• Annals of Hepato-Biliary-Pancreatic Surgery
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• v.28 no.2
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• pp.238-247
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• 2024

Backgrounds/Aims: Prolonged use of steroids after liver transplantation (LT) significantly increases the risk of diabetes or cardiovascular disease, which can adversely affect patient outcomes. Our study evaluated the effectiveness and safety of early steroid withdrawal within the first year following LT. Methods: This study was conducted as an open-label, multicenter, randomized controlled trial. Liver transplant recipients were randomly assigned to one of the following two groups: Group 1, in which steroids were withdrawn two weeks posttransplantation, and Group 2, in which steroids were withdrawn three months posttransplantation. This study included participants aged 20 to 70 years who were scheduled to undergo a single-organ liver transplant from a living or deceased donor at one of the four participating centers. Results: Between November 2012 and August 2020, 115 patients were selected and randomized into two groups, with 60 in Group 1 and 55 in Group 2. The incidence of new-onset diabetes after transplantation (NODAT) was notably higher in Group 1 (32.4%) than in Group 2 (10.0%) in the per-protocol set. Although biopsy-proven acute rejection, graft failure, and mortality did not occur, the median tacrolimus trough level/dose/weight in Group 1 exceeded that in Group 2. No significant differences in safety parameters, such as infection and recurrence of hepatocellular carcinoma, were observed between the two groups. Conclusions: The present study did not find a significant reduction in the incidence of NODAT in the early steroid withdrawal group. Our study suggests that steroid withdrawal three months posttransplantation is a standard and safe immunosuppressive strategy for LT patients.

• Radiation Oncology Journal
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• v.6 no.2
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• pp.211-226
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• 1988

Among 165 patients of esophagus cancer treated by either radiation alone or postoperative radiation, median survival period was 6.6 months, $16\%$ 3 years and $8\%$ 5years crude survival. In biphasic plotting of survival curve semilogarithmically all nonresponder died within one year regardless of treatments and in responder each 1, 2, 3 years survival rate was $80\%,\;70\%,\;60\%$ in the group of postoperative radiation among 20 patients ($54\%$ of 37 patients) respectively and $62\%,\;38\%,\; 23\%$ each in the group of radiation alone among 61 patients ($48\%$ of 128 patients) respectively, better survival rate of postoperative radiation vs radiation alone in 3 year (P<0.01). The most common cause of death was dysphagia $55\%$, and majority of patients died by failure to control the disease locally $62\%,\;88\%$ of stricture were associated with persistenece of cancer in esophagus. $50\%$ of patients was found to have locoregional metastatc nodes. Preoperative diagnostic failure rate was for metastatic locoregional nodes was $54\%$, for grossly metastatic nodes $29.7\%$, for blood borne organ metastasis $13.5\%$, and for local extent of the disease $14\%$. The residual cancer at surgical margin o. postitive node was not effectively killed by either 5000 to 5500 cGy conventional radiation or 5290 to 5750 cGy with 115 cGy fraction in 2 times daily; hyperfractionated radiation. However hyperfractionation schedule decreased the both acute and late complications in this study.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.3
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• pp.148-154
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• 2016

A urea cycle disorder is a condition caused by a defect of the enzymes in the urea cycle, and deficiency of ornithine transcarbamylase (OTC), which converts carbamoyl phosphate and ornithine into citrulline, is the most common type of the disorder. OTC deficiency induces the accumulation of precursors of urea, ammonia, and glutamine, leading to neurological symptoms including hypotonia, respiratory failure, seizure, lethargy, and coma and sometimes to death. Because OTC deficiency is inherited in an X-linked manner, typical symptoms such as vomiting, poor feeding, and lethargy appear mainly in male neonates. We recently had a case that presented with neonatal onset lethargy, vomiting, and apnea in a 4-day-old boy. He was diagnosed with OTC deficiency by biochemical phenotype, including hyperammonemia and an increased orotic acid level in the urine. Genetic analysis of the OTC gene showed a novel mutation c.780_781insCAGGCAGTGT (p.Ile261Glnfs*35). He was treated for hyperammonemia using continuous venovenous hemofiltration (CVVH) at 118 hours after birth. After 4 days of CVVH, his consciousness and blood ammonia concentration were normalized, and he was discharged at the age of 53 days. At around 12 months of age, bilateral femur fractures and osteomyelitis occurred in this patient. Two months after the fracture, he died of septic shock, insulin-resistant hyperglycemia, and multi-organ failure.

• Journal of Chest Surgery
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• v.29 no.11
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• pp.1212-1217
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• 1996

From Dec. 1993 to May 1995, 9 male and 5 female patients ranging in age from 25 to 65 years, were operated on for ascending aorta and/or aortic arch diseases. Six patients had acute aortic dissection, type A(ruptured in 4 cases); four had ruptured ascending aortic aneurysm; three had annuloaortic ectasia(ruptured in 1 cases); one had aortic arch aneurysm. The diagnostic procedures were echo cardiography and dynamic CT scan in all patients having acute dissection or rupture. The aortic angiography was performed in two cases. Indications for operations were rupture in five cases, acute aortic dissection in five cases, severe congestive heart failure in two cases, progressive aortic insufficiency in one case and impending rupture in one case. The emergent repair was performed in ten cases(71%). The surgical treatment consisted of 6 Cabrol operations, a Cabrol operation combined with arch replacement, a modified Bentall operation, 4 replacement of ascending aorta, a replacement of aortic arch, and a replacement of ascending aorta and aortic arch. Complications were a hypoxic encephalopathy, two atrial fibrillations, a sternal deheiscence, and a mediastinitis. Two early mortality(14%) were due to intractable bleeding and multiple organ failure, and one late mortality(7%) was due to ventricular arrhythmia. In eleven survivors, follow-up period was from 2 months to 12 months and the course was uneventful.

• Clinical and Experimental Pediatrics
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• v.52 no.10
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• pp.1153-1160
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• 2009

Purpose:To evaluate the risk factors for mortality and prognostic factors in pediatric hemato-oncology patients admitted to the pediatric intensive care unit (PICU). Methods:We retrospectively reviewed the medical records of pediatric hemato-oncology patients admitted at the PICU of the Asan Medical Center between September 2005 and July 2008. Patients admitted at the PICU for perioperative or terminal care were excluded. Results:Total 88 patients were analyzed. Overall ICU mortality rate was 34.1%. Mean age at PICU admission was $7.0{\pm}5.7$ years and mean duration of PICU stay was $18.1{\pm}22.2$ days. Hematologic diseases contributed to 77.3% of all the primary diagnoses, and the primary cause of admission was respiratory failure (39.8%). The factors related to increased mortality were C-reactive protein level (P<0.01), ventilation or dialysis requirement (P<0.01), and hematopoietic stem cell transplantation (P<0.05). In all, 3 scoring systems were investigated [Number of Organ System Failures (OSF number), the Pediatric Risk of Mortality III (PRISM III) score, and the Sequential Organ Failure Assessment (SOFA) score]; higher score correlated with worse outcome (P<0.01). The Oncological Pediatric Risk of Mortality (O-PRISM) scores of the 21 patients who had received hematopoietic stem cell transplantation were higher among the non-survivors, but not statistically significant (P=0.203). Conclusion:The PRISM III and SOFA scores obtained within 24 hours of PICU admission were found to be useful as early mortality predictors. The highest OSF number during the PICU stay was closely related to poor outcome.

• 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.

• Tuberculosis and Respiratory Diseases
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• v.54 no.4
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• pp.429-438
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• 2003

Background : The mortality from acute respiratory distress syndrome(ARDS) is >40-50%. Although some prospective trials have failed to demonstrate a survival benefit of steroids in the early stages of ARDS, there are some reports showing some success with steroids in the later stages. This study observed the changes in the physiologic parameters with time in late ARDS patients who were treated with steroids. Methods : The medical charts of 28 intensive care unit patients(male:female=24:4; mean age 64 years), who had been diagnosed with refractory late ARDS ($PaO_2/FIO_2$ <200) and were treated with corticosteroids from December 1999 to July 2002, were retrospectively reviewed. The patients were divided into two groups: the weaned group(n=14), which included the patients who had been successfully weaned from a ventilator after corticosteroid therapy, and the failed group(n=14), which included the patients who had failed weaning. The physiologic parameters included the $PaO_2/FIO_2$ ratio, the positive end-expiratory pressure(PEEP) level, the $PaCO_2$, compliance, the sequential organ failure assessment(SOFA) score, the acute physiologic and the chronic health evaluation(APACHE) II score, and the Murray Lung Injury Score(LIS) in the two groups were compared from the day of mechanical ventilation(Dmv) to 7 days after the corticosteroid therapy. Results : There was no significant difference in the clinical characteristics and the physiologic parameters between the two groups prior to the corticosteroid therapy except for the SOFA score at Dmv(weaned group : $6.6{\pm}2.5$ vs failed group : $8.8{\pm}2.9$, p=0.047). However, within 7 days after corticosteroid therapy, there was significant improvement in the $PaO_2/FIO_2$ ratio, the PEEP level, the $PaCO_2$, the SOFA score, the APACHE II score, and the LIS of the weaned group compared to the failed group. Conclusions : During corticosteroid therapy in late ARDS, the continuation of corticosteroid therapy should be determined carefully in patients who do not show improvement in their physiologic parameters by day 7.

• Clinical and Experimental Pediatrics
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• v.48 no.1
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• pp.68-74
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• 2005

Purpose : There is growing use of continuous renal replacement therapy(CRRT) for pediatric patients, but reports about the use and outcome of CRRT in children is rare in Korea. We report our experiences of CRRT in critically ill pediatric patients. Methods : We reviewed the medical records of 23 pediatric patients who underwent CRRT at Asan Medical Center between May 2001 and May 2004. We evaluated underlying diseases, clinical features, treatment courses, CRRT modalities and outcomes. Results : Ages ranged from three days to 16 years with a median of five years. Patients weighed 2.4 to 63.9 kg(median 23.0 kg; 10 patients ${\leq}20kg$). The underlying diseases were malignancy(nine cases), multiple organ dysfunction syndrome(five cases), hyperammonemia(four cases), acute renal failure associated with liver failure(three cases), dilated cardiomyopathy(one case) and congenital nephrotic syndrome(one case). Pediatric Risk of Mortality(PRISM) III score was $17.6{\pm}7.6$ and the mean number of failing organs was $3.0{\pm}1.7$. Duration of CRRT was one to 27 days(median : nine days). Eleven patients(47.8%) survived. Chronic renal failure developed in two cases, intracranial hemorrhage in one case, and chylothorax in one case among the survivors. PRISM III score and the number of vasopressor before the start of CRRT was significantly lower in the survivors($12.7{\pm}4.2$ and $0.9{\pm}1.1$) compared with nonsurvivors($22.1{\pm}7.8$ and $2.4{\pm}1.4$)(P<0.05). Conclusion : CRRT driven in venovenous mode is an effective and safe method of renal support for critically-ill infants and children to control fluid balance and metabolic derangement. Survival is affected by PRISM III score and the number of vasopressors at the initiation of CRRT.

• Childhood Kidney Diseases
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• v.11 no.2
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• pp.247-254
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• 2007

Purpose : Continuous renal replacement therapy(CRRT) has been the first choice for the treatment of acute renal failure in critically ill children not only in western countries but also in Korea. However, there are very few studies that have analyzed the outcome and prognosis of this modality in Korean children. We performed this study to evaluate the factors associated with the outcome and prognosis of patients treated with CRRT. Methods : We retrospectively reviewed the medical records of 32 children who had received CRRT at Severance hospital from 2003 to 2006. The mean age was 7.5 years(range 4 days-16 years) and the mean body weight was 25.8 kg (range 3.2-63 kg). Results : Eleven(34.4%) of the 32 patients survived. Bone marrow transplantation and malignancy were the most common causes of death and underlying disease leading to the need for CRRT Mean patient weight, age, duration of CRRT, number of organ failures, urine output, estimated glomerular filtration rate(eGFR), C-reactive protein, and blood urea level did not differ significantly between survivors and nonsurvivors. (1) Pediatric risk of mortality(PRISM) III score at CRRT initiation($9.8{\pm}5.3$ vs. $26.7{\pm}7.6$, P<0.0001), (2) maximum pressor number ($2.1{\pm}1.2$ vs. $3.0{\pm}1.0$, P=0.038), and (3) the degree of fluid overload($5.2{\pm}6.0$ vs. $15.0{\pm}8.9$, P=0.002) were significantly lower in survivers than in nonsurvivors. Multivariate analysis revealed that fluid overload was the only independent factor reducing survival rate. Conclusion : CRRT was successfully applied to the treatment of acute renal failure in a wide range of critically ill children. To improve survival, we suggest the early initiation of CRRT to prevent the systemic worsening and progression of fluid overload in critically ill children with acute renal failure. (J Korean Soc Pediatr Nephrol 2007;11:247-254)

• Radiation Oncology Journal
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• v.10 no.2
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• pp.205-212
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• 1992

Ninety five patients of rectal cancer treated with surgery with or without adjuvant radiation therapy since January 1982 to December 1990 at the Chonnam University Hospital were analysed retrospectively regarding local failure. Of these 95 patients 72 patients were treated with surgery alone and remaining 23 patients received postoperative radiation therapy to pelvis. There were 45 men and 50 women with 53 years of median age. Minimum follow-up period was 19 months and median was47 months (range, 19-125 months). Kaplan-Meier method was used to calculate actuarial risk of local recurrence and survival rate. Comparison between two groups was evaluated by Log rank test. Of total 95 patients twenty seven patients ($28.4\%$) developed local recurrence and 13 patients ($17.3\%$) developed local and distant metastasis concomitantly. Eighty nine percent (24/27) of patients developed local recurrence within 24 months. Pelvic organ adjacent to the primary tumor area was the most common site of initial local recurrence. Of 72 patients treated with surgery alone local recurrence developed in 24 patients. Of 17 patients with stage A and 81 (Gunderson-Sosin modification of Dukes' staging system) 6 patients experienced local recurrence ($31.2\%$). The local recurrence rate of B2 and B3 group was $29.9\%$ (7/33) and that of C2 and C3 was $54.7\%$ (11/19), respectively. There was statistically significant difference between two groups (p<0.05). Of 23 patients treated with definitive surgery and radiation therapy $10\%$ (1/10) recurred in B2 and B3 patients. This was slightly lower than C2 and C3 patients ($22.2\%$, 2/10) of similar policy, but revealed no statistically significant difference (p>0.05). In the patients of B2+3 local failure rate decreased when radiation therapy was added ($29.9\%$ vs $10\%$, p>0.05) and also similar results in C2+3 group ($34.7\%$ vs $22.2\%$, p<0.05). The local failure rate in relation to distance from the anal verge had no statistically significant difference.