• Clinical and Experimental Pediatrics
• /
• v.51 no.3
• /
• pp.329-334
• /
• 2008

Kwashiorkor is a syndrome of severe protein malnutrition, which manifests itself in hypoalbuminemia, diarrhea, dermatitis, and edema. It can be life-threatening due to associated immune deficiency and an increased susceptibility to infections. Kwashiorkor should be treated early with nutritional support and the control of infection. Dilated cardiomyopathy may develop during the treatment and in such cases a poor prognosis is expected. Kwashiorkor has been known as a common disease of poor countries. To date, in fact, there has been no report of kwashiorkor leading to death in technically advanced countries. We here report a fatal case of a baby girl admitted with kwashiorkor. She had been fed only with cereal grain mixed with juice, without any protein supplement, for 2 months. This diet was deficient not because of poverty, but due to the illiteracy of her parents. The patient suffered from diarrhea, whole body edema, hypothermia, and dermatitis. Laboratory findings revealed an immune-deficient state featuring leukopenia and decreased immunoglobulin. Blood and urine cultures revealed Alcaligenes Xylosoxidans growth. The patient was fed frequent small amounts of protein-containing formula and intravenous albumin and micronutrients were administered for nutritional support. She was also treated with intravenous immunoglobulin and antibiotics in order to control infection. Nevertheless, she developed dilated cardiomyopathy and multi-organ failure and died. We review this case in light of the literature.

• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.15 no.3
• /
• pp.165-170
• /
• 2015

Ornithine transcarbamylase (OTC) deficiency is a rare X-linked genetic disorder of urea synthesis in newborns. It is the most common urea cycle disorder and leads to elevated levels of ammonia in the blood. Excessive ammonia can cause various symptoms, including vomiting, lethargy, and coma. Boys have a more serious form of OTC deficiency than girls. If not treated immediately, severe OTC deficiency can lead to neurologic abnormalities, hyperammonemic coma, and death. Because late-onset OTC deficiency, which is more common in girls, presents mild symptoms, it is easy to miss diagnosis and prompt treatment. We describe an 11-month-old girl who presented periodic vomiting, intermittent lethargy, and seizure. She was diagnosed with OTC deficiency by elevated serum ammonia and urine orotic acid levels. Genetic analysis of the OTC gene revealed a missense mutation in exon 5 (c.418G>C). We reported an experience of exact diagnosis and successful treatment of late-onset OTC deficiency in our patient.

• The Journal of Korean Academy of Sensory Integration
• /
• v.17 no.1
• /
• pp.43-53
• /
• 2019

Objective : This study was designed to find the latest intervention trends in treatment of children with attention deficit hyperactivity disorder (ADHD) in South Korea. Methods : Studies on ADHD published in Korean journals from January 2004 to December 2018 were reviewed. The databases used were DBpia, Google Scholar, RISS, and KISS. The search terms were "attention deficit hyperactivity," "child," and "intervention." A total of 14 studies were selected. The results of this study were as follows: Patient, Intervention, Comparison, Outcome. Results : The level of evidence was the highest in the single subject study of level IV, and the methodological quality was the most common study. The interventional metronome (IM) intervention was the most frequently used, and the evaluation tools used in the measurement were the Long Form Assessment of the Interactive Metronome and the Korean Behavior Child Checklist. The Korean ADHD interventions showed statistically significant improvement in concentration, hyperactivity, task performance, excessive language use, and timing. Conclusions : Various types of interventions were provided for children with ADHD in South Korea. The interventions showed positive effects, and provided basic data for evidence-based implementation in clinical practice. In the future, a model study should be conducted, to establish an effective service delivery system, as well as ongoing research on children with ADHD.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
• /
• v.16 no.1
• /
• pp.124-131
• /
• 2005

Childhood and adolescent onset-bipolar disorders have higher rate of comorbidity with anxiety disorders as well as attention deficit hyperactivity disorder and conduct disorder. Obsessive compulsive disorder, social phobia, panic disorder, and separational anxiety disorder are common comorbid anxiety disorders with bipolar disorders in child and adolescent. Prospective and reliable data on temporal and causal relationship between the onset of bipolar disorders and the onset of comorbid anxiety disorders are still in debate. The authors report 2 adolescent cases with antidepressant induced-manic episodes with preceding anxiety symptoms. The authors suggest careful prescription of antidepressants for anxiety disorders, even for those who do not have definite past history or family history of bipolar disorders. Further comprehensive and prospective studies are requested for the temporal relationship and pharmacological guideline for comorbid bipolar disorders and anxiety disorder in child and adolescent.

• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.18 no.3
• /
• pp.95-98
• /
• 2018

A striking feature of mitochondrial disorders is the vast heterogeneity in their clinical symptoms that ranges from a single organ to severe multisystem involvement. Though a variety of ocular symptoms such as ptosis, pigmentary retinal degeneration, external ophthalmoplegia, and optic nerve atrophy can occur in association with mitochondrial cytopathies, progressive bilateral cataracts are rare among their ocular findings. A 5-year-old girl with no previous medical history came to our hospital presenting symptoms of seizure. She started showing progressive developmental regression, increased seizure frequency, hypotonia, general weakness, dysphagia and decreased vision. Lactic acidosis was noted in metabolic screening test and we confirmed mitochondrial respiratory chain complex I defect in spectrophotometric enzyme assay using the muscle tissue. Progressive bilateral cataracts then developed and were fully evident at the age of 7. She underwent cataract extraction with posterior chamber lens implantation. We are reporting a case of mitochondrial respiratory chain defect with multiorgan involvements including bilateral progressive cataract, an uncommon ocular manifestation. Ophthalmologic evaluation is highly recommended not to overlook the possible ocular manifestations in mitochondrial disorders.

• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.23 no.2
• /
• pp.8-14
• /
• 2023

The hepatic glycogen storage disease type 0 (GSD type 0) is an autosomal recessive disorder caused by a deficiency of hepatic glycogen synthase encoded by the glycogen synthase 2 (GYS2) gene, leading to abnormal synthesis glycogen. The clinical findings of GSD type 0 are hyperketotic hypoglycemia at fasting state and accompanying postprandial hyperglycemia and hyperlactatemia. GSD type 0 has only been reported in a very small number so far, and the diagnosis is likely to be missed because symptoms are mild, severe hypoglycemia is rare or asymptomatic, or symptoms gradually disappear with age. Essential management strategies include feeding high-protein meals to stimulate gluconeogenesis, frequent meals to prevent hypoglycemia during the day and feeding complex carbohydrates such as uncooked cornstarch to slowly release glucose during nignt. GSD type 0 has a good prognosis, with appropriate treatment, normal growth can be achieved and no complications occur. Significant hypoglycemia occurs less common in adulthood, but ongoing dietary management may be necessary.

• Proceedings of the Korean Society for Bio-Environment Control Conference
• /
• 1997.05a
• /
• pp.11-16
• /
• 1997

본 연구는 양액재배에서 영양진단에 기초한 배양액관리를 하기 위해 유효한 수단으로 이용되고 있는 신속하고 간편하며 정확한 즙액분석 방법(질산태질소의 정량방법, 인산정량방법, 즙액추출법, 추출비율, 마쇄시간 및 샘플채취 부위 등)들에 관한 비교검토를 하였다. 'Earl's Favorite 춘계 F$_1$호'를 1993년 1월 11일에 파종하여 본엽 3~4매의 묘를 스티로폼베드내 암면슬라브에 정식하여 비순환방식으로 재배했다. 즙액분석법은 결핍이나 과잉증상이 외관적으로 나타나기 전에 신속하고 간이한 즙액분석법에 의해 작물체의 영양상태를 진단하여 대응이 가능하다고 판단되었다. 즙액분석시 질산태질소의 분석에는 자외부흡광도법, 인산분석에는 바나드몰리브덴산법이 추천되었고, 추출비율은 1:4로, 엽병의 마쇄시간은 60초, 그리고 채취 부위는 일정부위로 하는 것이 바람직하다고 판단되었다. 그러나 샘플채취시간에 대해서는 채취시간에 따른 차이가 크기 때문에 앞으로 보다 상세한 검토가 요청되었다.

• Journal of Korean Society of Industrial and Systems Engineering
• /
• v.33 no.2
• /
• pp.105-111
• /
• 2010

The purpose of this study was to detect early children with hyperactivity which is one of the symptoms of Attention Deficit-Hyperactivity Disorder (ADHD). This study used two methods: K-CBCL and observation of children's behavior. K-CBCL was done online by parents at home. For observation of children's behavior, the school asked children to wear a 3-axis accelerometer on their wrists. The data from K-CBCL and 3-axis accelerometer were analyzed and clustered to separate hypersensitive children from ordinary children. This experiment confirmed that 3-axis accelerometer which is one of Ubiquitous techniques and the K-CBCL questionnaire were helpful for detection of hypersensitive children.

• Korean Journal of Psychosomatic Medicine
• /
• v.13 no.2
• /
• pp.95-101
• /
• 2005

Objectives : This study was designed to investigate depression, anxiety, alexithymia, stress res ponses in caregivers of patients with attention deficit hyperactivity disorder. Methods : The subjects were 38 attention deficit hyperactivity disorder patients caregivers(38 women, mean age $37.5{\pm}6.5$). Patients were diagnosed with DSM-IV ADHD criteria. Korean version of Beck Depression Inventory(BDI), State and Trait Anxiety Inventory(STAI), Toronto Alexithymia Scale(TAS) and Stress Response Inventory(SRI) were used for assessment. Results 1) The BDI scores of ADHD patients caregiver group were significantly higher than control group$(16.4{\pm}7.1\;vs.\;10.9{\pm}5.5)(p=0.011)$. 7 of the 38 caregivers(18.4%) and none of control group(0%) had BDI scores over 20 points(p=0.021). Calculated relative risk for ADHD in the presence of caregivers' depression was 1.516 overall(95% confidence interval, 1.234-1.862). 2) In ADHD patient's caregiver group, the scores of Stress Response Inventory were significantly higher than control group$(44.2{\pm}20.2\;vs.\;26.5{\pm}16.8)(p=0.006)$. 3) No significant differences were found in the score of STAI, SIAIS, SIAI-T, TAS between caregiver and control group. Conclusion : This study suggest that ADHD patients' caregivers are likely to have more depressive symptoms and higher stress response level than control group. We propose that physicians should consider integrated approaches for caregiver's psychopathology in the management of ADHD.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
• /
• v.3 no.1
• /
• pp.26-45
• /
• 1992

This study presents the behavioral and cognitive changes by administration of methylphenidate(MPH) and imipramine(IMI) for the treatment of attention-deficit hyperactivity disorder(ADHD) in $5_{1/2}{\sim}12$ years old children referred to child psychiatric clinics. Behavioral changes are assessed with parent's and teacher's ratings. Drug effects on attention. short-term memory, and impulsivity are evaluated with psychological tests in laboratory. The changes were assessed twice in a 8-week periods. The data were analyzed seperately for 15 subjects each drug using repeated measured analysis of variance(ANOVA). The findings indicates that behavioral and cognitive impairments are improved by both drugs, but impulsivity is not. And MPH is superior to IMI on the improvement of attentional problem ; especially the findings indicates important differences between simple task and complex. perceptual-search task. These data confirm the effectiveness of MPH for treatment of ADHD, also raise questions regarding assessment method of attention and impulsivity as fell as importance of impulsivity in ADHD.