of twin anemia-polycythemia sequence (0% vs. 43.8%, P<0.001). CONCLUSION: Since the introduction of FLS, the survival rate of TTTS patients has improved. The Solomon technique resulted in better dual survival rates than selective FLS.

Newly Detected PKHD1 Gene Mutation in a Newborn with Fatal Autosomal Recessive Polycystic Kidney Disease

Autosomal recessive polycystic kidney disease is among the most common inherited ciliopathies and is caused by mutations in the polycystic kidney and hepatic disease 1 (PKHD1) gene. Despite its great phenotypic variability, this condition is usually diagnosed during the neonatal and early infantile periods. We report a 37+3 -gestational-week neonate presenting with fatal autosomal recessive polycystic kidney disease who died at 28 hours of life from severe respiratory failure. The familial history is significant because a previous sibling died in utero at 24+2 weeks of gestational age and was diagnosed with polycystic kidney disease based on prenatal ultrasonography and autopsy. Our patient's autopsy revealed findings compatible with polycystic kidney disease. In addition, a PKHD1 gene study of peripheral blood leukocytes identified the compound heterozygote mutation c.274C>T(p.Arg92Trp), as well as the novel heterozygous nonsense mutation c.2770C>T(p.Gln924*).

Metastatic Spermatic Cord Tumor From Colorectal Cancer

Metastatic tumors of the spermatic cord are extremely rare, and the prognosis for patients is typically poor. In the majority of cases, the primary tumor occurs in the gastrointestinal tract. We report a case of a 62-year-old man with a metastatic spermatic cord tumor. The patient complained of groin discomfort with a tender mass in the right inguinal area. An excisional biopsy was performed, and the pathologic finding was a metastatic mucinous adenocarcinoma. We performed a systemic evaluation including colonoscopy, abdominal computed tomography, and total-body positron emission tomography, and the primary tumor was confirmed to involve the total colon, including the cecum, sigmoid colon, and rectum. The pathologic finding for rectum revealed a mucinous adenocarcinoma compatible with a metastatic spermatic cord tumor.