• Clinical and Experimental Pediatrics
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• v.45 no.8
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• pp.1028-1032
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• 2002

Subcutaneous pannicultis-like T cell lymphoma is a rare cutaneous T cell lymphoma. It presents with multiple subcutaneous nodules or plaques involving the extremities or trunk, and with constitutional symptoms that include fever, malaise, fatigue, myalgia, chills and weight loss. Histologically, the lesions of this disease are reminiscent of panniculitis and are composed of a mixture of small and large atypical lymphoid cells infiltrating between adipocytes. The optimal treatment for this disease is undefined and prognosis of this disease is poor, even when treated with multiagent chemotherapy regimens considered optimal for agressive lymphoma of other types. Poor prognosis factors include clinical features such as anemia, leukocytopenia, hepatosplenomegaly, lymphadenopathy and coagulopathy, which are suggestive of hemophagocytosis. Much of the mortality of this disease is due not to disseminated lymphoma with organ failure, but rather to complications of the cytopenias associated with the hemophagocytic syndrome. We report a case of subcutaneous panniculitis-like T cell lymphoma in a 12 year-old boy who presented with initial complaints of fever and multiple subcutaneous nodules, and briefly review the related literature.

• Clinical and Experimental Pediatrics
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• v.46 no.1
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• pp.42-50
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• 2003

Purpose : The acute respiratory distress syndrome(ARDS), an acute form of severe alveolar-capillary injury evolving after a direct or indirect lung insult is thought to be a common cause of respiratory failure though not many clinical studies on the subject have been made yet. Methods : Between January 1992 and December 2001, we conducted a retrospective study on 33 children who fulfilled the definition of the ARDS recommended by the American-European Consensus Conference in 1994. Results : A total of 33 patients(20 boys and 13 girls) were selected. Their age ranged from 4 months to 12 years with seven children younger than 1 year. The overall mortality rate was 78.8% and no significant difference was noted based on age or sex. Concerning seasonal variation, incidence of the ARDS increased in spring, especially in May(21.2%). Pneumonia(66.7%) was found to be the most common risk factor of the ARDS followed by sepsis(24.2%) and aspiration(3.0%). In immune compromised children(six cases), including a recipient of bone marrow transplantation, the mortality rate was 100%. Compared with children with multiple organ failure recording a 83.3% mortality rate, those with isolated respiratory failure, showed a lower mortality rate of 33.3%, although stastistically insignificant. Between survivor and non-survivor groups, significant differences were shown in hematocrit, $PaO_2$, $PaCO_2$, PEEP, and $PaO_2/FiO_2$ on the seventh day after the onset of the ARDS. Conclusion : According to our study, respiratory failure proved to have a great effect on mortality rate in the ARDS. More aggressive intervention and further studies on this subject should be done to improve the survival rate.

• 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.

• Journal of Chest Surgery
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• v.18 no.2
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• pp.182-192
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• 1985

[here are so many reports that pulsatile blood flow provides physiologic organ perfusions during cardiopulmonary bypass. So, we compared the recent 30 cases undergoing cardiac surgery by Cobe-Stckert pulsatile roller pump with another 30 cases by Polystan nonpulsatile roller pump. Pulsatile flow was applied during aortic-cross clamping period when synchronized to internal EKG simulator, and perfusion mode was changed to continuous nonpulsatile flow after declamping of aorta. Age, sex, weight, and disease entities were comparable and operative techniques were similar between two groups. 1. There were no differences in average ACC time, ECC time, and Operation time. 2. Postoperative artificial respiration time was 6hrs 30mins in nonpulsatile group and 4hrs 48mins in pulsatile group, and detubation time after ventilator weaning was 2hrs 44mins in nonpulsatile group and 1hrs 43mins in pulsatile group. 3. Average pulse pressure was 8mmHg in nonpulsatile group and 55mmHg in pulsatile group, and a mean arterial pressure was 66.0mmHg in nonpulsatile group and 60.7mmHg in pulsatile group. 4. Mean urine-output during ACC;ECC period was 9.717.3;9.913.2ml/kg/hr in nonpulsatile group and 14.215.0;15.817.5 in pulsatile group [p<0, 05], and thereafter progressive decrease of differences in urine output between two groups until POD 2, and lesser amounts of diuretics was needed in pulsatile group during same postoperative period. Serum BUN/Cr level showed no specific difference and urine concentration power was well preserved in both groups. 5. Plasma proteins and other Enzymes showed no differences between two groups, but serum GOT/GPT level was higher in nonpulsatile group till POD 2. 6. Serum Electrolytes showed no differences between two groups. 7. WBC, RBC, Platelet counts, Hgb and Hct were not different and Coagulogram was well preserved in both groups. 8. Plasma free Hgb level was 7.09mg% in pulsatile group compared with 3.48mg% in pulsatile group on POD 1 but was normalized on POD 2. Gross hemoglobinuria after ECC was noted in 6 cases [20%] of pulsatile group and 4 cases [13%] of nonpulsatile group. 9. In both groups, most patients were included in NYHA class III to IV [28 cases;93% in nonpulsatile group, 22 cases;73% in pulsatile group] preoperatively, and well improved to class I to 11[22 cases; 73% in nonpulsatile group, 30 cases; 100% in pulsatile group] postoperatively. There were 7 operative mortalities in nonpulsatile group only, which were 5 cases of TOF with hepatic failure, 1 case of multiple VSDs with low out-put syndrome, and 1 case of mitral valvular heart disease with cardiomyopathy. We concluded that the new, commercially available Cobe-Stckert pulsatile roller pump device was safe, simple, and reliable.

• Journal of The Korean Society of Clinical Toxicology
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• v.13 no.2
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• pp.62-70
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• 2015

Purpose: Cardiac complications may occur in cases of organophosphate (OP) poisoning. However, a few studies regarding patterns of cardiac toxicity as determined by transthoracic echocardiography (TTE) after exposure to OP have been reported. In the current study, the authors examined cardiac functions using TTE in patients with myocardial injury caused by exposure to OP. Methods: A retrospective review was conducted on 16 consecutive cases of OP poisoning with myocardial injury (defined as elevated troponin I within 48 hours of arrival at the regional emergency center in South Korea and diagnosed and treated at the center from January 2012 to November 2014. Results: TTE was performed in 11 (69%) of the 16 patients with an elevated troponin I (TnI) level within 48 hours. Of these 11 patients, 5 patients (45.5%) exhibited reduced ejection fraction (EF), and 3 exhibited regional wall motion abnormality (RWMA). Two patients (18.2%) had both reduced systolic function and RWMA. Two of the 5 patients with reduced EF returned to normal systolic function, however two patients did not regain normal systolic function after admission. One patient expired due to multiple organ failure, and 4 patients were transferred with a moribund status. Twelve of 15 patients who survived to discharge (at 4 to 35 months) were followed. Five of these patients died during follow-up and 7 survived without further complications. Conclusion: OP can cause reversible cardiac dysfunction including reduced systolic function and RWMA. Serum TnI may be useful for initial assessment of cardiac function during the workup of patients suffering from OP poisoning. After the initial assessment of cardiac enzyme, further evaluation with TTE in patients with abnormal cardiac enzyme will be necessary to understand the cardiac toxicity.

• Tuberculosis and Respiratory Diseases
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• v.46 no.5
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• pp.729-734
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• 1999

Broncho-esophageal fistula(BEF) is an uncommon clinical entity which can cause severe suppurative lung disease. Acquired fistulas between the esophagus and tracheobronchial tree are relatively uncommon. They are caused by many diseases including malignancy and chronic inflammation such as tuberculosis and have favorable outcome with proper treatment To our knowledge, there has been no description of patients with BEF due to the bronchiectasis. We report a case of broncho-esophageal fistula in association with bronchiectasis in a 35-year-old male patient with hemoptysis. Bronchoscopy revealed mild bleeding from the superior segment of the right lower lobe without specific endobronchial lesion. Barium esophagogram could not confirm the fistula. The diagnosis of a broncho-esophageal fistula was established by an esophagogastroscopy using fistulogram and subsequent bronchoscopy, in which the communication between the bronchial tree and the esophagus was demonstrated by instilling dye selectively through the fistulous opening using esophagogastroscopy and visualizing the fistula and the bronchial tree. The patient was treated with resection of the right lower lobe, extirpation of the diverticulum and surgical closure of the bronchial defect and fistula, but he suffered from pneumonia thereafter and eventually expired due to sepsis and multiple organ failure.

• Journal of Hospice and Palliative Care
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• v.17 no.2
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• pp.57-65
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• 2014

Purpose: Since most terminally ill cancer patients die of multiple organ failure, plasma endotoxin concentration levels may be used to predict the life expectancy. This study was performed to evaluate the clinical significance of endotoxin level in plasma as a prognostic factor for survival in patients with terminal cancer. Methods: This study was conducted with 56 terminally ill cancer patients, above 20 years old, from April 2009 through October 2009. Demographic characteristics, Karnofsky performance status, and survival time were evaluated. We analyzed blood levels of white blood cell hemoglobin, hematocrit, aspartate aminotransferase, alanine aminotransferase, c-reactive protein, total bilirubin and endotoxin in each patient. Results: We considered following variable for univariate analysis: plasma endotoxin level, sex, age, WBC, hemoglobin, hematocrit, AST, ALT, total bilirubin, CRP and severity of pain. Univariate analysis did not show a significant association between plasma endotoxin level and survival time. However, in a multivariate analysis with factors that were found to be significantly associated with survival sex, WBC count and total bilirubin level in univariate analysis, high levels of plasma endotoxin and short survival time were significantly related. Conclusion: Plasma endotoxin level could be used as a prognostic factor to predict the life expectancy of terminally ill cancer patients.

• Clinical and Experimental Pediatrics
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• v.57 no.1
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• pp.50-53
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• 2014

Hemophagocytic lymphohistiocytosis (HLH) occurs in the primary form (genetic or familial) or secondary form (acquired). The familial form of HLH (FHL) is a potentially fatal autosomal recessive disorder that occurs because of constitutional defects in cell-mediated cytotoxicity. Here, we report a fatal neonatal case of type 2 FHL (FHL2) that involved a novel frameshift mutation. Clinically, the newborn presented with severe sepsis-like features and required mechanical ventilation and continuous venovenous hemodiafiltration. Flow cytometry analysis showed marked HLH and complete absence of intracytoplasmic perforin expression in cytotoxic cells; therefore, we performed molecular genetic analyses for PRF1 mutations, which showed that the patient had a compound heterozygous mutation in PRF1, that is, c.65delC ($p.Pro22Argfs^*2$) and c.1090_1091delCT ($p.Leu364Glufs^*93$). Clinical and genetic assessments for FHL are required for neonates with refractory fever and progressive multiple organ failure, particularly when there is no evidence of microbiological or metabolic cause.

• Journal of Trauma and Injury
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• v.25 no.4
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• pp.247-253
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• 2012

Purpose: Hemorrhage is a main cause of death in trauma patients. The goal of this study is to describe the characteristics of trauma patients with massive bleeding and to evaluate the prognostic factors concerning their survival. Methods: This study was performed retrospectively and included trauma patients with massive bleeding who had been treated from March 2007 to August 2012. The inclusion criterion was patients who received more than 10 U of packed red blood cells within the first 24 hours after visiting the emergency department. Based on their medical records, we collected data in terms of demographic findings, mechanisms of injury, initial clinical and laboratory findings, methods for hemostasis (emergency surgery and/or angioembolization), transfusion, injury severity score (ISS), revised trauma score (RTS) and trauma and injury severity score (TRISS). We used the Mann-Whitney U test and Fisher's exact test to compare the variables between the patients that survived and those that did not. We performed a logistic regression analysis with the significant variables from the univariate test. Results: Thirty-two(32) patients were enrolled. The main mechanisms of injury were falls and motor vehicle accidents. The mean transfusion amount of packed red blood cells (PRBC) was 17.4 U. The mean elapsed time for the first hemostasis (surgery or embolization) was 3.5 hours. The initial technical success rates were 83.3%(15/18) in angioembolization and 66.7%(8/12) in surgery. The overall mortality rate was 34.4%(11/32). The causes of death were bleeding, brain swelling and multiple organ failure. The ISS(25.5 vs 46.3, p=0.000), TRISS(73.6 vs 45.1, p=0.034) and base excess(<-12 mmol/L, p=0.020) were significantly different between the patients who survived and those who did not. Conclusion: The ISS was a prognostic factor for trauma patients with massive bleeding.