• Clinical and Experimental Pediatrics
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• 제50권6호
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• pp.580-584
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• 2007

다발성 경화증은 중추신경계 백질의 여러 부위를 시간 간격을 두고 침범하는 만성재발성 질환이다. 주로 20세에서 40세 사이에 발병하며 15세 이하 소아에서도 3-5% 정도 발병하는 소아기에는 매우 드문 질환이다. 원인은 아직 정확히 밝혀지지 않았으나 유전적, 환경적 및 감염과 연관된 자가면역반응 등 여러요인이 복합적으로 작용하는 것으로 생각하고 있다. 임상증상은 침범된 백질 부위에 따라 다양한데 사지근력 약화나 저림, 시력장애, 감각장애, 운동실조 등 다양한 증세로 호전과 재발을 반복한다. 본 저자들은 경련, 왼쪽 편마비 등의 증상으로 6세에 첫 발병 후 메틸프레드니솔론(methylprednisolon) 치료 후 증상 완전 회복 있었으나 6개월 후 경련, 두통, 왼쪽 안와주위 통증 등으로 다시 입원하는 등 4년간 추적 관찰 중 4차례 메틸프레드니솔론 치료 실시하였으나 다른 양상의 신경학적 증상으로 재발하여 Interferon-${\beta}$-1b($Betaferon^{(R)}$, SheringAG, Germany) 예방 치료를 실시한 다발성 경화증 환자 1례를 경험 하였기에 문헌 고찰과 함께 보고하는 바이다.

• Clinical and Experimental Pediatrics
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• 제53권10호
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• pp.892-897
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• 2010

Purpose: This study evaluated the prevalence of the metabolic syndrome (MetS) and risk factors for metabolic derangement in young adults with childhood-onset hypopituitary growth hormone deficiency (ACOHGHD). Methods: Thirty patients with ACOHGHD who were treated with hormone-replacement therapy, aged 18 to 29 years, who visited the Seoul National University Children's Hospital between September 2009 and February 2010 were enrolled. Height, weight, waist circumference, hip circumference, and blood pressure were measured, and the clinical and hormonal features were reviewed retrospectively. We evaluated measures of metabolic derangement in the enrolled patients and in the data of healthy adults aged 20 to 29 years taken from the 2005 Korean National Health and Nutrition Examination Survey (KNHANES) as part of the National Cholesterol Education Program-the Adult Treatment Panel III. Results: Compared with the KNHANES participants, patients with ACOHGHD had significantly large waist circumference (men and women), high systolic blood pressure (BP) (women) and diastolic BP (men), and high serum triglyceride levels (women). The duration of illness correlated significantly with central obesity ($r^2$=0.546, $P$=0.003). The prevalence of MetS was 10% in patients with ACOHGHD and 2.3% in KNHANES participants. The prevalence of central obesity and MetS was higher in patients with ACOHGHD than in KNHANES participants ($P$<0.001 and $P$=0.042, respectively). Conclusion: Abdominal obesity correlated with the duration of illness in patients with ACOHGHD. Waist circumference should be measured in the clinic to prevent MetS, particularly in patients with a long history of ACOHGHD, regardless of age or sex.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• 제16권2호
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• pp.183-191
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• 2005

뚜렛 장애는 음성틱과 운동틱을 특징으로 하는 소아기의 대표적인 신경발달학적 행동장애이다. 소아기 발병 강박장애는 강박장애의 한 아형으로써 틱장애와의 연관성이 알려져 있다. 두 질환은 처음 진단시, $40\~75\%$에서 서로 공존질환으로서 발견되며, 유전학적으로도 관련성이 있으며 신경해부학적으로도 피질-선초체-시상 회로(cortico-striato-thalmic circuit)의 이상이 보고되며 증상적인 면에서도 유사점이 발견되고 있다. 최근 10여년 동안 틱장애와 소아기 발병 강박장애 영역에서는 놀라울 정도로 많은 연구들이 진행되었다. 본 연구는 뚜렛 장애를 포함하는 틱장애와, 소아 청소년 발병 강박 장애에서 1) 임상 양상 2) 유전학 및 역학 연구 3) 뇌영상 연구 4)신경화학 5) 연쇄구균 감염 관련 소아기 자가면역성 신경정신과적 질환(pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection : PANDAS)에 대해 고찰하고자 한다.

• Childhood Kidney Diseases
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• 제13권1호
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• pp.1-13
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• 2009

소아청소년기의 당뇨병은 대부분 제1형 당뇨병이나 최근 우리나라를 포함한 서구 사회에서는 제2형 당뇨병의 빈도가 증가하고 있다. 임상소견 상 제1형 당뇨병은 여러 위험인자에 의하여 비교적 전형적인 단계를 거치면서 미세알부민뇨와 당뇨병성 신병증으로 진행하면서 만성 신질환으로 발전하게 되며, 제2형 당뇨병은 비전형적 임상경과를 거치나 신병증 진행율이 높아서, 실제로 당뇨병성 신병증은 전세계 신장대체요법이 필요한 말기 신질환의 가장 많은 원인이며 국내에서도 꾸준히 원인 질환으로서 증가 중이다. 당뇨병이 사춘기 전에 발생하는 경우보다 사춘기나 그 이후에 발생하는 경우에 혈관합병증의 발생이 증가하므로, 사춘기가 위험인자로 작용하며, 이것은 유병기간과 함께 사춘기 전에 소아 당뇨병성 신병증이 발생하는 경우는 매우 드문 이유이다. 제1형과 제2형 당뇨병에서 신병증은 비슷하게 15-25%에서 발병하며, 당뇨병성 신병증과 만성 신질환으로 진행하는 과정 중에 가장 중요한 표식자인 미세알부민뇨는 위험인자이고 병리학적 소견과 관련이 있다.

• Clinical and Experimental Pediatrics
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• 제54권1호
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• pp.22-28
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• 2011

Purpose: To investigate the clinical characteristics of late-onset epilepsy combined with autism spectrum disorder (ASD), and the relationship between certain types of electroencephalography (EEG) abnormalities in ASD and associated neuropsychological problems. Methods: Thirty patients diagnosed with ASD in early childhood and later developed clinical seizures were reviewed retrospectively. First, the clinical characteristics, language and behavioral regression, and EEG findings of these late-onset epilepsy patients with ASD were investigated. The patients were then classified into 2 groups according to the severity of the EEG abnormalities in the background rhythm and paroxysmal discharges. In the severe group, EEG showed persistent asymmetry, slow and disorganized background rhythms, and continuous sharp and slow waves during slow sleep (CSWS). Results: Between the two groups, there was no statistically significant difference in mean age (P=0.259), age of epilepsy diagnosis (P=0.237), associated family history (P=0.074), and positive abnormal magnetic resonance image (MRI) findings (P=0.084). The severe EEG group tended to have more neuropsychological problems (P=0.074). The severe group statistically showed more electrographic seizures in EEG (P=0.000). Rett syndrome was correlated with more severe EEG abnormalities (P=0.002). Although formal cognitive function tests were not performed, the parents reported an improvement in neuropsychological function on the follow up checkup according to a parent's questionnaire. Conclusion: Although some ASD patients with late-onset epilepsy showed severe EEG abnormalities, including CSWS, they generally showed an improvement in EEG and clinical symptoms in the longterm follow up. In addition, severe EEG abnormalities tended to be related to the neuropsychological function.

• 대한유전성대사질환학회지
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• 제3권1호
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• pp.32-37
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• 2003

저자들은 반복적이며 간헐적인 기면, 정신착란과 운동실조를 주소로 내원한 6세 여아에서, 고암모니아혈증과 혈장 glutamine, 요 orotic acid의 증가를 보여 지발형 OTC 결핍증으로 진단하고, 분자유전학적 검사상 exon 6에서 221번째 아미노산 lysine에 해당하는 염기 AAG가 AAT(asparagine)로 치환된 돌연변이를 이형접합자(heterozygote)로 보인하였던 1례를 경험하였기에 보고하는 바이다.

• Clinical and Experimental Pediatrics
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• 제57권5호
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• pp.211-216
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• 2014

Asthma comprises a heterogeneous group of disorders characterized by airway inflammation, airway obstruction, and airway hyperresponsiveness (AHR). Airway inflammation, which induces AHR and recurrence of asthma, is the main pathophysiology of asthma. The fractional exhaled nitric oxide (FeNO) level is a noninvasive, reproducible measurement of eosinophilic airway inflammation that is easy to perform in young children. As airway inflammation precedes asthma attacks and airway obstruction, elevated FeNO levels may be useful as predictive markers for risk of recurrence of asthma. This review discusses FeNO measurements among early-childhood wheezing phenotypes that have been identified in large-scale longitudinal studies. These wheezing phenotypes are classified into three to six categories based on the onset and persistence of wheezing from birth to later childhood. Each phenotype has characteristic findings for atopic sensitization, lung function, AHR, or FeNO. For example, in one birth cohort study, children with asthma and persistent wheezing at 7 years had higher FeNO levels at 4 years compared to children without wheezing, which suggested that FeNO could be a predictive marker for later development of asthma. Preschool-aged children with recurrent wheezing and stringent asthma predictive indices also had higher FeNO levels in the first 4 years of life compared to children with wheezing and loose indices or children with no wheeze, suggesting that FeNO measurements may provide an additional parameter for predicting persistent wheezing in preschool children. Additional large-scale longitudinal studies are required to establish cutoff levels for FeNO as a risk factor for persistent asthma.

• 대한한방소아과학회지
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• 제15권2호
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• pp.141-165
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• 2001

Attention-deficit/hyperactivity disorder(ADHD) is one of the most common childhood-onset psychlatric disorders. It is distinguished by symptoms of inattention, hyperactivity, and impulsivity. ADHD may be accompanied by learning disabilities, depression, anxiety, conduct disorder, and oppositional defiant disorder. The etiology of ADHD is unknown, and the disorder may have several different causes. Individual with ADHD present in childhood and may continue to show symptoms as they enter adolescence and adult life. Public interest in ADHD has increased along with debate in the media concerning the diagnostic process and treatment strategies. The purpose of this study is oriental medical approach to ADHD. This study was progressed for oriental diagnosis and treatment for ADHD. In oriental medicine, the reason of ADHD was deficiency of the kidney, hyperactivity of the liver(腎虛肝亢), deficiency of the heart and the spleen(心脾不足), heart disturbed by phlegm and heat(痰熱擾心). The method of medical treatment was nourishing the kidney and checking exuberance of yang(滋腎潛陽), relieving mental stress and promoting wisdom(寧神益智), nourishing the heart and strengthening the spleen(養心健脾), tranquilzation(安神定志). removing heat-phlegm(淸熱化痰), inducing resuscitation and tranquilzation(開窮安神). The prescription was commonly used as Liuwei Dihuang Wan jiajian(六味地黃丸加減), Guipi Tang he Ganmai Dazao Tang jiajian(歸脾湯合甘麥大棗湯加減), Huanglian Wendan Tang jiawei(溫黃連溫膽湯加味). It should help primary care providers in their assessment of a common child health problem.

• Childhood Kidney Diseases
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• 제1권1호
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• pp.79-81
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• 1997

Minimal change nephrotic syndrome is characterized by proteinuria, hypoproteinemia, edema, and hyperlipidemia. Children with onset of nephrotic syndrome between the age of 1 and 8 year are likely to have steroid response to minimal chage disease, but we experienced one case of minimal change disease which failed to respond to steroid therapy at beginning and subsequently developed acute renal failure. It was seen in a 5 year-old male child that presented with edema and gross hematuria. Peritoneal dialysis was performed for acute renal failure for 11 days. Patient was completely recorvered from acute renal failure and renal biopsy was done at 27th day after onset of disease which revealed typical picture of minimal change disease complicated by acute tubular necrosis. We beleive this case is very unusual and it may be the first case in the literature in terms of pediatric cases.

• 수면정신생리
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• 제2권1호
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• pp.13-22
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• 1995

To provide the physician with adequate information to diagnose and treat sleepwalking and sleep terrors, the author reviewed clinical features, epidemiology, causative and precipitating factors, polysomnography, diagnosis, differential diagnosis, and treatment for these disorders. Sleepwalking and sleep terrors have been defined as disorders of arousal that occur early in the night and have their onset during stage 3 or 4 sleep. In both disorders, patients are difficult to arouse, and complete amnesia or minimal recall of the episode is frequent. Genetic, developmental, and psychological factors have been identified as causes of both sleepwalking and sleep terrors. Sleepwalking and sleep terrors typically begin in childhood or early adolescence and are usually outgrown by the end of adolescence. When sleepwalking or sleep terrors have a post-pubertal onset or continue to adulthood, psychopathology is a more significant causative factors. The behavior that occur from deep slow-wave sleep can be painful or dangerous to the individual and/or disturbing to those close to that individual. The assessment of patients suspected of having these conditions requires a thorough medical and sleep history. The most important consideration in managing patients with sleepwalking or sleep terrors episodes is protection from injury.