• Clinical and Experimental Pediatrics
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• 제56권7호
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• pp.275-281
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• 2013

Temporal lobe epilepsy (TLE) is the most common type of medically intractable epilepsy in adults and children, and mesial temporal sclerosis is the most common underlying cause of TLE. Unlike in the case of adults, TLE in infants and young children often has etiologies other than mesial temporal sclerosis, such as tumors, cortical dysplasia, trauma, and vascular malformations. Differences in seizure semiology have also been reported. Motor manifestations are prominent in infants and young children, but they become less obvious with increasing age. Further, automatisms tend to become increasingly complex with age. However, in childhood and especially in adolescence, the clinical manifestations are similar to those of the adult population. Selective amygdalohippocampectomy can lead to excellent postoperative seizure outcome in adults, but favorable results have been seen in children as well. Anterior temporal lobectomy may prove to be a more successful surgery than amygdalohippocampectomy in children with intractable TLE. The presence of a focal brain lesion on magnetic resonance imaging is one of the most reliable independent predictors of a good postoperative seizure outcome. Seizure-free status is the most important predictor of improved psychosocial outcome with advanced quality of life and a lower proportion of disability among adults and children. Since the brain is more plastic during infancy and early childhood, recovery is promoted. In contrast, long epilepsy duration is an important risk factor for surgically refractory seizures. Therefore, patients with medically intractable TLE should undergo surgery as early as possible.

• Childhood Kidney Diseases
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• 제13권2호
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• pp.99-117
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• 2009

당뇨병성 신병증은 최근 우리나라를 포함한 서구 사회에서는 만성 신부전의 가장 많은 원인 중 하나이며, 꾸준히 증가추세이다. 조직학적으로는, 사구체, 세뇨간질, 소동맥 등 신장의 주요 부위에 병변이 나타나는데, 특징적으로 사구체 기저막의 비후와 족세포의 변화와 함께 사구체 경화, 소동맥의 유리질 경화와 세뇨간질 부위에 섬유화 등이 나타난다. 당뇨병성 신병증은 혈역학적 인자들과 대사성 인자들 간의 복잡한 상호작용의 결과로 일어난다. 고혈당은 혈류역학적 요소로 고혈압과 함께 사구체내압의 증가와 혈관활성물질의 활성화에 함께, 비혈역학적 경로들, 예를 들면, 후기 당화합 최종생성물 생성, 세포 내 신호전달체계와 물질, 시토카인, 산화 스트레스 등 다양한 원인들을 활성화시킨다. 이러한 대사적, 혈류역학적인 인자들은 신장의 알부민 투과성을 증가시키고 세포 외 기질의 축적을 일으키며 결과적으로 증가된 단백뇨, 사구체경화와 세뇨관간질의 섬유화를 일으키게 된다.

• Asian Pacific Journal of Cancer Prevention
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• 제17권3호
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• pp.1201-1207
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• 2016

Background: Excessive exposure to the sun during childhood is strongly associated with the development of skin cancer in the future. The only way to prevent the development of skin cancer is to protect against ultraviolet radiation, which can be achieved through strategic awareness during childhood and adolescence. Objective. The aim of this work was to evaluate the impact of educational activities for rural and urban students to promote the use of sunscreens and prevent skin cancer. Materials and Methods: This study was carried out with students (9-12 years) of rural (n=70) and urban (n=70) schools in Rio Grande do Sul state, Brazil. The educational interventions were lectures and games. The impact of this strategy was evaluated through the application of a questionnaire before and after the interventions. Results: Before the intervention, it was found around 50% of rural and urban students were not aware of the damage caused by sun exposure, often exposing themselves to UV radiation without use sunscreen ( ~ 25 %) and at the most critical times of the day/year. After the lectures we observed an improvement in the behavior of the students with regard to sun exposure and knowledge about skin cancer. Conclusions: The results of this study emphasize the importance of prevention strategies for skin cancer and promoting the use of sunscreens based educational strategies. The interventions were of great value in relation to disseminating knowledge on the subject.

• Asian Pacific Journal of Cancer Prevention
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• 제14권2호
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• pp.1101-1106
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• 2013

Background: Osteosarcoma is the most common primary bone tumor in childhood and adolescence. Carboplatin, a platinum-derived agent, is used as neoadjuvant chemotherapy for pediatric osteosarcoma because of its anti-tumor activity and had low toxicity as compared to cisplatin. Objective: To determine demographic data, prognostic factors and outcome of childhood osteosarcoma treated with a carboplatin-based chemotherapeutic protocol at Chiang Mai University. Method: A retrospective analysis was conducted on 34 osteosarcoma patients aged less than 18 years and treated between 2003 and 2011. Results: Overall limb-salvage and amputation rates were 23.5% and 70.6%, respectively. With the mean follow-up time of 29.5 months (1.5-108.9), the Kaplan-Meier analysis for 3-year disease-free survival (DFS) and 3-year overall survival (OS) were $20.2{\pm}7.7%$ and $47.1{\pm}9.5%$ respectively. Patients who had initial pulmonary metastasis were at significantly greater risk for developing recurrence (p=0.02, OR=7; 1.2-40.1) and had a tendency to have lower 3-year OS compared to those without initial pulmonary metastasis ($28.1{\pm}13%$, $63.1{\pm}12.3%$, respectively, p=0.202). On univariate analysis, age at diagnosis >14 years and patients who were declined surgery were significantly associated with lower 3-year OS (p=0.008 and <0.05, respectively). However, age at diagnosis, sex, tumor size and histological subtypes were not found to significantly affect recurrence or survival. Conclusions: In our study, the survival rate was far lower than those reported from developed countries. These might indicate the ineffectiveness of carboplatin in combination with doxorubicin as frontline treatment of pediatric osteosarcoma, especially in those with initial pulmonary metastasis. Refinement in risk and treatment stratification and dose intensification for pediatric osteosarcoma constitutes a future challenge to improve outcomes, especially in metastatic patients who may need a more intensive regimen.

• 한국가구학회지
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• 제27권2호
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• pp.111-121
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• 2016

In the structure of modern houses, a living room is the space most frequently used by family members. In a living room, they rest, talk together, watch TV, study, read books and enjoy hobbies. A living room is furnished with a TV cabinet, a storage closet, a sofa, a bookshelf, a desk and etc. depending on the purposes of activities in it, which can be different in each house. Furniture is the required tool for indoor architectural space and humans to lead a life. In most families, a man and a woman marry each other and become man and wife, go through the honeymoon phase, give birth to one or two children and become a family with three or four members. According to the children's growth cycle from birth to infancy, early childhood, childhood, adolescence, youth and adulthood, furniture layout and kinds of furniture in a living room change. Depending on the family life cycle, most parents of young children try to help their children to form good habits of reading books and studying for their future. As for the environmental elements interrupting reading and studying, watching TV and using smart phones excessively are being regarded as problems. As the number of two-income families is increasing in this era when many women are participating in the social activities with the government's encouragement, children are having more time.

• 감성과학
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• 제9권4호
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• pp.307-319
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• 2006

본 연구에서는 정서능력과 뇌반구 기능이 문제행동에 미치는 영향을 밝히는 것이 주된 목적이었다. 남자 중학생 1,050명을 연구의 대상으로 하여 정서능력척도검사(Trait Meta-Mood Scale), 뇌 정보처리 특성 검사(Brain Preference Indicator Test), 그리고 공격성과 비행은 한국판 아동자기보고 검사(Korea Youth Self Report-Child Behavior Check List)를 사용하여 조사 연구하였다 연구 대상은 초기청소년기에 해당하는 남녀 중학생 1,479명을 표집하였다. 연구결과 문제행동을 많이 가진 집단은 보통 아동들에 비해 정서능력 요인중 정서의 명확성이 유의미하게 낮았으며, 자신에 대한 정서 주의는 많이 기울이는 것으로 나타났다. 뇌정보 처리에서는 주로 우반구 우세적 정보처리를 많이 하는 것으로 나타났다 뇌정보처리 특성과 정서 명확성 그리고 정서주의가 문제행동의 하위 요인인 공격성향과 비행성향 모두 유의미하게 설명하는 것으로 나타났다. 반면, 성별과 정서개선, 성별과 정서명료의 상호작용효과가 나타나, 이 두 요인이 공격성과 비행에 영향을 주는 효과가 남자와 여자학생 간에 다르게 나타났다.

• 대한유전성대사질환학회지
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• 제13권1호
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• pp.48-53
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• 2013

Tyrosinemia I (fumarylacetoacetate hydrolase deficiency) is an autosomal recessive inborn error of tyrosine metabolism that produces liver failure in infancy or a more chronic course of liver disease with cirrhosis, often complicated by hepatocellular carcinoma in childhood or early adolescence. We studied a 37-year-old woman with tyrosinemia I whose severe liver disease in infancy and rickets during childhood were resolved with dietary therapy. From 14 years of age, she resumed unrestricted diet with the continued presence of the biochemical features of tyrosinemia, yet maintained normal liver function. In adult years, she accumulated only a small amount of succinylacetone. Despite this evolution to a mild biochemical and clinical phenotype, she eventually developed hepatocellular carcinoma. Her fumarylacetoacetate hydrolase genotype consists of a splice mutation, IVS6-1G>T, and a novel missense mutation, p.Q279R. Studies of resected liver revealed the absence of hydrolytic activity and immunological expression of fumarylacetoacetate hydrolase in tumour. In the non-tumoral areas, however, 53% of normal hydrolytic activity and immunologically present fumarylacetoacetate hydrolase were found. This case demonstrates the high risk of liver cancer in tyrosinemia I even in a seemingly favorable biological environment. In this study of tyrosinemia I, Case 2 with negative succinylacetone accumulation and the recovery of acute liver failure was compared with Case 1. Diet restriction and NTBC treatment are crucial to prevent hepatocellular carcinoma until liver transplant can take place and cure the condition. Further studies are needed to examine cases where liver cancer did not result despite clinical symptoms/signs of tyrosinemia type I.

• 대한한방소아과학회지
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• 제19권2호
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• pp.197-213
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• 2005

Objective : The purpose of this study was to investigate chief complaints of pediatric outpatients .Fin the oriental hospital and search for a trend of patients and their symtoms. Method : The study was composed of 2,915 new patients aged between 0 and 20 who had been visited pediatrics in ${\bigcirc}{\bigcirc}$ university oriental hospital from 2001 to 2004. Results : The percentage of new patients decreased every year with 30.7% in 2001, 25.5% in 2002, 24.1% in 2003, 17.8% in 2004. The age distribution showed 9.9% in infancy patients, 62.6% in early childhood, 16.8% in late childhood, 10.6% in adolescence. The percentage of infants decreased every year with 13.3% in 2001, 9.4% in 2002, 8.8% in 2003, 7.1% in 2004. The percentage of adolescents increased every year with 8.0% in 2001, 9.6% in 2002, 12.8% in 2003, 13.5% in 2004. Systemic division of chief complaints was respiratory symtoms(37.6%), general symtoms(26.1%), digestive symtoms(19.2%), skin symtoms(6.8%), nervous and mental symtoms (6.1%), urinary symtoms(2.2%), musculoskeletal symtoms(1.3%) and others(0.7%) in order of frequency. The main chief complaints were nasal mucus/obstruction(17.5%), weakness(16.9%), cough(12.8%), loss of appetite(9.8%), inappropriate growth(4.3%), atopic skin problem(3.4%), repeated common cold(2.4%), abdominal pain(2.4%), vomiting (2.2%), diarrhea(2.0%) and fever (1.9%). Loss of appetite, inappropriate growth and repeated common cold increased every year. Diarrhea and convulsion decreased every year. Conclusions : The two main causes of visit of patients were respiratory problems and improvement of general condition. More data of patients should be accumulated for further study. Not only analysis according to chief complaint, but also analysis according to diagnosis will accomplish more aaccurate information about disease of childrens in the future.

• Journal of Genetic Medicine
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• 제17권1호
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• pp.39-42
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• 2020

The Senior-Loken syndrome was first described in 1961 as an oculo-renal disease consisting of familial juvenile nephronophthisis and Leber congenital amaurosis. It is a rare autosomal recessive disorder with a prevalence of 1:1,000,000 caused by mutations in nine genes (NPHP 1-8 and NPHP 10). Ocular manifestations (e.g., photophobia, nystagmus, and extreme hyperopia) occur within the first few years of life while renal manifestations (e.g., formation of multiple cysts impairing kidney function and end-stage renal disease) appear in late childhood to adolescence. Here, we report a case of a Filipino male presenting with rotatory nystagmus and progressive deterioration of vision since childhood. He had congenital amaurosis and juvenile nephronophthisis that progressed to end stage renal disease by age 19. All laboratory and imaging findings were consistent with chronic kidney disease. Molecular genetic testing of ciliopathy-related genes was performed revealing a homozygous mutation in exon 11 of the IQCB1/NPHP5 gene, c.1090C>T (p.Arg364^⁎). This sequence change created a premature translational stop signal resulting in a truncated protein product, nephrocystin-5 and its consequent loss of function. His symptoms eventually improved with initiation dialysis. The prognosis of Senior-Loken syndrome remains dismal and a high index of suspicion, early diagnosis and timely intervention of renal complications are warranted.

• 한국가족복지학
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• 제23호
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• pp.43-70
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• 2008

본 연구는 지적 장애인을 돌보는 어머니의 사회적 지지가 돌봄 자기효능감에 주는 영향을 분석하였으며, 이 영향이 장애인 자녀가 속한 생애주기에 따라 달라지는가를 탐색하였다. 530명의 사례에 대한 분석 결과, 전체집단에서 어머니가 지각한 사회적 지지가 많을수록 돌봄 자기효능감이 높은 것으로 나타났다. 그런데 자녀 생애주기의 조절효과를 살펴본 결과, 아동의 어머니 집단에 비교해 청소년의 어머니 집단과 성인의 어머니 집단에서 효과의 크기가 통계적으로 유의하게 달랐다. 각 집단에 대한 분석 결과, 아동의 어머니 집단에서는 사회적 지지가 돌봄 자기효능감에 영향을 주지 않은 반면, 청소년의 어머니 집단과 성인의 어머니 집단에서는 사회적 지지가 돌봄 자기효능감에 영향을 주는 것으로 나타났다. 본 연구를 통해 장애인 가족 연구에서 생애발달적 관점이 지지되었으며, 실천적으로는 청소년기와 성인기의 자녀를 둔 장애인 가족에 대한 사회적 지지의 중요성이 더욱 강조되어야 할 것으로 생각되었다.