• 동의신경정신과학회지
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• 제20권3호
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• pp.1-13
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• 2009

Objectives : The glial cell, located in between the blood vessel and nerve cell, takes charge of the cell support, nutrition supply, elimination of body waste, and cell action. GagamSohabhwangwon(GGSH), a chinese traditional medicinal prescription has been used orally for the treatment of seizures, infantile, convulsion, stroke and so forth. This paper examines the effect of the GagamSohabhwangwon(GGSH) essential oil on cell activity and anti oxidation. Methods : MTT assay methods were employed to measure the cell activity based on the amount of the GagamSohabhwangwon(GGSH) essential oil by using primarily cultivated glial cell. In addition, this paper measured a viability of the glial cell after a protein active retarder control to confirm the multiplication of the cell and examined the cell extinction by the active oxygen, an extinction shielding effect with different amount of the GagamSohabhwangwon(GGSH) essential oil to observe anti oxidation. Furthermore, this paper measured a viability of the cell and phosphorylation(phosphorylation) of the protein which affects the multiplication of the glial cell. Results : When controlling the amount of the GagamSohabhwangwon(GGSH), there was a multiplication effect of the primary glial cell, the multiplication of the cell was dependent on the density of the GagamSohabhwangwon. The multiplication power of the primary glial cell was suppressed by PKA inhibiter (H89). In compliance with the active oxygen the extinction of the primary glial cell was dependent on the density of the GagamSohabhwangwon, there was a shielding effect of the cell extinction when GagamSohabhwangwon(GGSH) was preprocessed. When inducing the multiplication of the primary glial cell, phosphorylation of the Akt, BDNF, CREB, ERK and ERM were increased. Conclusions: Based on the results, GagamSohabhwangwon essential oil will have the effect which activates the nervous system cell and protects the cell through anti oxidation.

• Neonatal Medicine
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• 제18권2호
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• pp.404-408
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• 2011

Scimitar증후군은 우측 폐정맥이 하대정맥을 통하여 좌심방이 아닌 우심방으로 연결되는 부분 폐 정맥 환류이상을 보이면서 우측 폐의 발육부전, 우폐동맥의 발육부전 및 폐 분리증 등과 같은 동반 기형을 보이는 매우 드문 심폐기형 가운데 하나이다. 또한 선천성 항문 막힘증은 출생 후 조기에 잘록 창자 창냄술과 같은 적극적인 수술적 치료가 요구되는 항문 직장 기형의 하나로 비뇨기계 이상, 척추 이상, 기관지 식도루, 식도 폐쇄증 혹은 심혈관계 이상 등이 동반될 수 있다. 그러나, 부분 폐 정맥 환류 이상의 하나인 scimitar증후군이 항문 막힘증과 함께 동반됨을 보고한 예는 아직까지 국내외적으로 없었다. 이에 저자들은 호흡 곤란 및 선천성 편측 폐 발육부전 및 항문 막힘증을 주소로 입원한 신생아에서 진단된 scimitar 증후군 1례를 경험하였기에 보고하는 바이다.

• 패션비즈니스
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• 제7권1호
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• pp.84-100
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• 2003

The purpose of this study is to analyze the meanings of signs represented in contemporary fashion based on the Peirce's trisection theory in order to present various types of sign representation that satisfies aesthetic value and characteristics of the age of information and image. Types of signs represented in contemporary fashion can be summarized into letters, numbers, codes, pictures, logos, characters and emblems. The meanings of signs were analyzed based on the Peirce's trisection theory, resulting in representation of play, brandsymbol, eroticism and suggestion. The play was categorized into infantile and parodic. The former inspires unexpected interest and laughter from contemporary people in the age of disorder through humorous characters, cartoons and illustrations. The latter inspires laughter by parodying things and it's intention is to release the isolation of people and their relative poverty through pictures for which techniques of parody and surrealism were used. Most of the brandsymbols represented by logos, used for textile and clothing design as well as accessories are optimal signs that construct brand images. Eroticism means emotion, impulses, senses of value and sexual attraction. A desire to represent sexual attraction is an expression of eroticism and self-expression to adjust in a changing society. Suggestion tends to pursue the image of harmony with nature in a world which is pressed by material civilization. Humans represent illuminating messages through direct slogans by characters, natural objects and motives of social phenomenon in pursuit of the benefits of abundant material civilization in the structure of a highly advanced scientific civilization and industrial society.

• Pediatric Infection and Vaccine
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• 제23권1호
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• pp.72-76
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• 2016

골화석증은 골격의 경화증이 특징적으로 나타나는 드문 유전 질환으로 뼈 흡수 기전에 손상이 오며 조기 사망하는 질환이다. 반면 거대세포바이러스 감염은 가장 흔한 선천성 감염 중 하나로 빈혈, 혈소판 감소증과 간비장종대, 뇌 석회화 등이 나타날 수 있다. 심한 간비비대, 혈소판 감소증 및 저칼슘혈증과 발달지연으로 내원한 환자에서 두 가지 질환이 함께 있어 항바이러스제 치료 및 대증치료를 시행하였고, 치료 반응이 빠르게 나타나지는 않았으나 지속적인 치료 결과 대부분의 수치가 정상화 되는 것을 확인하였다. 본 증례는 골화석증 신생아에게 동반된 거대세포바이러스 감염의 첫 증례 보고로, 거대세포바이러스 감염에 대한 항바이러스제의 장기 치료로 호전된 사례이다.

• 대한구순구개열학회지
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• 제5권1호
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• pp.43-58
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• 2002

A General survey on the cleft lip and/or palate patients in Pusan National University Hospital was done. The conclusions were as follow. 1. In gender ratio, male was higher than female. 2. In the cleft type, unilateral cleft lip and palate, cleft lip and alveolus, and bilateral cleft lip and palate in higher ratio order. Left unilateral cleft lip and palate was higher than right in ratio. 3. In first hospital visiting age for dental treatment, the group from 6 years old to 12 years old was most and in that the age group for attending elementary school (about 7-8 years old) showed especially higher ratio. The large majority of patient were born in 1980's and lived in Pusan metropolitan city, KyungSangNamDo. Especially, most of them lived in the neighboring Held of Pusan National University Hospital. 4. Class III skeletal pattern and Angle's Class III molar relationship was most prevalent. Angle's Class II molar relationship showed relative higher ratio because tooth loss and malposition. 5. Primary lip closure in 3 months, secondary lip closure in 6-7 years old, palatal closure in 12-16 months and bone graft in 8-10 years old were operated mostly. Rhinoplasty and scar revision were operated in too early age so the need of infantile orthopedics must be considered. 6. Maxillary expansion and protraction were operated mostly in 8-11 years old but operating in primary dentition must be considered. 7. Tooth alignment were started mostly in mixed dentition and consideration about prosthodontic treatment and retention will be need. 8. In tooth anomaly, tooth malformation and missing were most prevalent.

• Clinical and Experimental Pediatrics
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• 제49권12호
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• pp.1358-1362
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• 2006

Niemann-Pick병은 lysosome내에 sphingomyelinase의 결핍으로 sphingomyelin이란 지질이 축적된 세포들이 간, 비장, 골수, 폐, 및 뇌 등에 침착되어 간, 비장 종대 및 신경증상을 나타내며, 상염색체 열성으로 유전되는 대사성 질환이다. 1914년 Niemann에 의해 처음 보고되어 Gaucher병의 한 변형으로 분류되어 있다가, 1927년 Pick에 의해 새로 분류되어 Niemann-Pick 병으로 명명되었다. 세계적으로도 희귀한 질환으로 국내에서는 1962년 정 등이 처음 보고한 이래 현재까지 저자가 조사한 바로 총 7례 정도가 보고되었다.저자들은 18개월 된 남아에서 임상증상 및 검사 소견으로 A 형 Niemann-Pick병으로 생각되는 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• Clinical and Experimental Pediatrics
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• 제57권7호
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• pp.310-316
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• 2014

Purpose: Prader-Willi syndrome (PWS) is a complex genetic disorder that results from the lack of paternally expressed genes in the chromosome 15q11-q13 region. This study was performed to delineate the clinical features of PWS infants and toddlers and the effects of two-year growth hormone (GH) treatment according to gender and age at the start of treatment. Methods: The clinical characteristics and the results of the GH treatment were reviewed retrospectively for 30 PWS patients diagnosed by molecular genetic testing and clinical manifestations. Results: The mean age at diagnosis with PWS was 13.7 months (2-47 months of age). All patients showed the characteristics of facial dysmorphism, including brown hair and almond-shaped eyes. Most patients showed developmental delays/mental retardation (93.3%), cryptorchidism (75%), feeding problems in infancy (73.3%), and neonatal or infantile hypotonia (66.7%). Among 30 patients, 14 PWS infants and toddlers had been treated with GH for more than two years. Two years of GH treatment resulted in an improvement in head circumference-standard deviation score (HC-SDS), body weight-SDS, insulin-like growth factor-1 (IGF-1) SDS, IGF binding protein-3 (IGFBP-3) SDS, lean body mass, and bone mineral content, especially in IGFBP-3 SDS and motor development in PWS patients younger than two years of age. There was significant increase in IGF-1 SDS and IGFBP-3 SDS among male PWS patients after GH treatment. Conclusion: Our study showed increases in IGFBP-3 SDS and an improvement in motor development among individuals under two years of age after GH treatment, and significant difference in IGF-1 SDS and IGFBP-3 SDS by gender.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제23권5호
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• pp.423-429
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• 2020

Purpose: Alimentary tract duplication (ATD) is a rare congenital condition that may occur throughout the intestinal tract. Clinical symptoms are generally related to the involved site, size of duplication, or associated ectopic mucosa. This study aimed to identify clinical implications by anatomical locations and age group and then suggest a relevant management according to its distinct features. Methods: We retrospectively reviewed the clinical data of pediatric patients who received a surgical management due to ATD. Furthermore, data including patients' demographics, anatomical distribution of the duplication, clinical features according to anatomical variants, and outcomes were compared. Results: A total of 25 patients were included in this study. ATD developed most commonly in the midgut, especially at the ileocecal region. The most common clinical presentation was abdominal pain, a sign resulting from intestinal obstruction, gastrointestinal bleeding, and intussusception. The non-communicating cystic type was the most common pathological feature in all age groups. Clinically, prenatal detection was relatively low; however, it usually manifested before the infantile period. A laparoscopic procedure was performed in most cases (18/25, 72.0%), significantly in the midgut lesion (p=0.012). Conclusion: ATD occurs most commonly at the ileocecal region, and a symptomatic one may usually be detected before the early childhood period. Surgical management should be considered whether symptom or not regarding its symptomatic progression, and a minimal invasive procedure is the preferred method, especially for the midgut lesion.

• Asian Pacific Journal of Cancer Prevention
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• 제15권3호
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• pp.1211-1217
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• 2014

Background: Cancer in infants younger than one year of age represents a unique problem with distinct epidemiological, clinical and genetic characteristics compared with older age groups. No report is yet available from Iran regarding epidemiological and survival rate of cancers diagnosed in this age group. Materials and Methods: The population under study comprised of patients which were diagnosed and admitted to Ali-Asghar hospital between years 1996-2005. In total, 287 infants were included in the retrospective descriptive survey. Patient files were evaluated for age of patient at the time of diagnosis, sex, geographical residence, consanguinity of parents, histological diagnosis, site of cancer involvement, type of therapy, date of last follow-up and cause of death (if applicable). Results: The average age at the time of diagnosis was 7.2 months old. The most frequent malignancy was retinoblastoma (44%), followed by leukemia (19%) and neuroblastoma (10%), with five-year overall survival rates of 77.7%, 41% and 90%, respectively Parents of 40 infants (13.9%) had consanguinity relationships. Conclusions: Although we cannot make any conclusions regarding the incidence of infant cancer subtypes based on this study, survival rates for major types were similar to the developed countries, which signifies strict adherence to standards of care in Ali-Asghar hospital, the main infant cancer care centre in Iran. A Childhood Cancer Registry with high-resolution data collection and also advanced genetic testing is advocated for in-depth analysis of variation in incidence and survival.

• 대한한방소아과학회지
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• 제14권1호
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• pp.197-204
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• 2000

Neuromuscular disorders are common causes of weakness and hypotonia in the infantile period and in childhood. Accurate diagnosis of specific neuromuscular disorders depends first on identification of which aspect of the peripheral neuromuscular system is affected-the motor neuron in the spinal cord, the nerve root or peripheral nerve, the neuromuscular junction, or the muscle-and then on the determination of the etiology and specific clinical entity. Spinal muscular atrophy(SMA) is the most common autosomal-recessive genetic disorder lethal to infants. The three major childhood-onset forms of SMA are now usually called type I, type II and typeⅢ. Progression of the disease is due to loss of anterior horn cells, thought to be caused by apoptosis. Diagnosis is based on the course of the illness, as well as certain changes seen on nerve and muscle biopsy and electrodiagnostic studies. More recently, our understanding of the genetics of this disorder has provided a noninvasive approach to diagnosis. We report on a 3-year-old male patient with spinal muscular atrophy type II. He had progressive muscular weakness since 18 months of age. The upper arms were slightly, and the thighs moderately atrophic. There was muscle weakness of both the upper and lower limbs, being more proximal in distribution. Electromyogram revealed a neurogenic pattern.