• Journal of The Korean Society of Inherited Metabolic disease
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• v.13 no.2
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• pp.75-80
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• 2013

Specific genetic conditions may lead to sudden unexpected deaths in infancy, such as inborn errors of fatty acid oxidation and genetic disorders of cardiac ion channels. The disease may present dramatically with severe hypoketotic hypoglycemia, Reye syndrome or sudden death, typically with a peak of frequency around 3-6 month, whilst neonatal sudden death is quite rare. When undetected, approximately 20-25% of infants will die or suffer permanent neurologic impairment as a consequence of the first acute metabolic decompensation. Meanwhile, the advent of newborn screening for metabolic diseases has revealed populations of patients with disorders of fatty acid oxidation (FAO), the most frequent of which is medium chain acyl-CoA dehydrogenase (MCAD) deficiency. Without this screening, affected individuals would likely succumb to sudden infant death syndrome (SIDS). Here we describe an overview of sudden infant death syndrome and inherited metabolic disorder.

• Clinical and Experimental Pediatrics
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• v.54 no.11
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• pp.443-455
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• 2011

Neonatal mortality rate (NMR) or infant mortality rate (IMR) are the rate of deaths per 1,000 live births at which babies of either less than four weeks or of one year of age die, respectively. The NMR and IMR are commonly accepted as a measure of the general health and well-being of a population. Korea's NMR and IMR fell significantly between 1993 and 2009 from 6.6 and 9.9 to 1.7 and 3.2, respectively. Common causes of infantile death in 2008 had decreased compared with those in 1996 such as other disorders originating in the perinatal period, congenital malformation of the heart, bacterial sepsis of newborns, disorders related to length of gestation and fetal growth, intra-uterine hypoxia, birth asphyxia. However, some other causes are on the increase, such as respiratory distress of newborn, other respiratory conditions originating in the perinatal period, other congenital malformation, diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism. In this study, we provide basic data about changes of NMR and IMR and the causes of neonatal and infantile death from 1983 to 2009 in Korea.

• Clinical and Experimental Pediatrics
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• v.58 no.6
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• pp.230-233
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• 2015

Lethargy in newborns usually indicates central nervous system dysfunction, and many conditions such as cerebrovascular events, infections, and metabolic diseases should be considered in the differential diagnosis. Nonketotic hyperglycinemia is an autosomal recessive error of glycine metabolism, characterized by myoclonic jerks, hypotonia, hiccups, apnea, and progressive lethargy that may progress to encephalopathy or even death. Cerebral sinovenous thrombosis is a rare condition with various clinical presentations such as seizures, cerebral edema, lethargy, and encephalopathy. Here, we report the case of a newborn infant who presented with progressive lethargy. An initial diagnosis of cerebral venous sinus thrombosis was followed by confirmation of the presence of nonketotic hyperglycinemia.

• Korean Journal of Social Welfare
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• v.65 no.2
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• pp.131-154
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• 2013

Although fatalities due to child abuse are frequently happened, there has been a dearth of research on the topic. The present research analyzes deaths due to child abuse in representative newspapers in order to examine incidences, causes, and characteristics of child abuse. The results showed that from 2000 to 2012 child abuse deaths occurred 141 cases. Most deaths due to child abuse were committed by single mothers, biological mothers, cohabiting women and stepmothers, and biological fathers. As regards the reasons of deaths due to child abuse, the frequency of newborn infant deaths as the result of abuse by single mother or single parent was the highest, and lack of parenting skills, postpartum depression, hardships of living and lack of financial abilities were followed. Also, subjects received extensive media coverage were crime compositions-stereotypes of child abusers, habitual and continual child abuse, lack of child abuse reporting and prevention system, and mild penalties. Finally, based on the results of the study, the implications to prevent and resolve deaths due to child abuse were discussed.

• Clinical and Experimental Pediatrics
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• v.64 no.4
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• pp.180-187
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• 2021

Background: Severe perinatal asphyxia results in multiple organ involvement, neonate hospitalization, and eventual death. Purpose: This study aimed to investigate the predictive factors of death in newborns with hypoxic-ischemic encephalopathy (HIE) receiving selective head cooling. Methods: This cross-sectional descriptive-retrospective study was conducted from 2013 to 2018 in Fatemieh Hospital of Hamadan and included 51 newborns who were admitted to the neonatal intensive care unit with a diagnosis of HIE. Selective head cooling for patients with moderate to severe HIE began within 6 hours of birth and continued for 72 hours. The required data for the predictive factors of death were extracted from the patients' medical files, recorded on a premade form, and analyzed using SPSS ver. 16. Results: Of the 51 neonates with moderate to severe HIE who were treated with selective head cooling, 16 (31%) died. There were significant relationships between death and the need for advanced neonatal resuscitation (P=0.002), need for mechanical ventilation (P=0.016), 1-minute Apgar score (P=0.040), and severely abnormal amplitude-integrated electroencephalography (a-EEG) (P=0.047). Multiple regression of variables or data showed that the need for advanced neonatal resuscitation was an independent predictive factor of death (P=0.0075) and severely abnormal a-EEG was an independent predictive factor of asphyxia severity (P=0.0001). Conclusion: All cases of neonatal death in our study were severe HIE (stage 3). Advanced neonatal resuscitation was an independent predictor of death, while a severely abnormal a-EEG was an independent predictor of asphyxia severity in infants with HIE.

• Neonatal Medicine
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• v.28 no.3
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• pp.124-132
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• 2021

Purpose: Neonatal near miss (NNM) allows for the detection of risk factors associated with serious newborn complications and death, the prevention of which could reduce neonatal mortality. This study was conducted with the objective of identifying predictors for NNM in a tertiary hospital in Bangalore city. Methods: This was an unmatched case-control study involving 120 NNM cases and 120 controls. NNM was determined using Pileggi-Castro's pragmatic and management criteria. Data was collected from in-patient hospital records and interviews of postpartum mothers. Multiple logistic regression of exposure variables was performed to calculate adjusted odds ratio (AOR) with 95% confidence interval (CI). Results: Significant predictors were maternal age ≥30 years (AOR, 5.32; 95% CI, 1.12 to 9.29; P=0.041), inadequate antenatal care (ANC) (AOR, 8.35; 95% CI, 1.98 to 51.12; P=0.032), <3 ultrasound scans during pregnancy (AOR, 12.5; 95% CI, 1.60 to 97.27; P=0.016), maternal anaemia (AOR, 18.96; 95% CI, 3.10 to 116.02; P=0.001), and any one obstetric complication (hypertensive disorder in pregnancy, diabetes in pregnancy, preterm premature rupture of membranes, prolonged labour, obstructed labour, malpresentation) (AOR, 4.34; 95% CI, 1.26 to 14.95; P=0.02). Conclusion: The predictors of NNM identified has important implications for public health policy and practice whose modifications can improve NNM. These include expanding essential ANC package to include ultrasound scans, ensuring World Health Organization recommendations of eight ANC visits, capacity building at all levels of health care to strengthen routine ANC and obstetric care for effective screening, referral and management of obstetric complications.

• Neonatal Medicine
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• v.15 no.1
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• pp.84-88
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• 2008

The Jarcho-Levin syndrome is a rare genetic disorder characterized by a short neck, short trunk, and a constricted thorax, and is due to multiple vertebral and rib defects. The small size of the thorax frequently leads to respiratory insufficiency and death in neonates or infants. This syndrome also combines with various kinds of anomalies, especially renal anomalies. We report an infant with Jarcho-Levin syndrome combined with fusion of both kidneys who was referred from a local obstetric clinic for cyanosis and respiratory difficulty.

• Clinical and Experimental Pediatrics
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• v.63 no.4
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• pp.125-132
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• 2020

Coronavirus disease-2019 (COVID-19), which started in Wuhan, China, in December 2019 and declared a worldwide pandemic on March 11, 2020, is a novel infectious disease that causes respiratory illness and death. Pediatric COVID-19 accounts for a small percentage of patients and is often milder than that in adults; however, it can progress to severe disease in some cases. Even neonates can suffer from COVID-19, and children may spread the disease in the community. This review summarizes what is currently known about COVID-19 in children and adolescents.

• Journal of Chest Surgery
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• v.18 no.4
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• pp.785-791
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• 1985

The congenital cystic adenomatoid malformation [C.C.A.M.] of the lung is a rare lesion consisted of multiple cysts in the pulmonary parenchyma lined cuboidal or columnar epithelium. The C.C.A.M. presents clinically in three ways:[1] stillborn or perinatal death, [2] progressive respiratory distress in the newborn, and [3] acute and chronic pulmonary nfections in the older infant and child. We recently experienced two cases of C.C.A.M. of the lung, and have good surgical results. The first case was 29 days old male and the second case was 16 month old female who have been suffered from severe respiratory difficulty and pulmonary infection repeatedly each other. The lobectomies were performed, and the postoperative course was uneventful.

• Clinical and Experimental Pediatrics
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• v.57 no.6
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• pp.251-256
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• 2014

Severe intraventricular hemorrhaging (IVH) in premature infants and subsequent posthemorrhagic hydrocephalus (PHH) causes significant mortality and life-long neurological complications, including seizures, cerebral palsy, and developmental retardation. However, there are currently no effective therapies for neonatal IVH. The pathogenesis of PHH has been mainly explained by inflammation within the subarachnoid spaces due to the hemolysis of extravasated blood after IVH. Obliterative arachnoiditis, induced by inflammatory responses, impairs cerebrospinal fluid (CSF) resorption and subsequently leads to the development of PHH with ensuing brain damage. Increasing evidence has demonstrated potent immunomodulating abilities of mesenchymal stem cells (MSCs) in various brain injury models. Recent reports of MSC transplantation in an IVH model of newborn rats demonstrated that intraventricular transplantation of MSCs downregulated the inflammatory cytokines in CSF and attenuated progressive PHH. In addition, MSC transplantation mitigated the brain damages that ensue after IVH and PHH, including reactive gliosis, cell death, delayed myelination, and impaired behavioral functions. These findings suggest that MSCs are promising therapeutic agents for neuroprotection in preterm infants with severe IVH.