• Clinical and Experimental Pediatrics
• /
• v.53 no.6
• /
• pp.669-679
• /
• 2010

The risk of mortality and morbidity of patients with congenital heart defects (CHDs) is highest during neonatal period and increases when diagnosis and proper management are delayed. Neonates with critical CHDs may present with severe cyanosis, respiratory distress, shock, or collapse, all of which are also frequent clinical presentations of various respiratory problems or sepsis in the newborn. Early diagnosis and stabilization and timely referral to a tertiary cardiac center are crucial to improve the outcomes in neonates with CHDs. In this review, the clinical presentation of critical and potentially life-threatening CHDs is discussed along with brief case reviews to help understand the hemodynamics of these defects and ensure proper decision-making in critically ill patients.

• Advances in pediatric surgery
• /
• v.3 no.2
• /
• pp.164-167
• /
• 1997

Conjoined twins are one of the rarest and most challenging congenital anomalies in pediatric surgery. Successful surgical separation is difficult because it the majority of conjoined twins in is technically typical to separate shared vital organs successfully. The timing of separation is variable, but separation is usually delayed until such infants are relatively mature(i.e, 9-12 months of age). Operative survival was 50 % in the neonatal period, but 90 % in those over 4 months of age. The present case was successfully separated early beacase of cardiac problems in one of the twins. These twins were omphalopagus and only the liver was shared through a bridge. The vascular and biliary trees were independent from each other. Successful surgical separation was on 11th day of life, but one died of sepsis 18 days after operation.

• Journal of Korea Association of Health Promotion
• /
• v.1 no.1
• /
• pp.21-25
• /
• 2003

Vomiting in pediatric patient is frequently encountered problem in emergency room or outpatient clinic. In differential diagnosis, age of the patient or accompanying symptoms should be considered in the differential diagnosis. Accurate diagnosis is very important because surgical treatment is necessary in some of the conditions. Imaging diagnosis of conservative modality such as upper gastrointestinal series or colon study is still important radiological examination in the initial differential diagnosis, but recently ultrasonography offers accurate diagnosis in many situations. The cause of vomiting in pediatric are diverse according to the age group :neonatal sepsis, necrotizing enterocolitis, or hypertrophic pyloric stenosis in neonates : gastroesophageal reflux, viral enteritis, or intussusception in infant: midgut volvulus, appendicitis, metabolic disorders, or increased intracranial pressure also an be the cause. knowledge of radiological findings of normal gastrointestinal tract is important to recognize abnormalities. A discussion of radiological findings in variable surgical conditions to present as vomiting in pediatric patients is offered.

• Perinatology
• /
• v.29 no.4
• /
• pp.175-179
• /
• 2018

Pneumatocele (PC) is a thin-walled cyst of the lung that can occur at all ages and with various etiologies. However, there is no fully accepted consensus for the management of PC in a neonatal intensive care unit. Although the management of PC is generally expectant, it is difficult to decide how long conservative management should be maintained, especially under Korea's medical care environment and the parents' worry and anxiety. We report a male neonate, born at $27^{+5}weeks$ gestation, weighing 1,000 g, who had a post infectious PC caused by methicillin-resistant Staphylococcus aureus sepsis. We treated conservatively for about 100 days (roughly 14 weeks), but unfortunately after a few days of chest retraction, acute exacerbation occurred, video assisted thoracoscopic surgery (VATS) was deemed necessary and performed. The purpose of this publication is to describe the clinical course, aggravation and relief after VATS management with a review of the literature.

• Journal of Korean Neurosurgical Society
• /
• v.66 no.3
• /
• pp.316-323
• /
• 2023

Objective : Owing to advances in critical care treatment, the overall survival rate of preterm infants born at a gestational age (GA) <32 weeks has consistently improved. However, the incidence of severe intraventricular hemorrhage (IVH) has persisted, and there are few reports on in-hospital morbidity and mortality. Therefore, the aim of the present study was to investigate trends surrounding in-hospital morbidity and mortality of preterm infants with severe IVH over a 14-year period. Methods : This single-center retrospective study included 620 infants born at a GA <32 weeks, admitted between January 2007 and December 2020. After applying exclusion criteria, 596 patients were included in this study. Infants were grouped based on the most severe IVH grade documented on brain ultrasonography during their admission, with grades 3 and 4 defined as severe. We compared in-hospital mortality and clinical outcomes of preterm infants with severe IVH for two time periods : 2007-2013 (phase I) and 2014-2020 (phase II). Baseline characteristics of infants who died and survived during hospitalization were analyzed. Results : A total of 54 infants (9.0%) were diagnosed with severe IVH over a 14-year period; overall in-hospital mortality rate was 29.6%. Late in-hospital mortality rate (>7 days after birth) for infants with severe IVH significantly improved over time, decreasing from 39.1% in phase I to 14.3% in phase II (p=0.043). A history of hypotension treated with vasoactive medication within 1 week after birth (adjusted odds ratio, 7.39; p=0.025) was found to be an independent risk factor for mortality. When comparing major morbidities of surviving infants, those in phase II were significantly more likely to have undergone surgery for necrotizing enterocolitis (NEC) (29.2% vs. 0.0%; p=0.027). Additionally, rates of late-onset sepsis (45.8% vs. 14.3%; p=0.049) and central nervous system infection (25.0% vs. 0.0%; p=0.049) were significantly higher in phase II survivors than in phase I survivors. Conclusion : In-hospital mortality in preterm infants with severe IVH decreased over the last decade, whereas major neonatal morbidities increased, particularly surgical NEC and sepsis. This study suggests the importance of multidisciplinary specialized medical and surgical neonatal intensive care in preterm infants with severe IVH.

• Neonatal Medicine
• /
• v.16 no.2
• /
• pp.154-162
• /
• 2009

Purpose: Death is an important problem for physicians and parents in neonatal intensive care unit. This study was intended to evaluate the mortality rate, causes of death, and the change of mortality rate by year for infants admitted to the neonatal intensive care unit. Methods: We retrospectively surveyed the medical records of the infants who were admitted to the neonatal intensive care unit at Asan Medical Center and who died before discharge between 1998 and 2007. Gestational age, birth weight, gender, time to death and the underlying diseases related to the causes of infant deaths and obtained from the medical records and analyzed according to year. Results: A total of 6,289 infants were admitted and 264 infants died during the study period. The overall mortality rate was 4.2%. For very low and extremely low birth weight infants, the mortality rate was 10.6% and 21.4%, respectively. There was no significant change in the mortality rate during the study period. Prematurity related complications and congenital anomalies were the conditions most frequently associated with death in the neonatal intensive care unit. of the infant deaths 37.1% occurred within the first week of life. Conclusion: Even though a remarkable improvement in neonatal intensive care has been achieved in recent years, the overall mortality rate has not changed. To reduce the mortality rate, it is important to control sepsis and prevent premature births. The first postnatal week is a critical period for deaths in the neonatal intensive care unit.

• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.16 no.1
• /
• pp.47-51
• /
• 2016

Propionic acidemia (PA) is an autosomal recessively inherited disorder of the organic acid metabolism. It is caused by a deficiency of propionyl-CoA carboxylase (PCC). PCC is a heteropolymeric enzyme composed of ${\alpha}$- and ${\beta}$-subunits. The clinical symptoms of PA are heterogeneous and present vomiting, dehydration, hypotonia, and lethargy, and it can result in death. The typical presentations of neonatal onset PA are life-threatening metabolic acidosis and hyperammonemia. Here, we described a case of neonatal onset PA with mild clinical presentations. She was born to a healthy mother without complications. No significant illness was observed until nine days after birth. She started exhibiting poor oral feeding, vomiting, lethargy, and hypotonia at ten days old. Her laboratory results showed mild hyperammonemia and acidosis. The initial diagnosis was neonatal sepsis and she was treated with antibiotics. However, her clinical symptoms didn't improve. So we considered a metabolic disease. She was given nothing by mouth and intravenous hydration and nutrition support was performed. Propionylglycine and 3-hydroxypropionic acid were showed high concentrations in urine by gas chromatograph mass spectrometry (GC-MS). C3 level of acylcarnitine analysis elevated 10.4 uM/L (range, 0.200-5.00) in plasma. We took gene analysis for PA to be based on the symptoms and laboratory results. We detected PCCB gene mutation and diagnosed PA. She survived without severe neurologic defects and complications and was hospitalized only three times with upper respiratory tract infections for 7 years. We report a case of a ten days old neonate with PA presenting without severe metabolic acidosis and hyperammonemia who was effectively treated with early aggressive care and conventional methods.

• Pediatric Infection and Vaccine
• /
• v.25 no.1
• /
• pp.45-49
• /
• 2018

Group B streptococcus (GBS) is the leading cause of neonatal morbidity and mortality. Late-onset GBS disease commonly manifests as occult bacteremia or meningitis. Approximately 50% of survivors of late-onset meningitis have long-term neurologic sequelae. Cerebrovascular complications are often associated with unfavorable clinical outcomes of GBS meningitis. There have been a few reports of cerebral infarction accompanied by GBS meningitis. We report a 29-day-old girl with severe, widespread cerebral infarction due to late-onset GBS meningitis. Isolated GBS strain from this patient was serotype III, ST-19. Currently, she has cortical blindness and significant developmental delay.

• Neonatal Medicine
• /
• v.15 no.1
• /
• pp.105-110
• /
• 2008

Neonatal liver abscesses are rare, carry a high mortality rate, and are difficult to diagnose. The diagnosis of liver abscesses in the neonate cannot be established from the clinical presentation alone. Risk factors for liver abscesses in neonates are maternal infection, sepsis, umbilical venous catheterization, omphalitis, and necrotizing enterocolitis. In this report, we describe a preterm infant (32 weeks, 1,580 g) who presented with abdominal distension, respiratory difficulties, and a persistent inflammatory response in spite of broad spectrum antibiotic treatment; a large (6${\times}$5 cm) solitary pyogenic liver abscess was identified at 9 days of age. It appeared that the liver abscess had originated in the uterus and umbilical venous catheterization facilitated its spread. Percutaneous drainage under abdominal ultrasound guidance was performed and prolonged antibiotics were treated for 5 weeks, effecting a cure.

• The Journal of Korea Assosiation for Disability and Oral Health
• /
• v.3 no.1
• /
• pp.22-25
• /
• 2007

Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) characterized by severe hypoglycemia caused by inappropriate over secretion of insulin is the most common cause of hypoglycemia in early infancy. The symptoms of hypoglycemia in neonate and infancy are neonatal sepsis, respiratory difficulty, tachypnea, apnea, cyanosis, and seizure. Especially the recurrent and severe hypoglycemia within $1^{st}$ year of life is responsible for severe and irreversible brain damage. To prevent it aggressive treatment is required. Due to severe and irreversible brain damage these children frequently require anesthesia during imaging procedures such as MRI or during various dental surgical procedures. Because of frequent hypoglycemia and dental phobia in children with neurologic disorder, anesthesiologists should pay attention to patient. We report a successful anesthetic management in a patient with PHHI for dental procedures.