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1.
Pediatric Gastroenterology, Hepatology & Nutrition ; : 453-460, 2022.
Article in English | WPRIM | ID: wpr-968493

ABSTRACT

Purpose@#Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is a congenital anomaly that can cause frequent digestive and nutritional problems, even after repair. The most common complication is anastomotic stricture, for which reoperation or balloon dilatation is performed. This study aimed to evaluate the postoperative complications of EA and the role of endoscopic balloon dilatation (EBD) in cases of anastomotic stricture. @*Methods@#We retrospectively analyzed patients diagnosed with EA with or without TEF between January 2000 and February 2021. Patients’ baseline characteristics, associated anomalies, and postoperative complications were reviewed. @*Results@#Among 26 patients, 14 (53.8%) were male, 12 (46.2%) had coexisting anomalies, and the median follow-up was 6.1 years (range, 1.2–15.7 years). In univariate analysis, prematurity, low birth weight, and long-gap EA were associated with postoperative complications in 12 (46.2%) patients. Among the 10 (38.5%) patients with anastomotic stricture, nine (90.0%) required EBD. Regarding the first EBD, it was performed at a median of 3.3 months (range, 1.2–7.6 months) post-repair, while the average patient weight was 4.6 kg. The mean diameter ranged from 3.3 to 9.1 mm without major complications. In univariate analysis, long-gap EA alone was significantly associated with EBD. @*Conclusion@#Approximately half of the patients experienced complications after EA repair.In particular, patients with a long-gap EA had a significantly increased risk of complications, such as anastomotic strictures. EBD can be safely used, even in infants.

2.
Journal of Genetic Medicine ; : 147-151, 2021.
Article in English | WPRIM | ID: wpr-915020

ABSTRACT

The Say–Barber–Biesecker–Young–Simpson variant of Ohdo syndrome (SBBYSS) (Online Mendelian Inheritance in Man #603736) is a rare autosomal dominant disorder and clinically features blepharophimosis with ptosis, a mask-like facial appearance, cryptorchidism, congenital heart defect, long thumbs/great toes, and thyroid dysfunction. The etiology of SBBYSS has been shown to be due to heterozygous KAT6B gene mutation. Here we report a case of a neonate with SBBYSS identified a novel mutation in KAT6B gene. The patient showed typical dysmorphic facies, cryptorchidism with micropenis, overriding fingers, and long thumbs and toes at birth. He had also hypothyroidism, large atrial septal defect, and sensorineural hearing loss. The next generation sequencing identified a heterozygous novel variant, c.5206C>T (p.Gln1736Ter) in KAT6B gene. At the 9 months of age, he underwent patch closure for atrial septal defect. Until the 12-month follow-up, he was under-developed.

3.
Neonatal Medicine ; : 37-43, 2020.
Article | WPRIM | ID: wpr-837006

ABSTRACT

Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular neoplasm that often develops a coagulopathy known as Kasabach­Merritt phenomenon (KMP). Visceral involvement denotes a poor prognosis. We report a case of visceral KHE with KMP successfully treated with corticosteroids and vincristine. The infant had been born vaginally at 34 weeks 6 days’ gestation, weighing 2,360 g. He was admi tted for the management of respiratory failure. Blood tests showed anemia and thrombocytopenia 1 hour after delivery. Additional blood tests revealed a prothrombin time of 12.1 seconds, activated partial thromboplastin time of 60.6 seconds, fibrinogen levels of 72.4 mg/dL, and D­dimer levels >3,200 ng/mL. Despite supportive measures and daily transfusions, the clinical condition and coagulopathy gradually worsened. Renal ultrasonography performed to find the origin of the coagulopathy revealed an echogenic mass measuring >3 cm in the abdominal cavity. A magnetic resonance imaging scan showed an ill­marginated, infiltrative mass like lesion in the right anteromedial and posteromedial perirenal space that was hypointense with mild enhance ment on T1­ and T2­weighted images. Large vascular tumors adherent to other visceral organs were noted during exploratory laparotomy but could not be resected. Treatment with methylprednisolone was ineffective. Vincristine was administered weekly from the 17th hospital day, and the coagulation profile showed gradual impro vement after its initiation. Intravenous methylprednisolone was switched to oral prednisolone on the 57th hospital day. He was discharged on the 73rd hospital day and continued vincristine treatment every 2 weeks and oral prednisolone administration as an outpatient treatment for 8 weeks. He remained symptom­free at the 39­month follow­up.

4.
Journal of Genetic Medicine ; : 108-111, 2020.
Article in English | WPRIM | ID: wpr-899315

ABSTRACT

Short stature homeobox-containing gene (SHOX) is a well-known causative gene for the short stature in Turner syndrome. The clinical manifestation of SHOX gene related disorders varies from SHOX haploinsufficiency, presenting with idiopathic short stature, disproportionate short stature, or Leri-Weill dyschondrosteosis (LWD) to recessive form of extreme dwarfism and limb deformity in Langer mesomelic dysplasia. LWD is usually diagnosed upon suspicion based on short stature and skeletal abnormalities, and it is rarely accompanied with respiratory failure in the neonatal period. Here, we report the case of a newborn infant with LWD presenting with severe micrognathia that caused respiratory distress, which was diagnosed using microarray testing. Even when the manifestation of Madelung deformity is not yet apparent, LWD should be considered as one of underlying diseases related to congenital micrognathia.

5.
Journal of Genetic Medicine ; : 108-111, 2020.
Article in English | WPRIM | ID: wpr-891611

ABSTRACT

Short stature homeobox-containing gene (SHOX) is a well-known causative gene for the short stature in Turner syndrome. The clinical manifestation of SHOX gene related disorders varies from SHOX haploinsufficiency, presenting with idiopathic short stature, disproportionate short stature, or Leri-Weill dyschondrosteosis (LWD) to recessive form of extreme dwarfism and limb deformity in Langer mesomelic dysplasia. LWD is usually diagnosed upon suspicion based on short stature and skeletal abnormalities, and it is rarely accompanied with respiratory failure in the neonatal period. Here, we report the case of a newborn infant with LWD presenting with severe micrognathia that caused respiratory distress, which was diagnosed using microarray testing. Even when the manifestation of Madelung deformity is not yet apparent, LWD should be considered as one of underlying diseases related to congenital micrognathia.

6.
Journal of Genetic Medicine ; : 31-38, 2019.
Article in English | WPRIM | ID: wpr-764506

ABSTRACT

Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is a maternally inherited mitochondrial disorder of which m.3243A>G is the most commonly associated mutation, resulting in an inability to meet the energy requirements of various organs. MELAS poses a diagnostic challenge owing to its multiple organ involvement and great clinical variability due to its heteroplasmic nature. We report three cases from a family who were initially misdiagnosed with myasthenia gravis or undiagnosed. Although there is no optimal consensus treatment approach for patients with MELAS because of the disease's heterogeneity, our 21-year-long therapy regimen of l-arginine, l-carnitine, and coenzyme Q10 supplementation combined with dietary management appeared to provide noticeable protection from the symptoms and complications. Prompt early diagnosis is important, as optimal multidisciplinary management and early intervention may improve outcomes.


Subject(s)
Humans , Acidosis, Lactic , Arginine , Carnitine , Consensus , DNA, Mitochondrial , Early Diagnosis , Early Intervention, Educational , Follow-Up Studies , MELAS Syndrome , Mitochondrial Diseases , Myasthenia Gravis , Population Characteristics
7.
Neonatal Medicine ; : 58-65, 2018.
Article in English | WPRIM | ID: wpr-714585

ABSTRACT

PURPOSE: The purpose of this study is to describe the rate of cytomegalovirus (CMV) virolactia, and the prevalence of breast milk (BM)-transmitted postnatal CMV infection among premature infants after freeze-thawing (FT) and Holder pasteurization (HP) of breast milk. METHODS: This is a single-center, retrospective study of 312 infants born at less than 32 weeks of gestation, or with a birth weight less than 1,500 g from January 2013 to June 2017. All infants were screened for CMV-specific immunoglobulin (Ig) G and IgM at birth. Initial CMV specific polymerase chain reaction (PCR) and CMV culture were performed on mothers' BM and babies' urine within the first 21 days of life. FT and HP of BM was used to prevent the transmission of CMV. For the surveillance of postnatal CMV infection, CMV culture and CMV specific PCR of urine from babies were repeated one to two months after the initial screening. Screening for viremia and viruria was performed if postnatal CMV infection was suspected. RESULTS: Among 178 BM samples obtained from mothers of CMV-IgG-seropositive infants, 80 (44.9%) were CMV PCR positive. CMV deoxyribonucleic acid (DNA) was detected in five of the 22 BM samples (22.7%) obtained from the mothers of CMV-IgG seronegative infants. When CMV DNA load in BM was measured before and after HP, various results were shown. Sixty-three infants out of 232 (27.2%) were evaluated for postnatal CMV infection and four infants out of 63 (6.3%) were infected. CONCLUSION: Interventions to prevent BM-transmitted CMV infection can reduce the chance of postnatal CMV infection, but not completely eliminate it.


Subject(s)
Humans , Infant , Infant, Newborn , Pregnancy , Birth Weight , Breast , Cytomegalovirus Infections , Cytomegalovirus , DNA , Immunoglobulin M , Immunoglobulins , Infant, Premature , Mass Screening , Milk, Human , Mothers , Parturition , Pasteurization , Polymerase Chain Reaction , Prevalence , Retrospective Studies , Viremia
8.
Korean Journal of Pediatrics ; : 165-173, 2016.
Article in English | WPRIM | ID: wpr-44141

ABSTRACT

PURPOSE: To identify the effects of modified parenteral nutrition (PN) and enteral nutrition (EN) regimens on the growth of very low birth weight (VLBW) infants. METHODS: The study included VLBW infants weighing <1,500 g, admitted to Chungnam National University Hospital between October 2010 and April 2014, who were alive at the time of discharge. Subjects were divided according to 3 periods: period 1 (n=37); prior to the PN and EN regimen being modified, period 2 (n=50); following the PN-only regimen modification, period 3 (n=37); following both PN and EN regimen modification. The modified PN regimen provided 3 g/kg/day of protein and 1 g/kg/day of lipid on the first day of life. The modified EN regimen provided 3.5-4.5 g/kg/day of protein and 150 kcal/kg/day of energy. We investigated growth rate, anthropometric measurements at 40 weeks postconceptional age (PCA) and the incidence of extrauterine growth restriction (EUGR) at 40 weeks PCA. RESULTS: Across the 3 periods, clinical characteristics, including gestational age, anthropometric measurements at birth, multiple births, sex, Apgar score, surfactant use and PDA treatment, were similar. Growth rates for weight and height, from time of full enteral feeding to 40 weeks PCA, were higher in period 3. Anthropometric measurements at 40 weeks PCA were greatest in period 3. Incidence of weight, height and head circumference EUGR at 40 weeks PCA decreased in period 3. CONCLUSION: Beginning PN earlier, with a greater supply of protein and energy during PN and EN, is advantageous for postnatal growth in VLBW infants.


Subject(s)
Humans , Infant , Apgar Score , Enteral Nutrition , Gestational Age , Head , Incidence , Infant, Very Low Birth Weight , Multiple Birth Offspring , Parenteral Nutrition , Parturition , Passive Cutaneous Anaphylaxis
9.
Neonatal Medicine ; : 127-130, 2016.
Article in English | WPRIM | ID: wpr-61669

ABSTRACT

Neonatal hydrocolpos is a rare condition that involves fluid accumulation in the vagina. On diagnostic imaging, the dilated vagina, along with the compressed uterus, can simulate a mature cystic teratoma with a mural nodule. Herein, we report the case of a newborn girl with congenital hydrocolpos that was caused by an imperforate hymen; the hydrocolpos mimicking a mature cystic teratoma on abdominal ultrasonography and magnetic resonance imaging. Any newborn girl with a pelvic cystic mass should be suspected as having a congenital vaginal obstruction manifesting as hydrocolpos or hydrometrocolpos. Thorough examination of the external genitalia, as well as imaging of the uterus and vagina, enables correct diagnosis and optimal treatment.


Subject(s)
Female , Humans , Infant , Infant, Newborn , Diagnosis , Diagnostic Imaging , Genitalia , Hydrocolpos , Hymen , Magnetic Resonance Imaging , Pelvis , Teratoma , Ultrasonography , Uterus , Vagina
10.
Korean Journal of Pediatric Gastroenterology and Nutrition ; : 393-397, 2011.
Article in Korean | WPRIM | ID: wpr-214463

ABSTRACT

Dieulafoy's lesion is defined as a small mucosal defect overlying an abnormal, large caliber submucosal artery that protrudes through the gastrointestinal mucosa. This lesion is a rare cause of massive upper gastrointestinal bleeding in the pediatric population and extremely rare in neonates. We report a 1-day-old neonate who presented with massive gastrointestinal bleeding caused by a gastric Dieulafoy lesion, which was successfully treated by endoscopic hemoclipping without any complications.


Subject(s)
Humans , Infant, Newborn , Arteries , Hemorrhage , Mucous Membrane
11.
Korean Journal of Perinatology ; : 310-318, 2011.
Article in Korean | WPRIM | ID: wpr-75134

ABSTRACT

PURPOSE: We evaluated the brain magnetic resonance imaging (MRI) findings and significance in newborns with severe hyperbilirubinemia. METHODS: This study included 60 newborns with severe hyperbilirubinemia at the neonatal intensive care unit (NICU) of the Chungnam National University Hospital from January 2006 to April 2011. The patients were grouped according to with or without high signal intensities in the globus pallidus (GP) on T1-weighted MRI. We compared the clinical characteristics and neurodevelopmental outcomes between the groups. RESULTS: Thirty-seven of the 60 newborns (62%) showed symmetric high signal intensities in the GP on T1-weighted MRI or intracranial hemorrhage. The group that had high signal intensities in the GP on T1-weighted MRI showed a higher tendency of hemolysis, anemia and exchange transfusion. Demographic characteristics and neurodevelopmental outcomes were similar between the groups. CONCLUSION: We suggest that the GP involvement in severe hyperbilirubinemia of newborn is described as high signal intensities on T1-weighted MRI in the early stage but this lesion is a transient phenomenon that have relatively good long-term prognosis.


Subject(s)
Humans , Infant , Infant, Newborn , Anemia , Brain , Globus Pallidus , Hemolysis , Hyperbilirubinemia , Intensive Care, Neonatal , Intracranial Hemorrhages , Magnetic Resonance Imaging , Magnetic Resonance Spectroscopy , Magnetics , Magnets , Prognosis
12.
Journal of the Korean Society of Neonatology ; : 320-327, 2011.
Article in Korean | WPRIM | ID: wpr-59461

ABSTRACT

PURPOSE: To determine the effect of changing practice guidelines designed to avoid hyperoxia or hypoxia in very low birth weight or very preterm infants. METHODS: We analyzed a database of <1,500 g birth weight or <32 weeks of gestation infants who were born and admitted to the neonatal intensive care unit of Chungnam National University Hospital from January 2007 to July 2010. First, we defined the relationship between arterial partial pressure of oxygen (PaO2) and pulse oxygen saturation (SpO2). When we evaluated 96 pairs of PaO2 and SpO2 measurements, oxygen saturation was 90-94% at a PaO2 of 43-79 mmHg on the oxyhemoglobin dissociation curve, according to pulse oximetry. Based on this observation, a change in practice was instituted in August 2008 with the objective of avoiding hypoxia and hyperoxia in preterm infants with targeting a SpO2 90-94% (period II). Before the change in practice, high alarms for SpO2 were set at 100% and low alarms at 95% (period I). RESULTS: Sixty-eight infants the met enrollment criteria and 38 (56%) were born during period II, after the change in SpO2 targets. Demographic characteristics, except gender, were similar between the infants born in both periods. After correcting for the effect of confounding factors, the rates for mortality, severe retinopathy of prematurity, and IVH attended to be lower than those for infants in period II. No difference in the rate of patent ductus arteriosus needed to treat was observed. CONCLUSION: A change in the practice guidelines aimed at avoiding low oxygen saturation and hyperoxia did not increase neonatal complication rates and showed promising results, suggesting decreased mortality and improvements in short term morbidity. It is still unclear what range of oxygen saturation is appropriate for very preterm infants but the more careful saturation targeting guideline should be considered to prevent hypoxemic events and hyperoxia.


Subject(s)
Humans , Infant , Infant, Newborn , Pregnancy , Hypoxia , Birth Weight , Blood Gas Analysis , Dissociative Disorders , Ductus Arteriosus, Patent , Hyperoxia , Infant, Premature , Infant, Very Low Birth Weight , Intensive Care, Neonatal , Oximetry , Oxygen , Oxyhemoglobins , Partial Pressure , Retinopathy of Prematurity
13.
Korean Journal of Perinatology ; : 40-47, 2010.
Article in Korean | WPRIM | ID: wpr-19112

ABSTRACT

OBJECTIVE: This study was aimed to analyze the effect of Insulin-like growth factor (IGF)-I and Insulin-like growth factor binding protein (IGFBP)-3 on early catch-up growth in small for gestational age (SGA) infants. METHODS: We prospectively studied in 18 SGA infants who were admitted at Chungnam National University Hospital from January 2004 to January 2006. We divided them into two groups of intrauterine growth retardation; symmetric and asymmetric type. Maternal and neonatal characteristics and the value of IGF-1 and IGFBP-3 at birth were compared between the two groups. After 12 months later, we checked their body weight and height to evaluate catch-up growth. RESULTS: Among 18 SGA infants, 5 were male and 13 were female. Average gestational age at birth was 38.4+/-1.1 weeks. Average birth weight was 2,359+/-154 g. Nine babies showed symmetric intrauterine growth retardation. Thirteen babies achieved catch-up growth at 12 month of age. There was no correlation between the characteristics of SGA and catch-up growth. However, the IGF-I and IGFBP-3 values were significantly higher in babies who showed catch-up growth. CONCLUSION: We were able to predict catch-up growth at early infancy by evaluating serum IGF-I and IGFBP-3 values at birth in SGA infants.


Subject(s)
Female , Humans , Infant , Male , Birth Weight , Body Weight , Carrier Proteins , Fetal Growth Retardation , Gestational Age , Insulin-Like Growth Factor Binding Protein 3 , Insulin-Like Growth Factor I , Parturition , Prospective Studies
14.
Korean Journal of Perinatology ; : 182-185, 2007.
Article in Korean | WPRIM | ID: wpr-196118

ABSTRACT

Gardnerella vaginalis is a normal component of the vaginal flora and is one of the organisms associated with bacterial vaginosis. It is rarely involved in neonatal infection. Although it is possible that G. vaginalis plays an etiologic role in bacteremia, facial cellulitis and abscess, conjunctivitis, infected cephalhematoma, scalp abscess, respiratory disease and meningitis in newborns, G. vaginalis is an uncommon pathogen of neonatal sepsis and meningitis. We report a 3,830 g term neonate with sepsis and meningitis due to G. vaginalis and review the characteristics of neonatal G. vaginalis infection reported in the literatures.


Subject(s)
Humans , Infant, Newborn , Abscess , Bacteremia , Cellulitis , Conjunctivitis , Gardnerella vaginalis , Gardnerella , Meningitis , Scalp , Sepsis , Vaginosis, Bacterial
15.
Korean Journal of Pediatrics ; : 268-271, 2007.
Article in Korean | WPRIM | ID: wpr-198448

ABSTRACT

PURPOSE: Premature narrowing of the foramen ovale is rare but serious clinical entity. Prenatal narrowing or obstruction of the foramen ovale shows symptoms such as right heart failure, fetal hydrops, triscupid regurgitation, left heart obstructive disease, and supraventricular tachycardia. This study aimed to assess the prenatal diagnosis and postnatal clinical course of restrictive foramen ovale in utero in otherwise normal heart. METHODS: The subjects were five patients diagnosed with restrictive foramen ovale in utero from January 2001 to June 2005 at Chungnam National University Hospital. The diagnostic criteria was defined when the maximum diameter in a 4-chamber view is less than 2.5 mm and there is a continuous doppler velocity at the foramen ovale of more than 0.6m/s. RESULTS: At the time of diagnosis of restrictive foramen ovale, gestation age was 34~37 wks, and chief complaints were fetal arrhythmia(2 cases), pericardial effusion, Ebstein anomaly and subaortic stenosis. Two cases which were diagnosed fetal hydrops and supraventricular tachycardia delivered by emergent cesarian section. Five cases were found to have right heart dilatation on echocardiogram after birth, but right heart dilatation became normalized at day 7 after birth and the clinical courses were not eventful. CONCLUSION: Identifying an obstructed foramen ovale in the fetus warrants the further search for additional cardiac and extracardiac anomalies, which may alter the prognosis. Delivery should be induced if possible in cases of foramen ovale obstruction with signs of cardiac decompensation.


Subject(s)
Humans , Pregnancy , Constriction, Pathologic , Diagnosis , Dilatation , Ebstein Anomaly , Fetal Heart , Fetus , Foramen Ovale , Heart Failure , Heart , Hydrops Fetalis , Parturition , Pericardial Effusion , Prenatal Diagnosis , Prognosis , Tachycardia, Supraventricular , Ultrasonography, Prenatal
16.
Journal of the Korean Child Neurology Society ; : 68-78, 2006.
Article in Korean | WPRIM | ID: wpr-32206

ABSTRACT

PURPOSE: This study was designed to compare the follow up results with clinical and laboratory findings in patients with seizures in the neonatal period. Also, it was aimed to evaluate the usefulness of scoring prognostic factors and its efficacy as a prognostic index. METHODS: The subjects are 67 neonates who were admitted to Chungnam National University Hospital from January 1999 to April 2005 for seizures which occurred within 28 days of birth. These patients were investigated retrospectively from the hospital records. We scored clinical manifestations including gestational age, birth weight, Apgar score at 5 min, etiology, type, onset, duration, and frequency of seizures, neurologic examination results, EEG background activities, and treatment responses. Follow-up examinations were done to analyze the presence of epilepsy and neurologic deficits. A sum of the factors associated with outcomes regarded as a prognostic index, were studied. RESULTS: 50 cases(74.6%) showed normal outcomes, while 7 cases(10.4%) had unfavorable neurologic outcomes and 4 cases(6.0%) favorable neurologic outcomes with epilepsy. 6 cases(9.0%) showed unfavorable neurologic outcomes with epilepsy. All of the data including gestational age, Apgar score at 5 min, etiology, type, duration, and frequency of seizures, neurologic examination results, EEG background activities, and treatment responses were used as a prognostic index by a scoring system. When the cut-off point was 7, sensitivity, specificity, positive predictive value and negative predictive value were 76.5%, 78.0%, 54.2%, and 90.7% respectively. CONCLUSION: A scoring system for neonatal seizures earned high scores in sensitivity, specificity and negative predictive value, so that it seems to be useful as a predicitive prognostic index.


Subject(s)
Humans , Infant, Newborn , Apgar Score , Birth Weight , Electroencephalography , Epilepsy , Follow-Up Studies , Gestational Age , Hospital Records , Neurologic Examination , Neurologic Manifestations , Parturition , Retrospective Studies , Seizures , Sensitivity and Specificity
17.
Korean Journal of Pediatrics ; : 851-856, 2006.
Article in Korean | WPRIM | ID: wpr-47620

ABSTRACT

PURPOSE: The purpose of this study is to find out the diagnostic significance of serum bile acid on total parenteral nutrition induced cholestasis in premature infants. METHODS: Infants without cholestasis were classified into postnatal days and each change of serum bile acid was measured and analyzed. Also, the serum direct bilirubin, serum bile acid, gamma-glutamic acid transferase, and alkaline phosphatase of premature infants with total parenteral nutrition induced cholestasis were measured for comparison and analysis of their correlation. RESULTS: Changes of serum bile acid analysis after birth showed no significant difference between boys and girls, between premature infants and term infants without cholestasis. Serum bile acid levels are constant after two weeks after birth in neonates without cholestasis. In premature infants with total parenteral nutrition induced cholestasis, the increase of serum direct bilirubin over 2 mg/ dL was 34.9+/-18.3 days after birth, and the increase of serum bile acid was 28.1+/-18.3 days. Its increase was about 1 week faster than serum direct bilirubin, however, there was no statistical significance(P=0.114). Comparing analysis of serum bile acid, gamma-glutamic acid transferase, and alkaline phosphatase, serum bile acid showed the highest correlation to serum direct bilirubin(r=0.487, P= 0.000). CONCLUSION: Serum bile acid is an important parameter of total parenteral nutrition induced cholestasis in premature infants and will be useful for early diagnosis and treatment.


Subject(s)
Female , Humans , Infant , Infant, Newborn , Alkaline Phosphatase , Bile , Bilirubin , Cholestasis , Early Diagnosis , Infant, Premature , Parenteral Nutrition, Total , Parturition , Transferases
18.
Journal of the Korean Society of Neonatology ; : 90-96, 2006.
Article in Korean | WPRIM | ID: wpr-70652

ABSTRACT

PURPOSE: This study was aimed to analyze the level of serum amino acids according to the sex, birth weight, gestational age in neonates. METHODS: Amino acid was measured by tandem mass spectrometry from the dried blood spots. We measured serum alanine, citrulline, glycine, methionine, ornitine, tyrosine, valine, leucine, phenylalanine levels in 172 neonates admitted to the NICU at Chungnam National University hospital from March 2003 to September 2003 and the data was analyzed according to the sex, birth weight, gestational age. RESULTS: There were no differences of serum amino acid level between term and preterm neonates according to the sex. However, there were significant statistical differences in serum amino acid level according to the birth weight (> or =2,500 g vs. or =37 weeks vs. <37 weeks). The level of alanine, citrulline, glycine, methionine, ornitine, tyrosine, valine, leucine was low in under 2,500 g (P<0.05), and in preterm neonates (P<0.05). Especially, preterm neonates under 1,800 g had low levels of valine and leucine (P<0.05). The serum levels of methionine, ornitine, valine, leucine were low in neonates with gestational age of less than 34 weeks (P<0.05). CONCLUSION: Awareness of low serum amino acid levels in preterm neonates is essential to improve nutritional supplements and catch-up growth.


Subject(s)
Humans , Infant, Newborn , Alanine , Amino Acids , Birth Weight , Citrulline , Gestational Age , Glycine , Leucine , Methionine , Phenylalanine , Tandem Mass Spectrometry , Tyrosine , Valine
19.
Korean Journal of Pediatrics ; : 1256-1259, 2005.
Article in English | WPRIM | ID: wpr-174020

ABSTRACT

Pulmonary atresia with intact ventricular septum (PAIVS) is rare, less than 1% of congenital heart disease. It needs a therapeutic approach according to its individual morphologic feature. Surgical treatment of valvotomy and modified Blalock-Taussig shunt or non-surgical interventional catheter balloon valvuloplasty can be used for mild to moderate hypoplasia of right ventricle. Fontan operation can be considered for less optimum morphological substrate of two ventricular repair. A 3- day-old male neonate was admitted with cyanosis and cardiac murmur. On echocardiogram, he had membranous pulmonary atresia with intact ventricular septum, normal sized tripartite right ventricle, large atrial septal defect with right-to-left shunt, small sized patent ductus arteriosus, and moderate tricuspid regurgitation. He was treated with intravenous continuous infusion of prostaglandin E1 (PGE1) at once. On the third day of hospitalization, Balloon valvuloplasty was performed. After insertion of patent ductus arteriosus stent on the tenth day, PGE1 infusion was discontinued. On the fifteenth day, he was discharged. Now, he is 9 months old and has nearly normal cardiac structure and function with 97% of percutaneous oxygen saturation.


Subject(s)
Male , Infant, Newborn , Humans
20.
Journal of the Korean Society of Pediatric Nephrology ; : 69-75, 2005.
Article in Korean | WPRIM | ID: wpr-145623

ABSTRACT

PURPOSE: The most important management of congenital hydronephrosis consists of the early diagnosis and evaluation of the pathologic abnormalities of congenital hydronephrosis. This study was conducted to investigate the different causes of hydronephrosis and its clinical outcome. METHODS: 54 live neonates who were hospitalized and diagnosed with congenital hydronephrosis at Chungnam National University Hospital from Aug. 1998 to Aug. 2003 were retrospectively analyzed. RESULTS: Hydronephrosis(renal pelvic AP diameter >5 mm) was postnatally detected in 54 cases(2.1%) among 2,539 neonates who were hospitalized from Aug. 1998 to Aug. 2003. There were three times more males than females. Additional imaging studies revealed that ureteropelvic junction obstruction was the most common postnatal diagnosis(48.7%), followed by multicystic dysplastic kidney, vesicoureteral reflux and duplication kidney with ureterocele. Spontaneous regression of hydronephrosis was revealed in 25 renal units(75.8%) of mild hydronephrosis, 14 renal units of moderate hydronephrosis and 1 renal unit of severe hydronephrosis. Operative correction were carried out in 14 renal units(70%) of severe hydronephrosis. CONCLUSION: The most common established cause of congenital hydronephrosis in this study was ureteropelvic junction obstruction. There are many cases of spontaneous regression in mild to moderate congenital hydronephrosis. Urinary tract infections occur in many neonates with hydronephrosis. Therefore, early detection and evaluation of congenital hydronephrosis and continuous follow-up at regular intervals are necessary for conservation of renal function.


Subject(s)
Female , Humans , Infant, Newborn , Male , Early Diagnosis , Follow-Up Studies , Hydronephrosis , Kidney , Multicystic Dysplastic Kidney , Retrospective Studies , Ureterocele , Urinary Tract Infections , Vesico-Ureteral Reflux
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