No abstract available.
Diagnosis ; Ductus Arteriosus, Patent ; Endarteritis ; Pulmonary Embolism

PURPOSE: This study aimed to evaluate the clinical and radiologic findings suggestive of spontaneous intestinal perforation (SIP) in extremely-low-birth-weight infants (ELBWIs) with persistent gasless abdomen, and to investigate the usefulness of abdominal ultrasonography for the diagnosis of SIP. METHODS: In total, 22 infants with birth weights less than 1,000 g who showed persistent gasless abdomen on simple abdominal radiography were included. Perinatal, neonatal, and perioperative clinical findings were retrospectively reviewed, and the risk factors for intestinal perforation were evaluated. Abdominal sonographic findings suggestive of intestinal perforation were also identified, and postoperative short-term outcomes were evaluated. RESULTS: In total, eight of the 22 infants (36.4%) with gasless abdomen had SIP. The number of infants with patent ductus arteriosus who were treated with intravenous ibuprofen or indomethacin was significantly higher in the SIP group than in the non-SIP group (P<0.05). Greenish or red gastric residue, abdominal distension, or decreased bowel sound were more frequent in infants with SIP (P<0.05), in addition to gray or bluish discoloration of abdomen, suggestive of meconium peritonitis (P<0.05). Pneumoperitoneum on simple abdominal radiography was found in only one of the eight infants (12.5%) with SIP. Intramural echogenicity and echogenic extramural material on abdominal ultrasonography were exclusively observed in infants with SIP. Four infants (50%) with SIP died after surgical intervention. CONCLUSION: Intestinal perforation may occur in ELBWIs with gasless abdomen. As intramural echogenicity and extraluminal echogenic materials on abdominal ultrasonography are indicative of SIP, this technique could be useful for diagnosing SIP.
Abdomen ; Birth Weight ; Diagnosis ; Ductus Arteriosus, Patent ; Humans ; Ibuprofen ; Indomethacin ; Infant ; Infant, Extremely Low Birth Weight ; Infant, Low Birth Weight ; Infant, Newborn ; Intestinal Perforation ; Meconium ; Peritonitis ; Pneumoperitoneum ; Radiography, Abdominal ; Retrospective Studies ; Risk Factors ; Ultrasonography

No abstract available.
Diagnosis ; Ductus Arteriosus, Patent ; Humans ; Infant, Newborn ; Infant, Premature

BACKGROUND: Patent ductus arteriosus (PDA) ligation is usually performed by congenital cardiac surgeons. However, due to the uneven distribution of congenital cardiac surgeons in South Korea, many institutions depend solely on adult cardiac surgeons for congenital cardiac diseases. We report the outcomes of PDA ligations performed by adult cardiac surgeons at our institution. METHODS: The electronic medical records of 852 neonates at Chung-Ang University Hospital, Seoul, South Korea from November 2010 to May 2014 were reviewed to identify patients with PDA. RESULTS: Of the 111 neonates with a diagnosis of PDA, 26 (23%) underwent PDA ligation. PDAs were ligated within 28 days of birth (mean, 14.5±7.8 days), and the mean gestational age of these patients was 30.3±4.6 weeks (range, 26 to 40 weeks) with a mean birth weight of 1,292.5±703.5 g (range, 480 to 3,020 g). No residual shunts through the PDA were found on postoperative echocardiography. There was 1 case of 30-day mortality (3.8%) due to pneumonia, and 6 cases of in-hospital mortality (23.1%) after 30 days, which is comparable to results from other centers with congenital cardiac surgery programs. CONCLUSION: Although our outcomes may not be generalizable to all hospital settings without a congenital cardiac surgery program, in select centers, PDA ligations can be performed safely by adult cardiac surgeons if no congenital cardiac surgery program is available.
Adult* ; Birth Weight ; Cardiac Surgical Procedures ; Diagnosis ; Ductus Arteriosus, Patent* ; Echocardiography ; Electronic Health Records ; Gestational Age ; Heart Defects, Congenital ; Heart Diseases ; Hospital Mortality ; Humans ; Infant, Newborn ; Korea ; Ligation* ; Mortality ; Parturition ; Pneumonia ; Seoul ; Surgeons* ; Thoracic Surgery

Cantú syndrome (CS, OMIM 239850) is a very rare autosomal dominantly inherited genetic disease characterized by congenital hypertrichosis, neonatal macrosomia, a distinct facial features such as macrocephaly, and cardiac defects. Since the first description by Cantú et al. in 1982, about 50 cases have been reported to date. Recently, two causative genes for CS has been found by using exome sequencing analyses: ABCC9 and KCNJ8 . Most cases of clinically diagnosed CS have resulted from de novo mutations in ABCC9. In this study, we report three independent Korean children with CS resulting from de novo ABCC9 mutations. Our patients had common clinical findings such as congenital hypertrichosis, distinctive facial features. One of them showed severe pulmonary hypertension and hypertrophic cardiomyopathy, which require medical treatment. And, two patients had a history of patent ductus arteriosus. Although two of our patients had shown early motor developmental delay, it was gradually improved during follow-up periods. Although CS is quite rare, there are the concerns about development of various cardiac problems in the lifetime. Therefore, an accurate diagnosis followed by appropriate management and genetic counseling should be provided to CS patients.
Cardiomyopathy, Hypertrophic ; Child ; Databases, Genetic ; Diagnosis ; Ductus Arteriosus, Patent ; Exome ; Follow-Up Studies ; Genetic Counseling ; Humans ; Hypertension, Pulmonary ; Hypertrichosis ; Megalencephaly

OBJECTIVETo study the value of amino-terminal pro-brain natriuretic peptide (NT-proBNP) in predicting symptomatic patent ductus arteriosus (sPDA) in preterm infants.

METHODSPreterm infants born at a gestational age (GA) of ≤ 32 weeks and diagnosed with patent ductus arteriosus (PDA) by echocardiography within 48 hours after birth between June 2014 and April 2015 were selected as subjects. Their clinical manifestations were observed, and serum NT-proBNP levels were measured and echocardiography was performed at 3 and 5 days after birth. The infants were divided into sPDA group and asymptomatic PDA (asPDA) group based on their clinical manifestations and the results of echocardiography. The correlations between serum NT-proBNP level and echocardiographic indices were analyzed. Serum NT-proBNP levels were compared between the two groups. The receiver operator characteristic (ROC) curve was applied to determine the sensitivity and specificity of serum NT-proBNP in the prediction of sPDA.

RESULTSA total of 69 preterm infants were enrolled in this study, with 13 infants in the sPDA group and 56 infants in the asPDA group. Serum NT-proBNP level was positively correlated with the diameter of the arterial duct (r=0.856; P<0.05)and the ratio of left atrial diameter to aortic root diameter (LA/AO) (r=0.713; P<0.05). At 3 and 5 days after birth, the serum NT-proBNP levels in the sPDA group were significantly higher than those in the asPDA group (P<0.05). The area under the ROC curve (AUC) for the prediction of sPDA by NT-proBNP levels at 3 days after birth was 0.949 (95% CI: 0.892-1.000; P<0.001), with a cut-off value of 27 035 pg/mL (sensitivity: 92.3%; specificity: 94.6%); the AUC for the prediction of sPDA by NT-proBNP levels at 5 days after birth was 0.924 (95% CI: 0.848-1.000; P<0.001), with a cut-off value of 6 411 pg/mL (sensitivity: 92.3%; specificity: 92.9%).

CONCLUSIONSNT-proBNP may be a quantitative index for shunt volume. The measurement of serum NT-proBNP levels on 3 and 5 days after birth may be useful to predict sPDA in preterm infants.

Biomarkers ; Ductus Arteriosus, Patent ; diagnosis ; Female ; Humans ; Infant, Newborn ; Infant, Premature ; Male ; Natriuretic Peptide, Brain ; blood ; Peptide Fragments ; blood ; ROC Curve

OBJECTIVETo analyze the clinical features and prognosis of extremely low birth weight infants (ELBWI).

METHODData of totally 165 hospitalized ELBWI between August 1st, 2008 and November 30th, 2013 in Hunan Children's Hospital were analyzed. The information of general data and births, prenatal care, delivery room stabilization, transfer information, complications, treatment, outcome and follow up were summarized.

RESULT(1) One hundred and sixty-five ELBWI were involved, their mean gestational age was (28.4±2.4) weeks, mean birth weight(910.9±93.1) g. (2) Rate of delivery in tertiary hospital was 46.7% (77/165) and prenatal steroids exposure was found in 52.1% (86/165). None of the cases were treated with continuous positive airway pressure (CPAP) in delivery room. (3) Rate of infants who were transfered to the class III b neonatal ward within 12 hours after birth was 58.8% (97/165). (4) The main complications of them included neonatal respiratory distress syndrome(NRDS, 77.0%, 127/165), bronchopulmonary dysplasia (BPD, 70.1%, 75/107), patent ductus arteriosus (PDA, 50.0%, 40/80), preterm retinopathy (ROP, 43.0%, 46/107), sepsis 39.4% (65/165), intraventricular hemorrhage (IVH, 34.8%, 49/141), necrotizing enterocolitis (NEC, 8.0%, 7/88). (5) TREATMENT: 97.6% (161/165) received oxygen therapy and 66.1% (109/165) received mechanical ventilation, 55.2% (91/165) used CPAP. 89.8% (114/127) of the NRDS used PS; 44.0% (33/75) of the BPD used low dose dexamethasone, 32.0% (24/75) used low dose nitric oxide; 60.0% (24/40) of the PDA used medication. 32.6% (15/46) of the ROP received laser photocoagulation. The average time of beginning enteral feeding was 2.0 d, the mean time to achieve full gastrointestinal feeding was 43.4 d. (6) OUTCOME: rate of survival in 165 cases with ELBWI was 51.5% (85/165), treatment was abandoned in 37.6% (62/165), total mortality was 48.5% (80/165). There were significant difference in survival rate between different birth weight group, gestational age group and admission age group (χ2=11.498, 8.789, 13.157, all P<0.05); There was significant difference in rate of giving up treatment between different birth weight and admission age groups (χ2=10.448, 8.259, all P<0.05). The primary cause of death was economic factor and worrying about prognosis. (7) Follow up: rate of neurodevelopmental impairment (NDI) in part of follow up cases was 27.9% (12/43).

CONCLUSIONELBWI have many severe complications and sequelae, which need complex treatment and long hospital stay with a low survival rate. In order to improve the level of treatment, we need to improve the rate of prenatal steroids, carry out intrauterine and postnatal, transport of ELBWI to III b neonatal ward as early and rationally as possible, give professional, sophisticated and integrated treatment technologies as far as possible.

Birth Weight ; Bronchopulmonary Dysplasia ; diagnosis ; therapy ; Cerebral Hemorrhage ; diagnosis ; therapy ; China ; epidemiology ; Dexamethasone ; therapeutic use ; Ductus Arteriosus, Patent ; diagnosis ; therapy ; Enterocolitis, Necrotizing ; diagnosis ; therapy ; Gestational Age ; Humans ; Infant, Extremely Low Birth Weight ; Infant, Newborn ; Infant, Premature ; Infant, Premature, Diseases ; diagnosis ; therapy ; Prognosis ; Respiratory Distress Syndrome, Newborn ; diagnosis ; therapy ; Retinopathy of Prematurity ; diagnosis ; therapy ; Sepsis ; diagnosis ; therapy ; Survival Rate ; Treatment Outcome

Angiocardiography ; Anti-Infective Agents ; therapeutic use ; Aorta ; abnormalities ; surgery ; Bronchopneumonia ; diagnosis ; drug therapy ; Cardiac Surgical Procedures ; methods ; Ductus Arteriosus, Patent ; diagnosis ; surgery ; Female ; Heart Defects, Congenital ; diagnosis ; surgery ; Humans ; Infant ; Pulmonary Artery ; abnormalities ; surgery ; Tomography, Spiral Computed

The purpose of this study was to evaluate prognostic factors associated with surgical ligation for patent ductus arteriosus (PDA) in extremely preterm infants born at the limits of viability. Ninety infants who were born at 23-25 weeks of gestation and who received surgical ligation were included and their cases were retrospectively reviewed. Infants were classified into two different groups: survivors with no major morbidity (N), and non-survivors or survivors with any major morbidity (M). Clinical characteristics were compared between the groups. Possible prognostic factors were derived from this comparison and further tested by logistic regression analysis. The mean gestational age and the mean birth weight of M were significantly lower than those of N. Notably, the mean postnatal age at time of ligation in N was significantly later than that of the other group (17+/-12 vs 11+/-8 days in N and M, respectively). An adjusted analysis showed that delayed ligation (>2 weeks) was uniquely associated with a significantly decreased risk for mortality or composite morbidity after surgical ligation (OR, 0.105; 95% CI, 0.012-0.928). In conclusion, delayed surgical ligation for PDA (>2 weeks) is associated with decreased mortality or morbidities in extremely preterm infants born at 23-25 weeks of gestation.
Adult ; Birth Weight ; Demography ; Ductus Arteriosus, Patent/diagnosis/epidemiology/*surgery ; Female ; Gestational Age ; Humans ; Infant, Extremely Premature ; Infant, Newborn ; Logistic Models ; Male ; Odds Ratio ; Prognosis ; Retrospective Studies ; Time Factors

PURPOSE: Although discussion about active treatment of trisomy 18 is increasing, there are no previous articles regarding this subject in Korea. In order to provide objective data about the clinical characteristics and survival of patients with trisomy 18, based on the treatment policy, to medical teams and parents with trisomy 18, we reviewed the medical records of such patients at a single center in Korea. METHODS: This is a retrospective study of 22 patients diagnosed with trisomy 18 at the Samsung Medical Center between 1995 and 2013. We collected data about the clinical characteristics, including demographics, birth history, diagnosis method, and associated anomalies. We analyzed the survival in days, according to three broad categories of treatment policy: give-up, conservative management and active treatment. RESULTS: Of the 22 patients with confirmed trisomy 18, the majority were female (19, 86%). The median gestational age was 39 weeks (range, 31-41 weeks) and the median birth weight is 2,029 g (range, 1,130-2,990 g). Among the anomalies associated with trisomy 18, ventricular septal defect (86%) and patent ductus arteriosus (81%) were the most common cardiac anomalies; giant cisterna magna (59%) was the most common central nervous system anomaly; and clenched hands (73%) and low set ears (59%) were the most common structural anomalies. The survival based on the treatment policy was the highest in the active treatment group, followed by the conservative management group. The give-up group had the lowest survival. CONCLUSION: It is possible to achieve an improvement in both survival and symptom relief for patients with trisomy 18, despite poor neurological outcome and high mortality. Medical personnel need to provide objective data on trisomy 18 to the parents, and determine the treatment policy through careful discussion.
Birth Weight ; Central Nervous System ; Cisterna Magna ; Demography ; Diagnosis ; Ductus Arteriosus, Patent ; Ear ; Female ; Gestational Age ; Hand ; Heart Septal Defects, Ventricular ; Humans ; Korea ; Medical Records ; Mortality ; Parents ; Reproductive History ; Retrospective Studies ; Trisomy*