• Journal of Chest Surgery
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• v.40 no.1 s.270
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• pp.32-36
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• 2007

Background: Although acute renal failure (ARF) after coronary artery bypass graft (CABG) is relatively rare, but devastating complication with high mortality. Our study aims to evaluate the effectiveness of early application of CRRT in patients with ARF which developed after on-pump CABG. Material and Method: Two hundred and eighty seven patients underwent isolated on-pump CABG between May 2002 and Feb. 2006 at our institution, of whom 15 (5.2%) needed CRRT (11 patients for postoperatively developed ARF and the remaining 4 patients with preexisting dialysis-dependent chronic renal failure (CRF) for postoperative hemodynamic and metabolic control). Criteria for early application of CRRT were as follows; decreased urine output less than 0.5cc/h/kg for 2 consecutive hours and elevated serum creatinine level greater than 2.0 mg/dL. Result: The incidence of ARF requiring CRRT after on-pump CABG was 3.9% (11/283) and the overall hospital mortality of patient with CRRT was 33.3% (5/15). Of 5 deaths, 4 were patients with postoperatively developed ARF, and 1 was a patient with pre-existing dialysis-dependent CRF patient. The mean time between the operation and the initiation of CRRT was $25.8{\pm}5.8$ hours and the mean duration of CRRT was $62.1{\pm}41.2$ hours. Of the 7 survivors who were not on dialysis-dependent preoperatively, 6 patients fully recovered renal function during hospital stay and 1 patient required permanent renal supportive treatment after discharge from hospital. Conclusion: Early application of CRRT could maintain stable postoperative hemodynamic status and make outcomes better than those of previous reports in patients with ARF which developed after on-pump CABG.

• Journal of Chest Surgery
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• v.45 no.4
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• pp.236-241
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• 2012

Background: Prolonged usage of extracorporeal membrane oxygenation (ECMO) may induce multi-organ failure. This study is aimed to evaluate prognostic factors in the patients with ECMO. Also, the prognosis of ECMO with Kidney Injury Network Scoring system is studied. Materials and Methods: From May 2005 to July 2011, 172 cases of ECMO were performed. The cases of perioperative use of ECMO were excluded. Renal failure patient and younger than 15 years old one were also excluded. As a result, 26 cases were enrolled in this study. Male patients were 15 (57.7%), and mean age was $56.57{\pm}17.03$ years old. Demographic data, ECMO parameters, weaning from ECMO, and application of continuous renal replacement therapy are collected and Acute Kidney Injury Network (AKIN) scores were evaluated just before ECMO and day 1, day 2 during application of ECMO. Results: Venoarterial ECMO was applied in 22 cases (84.6%). The reasons for applications of ECMO were cardiac origin in 21 (80.8%), acute respiratory distress syndrome in 4, and septic shock in 1 case. Successful weaning from ECMO was achieved in 15 cases (57.7%), and survival discharge rate was 9 cases (34.6%). Mean duration of application of ECMO was $111.39{\pm}54.06$ hours. In univariate analysis, myocarditis was independent risk factors on weaning failure. Using the receiver operating characteristic curve, level of hemoglobin on 24 hours after ECMO, and base excess on 48 hours after ECMO were showed more than 0.7. AKIN score was not matched the prognosis of the patients with ECMO. Conclusion: In our study, the prognosis of the patients with myocarditis was poor. Hemoglobin level at first 24 hours, and degree of acidosis at 48 hours were useful methods in relating with prognosis of ECMO. AKIN scoring system was not related with the prognosis of the patients. Further study for prognosis and organ injury during application ECMO may be needed.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.24 no.3
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• pp.288-294
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• 2021

Purpose: Despite aggressive medical and nutritional management, patients with methylmalonic acidemia (MMA) often suffer from multi-organ damage. Early deceased donor liver transplantation (DDLT) has emerged as an intervention to prevent disease progression. We investigated the efficacy of living donor LT (LDLT) with a potential carrier of MMA and a small volume of graft in patients with MMA as an alternative to DDLT. Methods: Of five patients (three male, two female; median age 5.7 years; range, 1.3-13.7 years), four underwent carrier LDLT, while one underwent non-carrier auxiliary LDLT. All patients received pre- and post-LT continuous renal replacement therapy and were provided with minimal restriction diet according to serum MMA level after LT. MMA levels in the serum and urine, the incidence of metabolic crisis, and clinical findings before and after LT were compared. Results: The survival rate was 100% during 2.2 years of follow up period after LT. In all five cases, MMA titer in the serum after transplantation decreased with less restrictive diet. Metabolic crisis was not observed during the follow-up period. In addition, no patient showed progression of severe renal impairment requiring hemodialysis. Progression of delayed cognitive development was not observed. Social functioning with improved neuropsychiatric development was observed. Conclusion: This study showed that LDLT achieved improved quality of life with less restrictive diet, therefore it could be a feasible alternative option to DDLT for the treatment of patients with MMA, even with an auxiliary LT.

• 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.

• Journal of Yeungnam Medical Science
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• v.34 no.1
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• pp.123-127
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• 2017

Drug-induced immune hemolytic anemia (DIIHA) is a rare side effect of drugs. DIIHA may cause a systemic inflammatory response that results in acute multi-organ failure and death. Ceftizoxime belongs to the class of third generation cephalosporins, which are the most common drugs associated with DIIHA. Herein, we present a case of a 66-year-old man who developed fatal DIIHA after receiving a second dose of ceftizoxime. He was admitted to receive photodynamic therapy. He had a history of a single parenteral dose of ceftizoxime 3 months prior to admission. On the day of the procedure - shortly after the infusion of ceftizoxime - the patient's mental status was altered. The blood test results revealed hemolysis. Oliguric acute kidney injury developed, and continuous renal replacement therapy had to be applied. On the suspicion of DIIHA, the patient underwent plasmapheresis. Diagnosis was confirmed by a detection of drug-dependent antibody with immune complex formation. Although his hemolysis improved, his liver failure did not improve. He was eventually discharged to palliative care, and subsequently died.

• The Korean Journal of Physiology and Pharmacology
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• v.19 no.3
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• pp.229-234
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• 2015

Nafamostat mesilate (NM) is a serine protease inhibitor with anticoagulant and anti-inflammatory effects. NM has been used in Asia for anticoagulation during extracorporeal circulation in patients undergoing continuous renal replacement therapy and extra corporeal membrane oxygenation. Oxidative stress is an independent risk factor for atherosclerotic vascular disease and is associated with vascular endothelial function. We investigated whether NM could inhibit endothelial dysfunction induced by tumor necrosis factor-${\alpha}$ (TNF-${\alpha}$ ). Human umbilical vein endothelial cells (HUVECs) were treated with TNF-${\alpha}$ for 24 h. The effects of NM on monocyte adhesion, vascular cell adhesion molecule-1 (VCAM-1) and intracellular adhesion molecule-1 (ICAM-1) protein expression, p38 mitogenactivated protein kinase (MAPK) activation, and intracellular superoxide production were then examined. NM ($0.01{\sim}100{\mu}g/mL$) did not affect HUVEC viability; however, it inhibited the increases in reactive oxygen species (ROS) production and p66shc expression elicited by TNF-${\alpha}$ (3 ng/mL), and it dose dependently prevented the TNF-${\alpha}$ -induced upregulation of endothelial VCAM-1 and ICAM-1. In addition, it mitigated TNF-${\alpha}$ -induced p38 MAPK phosphorylation and the adhesion of U937 monocytes. These data suggest that NM mitigates TNF-${\alpha}$ -induced monocyte adhesion and the expression of endothelial cell adhesion molecules, and that the anti-adhesive effect of NM is mediated through the inhibition of p66shc, ROS production, and p38 MAPK activation.

• Journal of the Korea Academia-Industrial cooperation Society
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• v.19 no.11
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• pp.421-425
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• 2018

Methanol is a clear, colorless, volatile, and poisonous liquid that is commonly used as an industrial solvent. Visual impairment is a common symptom of methanol poisoning; however, visual impairment rarely occurs after exposure through inhalation. Therefore, visual loss after methanol intoxication via respiration has rarely been reported. We report a case of visual damage associated with methanol poisoning via respiratory exposure in an industrial setting. In this case in South Korea, a 28-year-old woman who worked at a cell phone factory was admitted to the emergency department with mental changes. She had blurred vision that began two days prior, but she did not come to the hospital until she experienced mental changes. She ranked 9 on the Glasgow Coma Scale and presented with severe metabolic acidosis. So, she was admitted to intensive care, and continuous renal replacement therapy was performed. Finally, she was discharged after recovery of her mental state, but had to undergo rehabilitation for six months. Also, her visual impairment was permanent. Methanol intoxication can occur through inhalation, which is difficult to detect initially. However, treatment of methanol poisoning is time-critical. Therefore, doctors should always keep in mind that methanol intoxication may occur via respiration. If in doubt, treatment should be given as soon as possible.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.2
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• pp.55-61
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• 2018

Orinithine transcarbamylase (OTC) deficiency is the most common inborn error of the urea cycle with resulting hyperammonemia, which is medical emergency in newborns.We recently had a case of a boy that presented with lethargy, seizure, hyperammonemia and hypocalcemia in neonatal period. He was diagnosed with OTC deficiency by two consequent ways which are initial biochemical phenotype including hyperammonemia and an increased orotic acid in his urine and genetic analysis of the OTC gene. The OTC gene showed a novel hemizygous mutation c.913C>T (p.Pro305Ser). He was treated by low protein intake, sodium benzoate, phenylbutyrate sodium, L-arginine, and continuous renal replacement therapy (CRRT). After discharge, he has a relatively good prognosis without notable developmental delay. For good prognosis, the duration of hyperammonemia should be shorten. And it can be reached by an early diagnosis. For early detection of OTC deficiency, targeted exome sequencing will be a important role as well as biochemical tests.

• Journal of Korean Neurosurgical Society
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• v.67 no.1
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• pp.84-93
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• 2024

Objective : We aimed to examine trends in critically ill neurology-neurosurgery (NNS) patients who were admitted to the intensive care unit (ICU) in South Korea and identify risk factors for in-hospital mortality after ICU admission in NNS patients. Methods : This nationwide population-based retrospective cohort study enrolled adult NNS adult patients admitted to the ICU from 2010 to 2019 extracted from the National Health Insurance Service in South Korea. The critically ill NNS patients were defined as those whose main admission departments were neurology or neurosurgery at ICU admission. The number of ICU admission, age, and total cost for hospitalization from 2010 to 2019 in critically ill NNS patients were examined as trend information. Moreover, multivariable logistic regression modeling was used to identify risk factors for in-hospital mortality among critically ill NNS patients. Results : We included 845474 ICU admission cases for 679376 critically ill NNS patients in South Korea between January 1, 2010 to December 31, 2019. The total number of ICU admissions among NNS patients was 79522 in 2010, which increased to 91502 in 2019. The mean age rose from 62.8 years (standard deviation [SD], 15.6) in 2010 to 66.6 years (SD, 15.2) in 2019, and the average total cost for hospitalization per each patient consistently increased from 6206.1 USD (SD, 5218.5) in 2010 to 10745.4 USD (SD, 10917.4) in 2019. In-hospital mortality occurred in 75455 patients (8.9%). Risk factors strongly associated with increased in-hospital mortality were the usage of mechanical ventilator (adjusted odds ratio [aOR], 19.83; 95% confidence interval [CI], 19.42-20.26; p<0.001), extracorporeal membrane oxygenation (aOR, 3.49; 95% CI, 2.42-5.02; p<0.001), and continuous renal replacement therapy (aOR, 6.47; 95% CI, 6.02-6.96; p<0.001). In addition, direct admission to ICU from the emergency room (aOR, 1.38; 95% CI, 1.36-1.41; p<0.001) and brain cancer as the main diagnosis (aOR, 1.30; 95% CI, 1.22-1.39; p<0.001) are also potential risk factors for increased in-hospital mortality. Conclusion : In South Korea, the number of ICU admissions increased among critically ill NNS patients from 2010 to 2019. The average age and total costs for hospitalization also increased. Some potential risk factors are found to increase in-hospital mortality among critically ill NNS patients.

• Journal of Chest Surgery
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• v.42 no.4
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• pp.464-472
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• 2009

Background: We analyzed the clinical results and the factors for survival of patients who underwent extracorporeal life support system after adult cardiovascular surgery. Material and Method: We retrospectively reviewed the medical record of 44 patients (1.6% of the total adult cardiovascular surgical cases) who underwent the use of a ventricular assisted device or extracorporeal membrane oxygenation from January 2002 to August 2008. There were 32 (72.7%) males and their mean age was 61.7$\pm$14.9(range: 20$\sim$73) years old. The mean duration of extracorporeal life support system was 5.3$\pm$3.0 (range: 1$\sim$12) days. Result: Of these 44 patients, 24 (54.5%) patients were successfully weaned from the extracorporeal device. Eighteen (40.9%) survivors were able to be discharged from the hospital. Complications were noted in 38 patients (86.4%). An emergency operation, no usage of a concomitant intraaortic balloon pump and major complications during use of the extra corporeal life support system such as bleeding, flow instability and renal failure were identified as significant risk factors for poor survival on univariated analysis. Owing to educational support and a continuous renal replacement therapy system, the clinical outcomes of these patients have improved since 2006. On multivariated analysis, renal failure and bleeding during extracorporeal life support were significant risk factors for poor survival. Conclusion: Although using. extracorporeal life support systems after adult cardiovascular surgery revealed acceptable clinical results, determining the optimal treatment strategy and further well designed larger studies are needed to improve the survival rate of patients who undergo extracorporeal life support after adult cardiovascular surgery.