• Title/Summary/Keyword: 발달장애청소년

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Expression of Nerve Growth Factor during Urinary Bladder Development (방광 발달과정에서 Nerve Growth Factor의 발현)

  • Lee, Kyoung Eun;Hong, Chang Hee;Kang, Hee Jung;Kim, Dug Ha
    • Clinical and Experimental Pediatrics
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    • v.48 no.4
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    • pp.411-415
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    • 2005
  • Purpose : This study was performed to determine the developmental expression pattern of nerve growth factor(NGF) in the urine of healthy children. It was hypothesized that NGF may contribute to the development of the spinobulbospinal micturition reflex that represents the adult micturition pattern. Methods : Voided urine was collected in 60 healthy children during the first 5 years of life(0-1 month, n=10; 1 month-1 year, n=10; 2 years, n=10; 3 years, n=10; 4 years, n=10). The urinary NGF was analyzed by using an enzyme linked immunosorbent assay. Results were normalized based on creatinine or total protein in urine. Results : NGF was significantly greater among neonates compared to other age groups(P<0.05). NGF levels declined during 1 month to 1 year and increased by age 2 years. NGF levels at age 3 years were less than in neonates examined. When comparing the NGF levels according to continence in children over 1 year old, NGF levels were significantly lower in children with continence than in children with incontinence(P<0.05). Conclusion : These data suggest that NGF is involved in the achievement of continence and in mechanisms of bladder nerve growth and in the reorganization of bladder reflex pathway.

Improved survival rate with decreased neurodevelopmental disability in extreme immaturity (초극소저출생체중아의 생존율 향상에 따른 장기 신경발달 장애의 감소)

  • Jeon, Ga Won;Kim, Myo Jing;Kim, Sung Shin;Shim, Jae Won;Chang, Yun Sil;Park, Won Soon;Lee, Mun Hyang
    • Clinical and Experimental Pediatrics
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    • v.50 no.11
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    • pp.1067-1071
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    • 2007
  • Purpose : The aim of this study was to determine whether improved survival of extremely low birth weight infants (ELBWI) was associated with decreased neurodevelopmental disability later in life, and also to identify the factors influencing this disability. Methods : ELBWI admitted to the neonatal intensive care unit of Samsung Medical Center, survived, and followed up until the corrected age of 18 months were enrolled. They were divided into two groups according to admission time: period I (1994-1999, n=36) and period II (2000-2004, n=98). Clinical data were collected retrospectively from the medical records. Results : Survival rates increased from 60.0% to 74.7%, cerebral palsy rates decreased from 22.2% to 8.2% and catch-up growth rate increased from 25.0% to 51.0% during period I and II. Despite less gestational age and birth weight, ELBWI during period II had less periventricular leukomalacia (PVL), sepsis and bronchopulmonary dysplasia compared to period I. The highest risk factors for cerebral palsy were intraventricular hemorrhage (IVH) (${\geq}$Grade III), failure of catch-up growth and PVL. Conclusion : In summary, improved viability was associated with decreased neurodevelopmental disability in ELBWI. Improved neonatal care with resultant decrease in PVL and IVH, and better nutritional support seem to be primarily responsible for this improved outcome.

Organic Acidopathies as Etiologic Diseases of Developmental Delay in Korean Childhood and Adolescent Age Group (한국인 소아청소년기 발달지연의 원인질환으로서의 유기산대사이상질환)

  • Lee, Jong Yoon;Lee, Ye Seung;Choi, Joong Wan;Bae, Eun Joo;Park, Won Il;Oh, Phil Soo;Lee, Hong Jin
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.12 no.2
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    • pp.85-93
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    • 2012
  • Purpose: Developmental delay is caused by very diverse etiologic diseases. Most chronic disorders has some influence on development. Chronic or acute disorders of CNS are main etiologic diseases of developmental delay. Up to now, over 60 diseases are included in organic acidopathies and most of them causes acute or chronic recurrent CNS damage and developmental delay. We have done this study to find out the importance of organic acidopathies causing developmental delay in Korean childhood and adolescent patients. Method: Retrograde analysis for 738 patients with developmental delay whose clinical informations are available and have done urine organic acid analysis for 5 years period, between Jan. 1st 2007 to Dec. 31th 2011. Statistical analysis was done with Student's t test using SPSS. Result: Out of 738 patients, 340 patients (46.1%) showed abnormalities on urine organic acid analysis. The most frequent disease was mitochondrial respiratory chain disorders (MRCD) (253, 34.3%), followed by ketolytic defects(39, 5.3%), 3-hydroxyisobutyric aciduria (26, 3.5%), glutaric aciduria type II (8, 1.1%), pyruvate dehydrogenase deficiency (3, 0.4%), 3-methylglutaric aciduria (2, 0.3%), glutaric aciduria type I (2, 0.3%), ethylmalonic aciduria (1, 0.15%), methylmalonic aciduria (1, 0.15%), HMG-CoA lyase deficiency (1, 0.15%), 3-methylcrotonylglycinuria (1, 0.15%), fatty acid oxidation disorders(1, 0.15%) and FAOD (1, 0.15%). Conclusion: Mitochondrial disorders are most frequent etiologic disease on all age group, followed by ketolytic defects and various organic acidopathies. The number and diversities of organic acidopathies emphasize meticulous evaluation of basic routine laboratory examinations and organic acid analysis with initial sample on every developmental patient.

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A CASE OF SEVERELY SELF-INJURED CHILD ASSOCIATED WITH PHYSICAL ILLNESS (신체 질환과 연관된 심한 자해로 입원한 아동 보고 1례)

  • Hong, Kang-E;Jeon, Seong-Ill
    • Journal of the Korean Academy of Child and Adolescent Psychiatry
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    • v.7 no.2
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    • pp.258-266
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    • 1996
  • Self-injurious behaviors are commoly founded in the case of the developmentally impaired, such as mental retardation and autistic disorder. These behaviors are primary serious sources of distress for both child and their parents, another obstacle to overcome within the family and society. The author has a case that a child, had never before shown self-injurious behaviors. He abruptly started to injury his face and heel. The beginnings of these harmful behaviors are associated with symptoms of physical illness, such as fever, chills and general aches. The self-injured wounds were very severe. After the patient was treated with haloperidol and improved his physical conditions, self-injurious behaviors disappeared. The author reports the child's clinical process, characteristics of self-injurious behaviors, and discuss the treatment factors, along with a literature review.

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ATTACHMENT-BASED INTERVENTION (애착장애의 치료)

  • Noh, Kyung-Sun;Lee, So-Hee
    • Journal of the Korean Academy of Child and Adolescent Psychiatry
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    • v.15 no.2
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    • pp.123-131
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    • 2004
  • We reviewed four types of Attachment intervention, 1) programs that seek to enhance sensitivity, 2) programs designed to alter parents' representations, 3) programs that provide and enhance social support, and 4) programs designed to enhance maternal mental health and well-being, and Project STEEP(Steps Toward Effective and Enjoyable Parenting). We have learned from the these studies that the emotional sensitivity and responsivity are very important factors for the development and treatment of Attachment relationships and the needs of the parents should also be addressed. Our recommendation for future attachment intervention is to design programs 1) that have the flexibility to meet participants' needs, 2) that involve other important people in the participants' lives, 3) that focus on fostering individual strengths, 4) that begin early, and 5) that are intensive and sufficiently long.

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A Case of Ring Chromosome 20 with Mental Retardation and Epilepsy (정신 지체와 간질을 동반한 20 환(Ring) 염색체 증후군 1례)

  • Jung, Yeon Kyung;Lee, Gyeong Hoon
    • Clinical and Experimental Pediatrics
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    • v.48 no.1
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    • pp.108-111
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    • 2005
  • Ring chromosome 20 mosaicism [r(20)] is a rare chromosomal anomaly associated with minor dysmorphism, mental retardation, autistic behavior, and intractable epilepsy. The proposed mechanism of ring formation is breakage of both short and long arms of a chromosome with subsequent end-to-end fusion. We encountered an 18-month-old boy who presented with developmental delay and mental retardation with seizure episodes, but showed normal brain magnetic resonance imaging. Chromosome study from peripheral blood showed 46,XY, r(20)(p13q13.3) karyotype. The authors report a case of ring chromosome 20 with mental retardation and epilepsy, with a review of the literature.

Motor delay : cerebral palsy (운동발달 장애)

  • Park, Ho Jin
    • Clinical and Experimental Pediatrics
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    • v.49 no.10
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    • pp.1019-1025
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    • 2006
  • Motor delay, when present, is usually the first concern brought by the parents of children with developmental delay. Cerebral palsy that is the most common motor delay, is a nonspecific, descriptive term pertaining to disordered motor function that is evident in early infancy and is characterized by changes in muscle tone, muscle weakness, involuntary movements, ataxia, or a combination of these abnormalities. A wide range of causative disorders and risk factors have been identified for cerebral palsy, and broadly classified into 5 groups; perinatal brain injury, brain injury related to prematurity, developmental abnormalities, prenatal risk factors, and postnatal brain injury. Delay in attaining developmental milestones is the most distinctive presenting complaint in children with cerebral palsy. A detailed history and thorough physical and neurologic examinations are crucial in the diagnostic process. The clinician should be cautious about diagnostic pronouncement unless the findings are unequivocal. Several serial examinations and history review are necessary. All children with cerebral palsy should undergo a neuroimaging study, preferably MRI, because an abnormality is documented on head MRI(89%) and CT(77%). The high incidence rates for mental retardation, epilepsy, ophthalmologic defects, speech and language disorders and hearing impairment make it imperative that all children with cerebral palsy be screened for mental retardation, ophthalmologic and hearing impairments, and speech and language disorders; nutrition, growth, and swallowing also should be closely monitored.

A Study on Secondary School Students' Current State on Harmful Information on the Internet and Educational Plan (인터넷 유해정보 실태 및 중등학생에 대한 교육적 보호방안 연구)

  • Han, Keun-Woo;Lee, Jae-Woon;Lee, YoungJun
    • The Journal of Korean Association of Computer Education
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    • v.9 no.2
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    • pp.1-11
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    • 2006
  • Harmful information on the Internet affects negatively on adolescents' mind and stunts their growth and personality. This paper aims to analyze the current state on harmful information, and to propose various educational plans for adolescents. For theses purposes, we analyzed harmful information by reviewing various literatures and previous researches, and presented the definition and types of harmful information. In addition, we showed the necessity of educational plans for secondary student. We developed educational protective plans for secondary school student, teacher and parents. The proposed educational plans can be used for IT ethics curricular design, education policy establishment, and etc.

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Costello syndrome: three sporadic cases (Costello 증후군 3례)

  • Kim, Ji Youn;Kim, Mi Jeong;Song, Eun Song;Cho, Young Kuk;Choi, Young Youn;Ma, Jae Sook
    • Clinical and Experimental Pediatrics
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    • v.50 no.10
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    • pp.1024-1029
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    • 2007
  • Costello syndrome (CS) is a rare multiple congenital abnormality syndrome characterized by a typical coarse face, developmental delay, psychomotor and growth retardation, neurologic abnormalities, cardiac and cutaneous anomalies, severe feeding difficulties with postnatal growth failure, and increased risk of tumors. Since Costello first described it in 1971 and again in 1977, over 100 cases have been reported worldwide. It was recently shown that CS is a congenital condition caused by heterozygous de novo missense mutations affecting the codon for glycine 12 or 13 of the HRAS gene. We experienced three unrelated cases with coarse faces, developmental delays, short statures, macrocephaly, and redundant skin with deep palmar and plantar creases, hypertrophic cardiomyopathy and atrial tachycardia, which are characteristic of CS.

Two cases of Smith-Magenis syndrome (Smith-Magenis 증후군 2예)

  • Jung, Seong Kwan;Park, Kyu Hee;Shin, Hae Kyung;Eun, So Hee;Eun, Baik-Lin;Yoo, Kee Hwan;Hong, Young Sook;Lee, Joo Won;Bae, Sook Young
    • Clinical and Experimental Pediatrics
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    • v.52 no.6
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    • pp.701-704
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    • 2009
  • SmithMagenis syndrome (SMS) is a rare disorder with multiple congenital anomalies caused by a heterozygous interstitial deletion involving chromosome 17p11.2, where the retinoic acid-induced 1 (RAI1) gene is located, or by a mutation of RAI1. Approximately 90% of the patients with SMS have a detectable 17p11.2 microdeletion on fluorescence in-situ hybridization (FISH). SMS is characterized by mental retardation, distinctive behavioral features, craniofacial and skeletal anomalies, speech and developmental delay, and sleep disturbances. Although there are some intervention strategies that help individuals with SMS, there are no reported specific interventions for improving the outcome in children with SMS. Here, we report two cases of SmithMagenis syndrome.