• Journal of Chest Surgery
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• 제48권1호
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• pp.7-12
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• 2015

Background: Mitral regurgitation is one of the leading causes of cardiovascular morbidity in pediatric patients with Marfan syndrome. The purpose of this study was to contribute to determining the appropriate surgical strategy for these patients. Methods: From January 1992 to May 2013, six patients with Marfan syndrome underwent surgery for mitral regurgitation in infancy or early childhood. Results: The median age at the time of surgery was 47 months (range, 3 to 140 months) and the median follow-up period was 3.6 years (range, 1.3 to 15.5 years). Mitral valve repair was performed in two patients and four patients underwent mitral valve replacement with a mechanical prosthesis. There was one reoperation requiring valve replacement for aggravated mitral regurgitation two months after repair. The four patients who underwent mitral valve replacement did not experience any complications related to the prosthetic valve. One late death occurred due to progressive emphysema and tricuspid regurgitation. Conclusion: Although repair can be an option for some patients, it may not be durable in infantile-onset Marfan syndrome patients who require surgical management during infancy or childhood. Mitral valve replacement is a feasible treatment option for these patients.

• 대한가정학회지
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• 제43권12호
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• pp.79-96
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• 2005

The purpose of this study is to explore the conceptions of children and their activities in everyday lives during the late period of the Goryo Dynasty. The historical method was used and the materials for analysis were the literatures written from the late thirteenth century to the fourteenth century in the Goryo Dynasty, such as 'the Ikjaijip'(익재집), 'Mokeunjip'(목은집), 'Yangchonjip'(양촌집). According to the results, the perspective on the nature of a chad was that children were inherently good with the true character which was given from natural laws and that each child was born with different natural endowment. Also, the notions on child development were that the infancy was a pure and perfect period which us not contaminated by temperament and worldly desires, instead of the developmental model that childhood is as a stage and the child must develop from the state of incompetence to the state of maturity. In addition, there were the principles for development that were keeping the innate ideal state and fostering it from the earliest time as infants were born with innate ideal state or ability. The concepts of children's characteristics were considered that they were inclined to make much noise and movement, but to have the plain and straightforward expressions and consistency in their feelings and everyday activities. And intelligent abilities for learning were often considered as desirable traits. In everyday lives, children did the household chores such as caring for livestock and silkworms or embroidering. Among their games were exploring activities, free plays using natural materials, pretend-plays, bamboo-stilts riding, and Yutnori.

• Journal of Korean Neurosurgical Society
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• 제41권4호
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• pp.248-251
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• 2007

Atlantoaxial rotatory dislocation [AARD] is an uncommon disorder of childhood in which clinical diagnosis is generally difficult and often made late. It is very rare in adults because of the unique biomechanical features of the atlantoaxial articulation. We report a case of post-traumatic AARD in an adult. Reduction was difficult to obtain by skull traction and gentle manipulation. Therefore, the patient was treated surgically by an open reduction, transpedicular screw fixation, and posterior C1-2 wiring with graft. The normal atlantoaxial relation was restored with disappearance of torticollis. Postoperatively, the patient remains neurologically intact and has radiographic documentation of fusion. Atlantoaxial transpedicular screw fixation can be one of the treatment options for the AARD.

• 대한유전성대사질환학회지
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• 제13권1호
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• pp.20-29
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• 2013

Inborn error of metabolism usually presents with a constellation of clinical pictures involving multiorgan systems. Because of its rarity and clinical diversity, it is difficult to make diagnosis accurately and efficiently. Many inborn error of metabolism shows predominantly hepatic symptoms and signs. The onset of symptoms is also varying depending the disease. The onset might be even prenatal, either neonatal or infantile, and late childhood. The major manifestation patterns are jaundice or cholestasis, hepatomegaly with or without splenomegaly, hypoglycemia and acute or chronic hepatocellular dysfunction. Based on pronounced hepatic symptoms and onset of symptoms, differential diagnosis can be more easily made with subsequent further laboratory investigation. In this review paper, major inborn error of metabolism with hepatic symptoms are described from the perspective of mode of clinical presentations.

• 아동학회지
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• 제30권5호
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• pp.103-117
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• 2009

This study examined patterns of delinquent behavior trajectory from late childhood to early adolescence and examined relationships between patterns of trajectory and individual, family, and school factors. Youth delinquent behavior trajectories were examined by mixed growth models using data from the 2nd to 5th year surveys of the Seoul Panel Study of Children. Relationships between patterns and effect factors were examined by multinominal logit models. Four patterns emerged: non-delinquency (80%); rapidly accelerating delinquency (3.3%); decelerating delinquency (6.0%); and moderately accelerating (10.7%) groups. Contacts with a delinquent peer group had persistent effects on more serious delinquent behavior trajectories. Increased levels of self-esteem and school achievement prevented increase in delinquent behaviors; close relationships with parents and parental supervision caused decrease in delinquent behaviors.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제44권4호
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• pp.198-203
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• 2018

Dyke-Davidoff-Masson syndrome is a non-inherited rare condition that presents during childhood and is characterized by seizures, hemiplegia, mental retardation, cerebral hemiatrophy, calvarial thickening, and hyperpneumatization of the frontal sinuses. The present article highlights a case of a 12-year-old male child with additional clinical findings of $caf{\acute{e}}$-au-late pigmentation and ocular lipodermoid. This is the first case report of DykeDavidoff-Masson syndrome to describe oral manifestations, such as unilateral delayed eruption of teeth, hypoplasia, and taurodontism, which could be unique and characteristic of this condition. Oral health care providers and physicians should be aware of these oral observations as dental referrals could warrant early dental prophylactic care and can be useful in diagnosing the possible time of injury and type of Dyke-Davidoff-Masson syndrome.

• Advances in pediatric surgery
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• 제14권1호
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• pp.75-82
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• 2008

Neuroblastoma arises from the primitive neural crest cells, and is a common malignancy in childhood. The clinical features are characterized by biological heterogeneity. Neuronal degeneration and differentiation occur in some patients. However treatment in the high risk group accounting for approximately half, has not been satisfactory despite a multimodal approach. Therefore, effective treatment is determined by the risk group of prognostic factors, such as age at diagnosis, stage of disease, pathological finding and N-myc amplification. Neuroblastoma can be diagnosed prenatally, which suggests its origin during the normal embryogenesis. Recent knowledge of molecular biology, such as Trk genes, and the concept of cancer stem cells have given us some improved understanding on this disease. Currently, targeted therapies based on the molecular biology of neuroblastoma are under investigation and increasing survival rate and decreasing late complications could be appreciated.

• Journal of Chest Surgery
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• 제16권3호
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• pp.310-315
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• 1983

The combined anomaly of pulmonary stenosis with atrial or ventricular septal defects is usually associated with decreased pulmonary blood flow and right to left shunt, and result in generalized cyanosis. Non-cyanotic pulmonary stenosis patients have generally been considered to have isolated pulmonary stenosis with intact septa. We are going to report a case of pulmonary stenosis with septal defects who have no frank cyanosis at rest because of the predominant intracardiac shunt from left to right. Recently, we managed surgically a case of pulmonary valvular stenosis combined with secundum type atrial septal defect, type II ventricular septal defect, and patent ductus arteriosus. The clinical manifestations of this patient were exertional dyspnea, frequent upper respiratory infection, chest discomfortness and lethargy since late childhood and these had been progressively aggravated. Pulmonary valvular stenosis, atrial septal defect and ventricular septal defect were closed through simply right atriotomy and patent ductus arteriosus through pulmonary arteriotomy. Immediate postoperative course was uneventful and one year follow up is excellent.

• Journal of Genetic Medicine
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• 제9권1호
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• pp.31-34
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• 2012

Spondyloepiphyseal dysplasia tarda (SEDT) is an X-linked skeletal dysplasia. Patients show disproportionate short stature with short trunk and barrel-shaped chest, which usually become pronounced in late childhood. The radiologic findings are characterized by narrow intervertebral disc spaces and moderate epiphyseal dysplasia of long bones. Here we report a case of SEDT with a novel frameshift mutation in TRAPPC2, the disease-causing gene of SEDT. This is the first Korean report with SEDT confirmed by genetic testing.

• 한방안이비인후피부과학회지
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• 제16권1호
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• pp.33-41
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• 2003

Infantile atopic dermatitis(AD) may be developed by food allergens due to immature intestinal epithelium and its mechanism, which may have no clear-cut evidence, is thought to be IgE mediated immediate and late phase hypersensitivity. It is not easy to diagnose AD caused by food allergens exactly so it is likely to be underestimated more than it be. But we must consider it as a major factor of not only infantile AD but childhood and adult AD. We can see similar theory at previous Oriental medicine. Allergens can be transmitted to a fetus through the placenta and infantile AD is inflammatory condition by food allergens and immature function of intestines. So we must consider those factors at infantile AD treatment. We expect a new model of infantile AD treatment combining the conventional therapy with the diet therapy based on the Oriental medical theory.