• Clinical and Experimental Pediatrics
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• v.46 no.3
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• pp.277-283
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• 2003

Purpose : Craniopharyngiomas are often accompanied by severe endocrine disorders. Although there is universal growth hormone deficiency(GHD), the resulting growth pattern is very heterogeneous. We report the growth and endocrine outcome of 44 children with craniopharyngioma, with emphasis on initial symptoms, growth before and during growth hormone(GH) treatment and spontaneous growth in spite of GHD. Methods : We performed a retrospective study of 44 children treated at our centre between 1984 and 2002. Results : About 30% of patients had symptoms suggesting endocrine disorder at diagnosis. After surgery, multiple endocrinopathies were almost universal. Before GH therapy, height velocity was $8.00{\pm}2.71cm/yr$ in the normal growth group(n=11) and $1.79{\pm}1.10cm/yr$ in the subnormal growth group(n=7) during the first year and during the second year, $6.76{\pm}2.49cm/yr$ and $2.29{\pm}1.33cm/yr$, respectively. There was no difference of body mass index(BMI) change between before and after surgery in the two groups. Height standard deviation score(SDS) was $-1.46{\pm}0.74$ in the normal growth group and $-0.43{\pm}0.97$ in the subnormal growth group. Before GH treatment height SDS was $-1.31{\pm}1.25$ and BMI was $20.46{\pm}3.60$. During GH treatment, height SDS increased to $-0.60{\pm}1.37$ in the first, and to $-0.41{\pm}1.54$ in the second year(P<0.05), but BMI did not change significantly. Conclusion : The endocrine morbidity could develop in most children with craniopharyngioma before and after the operation and should be managed properly. Although all treated patients benefit from GH therapy, further studies are necessary to find out the possible mechanism of growth regulation in normally growing children, despite GH deficient.

• Clinical and Experimental Pediatrics
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• v.46 no.3
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• pp.259-264
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• 2003

Purpose : We have performed an analysis on patients who received Rastelli operation in our institute and reviewed their progress postoperatively. Various factors with suspected relationship to the outcome have been considered to help in future treatment and follow-up. Methods : We analyzed retrospectively 43 patients who either received Rastelli operation in Yonsei University Cardiovascular Center from March 1995 to April 1997 or who received post-procedural cardiac catheterization and follow-up echocardiography in the out-patient department after the procedure. Results : No statistically valid relationships were found between the age of the patient, their body weight, preoperative pulmonary arterial index and pressure, presence of pulmonary branchial stenosis and postoperative results. Cases with atrioventricular concordance showed lower age and body weight, and discordant cases exhibited lower ejection fraction 3 days postoperatively. Upon follow up, lower NYHA score was seen in patients with severe residual stenosis. In the group that received cardiac catheterization after the procedure, residual stenosis and right ventricular pressure measurement in echocardiography showed good correlation with the catheterization data. Conclusion : In cases where conduit insertions of the right ventricular outflow tract are required to achieve total correction in complex cardiac deformity, early operation does not seem to provide a clear risk to the patient. In patients with atrioventricular discordance, careful postoperative observation of the ventricular function seems to be needed. Also, echocardiography appears to be a sound method in follow-up of patients after the correctional procedure.

• Journal of the Korean Arthroscopy Society
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• v.17 no.1
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• pp.24-30
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• 2013

Purpose: To evaluate the outcome of arthroscopic posterior cruciate ligament (PCL) augmentation using an autogenous hamstring tendon graft and the posterior-posterior triangulation technique with preservation of ligament remnant or elongated ligament. Materials and Methods: From January 2002 to December 2009, we performed PCL augmentation using an autogenous hamstring tendon graft and the posterior-posterior triangulation technique in 32 patients. The mean age was 35.2 years. Twenty two cases were male and 10 cases were female. Average follow-up period was 5 years and 5 months (range: 2-7.9). Subjective and objective parameters were utilized in analyses, such as the mean range of motion, post. drawer test, Lysholm knee score, Tegner activity score, International Knee Documentation Cominittee (IKDC) grade, and second look arthroscopic examination. Results: At last follow up posterior displacement by the Telos stress test decreased from $10.8{\pm}5.1\;mm$ to $2.8{\pm}3.7\;mm$ (p<0.05). The final Lysholm knee score improved from $60.4{\pm}5.8$ to $84.6{\pm}4.8$. Tegner activity score improved from 3.2 to 4.8. The final IKDC grade was A in 18, B in 11, C in 3. Postoperative Lysholm knee score, IKDC grades, Tegner activity scale, and posterior displacement demonstrated statistically significant improvement compared to the preoperative state (p<0.05). Conclusion: Arthroscopic PCL reconstruction using an autogenous hamstring tendon with preservation of ligament remnant showed a good clinical results and posterior stability.

• The Journal of Dong Guk Oriental Medicine
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• v.6 no.2
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• pp.167-190
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• 1998

Nowadays there were two tendencies of studies about prognostic factors in stroke. One way was to define prognostic factors according to the radiological features. And the other way was to define according to the mental state, recognition, perception, motors, language, urinary&bowel incontinence etc.. The former could be objectively investigated, while the latter was difficult. The purpose of this study was to determine which variables would be predictors of stroke and which factors would be affect predictions most. The subjects of this study were 32 patients who were admitted to the Dept. of Internal Medicine, Dongguk Univ. College of Oriental Medicine whthin 48 hours from attack, Medical records were reviewed FIM, CNS, NIH stroke scale. We compared each sub-items of FIM, CNS, NIH stroke scale about mental state, recognition, perception, motors, language, urinary&bowel incontinence with MBI score at 4 weeks from admission. Also, we analyzed the correlations of sub-items and groups which devided into 5 according to independence of MBI score. And we found out the most influent factors with multiple regression analysis. The major results were as follows; 1. In mean of MBI score at 4 weeks of each groups devided low, middle, high score at mental state, recognition, perception, motors, language, urinary&bowel incontinence items, there were statistical differences in all items. 2. The mental state and lim ataxia sub-items had no significant correlations with groups divided according to independence of MBI score. All the other items were significantly correlated. 3. The most influent factors was recognition. The second was sensory and the third was bowel incontinence. 4. The most influent scales was FIM, and the second was CNS, and NlH had no statistical significancy.

• Tuberculosis and Respiratory Diseases
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• v.59 no.1
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• pp.53-61
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• 2005

Background : Cardiac troponin I (cTnI) is a specific marker of myocardial injury. It is known that a higher level of cTnI is associated with a poor clinical outcome in patients with acute coronary syndrome. An elevation in cTnI is also observed in various noncardiac critical illnesses. This study evaluated whether cTnI is useful for predicting the prognosis in noncardiac critically ill patients. Methods : From June 2003 to July 2004 at Gyeongsang National University Hospital, we enrolled 215 patients (male:142, female:73, mean age:$63{\pm}15$ years ) who were admitted for critical illness other than acute coronary syndrome at the medical intensive care unit(ICU). The severity score of critical illness (SAPS II and SOFA) was determined and serum cTnI level was measured within 24 hours after admission to the ICU. The mortality rate was compared between the cTnI-positive (${\geq}0.1{\mu}g/L$) and cTnI-negative ($cTnI<0.1{\mu}g/L$) patients at the $10^{th}$ and $30^{th}$ day after admission to the ICU. The mean cTnI value was compared between the survivors and non-survivors at the $10^{th}$ and $30^{th}$ day after admission to the ICU in the cTnI-positive patients. The correlation between cTnI and the severity of the critical illness score (SAPS II and SOFA) was also analyzed in cTnI-positive patients. Results : 1) The number of cTnI-negative and positive patients were 95(44%) and 120(56%), respectively. 2) The mortality rate at the $10^{th}$ and $30^{th}$ day after admission to the ICU was significantly higher in the cTnI-positive patients (29%, 41%) than in the cTnI-negative patients (12%, 21%)(p<0.01). 3) In the cTnI-positive patients, the mean value of the cTnI at the $10^{th}$ and $30^{th}$ day after admission to the ICU was significantly higher in the non-survivors ($4.5{\pm}9.2{\mu}g/L$, $3.5{\pm}7.9{\mu}g/L$) than in the survivors($1.8{\pm}3.6{\mu}g/L$, $2.0{\pm}3.9{\mu}g/L$) (p < 0.05). 4) In the cTnI-positive patients, the cTnI level was significantly correlated with the SAPS II score (r=0.24, p<0.001) and SOFA score (r=0.30, p<0.001). Conclusion : The cTnI may be a useful prognostic marker in noncardiac critically ill patients.

• Journal of Gastric Cancer
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• v.7 no.4
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• pp.185-192
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• 2007

Purpose: RUNX3, a novel tumor suppressor, is frequently inactivated in gastric cancer. In the present study, we examined the pattern of RUNX3 expression in gastric cancer cells from gastric cancer specimens and the impact of its alteration on clinical outcome. Materials and Methods: A total of 124 samples of both gastric cancer and normal tissue were obtained from 124 patients who underwent curative gastrectomy at the Seoul Medical Center from January 2001 to December 2005. RUNX3 expression was determined by immunohistochemical staining, and the results were analyzed. Statistical analysis wabased on clinicopathological findings and differences in survival rates. Results: The mean age of the patients was 61 years, and the male:female ratio was 1.9:1. The expression rate of RUNX3 was 59.7% (74/124). The expression rate was higher in differentiated gastric cancers (nucleus: 9.1%, cytoplasm: 57.6%) than in the undifferentiated types (nucleus: 5.2%, cytoplasm: 46.6%) (P=0.133). The 5-year survival rates according to RUNX3 expression determined from cancer tissue were 88.9% for the nucleus $\pm$ cytoplasm(+) group of patients, 76.1% for the cytoplasm only (+) group of patients, and 65.3% for the RUNX3 negative expression group of patients (P=0.626). Only UICC TNM staging showed statistical significance related to the survival rate, as determined by multivariate analysis. Conclusion: The RUNX3 expression rate was higher in differentiated gastric cancer than in the undifferentiated types without significance. Although RUNX3 expression predicted better survival, based on multivariate analysis, the finding was not statistically significant. More cases should be further evaluated.

• The Journal of the Korean bone and joint tumor society
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• v.13 no.1
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• pp.60-66
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• 2007

Purpose: The purpose of this study was to evaluate the functional outcomes of periacetabular malignant bone tumors treated by internal pelvectomy without reconstruction. Materials and methods: Between January 1996 and December 2005, eight patients with primary malignant or metastatic periacetabular bone tumors were treated by internal pelvectomy without reconstruction. There were 6 men and 2 women. Mean age was 42 years old. There were 3 osteosarcomas, 3 chondrosarcomas and 2 metastatic carcinomas. The type of pelvic resections were 6 type I+II+III, 1 type I+II and 1 type II+II resection. The functional outcomes were evaluated with ISOLS revised criteria. The follow up period ranged from 6 to 84 months. Results: At last follow up, 5 patients showed CDF, 2 patients, AWD and the remained 1, DOD. The mean functional score for pain, functional activity, emotional acceptance, use of external support, walking ability and gait were 4.9, 2.9, 2.9, 1.5, 2.3 and 2.5 respectively. The total functional score ranged from 37% to 70%(average: 56%). There were three temporary nerve palsies. Conclusion: The internal pelvectomy without reconstruction for selective difficult periacetabular malignant tumors could be a viable option with fewer complications and fair functional outcomes.

• Clinical and Experimental Pediatrics
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• v.45 no.8
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• pp.1000-1006
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• 2002

Purpose : In the course of treatment, patients with hematological or oncological disorders often develop pulmonary complication. The patients who develop a severe pulmonary complication have a poor outlook. The causes of pulmonary complication are either infectious or non-infectious in origin. We have analyzed the etiology and outcome of these patients admitted to the pediatric intensive care unit of Asan Medical Center. Methods : Medical records of 95 patients on Pediatric oncology service who were admitted to pediatric intensive care unit(PICU) of Asan Medical Center from Jan 1997 to May 2000 were retrospectively reviewed. Results : The mean age of the patients was 8.5 years(2 months-18 years). The underlying malignancies of these 95 patients were as following; acute lymphoblastic leukemia(31 cases), lymphoma (11 cases), acute myeloid leukemia(nine cases), brain tumor(eight cases) and other solid tumors(25 cases). Pulmonary complications included pneumonia, acute respiratory failure, pneumothorax and pleural effusion. The most common cause of pulmonary complication was infection(88%) in etiology. The overall mortality rate was 56.8%. Pulmonary complications in these patients carried high rates of mortality regardless of whether they were immune compromised(76%) or not(69%). Even without pulmonary complications, the hematological or oncological patients admitted to PICU had high mortality rates of 43%. Conclusion : Pulmonary complications are frequent finding in the hematological or oncological patients admitted to Intensive Care Unit. The main etiology of these pulmonary complications was infection, which carried a high mortality rate regardless of their immune status at the time when they were admitted to PICU.

• Clinical and Experimental Pediatrics
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• v.53 no.1
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• pp.80-84
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• 2010

Purpose : The aims of this study were to investigate the long-term outcomes in children with infantile spasms (IS) and to identify the prognostic factors influencing their neurodevelopment. Methods : We retrospectively evaluated seventy two children over five years old who were treated for IS at Asan Medical Center, Seoul, Korea, between 1994 and 2007. Forty-three children were contacted by telephone or medical follow-up to assess their current neurodevelopmental status. Multiple logistic regression was used to calculate odds ratios (ORs) and 95% confidence interval (95% CIs) of risk factors for unfavorable outcomes.Results : The mean follow-up duration for these 43 children was $7.2{\pm}1.5$ years (range, 4.5 to 13.0 years). Of these, 13 (30.2%) had cryptogenic and 30 (69.8%) had symptomatic IS. Eleven (25.6%) children were initially treated with adrenocorticotrophic hormone (ACTH) therapy, with a mean treatment lag of $1.3{\pm}1.9$ months (range; 0.1 to 7.0 months). Eighteen (41.8%) children clinically responded to initial treatment, as shown by EEG response. Overall, 22 (51.2%) children had at least moderate neurodevelopmental disorders and 2 (4.8%) died. In univariate analysis, etiology (symptomatic) and poor electroclinical response to initial treatment were related to long-term unfavorable outcomes. In multivariate analysis, response to primary treatment was the sole significant independent risk factor with a high OR. Conclusion : Overall prognosis of children with IS was poor. Electroclinical non-responsiveness to initial treatment was related to unfavorable long-term outcomes, indicating that initial control of seizures may be important in reducing the likelihood of poor neurodevelopment.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.3
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• pp.92-95
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• 2017

Fabry disease (FD) is an X-linked lysosomal storage disorder caused by an ${\alpha}$-galactosidase A (GLA, MIM 300644) enzyme deficiency due to pathogenic variants in the ${\alpha}$-galactosidase A gene (GLA). The disease leads to accumulation of globotriaosylceramide (Gb3) and related glycophospholipids affecting nearly all major organ systems, with the primary sites damaged by Gb3 including renal glomeruli, myocardium, neurons of the dorsal ganglion and autonomic nervous system, and vascular endothelial and smooth muscle. Progressive deposition in these organ systems present with various clinical manifestations including acroparesthesia, renal failure and heart failure. Here, we report a Chinese male diagnosed with Fabry disease in his late $4^{th}$ decades showing improvement of acroparesthesia during enzyme replacement therapy (ERT). A 48-year-old Chinese man who presented with chronic recurrent severe burning pain in his fingers and toes since the age of 10, with worse involvement of the former visited to our clinic for further evaluation. His medical history included a transient ischemic attack aged 40 and diagnosed with stage 4-5 chronic kidney disease aged 47. In the family history, the patient's brother was found to be have Fabry disease 1 month before his visit. Except for his brother, all other members of the family are healthy. Based on his medical history and family history, he was strongly suspicious for Fabry disease. He was found to have a galactose-alpha-1,3-galactose level 4.96 (Reference range, 42.5-67.9) suggestive of Fabry disease. The followed sequencing of GLA coding region in our patient revealed hemizyosity for the mutation c.988C>T (Q330X) in Exon 7. Since ERT start, he showed significant improvement in his symptoms of burning sensation of fingers and toes. On the contrary, due to deteriorating kidney function even with ERT, he is considered for kidney transplantation. Despite of diagnostic delay until late 4th decades, ERT showed a potential improvement of acroparesthesia in our patient. However, late start of ERT can lead to poor outcome in multiorgan function. Therefore, early diagnosis with high index of suspicion followed by continuous ERT with regular monitoring have an impact on quality of life in Fabry disease.