• Journal of Chest Surgery
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• v.16 no.3
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• pp.322-330
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• 1983

TAPVC represents 1 to 4% of all congenital cardiac defects. Generally severe and refractory cardiac failure develops in the majority of patients in the early infancy. In a small minority of patient, they are relatively asymptomatic in infancy, but symptoms will develop of necessity In the later life. Our three cases had dyspnea on exertion and cyanosis and had a history of frequent respiratory infections. All 3 cases had the typical showman configuration on roentgenograms of the chest. Current surgical therapy has greatly altered the unfavorable course of these patients after institution of extra-corporeal circulation. We have experienced three cases from June, 1973 to May, 1983, the patients were undergone complete repair with extracorporeal circulation at the Department of Thoracic and Cardiovascular Surgery in the Yonsei University. The ages ranged from 12 years to 14 years, all cases were supracardiac type of TAPVC. Only one case died due to bleeding from anastomosis site between LA and pulmonary venous trunk. In the remaining two patients with complete corrections of TAPVC, there was excellent relief of symptoms such as dyspnea and cyanosis. Their postoperative course have been good during follow-up.

• Journal of Interdisciplinary Genomics
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• v.3 no.1
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• pp.13-20
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• 2021

Developmental and epileptic encephalopathies are the most devastating early-onset epilepsies, characterized by early-onset seizures that are often intractable, electroencephalographic abnormalities, developmental delay or regression, and various comorbidities. A large number of underlying genetic variants of developmental and epileptic encephalopathies have been identified over the past few decades. However, the most thorough sequencing studies leave 60-65% of patients without a molecular diagnosis. This review explores the genetic basis of developmental and epileptic encephalopathies that start within the first year of life, including Ohtahara syndrome, early myoclonic encephalopathy, epilepsy of infancy with migrating focal seizures, infantile spasms, and Dravet syndrome. The purpose of this review is to give an overview and encourage the clinicians to start considering genetic testing as an important investigation along with electroencephalogram for better understanding and management of developmental and epileptic encephalopathies.

• Journal of the Korea Academia-Industrial cooperation Society
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• v.22 no.2
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• pp.577-586
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• 2021

This study analyzed the research trends related to parenting stress of parents with infancy. To this end, 82 papers in journals registered in the KCI from 1998 to 2019 were reviewed. The frequency and percentage were calculated and analyzed for each research period, research topic, research type, and research subject. The research results were as follows. First, from 1998 to 2019, there was a continuous increase in each period. Second, regarding the research subject, the themes related to parents were the most researched, followed by the child, parenting environment, and parent-child. Among the topics related to parents, research on the variables related to parenting beliefs and behaviors was the highest. Third, quantitative research was absolute, and no qualitative research was conducted. Among the quantitative studies, the most common test method was through a questionnaire. Fourth, regarding each research subject, the research of mothers was dominant, followed by mother-infant, parent, and parent-infant-teacher. This study is significant because it provides an opportunity to broaden the scope of research and academic interest in the parenting stress of infancy parents.

• The Journal of Pediatrics of Korean Medicine
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• v.30 no.1
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• pp.45-58
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• 2016

Objectives The purpose of this study is to investigate about chief complaints of Korean pediatrics and adolescent outpatients in the ${\bigcirc}{\bigcirc}$ university hospital for their recent trend. Methods The study was composed of 4,677 new patients aged between 1 month and 20 years who had been visited pediatrics in ${\bigcirc}{\bigcirc}$ university hospital from 2012 January to 2015 December. Results 1. Among those patients, majority was early childhood as 51.4%, followed by early childhood, late childhood, infancy, and adolescence. 2. Chief complaints in this population have been recorded in the fall the most as 29.6%, but the distribution was inconsistent. 3. To categorize, digestive-related chief complaints were the highest as 30.6%, followed by respiratory, supplemental use, growth, psychiatrics. Respiratory-related chief complaints increased dramatically in the fall. 4. In infancy and early childhood, digestive/respiratory complaints were common, whereas, growth complaints were common in late childhood and adolescence. 5. Digestive/respiratory complaints have been over 50% out of all chief complaints annually. Psychiatric and growth-related complaints have been trending up, whereas, supplemental/skin complaints have decreased every year. 6. Specifically, anorexia were the most common in digestive complaints, and rhinitis/sinusitis were top among respiratory complaints. Precocious puberty and developmental disability increased every year. Conclusions The most common chief complaints were digestive and respiratory-related, and precocious puberty and growth issues have been trending up lately. Based on this result, more studies that targets precocious puberty and growth problems are needed.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.15 no.2
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• pp.78-86
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• 2015

Purpose: Homocystinuria (OMIM#236200) is a metabolic disease caused by mutation in the CBS gene. This study was conducted to identify the clinical features and prognosis of homocystinuria as well as to find out the CBS gene mutations of the six homocystinuria patients who were receiving treatment in the Pediatric Department at Soonchunhyang University Hospital. Methods: From January 1992 to March 2015, clinical, biochemical, and genetic analyses were performed retrospectively on the six patients diagnosed with classic homocystinuria at Soonchunhyang University Hospital. Results: A total of six patients were included in this study, including three who were diagnosed with homocystinuria at the mean age of $50{\pm}22.5$ days based on their abnormal newborn screening test results. The other three were diagnosed at the mean age of 7, when they visited the hospital for evaluation of developmental delay and lens dislocation. The group diagnosed at early infancy had normal cognitive function, but the other group had varying degrees of mental retardation. Major complications were found only in the group diagnosed after infancy. CBS gene mutation was found in all the patients, and they were all non-responsive to vitamin B6 treatment. At present, all patients' diets are controlled following a methionine-free formula and they are on medication with folic acid, betaine, pyridoxine, and methylcobalamin. Conclusion: Six homocystinuria patients were monitored for up to 23 years. The group diagnosed at early infancy exhibited no major complications. Therefore, early diagnosis is crucial in the prognosis, and homocystinuria must be included in the newborn screening program.

• Neonatal Medicine
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• v.15 no.1
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• pp.75-79
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• 2008

Purpose : There are many causes of hematochezia in neonates and during early infancy. Cow's milk allergy is considered to be the most common cause of bloody stools in infants; however, cow's milk allergy is diagnosed in relatively few patients. In a significant proportion of case investigations, the etiology of hematochezia is not determined. The objectives of this study were to evaluate the clinical course and prognosis of patients with hematochezia of unknown etiology. Methods : The patients were <4-month-old infants with hematochezia in whom infectious and surgical etiologies had been excluded. We investigate retrospectively the clinical course and laboratory finding. We also perform 2-years follow-up study and evaluate prognosis of the patients. Results : In all study patients, RAST milk was negative. All patients had self-limited hematochezia. In 2-years of follow-up, the prognosis of patients was good. There were no differences at 2-year regarding allergic tendencies of the patients, as determined by the increase in total eosinophil count. Conclusion : Hematochezia in neonates and during early infancy is generally a benign and self-limiting symptom. Based on 2-years of follow-up, the prognosis of the patients is good.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.12 no.sup1
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• pp.41-45
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• 2009

Optimal nutrition in infancy and early childhood is the success behind good health, growth, and development of children. Failure to thrive may be the end point of any combination of a nutritional disorder, poor growth, and psychosocial deprivation. Hospital admission is rarely necessary and may be counterproductive. Day attendance, for investigation and observation of child-parent interaction, may be more valuable. Nutritional supplementation, together with nutritional counseling, can improve food intake and growth in children with failure to thrive.

• Journal of Chest Surgery
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• v.19 no.1
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• pp.160-164
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• 1986

Tetralogy of Fallot with absent pulmonic valve is a rare cardiac malformation. The most significant symptoms during early infancy are secondary to bronchial compression resulting from the dilated pulmonary arteries. The clinical diagnosis was confirmed by echocardiography, cardiac catheterization and cardioangiography. A case of TOF with absent pulmonic valve was successful operated upon without insertion of the pulmonic valve. The narrow pulmonic valve annulus was enlarged with a transannular pericardial patch graft. The postoperative course was uneventful.

• Journal of Chest Surgery
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• v.32 no.6
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• pp.510-517
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• 1999

Background: Total anomalous pulmonary venous connection (TAPVC) is still one of the more challenging congenital heart defects in newborns and young infants. The purpose of the study is to evaluate the early and midterm results of the surgical corrections for patients in early infancy with isolated TAPVC. Material and Method: Hospital records of 15 consecutive patients in early infancy (January 1993 to August 1998) were retrospectively reviewed. There were 8 boys and 7 girls whose ages ranged from 4 days to 3.5 months (median age 22 days). Their body weight ranged from 1.75 kg to 4.9 kg (mean 3.54 kg). The abnormal anatomical connections were supracardiac in 11, cardiac in 3, and infracardiac in 1. In 6 patients (40%), the pulmonary venous drainage was obstructive. Total circulatory arrest was used in 13 patients. Anastomosis between the common pulmonary vein and the left atrium was performed with a continuous suture technique using a fine nonabsorbable polypropylene suture through a lateral approach behind the right atrium. Result: There was one hospital death (6.5%) caused by a sepsis 17 days after the operation in a neonate who had supracardiac drainage and was dependent on a ventilator preoperatively. There were 2 late deaths. One died sudde`nly of an unknown cause at home 2.5 years after the operation and the other died of a recurrent pulmonary hypertension 3 months after the reoperation due to pulmonary venous obstruction (PVO). Two patients required reoperations because of PVO 5 months and 10 months respectively after the initial operation. Of these patients, one patient is alive at the present time with persistent pulmonary hypertension. All survivors without postoperative PVO (78.6%) were in NYHA functional class I at mean follow-up of 25.8 months (0.5∼67 months). Conclusion: Surgical correction of TAPVC in early infancy can be performed at low risk. However, there were 2 postoperative PVOs (14.3%) which had bad results. The survivors without postoperative PVO had excellent functional status.

• Journal of the korean academy of Pediatric Dentistry
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• v.49 no.3
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• pp.348-356
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• 2022

Supernumerary teeth develop from excessive proliferation and development of the dental lamina. Supernumerary teeth can cause several problems, including ectopic eruption, delayed eruption, root resorption of adjacent teeth, and diastema. Supernumerary teeth in infancy are rare and have rarely been reported. Case of a 2-day-old infant with 3 supernumerary teeth is presented here and the patient was followed up for 21 months. The erupted supernumerary tooth in the primary dentition was extracted under moderate sedation at the age of 14 months. Microcomputed tomography analysis of the extracted tooth confirmed microscopic root malformation. After extraction, the midline diastema was reduced and oral hygiene improved. Early diagnosis and prompt treatment can prevent complications of supernumerary teeth.