• The Journal of Korea CHUNA Manual Medicine
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• v.5 no.1
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• pp.141-150
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• 2004

Objectives : The objective of this study is to evaluate the treatment effect of Oriental therapy including exercise therapy in chronic ankylosing spondylitis with total hip replacement. Methods : Oriental therapy and exercise therapy were performed for 64days admission in a patient who had chronic ankylosing spondylitis with total hip replacement and physical tests were examined. Result : Pain and ROM of cervical. lumbar spine, and left hip joint has improved with this treatment. And Anorexia, dyspepsia, and fatigue were also decreased. Conclusions: In this case, oriental therapy was proved to be effective in improving chronic symptoms and general prostration of Ankylosing Spondylitis. And It is necessary to carry out exercise therapy regulary.

• Journal of Chest Surgery
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• v.33 no.12
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• pp.941-947
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• 2000

Background: Central nervous system complication after coronary artery bypass grafting(CABG) is one of the major prognostic determinants and the use of the cardiopulmonary bypass(CPB) may increase the incidence of this devastating complication. In this study, the outcomes after off-pump CABG were studied and compared with those following the conventional CABG using CPB. Material and Method: Among the consecutive isolated CABG's performed in SNUH during Feb. 1995 and Jun. 1999, 338 coronary artery bypass grafting were divided into two groups. 223 patients underwent CABG using the CPB(Group I), and 115 patients underwent CABG without CPB(OPCAB)(Group II). All patients enrolled in this study received extensive preoperative examinations including thorough neurologic examination before and after surgery, transcranial doppler study, carotid duplex ultrasonography, and magnetic resonance angiography if necessary. Central nervous system(CNS) complications were defined as stroke, seizure, metabolic or hypoxic encephalopathy and transient delirium after surgery. Result: There were 61 cases(27.3%) who developed postoperative CNS complication in Group I, whereas 8 cases(7.0%) of CNS complications developed postoperatively in group II(p<0.05). Statistically significant predictors of postoperative CNS complications in group I were age and the use of cardiac assist devices perioperatively. Conclusion: This study suggested that omitting the use of CPB in CABG resulted in significant decrease of the postoperative CNS complications. OPCAB should be more widely applied especially to the elderly who have preexisting cerebrovascular disease.

• Radiation Oncology Journal
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• v.16 no.4
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• pp.389-398
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• 1998

Purpose : To evaluate the role of conventional postoperative adjuvant radiotherapy in the management of supratentorial malignant glioma and to determine favorable prognostic factors affecting survival. Materials and Methods : From Sep. 1985 to Mar. 1997, the number of eligible patients who received postoperative radiotherapy completely was 69. They ranged in age from 7 to 66 years (median, 47). Forty-two (61$\%$) patients were glioblastoma multiforme and the other 27 (39$\%$) were anaplastic astrocytoma. Twenty patients (29$\%$) had Karnofsky score equal or more than 80 preoperatively. Forty-three patients (62$\%$) had symptom duration equal or less than 3 months. Twenty-four patients (35$\%$) had gross total resection and forty patients(58$\%$) had partial resection, the remaining five patients (7$\%$) had biopsy only. Radiotherapy dose ranged from 50.4 Gy to 61.2 Gy (median, 55.8; mode, 59.4) with fraction size of 1 8 Gy-2.0 Gy for 33-83 days(median, 48) except three patients delivered 33, 36, 39 Gr, respectively with fraction size of 3.0 Gy due to poor postoperative performance status. Follow-up rate was 93$\%$ and median follow-up period was 14 months. Results : Overall survival rate at 2 and 3 years and median survival were 38$\%$, 20$\%$, and 16 months for entire patients; 67$\%$, 44$\%$, and 34 months for anaplastic astrocytoma; 18$\%$, 4$\%$, and 14 months for glioblastoma multiforme, respectively (p=0.0001). According to the extent of surgery, 3-year overall survival for gross total resection, partial resection, and biopsy only was 38$\%$, 11$\%$, and 0$\%$, respectively (p=0.02) The 3-year overall survival rates for patients age 40>, 40-59, and 60< were 52$\%$, 8$\%$, and 0$\%$, respectively (p=0.0007). For the variate of performance score 80< vs 80>, the 3-year survival rates were 53$\%$ and 9$\%$, respectively (p=0.008). On multivariate analysis including covariates of three surgical and age subgroups as above, pathology, extent of surgery and age were significant prognostic factors affecting overall survival. On another multivariate analysis with covariates of two surgical (total resection vs others) and two a9e (50> vs 50<) subgroups, then, pathology, extent of surgery and performance status were significant factors instead of age and 3-year cumulative survival rate for the five patients with these three favorable factors was 100$\%$ without serious sequela. Conclusion : We confirmed the role of postoperative conventional radiotherapy in the management of supratentorial malignant glioma by improving survival as compared with historical data of surgery only. Patients with anaplastic astrocytoma, good performance score, gross total resection and/or young age survived longest. Maximum surgical resection with acceptable preservation of neurologic function should be attempted in glioblastoma patients, especially in younger patients. But the survival of most globlastoma patients without favorable factors is still poor, so other active adjuvant treatment modalities should be tried or added rather than conventional radiation treatment alone in this subgroup.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.19 no.1
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• pp.20-25
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• 2019

Very-long-chain acyl-CoA dehydrogenase (VLCAD) deficiency (OMIM#201475) is an autosomal recessively inherited metabolic disorder of mitochondrial long-chain fatty acid oxidation. The clinical features of VLCAD deficiency is classified by three clinical forms according to the severity. Here, we report a case of later-onset episodic myopathic form of VLCAD deficiency whose diagnosis was confirmed by plasma acylcarnitine analysis and" multigene panel multigene panel sequencing. A 34-year old female patient visited genetics clinic for genetic evaluation for history of recurrent myopathy with intermittent rhabdomyolysis. She suffered first episode of rhabdomyolysis with acute renal failure requiring hemodialysis at twelve years old. After then, she suffered several times of recurrent rhabdomyolysis provoked by prolonged exercise or fasting. Physical and neurologic exam was normal. Serum AST/ALT and creatinine kinase (CK) levels were mildly elevated. However, according to her previous medical records, her AST/ALT, CK were highly elevated when she had rhabdomyolysis. In suspicion of fatty acid oxidation disorder, multigene panel sequencing and plasma acylcarnitine analysis were performed in non-fasting, asymptomatic condition for the differential diagnosis. Plasma acylcarnitine analysis revealed elevated levels of C14:1 ($1.453{\mu}mol/L$; reference, 0.044-0.285), and C14:2 ($0.323{\mu}mol/L$; 0.032-0.301) and upper normal level of C14 ($0.841{\mu}mol/L$; 0.065 -0.920). Two heterozygous mutation in ACADVL were detected by multigene panel sequencing and confirmed by Sanger sequencing: c.[1202G>A(;) 1349G>A] (p.[(Ser 401Asn)(;)(Arg450His)]). Diagnosis of VLCAD deficiency was confirmed and frequent meal with low-fat diet was educated for preventing acute metabolic derangement. Fatty acid oxidation disorders have diagnostic challenges due to their intermittent clinical and laboratorial presentations, especially in milder late-onset forms. We suggest that multigene panel sequencing could be a useful diagnostic tool for the genetically and clinically heterogeneous fatty acid oxidation disorders.

• Journal of Chest Surgery
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• v.30 no.8
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• pp.780-785
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• 1997

Mediastinal tumor had been fascinated by its location on heart, great vessels, esophagus, and nervous tissue, its convenience of surgical treatment and superiority of its operative result. Between January 1989 and June 1995, eighty-seven patients with mediastinal tumor which were treated surgically in the Department of Thoracic and Cardiovascular Surgery, Asan Medical Center, School of Medicine, University of Ulsan. To provide the appropriate surgical management of mediastinal tumor, the demographic data, diagnostic evaluation, clinical presentation, location, size, operative finding and histopathologic distribution were reviewed and we analyzed relativity between invasiveness in chest computed tomographic finding or invasiveness on operative finding and histopathologic invasiveness. The anterosuperior mediastinum was the most commonly involved site of a mediastinal tumor(57%), followed by the posterior mediastinum(35%) and middle mediastinum(8%). The most frequently encountered tumors were thymic neoplasia(31%), followed by primary cyst(22%), neurogenic tumor(22%) and teratoma(10%) in decreasing order of frequency. Histopathologically invasive tumors were present in 17 patients(20%) and its site included anterosuperior mediastinum(16%) and posterior mediastinum(4%). All patients in this study underwent chest CT. In chest CT's finding, 15 patients(17%) showed invasiveness. A total excision of the tumor was performed 80 patients(92%), subtotal excision 6 patients(7%) and biopsy only 1 patient(2%). In operative finding, 14 patients(16%) were suspected invasiveness. The mean size of the tumor was 6.0$\pm$ 3.2cm. In anterosuperior mediastinum, the mean size was 6.2$\pm$3.1cm, in middle mediastinum, it was 3.9$\pm$1.1cm, in posterior mediastinum, it was 5.8$\pm$2.6cm. In malignant tumors, the mean size was 7.3$\pm$4.6cm, in benign tumor, it was 5.5$\pm$2.6cm(P<0.05). Relativity between histopathological invasiveness(17 patients) and invasiveness in chest CT's finding(15 patients) included sensitivity 35%, specificity 87% and predictability 35%, relativity between histopathological invasiveness(17 patients) and invasiveness on operative finding included sensitivity 52%, specificity 93% and predictability 64%. In conclusion, since it was proved that the compatibility of preoperative chest CT findings or operative findings and histopathological invasiveness is quite low, it is considered that wide excision of the mediastinal tumor except cystic lesion including adjacent tissues would yield better postoperative results.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.1 no.1
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• pp.55-64
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• 1990

The purpose of the present study is to investigate cognitive characteristics of ADHD children by comparing their performances on KEDI-WISC according to age and EEG variables. Subjects were 56 ADHD children who visited Seoul National University Children's Hospital during the period from January, 1988 to March, 1989. Group differences on age and EEG variables were tested by ANOVA, and Hierarchical Cluster Analysis was performed to investigate how ADHD children were classified based on their performances on KEDI-WISC. The results Indicated that ADHD children showed low scores on Coding, Digit Span, and Comprehension subtests, suggesting their attention deficits and impulsivity. ADHD children were clustered Into three groups based on only FSIQ. In post-hoc tests three groups showed different cognitive strengths and weaknesses on KEDI-WISC. Group differences on age were not significant, and abnormal EEG group showed lower PIQ than normal EEG group, suggesting the possibility that their attention deficits were related to neurological factors.

• Pediatric Infection and Vaccine
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• v.12 no.2
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• pp.202-207
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• 2005

Intravenous immune globulin(IVIG) is widely used for immune thrombocytopenic purpura (ITP), Kawasaki disease and other autoimmune neuromuscular disease. Aseptic meningitis was one of the most serious neurologic complications reported following the use of IVIG. We experienced 4 episodes of aseptic meningitis associated with IVIG usage in 3 patients from 2003 to 2004. Underlying disease of each patients was ITP, Kawasaki disease and myathenia gravis and all of them received high dose IVIG treatment for their underlying disease. Within a days, they started to complain severe headache and diagnosed meningitis by cerebrospinal fluid analysis. Cerebrospinal fluid leukocyte counts varied from 92 to over a thound per microliter with dominance of polymorphonuclear leukocytes. Microbiologic studies revealed no organisms. All of them were free from headache within 2 days and did not suffer any neurological sequelae.

• Journal of the Korea Academia-Industrial cooperation Society
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• v.18 no.5
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• pp.211-218
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• 2017

This study examined the effects of a computer-assisted cognitive rehabilitation program on the depression, cognitive functions, and daily functions of stroke patients. Forty-four stroke patients were divided into the treatment group and control group, consisting of 22 patients each. The patients in the treatment group were treated with a computer-assisted cognitive rehabilitation program for 6 weeks, 5 times a week, for a total of 30 times. Depression, cognitive functions, and daily functions was gauged by the BDI, NSCE, and k-MBI, respectively. The treatment group showed decreases in depression, and increases in both cognitive functions and daily functions by the end of the rehabilitation program. Compared to the treatment group, the control group did not show significant improvements in depression, cognitive functions, and daily functions. Therefore, these findings indicate that a computer-assisted cognitive rehabilitation program has a positive effect on depression, cognitive functions, and daily functions in stroke patients.

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

• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.1
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• pp.30-34
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• 2018

Niemann Pick type C disease (NPC) is an inherited progressive neurodegenerative disorder, due to defects of intracellular lipid trafficking and storage. Hepatosplenomegaly may prevail, while progressive neurodegenerative symptoms such as cerebellar involvement, dystonia, vertical supranuclear ophthalmoplegia, cataplexy, and eventually seizures starting at juvenile or late infantile period may accompany after normal early development. Here we describe a 3-year-old Korean boy with NPC who presented with splenomegaly at age 3. Liver biopsy showed characteristic foamy cell stained by periodic acid-schiff, and molecular analysis for NPC1 identified the compound heterozygous mutations, novel mutation of c.1631G>A (p.Trp544Ter) and c.2662C>T (p. Pro888Ser) as a known mutation. Filipin was strongly stained with unesterified cellular cholesterol in the patient's skin fibroblasts. The patient has received migulstat since age 3 years and his long-term outcome is needed to be observed.