• Neonatal Medicine
• /
• v.18 no.2
• /
• pp.409-411
• /
• 2011

In Korea, pulmonary surfactant (PS) replacement therapy in respiratory distress syndrome (RDS) was started in 1991 since when Surfacten$^{(R)}$ was imported from Japan. At the present time, Surfacten$^{(R)}$, Newfactan$^{(R)}$, Curosurf$^{(R)}$, and Infasurf$^{(R)}$ are available in Korea. The governmental health insurance covers the expense for multiple dose treatment since 2002 and the early prophylactic treatment (BW: <1,250 g or GP: <30 wks) since 2011. We undertook a multi-institutional collective study to evaluate the outcomes of PS over past 20 years in Korea (Period-I; 1990/91, P-II; 1996, P-III; 2002, and P-IV; 2007, P-V; 2010). There were 60 RDS neonates with PS treatment in P-I (16 hospitals), 1,179 in P-II (64), 1,595 in P-III (62), 1,921 in P-IV (57), and 3,160 in P-V (72). Decreased mortality rate, defined as the percentage of neonates who died within 28 days of birth, was seen between periods, P-V vs P-I, II, III, and IV (mortality rate: 10.1% vs. 40.0%, 30.0%, 18.7%, and 14.3%). We conclude that PS therapy contributed to improve remarkable outcome in RDS neonates over the last 20 years in Korea. However, more efforts should be made to optimize PS therapy for better outcome. Multiple PS doses for relapse and poor response, early prophylactic use, and better supportive care for pre-term infants are mandatory.

• Neonatal Medicine
• /
• v.25 no.1
• /
• pp.7-15
• /
• 2018

We need to understand the outcomes into adulthood for survivors born either extremely low birthweight (ELBW; <1,000 g) or extremely preterm (EP; <28 weeks' gestational age), particularly their blood pressure and cardiovascular metabolic status,respiratory function, growth, psychological and mental health performance, and functional outcomes. Blood pressure is higher in late adolescence and early adulthood in ELBW/EP survivors compared with controls. In some studies, expreterm survivors have higher insulin and blood lipid concentrations than controls, which may also increase their risk for later cardiovascular disease. ELBW/EP survivors have more expiratory airflow obstruction than do controls. Those who had bronchopulmonary dysplasia (BPD) in the newborn period have even worse lung function than those who did not have BPD. As a group, they are unlikely to achieve their full lung growth potential, which means that more of them are likely to develop chronic obstructive airway disease in later life. Although they are smaller than term born controls, their weight gradually rises and ultimately reaches a mean z-score close to zero in late adolescence, and they ultimately attain a height z-score close to their mid-parental height z-score. On average, ex-preterm survivors have intelligence quotient (IQ) scores and performance on tests of academic achievement approximately 2/3 SD lower than do controls, and they also perform less well on tests of attention and executive function. They have similar high rates of anxiety and depression symptoms in late adolescence as do controls. They are, however, over-represented in population registries for rarer disorders such as schizophrenia and Autism Spectrum Disorder. In cohort studies, ex-preterm survivors mostly report good quality of life and participation in daily activities, and they report good levels of self-esteem. In population studies, they require higher levels of economic assistance, such as disability pensions, they do not achieve education levels as high as controls, fewer are married, and their rates of reproduction are lower, at least in early adulthood. Survivors born ELBW/EP will present more and more to health carers in adulthood, as they survive in larger numbers.

• Development and Reproduction
• /
• v.21 no.3
• /
• pp.317-325
• /
• 2017

Direct communication between neighboring cells through connexin (Cx)-based gap junction is a crucial biological manner to regulate functions of a tissue consisting of multi-cell types. The present research evaluated expressional changes of Cx isoforms in the caput epididymis of adult rat exposed to estradiol benzoate (EB) or flutamide (Flu) at the early postnatal age. A single subcutaneous administration of EB at a low-dose [$0.015{\mu}g/kg$ body weight (BW)] or a high-dose ($1.5{\mu}g/kg\;BW$) or Flu at a low-dose ($500{\mu}g/kg\;BW$) or a high-dose (5 mg/kg BW) was performed to an animal at 1 week of age. Quantitative real-time PCR analysis was employed to determine expressional changes of Cx isoforms. The transcript levels of Cxs30.3 and 37 were decreased by a low-dose EB treatment, while decreases of Cxs31, 31.1, 32, 40, and 45 transcript levels were observed with a low-dose EB treatment. The treatment of a high-dose EB resulted in expressional reduction of Cxs30.3, 31, 31.1, 37, 40, 43, and 45. The Flu treatment at a low dose caused increases of Cxs26, 37, and 40 transcript levels but decreases of Cxs31.1, 43, and 45 transcript levels. Increases of Cxs30.3, 31, 37, and 40 mRNA amounts were induced by a high-dose Flu treatment. However, exposure to a high-dose Flu produced expressional decreases of Cxs31.1, 32, and 43 in the adult caput epididymis. These observations suggest that exposure to EB or Flu at the neonatal period could lead to aberrant expression of Cx isoforms in the adult caput epididymis.

• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.20 no.1
• /
• pp.14-23
• /
• 2020

Classic galactosemia is a rare genetic disorder in Korea and the mutation spectrum in Koreans differs from that of Caucasians and non-Caucasian Americans. Classic galactosemia is considered a metabolic complication that is preventable by early detection via newborn screening and dietary treatment. In this most recent case of Korean galactosemia, the patient showed early initiation of clinical symptoms, which manifested during the neonatal period. The patient achieved normalization via diet management to correct metabolic complications. In addition, we assessed the characteristics of mutations in 25 Korean galactosemia cases via a literature review of studies associated with classic galactosemia.

• Korean Journal of Veterinary Research
• /
• v.41 no.3
• /
• pp.381-385
• /
• 2001

Neospora caninum infections have been associated with neonatal paresis as well as abortion around the world. Bovine abortion induced by N caninum was first reported in 1997 in Korea. Diagnosis of N caninum infection is usually based on histopathology and immunohistochemical detection of organism. However, often the tissues having lesion suggestive of N caninum infection were negative on immunohistochemistry. Here, we describe establishment of PCR-based diagnostic strategy for N caninum infection using DNA extracted from paraffin blocks containing the lesion. PCR was able to amplify N caninum-specific bands from the paraffin blocks containing at least moderate degree of inflammation. Compared to paraffin-blocks, DNA extracted from fresh tissues were less sensitive than that of paraffin blocks. This PCR-based method can be practically applicable for rapid diagnosis of bovine N caninum infection with high specificity and sensitivity. Based on this method, 17% of bovine abortion surveyed during a designated period was associated with N caninum infection.

• Advances in pediatric surgery
• /
• v.10 no.1
• /
• pp.17-21
• /
• 2004

Improvement in prenatal ultrasonography is leading to diagnose choledochal cyst before birth and before onset of classical symptom more frequently. But, there is a controversy about optimal timing for Cyst excision of prenatally diagnosed asymptomatic choledochal cyst. To identify the most appropriate timing for surgery in prenatally diagnosed choledochal Cysts, we analyzed 6 patients who had operation for choledochal cysts within 30days after birth at the division of Pediatric Surgery, Samsung Medical Center and Inha University School of Medicine, from June 1995 to June 2002. Males were four and females 2, the mean age at operation was 11.2 days, and the median age 8.0 days. The range of gestational ages of the antenatal diagnosis of bile duct dilatation was 24 weeks to 32 weeks, mean was 38.3 weeks, and mean birth weight was 3,298.3 g. After birth, abdominal ultrasonography, hepatobiliary scintigraphy, and magnetic resonance cholangiopancratography (MRCP) were performed. Mean age at operation was 11.2 days. All patients had the cyst excision and Rouxen-Y hepaticojejunostomy. Immediate postoperative complication was not found. During the median follow-up period of 41 months, one patient was admitted due to cholangitis, and the other due to variceal bleeding. Early operative treatment of asymptomatic newborn is safe and effective to prevent developing complications later in life.

• Clinical and Experimental Pediatrics
• /
• v.61 no.10
• /
• pp.315-321
• /
• 2018

Purpose: To investigate the effectiveness of transient intubation for surfactant administration and extubated to nasal continuous positive pressure (INSURE) for treatment of respiratory distress syndrome (RDS) and to identify the factors associated with INSURE failure in extremely premature infants. Methods: Eighty-four infants with gestational age less than 28 weeks treated with surfactant administration for RDS for 8 years were included. Perinatal and neonatal characteristics were retrospectively reviewed, and major pulmonary outcomes such as duration of mechanical ventilation (MV) and bronchopulmonary dysplasia (BPD) plus death at 36-week postmenstrual age (PMA) were compared between INSURE (n=48) and prolonged MV groups (n=36). The factors associated with INSURE failure were determined. Results: Duration of MV and the occurrence of BPD at 36-week PMA were significantly lower in INSURE group than in prolonged MV group (P<0.05), but BPD plus death at 36-week PMA was not significantly different between the 2 groups. In a multivariate analysis, a reduced duration of MV was only significantly associated with INSURE (P=0.001). During the study period, duration of MV significantly decreased over time with an increasing rate of INSURE application (P<0.05), and BPD plus death at 36-week PMA also tended to decrease over time. A low arterial-alveolar oxygen tension ratio (a/APO2 ratio) was a significant predictor for INSURE failure (P=0.001). Conclusion: INSURE was the noninvasive ventilation strategy in the treatment of RDS to reduce MV duration in extremely premature infants with gestational age less than 28 weeks.

• Clinical and Experimental Pediatrics
• /
• v.46 no.8
• /
• pp.826-830
• /
• 2003

ARCI syndrome consists of arthrogryposis, renal tubular acidosis, cholestatic jaundice and icthyosis. We experienced an ARCI syndrome case with corpus callosum hypoplasia and atrial septal defect. This case had oral feeding difficulty, multiple joint contracture, renal tubular acidosis and neurogenic muscular atrophy at neonatal period. At two months of age, icthyosis and cholestatic jaundice were diagnosed. The case was hospitalized due to pneumonia at four months of age. Corpus callosum hypoplasia and atrial septal defect were detected. The case was treated with a mechanical ventilator because pneumonia was aggravated and respiratory failure occurred. The patient expired at five months of age.

• Clinical and Experimental Pediatrics
• /
• v.53 no.8
• /
• pp.809-813
• /
• 2010

Bartter syndrome (BS) is an autosomal recessively inherited rare renal tubular disorder characterized by hypokalemic metabolic alkalosis and hyperreninemic hyperaldosteronism with normal to low blood pressure due to a renal loss of sodium. Genetically, BS is classified into 5 subtypes according to the underlying genetic defects, and BS is clinically categorized into antenatal BS and classical BS according to onset age. BS type I is caused by loss-of-function mutations in the $SLC12A1$ gene and usually manifests as antenatal BS. This report concerns a male patient with compound heterozygous missense mutations on $SLC12A1$ (p.C436Y and p.L560P) and atypical clinical and laboratory features. The patient had low urinary sodium and chloride levels without definite metabolic alkalosis until the age of 32 months, which led to confusion between BS and nephrogenic diabetes insipidus (NDI). In addition, the clinical onset of the patient was far beyond the neonatal period. Genetic study eventually led to the diagnosis of BS type I. The low urinary sodium and chloride concentrations may be caused by secondary NDI, and the later onset may suggest the existence of a genotype-phenotype correlation. In summary, BS type I may have phenotype variability including low urine sodium and chloride levels and later onset. A definitive diagnosis can be confirmed by genetic testing.

• Clinical and Experimental Pediatrics
• /
• v.54 no.2
• /
• pp.69-78
• /
• 2011

Purpose: The aim of this study was conducted to investigate the mean nRBC count in very low births weight infants (VLBWIs) and to determine the usefulness of the nRBC as an independent prognostic factors of perinatal complications in VLBWIs. Methods: This study was conducted on 112 VLBWIs who were hospitalized in the neonatal intensive care unit (NICU) of the author's hospital within the period from March 2003 to and May 2008. Based on the infants' nucleated red blood cells (nRBC) counts at birth, on the third day after birth, on the seventh day after birth, in the second week after birth, and in the fourth week after birth in the medical records, the correlation between nRBC or absolute nRBC counts with birth weight, gestational age, and other perinatal outcomes were retrospectively investigated. Results: In VLBWIs, their mean nRBC and absolute nRBC counts were showing a gradual decrease after birth, and they were consisteantly kept at low values since one week after and inversely proportional to the birth weights. The mean nRBC counts based on the stage after birth showed a significant correlation with perinatal death, necrotizing enterocolitis, and severe intraventricular hemorrhage. Conclusion: The increase in the nRBC count showed a significant correlation with having a severe intraventricular hemorrhage, necrotizing enterocolitis, and perinatal death in VLBWIs. If an increase or no decrease in the nRBC count after birth is observed, newborninfant care precautions should be required.