• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.14 no.1
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• pp.86-90
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• 2011

Wilson's disease is an autosomal recessive disorder of copper metabolism consequence of which leads to accumulation of copper in the liver, brain, cornea and other tissues. The manifestations are more likely to be hepatic in the early childhood and neurological in the adolescents. In addition, the abnormalities that develop during disease progression may result in other manifestations such as hematologic, endocrine, or renal findings. We report a thirteen year-old girl who manifested tetany shortly after the initial diagnosis of Wilson's disease. Despite aggressive calcium, magnesium and vitamin D replacement, the hypocalcemia and hypomagnesemia did not respond to the therapy promptly. It took more than three weeks for blood levels of the minerals to be normal. We concluded that tetany occurred in our patient because of hypoparathyroidism as a rare complication of Wilson disease, vitamin D deficiency resulting from various conditions, and inconclusive hypomagnesemia.

• Journal of Chest Surgery
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• v.43 no.2
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• pp.194-198
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• 2010

Intramural hematoma of the aorta (IMH) is the precursor or a variant of a classic aortic dissection where hemorrhage occurs within the aorta wall in the absence of an initial intimal tear. IMH has a high rate of mortality and morbidity. The optimal therapy for IMH is uncertain, yet the involvement of the ascending aorta is usually considered as an indication for surgery due to the associated risk of rupture or cardiac tamponade. We report here on a case of a 71-year-old man who presented with syncope. Because of misdiagnosis, he underwent computed tomography (CT) after 5 hrs from arriving to the ER. Computed tomography of the aorta revealed intramural hematoma of the ascending aorta with cardiac tamponade. He also had vascular complications such as acute renal failure and visceral ischemia. We performed emergency graft replacement of the total arch and ascending aorta. He was discharged without complication on postoperative day 14.

• Journal of Chest Surgery
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• v.45 no.2
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• pp.91-94
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• 2012

Background: Despite improved managements for acute respiratory distress syndrome (ARDS), its mortality remains high. Extracorporeal membrane oxygenation (ECMO) has emerged as the final option for the treatment of ARDS unresponsive to conventional measures. This study describes our experiences of venovenous ECMO support for the treatment of ARDS. Materials and Methods: Between 2007 and 2010, 56 patients (aged $56.6{\pm}13.4$ years, 43 males) received venovenous ECMO for the treatment of ARDS. The detailed clinical records were retrospectively reviewed. Results: Before the institution of ECMO support, 35 patients (55.4%) required nitric oxide inhalation, 35 patients (55.4%) received continuous renal replacement therapy, and 20 patients (35.7%) were in shock status. The median duration of ECMO support was 164 hours (range, 5 to 1,413 hours). 27 (48%) patients could be successfully weaned from ECMO. Of them, 7 (13%) survived to discharge. On logistic regression analysis, a requirement for higher inspiratory pressure before ECMO support was the only significant factor that could predict ECMO weaning failure. Conclusion: The outcome of venovenous ECMO support for the treatment of ARDS was suboptimal. Further improvements in outcomes should be made through the accumulation of experience and establishment of a standardized protocol for the management of ECMO.

• Journal of Genetic Medicine
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• v.7 no.1
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• pp.87-90
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• 2010

Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea Hyperammonemia in the newborn often leads to severe fatal illness associated with hyperammonemic encephalopathy. Transient hyperammonemia in newborns (THAN) is characterized by self-limiting, transient hyperammonemia during the neonatal period. THAN may have favorable long-term outcomes if it is diagnosed early and appropriately managed. However, severe hyperammonemia can develop even in newborns with THAN, which may require emergent management. Here we report a case of THAN with severe hyperammonemia during the neonatal period that was successfully treated with continuous renal replacement therapy and nitrogen-scavenging medications. Our patient went on to develop normally and has not re-experienced a hyperammonemic episode until 9 months of age without the administration of a protein restricted diet or medications.

• Journal of The Korean Society of Clinical Toxicology
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• v.17 no.1
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• pp.14-20
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• 2019

Purpose: This study estimated the incidence of delirium and associated risk factors and outcomes in ICU patients with acute poisoning. Methods: Data were collected from ICU patients over 18 years of age that were admitted via the emergency center after presenting with poisoning from 2010 to 2015. Delirium was assessed retrospectively using the Intensive Care Delirium Screening Checklist (ICDSC). Risk factors were evaluated by univariate and multivariate analysis. Results: A total of 199 patients participated in this study and 68 (34.2%) were diagnosed with delirium based on the ICDSC score. The delirium group showed a significantly higher association with prolonged length of stay in the hospital and ICU in comparison with the non-delirium group. The delirium group was associated with greater use of physical restraint. A statistically greater number of patients with pharmaceutical substance poisoning developed delirium over a short period of time than those with non-pharmaceutical substance poisoning. There was no significant difference between the two groups with respect to age, sex, past history, GCS score, vital signs, application of ventilator care and renal replacement therapy. Conclusion: The finding that the delirium group had a greater length of stay in both the hospital and the ICU is consistent with the results of previous worldwide studies of the effects of delirium on the prognosis of patients who were admitted to the ICU, suggesting the possibility for domestic application. Additionally, use of physical restraint was positively related to the incidence of delirium. Thus, interventions for minimizing the use of physical restraints and considering alternatives are needed.

• Journal of The Korean Society of Clinical Toxicology
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• v.19 no.2
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• pp.133-138
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• 2021

Glufosinate-containing herbicides is a non-selective herbicide commonly used worldwide. As the use of them increased gradually since paraquat was banned in 2012, the number of suicides by their ingestion is also increasing continuously. Complications of glufosinate-containing herbicide poisoning include various central nervous system (CNS) toxicities such as convulsions, loss of consciousness, memory impairment, and respiratory depression, which may be accompanied by hemodynamic changes such as bradycardia and hypotension. However, it is very rare that arrhythmias other than bradycardia occurred and Takotsubo cardiomyopathy was combined due to cardiotoxicity. A 71-year-old female patient was transferred to our hospital after ingesting 500 mL of glufosinate-containing herbicide and receiving 5 L of gastric lavage at a local hospital. A few hours later, she presented stuporous mentality, respiratory depression, and convulsions, and was accompanied by hypotension and bradycardia. On the second day of admission, electrocardiogram (ECG) showed bradycardia and QTc prolongation with hemodynamic Instability. Accordingly, we conducted the early treatment with continuous renal replacement therapy (CRRT) and the application of temporary cardiac pacemaker. An echocardiogram demonstrated decreased ejection fraction (EF) and Takotsubo cardiomyopathy on the third day of admission. Then, she was discharged safely with conservative treatment. At the follow-up after 1 year, Takotsubo cardiomyopathy, EF and QTc prolongation were recovered on echocardiogram and ECG. Because cardiac toxicity after glufosinate-containing herbicide poisoning may cause life-threatening consequences, caution is required while treating the patient. Therefore, if electrocardiogram changes are seen in the elderly with a large amount of glufosinate herbicide ingestion, additional cardiac function test through echocardiography should be concerned, and early treatment through CRRT or artificial cardiac pacing should be considered.

• Journal of Medicine and Life Science
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• v.19 no.2
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• pp.46-56
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• 2022

The incidence of fever complicating percutaneous coronary intervention (PCI) is rare. However, little is known regarding the cause of fever after PCI. Therefore, this study aimed to determine the clinical characteristics of patients with acute myocardial infarction (AMI), with or without fever, after PCI. We enrolled a total of 926 AMI patients who underwent PCI. Body temperature (BT) was measured every 4 hours or 8 hours for 5 days after PCI. Patients were divided into two groups according to BT as follows: BT<37.7℃ (no-fever group) and BT ≥37.7℃ (fever group). The 2 years clinical outcomes were compared subsequently. Fever after PCI was associated with higher incidence of major adverse cardiac events (MACE) (hazard ratio [HR], 1.56; 95% confidence interval [CI], 1.07-2.28; P=0.021), all-cause death (HR, 2.32; 95% CI, 1.18-4.45; P=0.014), cardiac death (CD) (HR, 2.57; 95% CI, 1.02-6.76; P=0.049), and any revascularization (HR, 1.69; 95% CI, 1.02-2.81; P=0.044) than without fever. In women, prior chronic kidney disease, lower left ventricular (LV) ejection fraction, higher LV wall motion score index, white blood cell count, peak creatine kinase-myocardial band level, and longer PCI duration were associated with fever after PCI. Procedures such as an intra-aortic balloon pump, extracorporeal membrane oxygenation, continuous renal replacement therapy, central and arterial line insertion, and cardiopulmonary resuscitation were related to fever after PCI. Fever after PCI in patients with AMI was associated with a higher incidence of MACE, all-cause death, CD, and any revascularization at the 2 years mark than in those without fever.

• Pediatric Infection and Vaccine
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• v.31 no.1
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• pp.136-139
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• 2024

Rhabdomyolysis is a syndrome that causes various complications due to the release of substances from muscle cells, often associated with preceding infectious diseases. We report the case of a 7-year-old Korean boy with recent severe acute respiratory syndrome coronavirus 2 infection, presenting with fever, chills, and generalized body aches, diagnosed as rhabdomyolysis. Additionally, we conducted a systematic review with the aim of delineating the disease spectrum, treatment, and outcomes. We identified seven reports that met the inclusion criteria. Among the cases, 5 had fever, with creatine kinase levels ranging from 3,717 and 274,664 IU/L. Two individuals received treatment in intensive care unit, 2 underwent renal replacement therapy, and 1 case has deceased. For children with coronavirus disease 2019 infection and muscle pain, a thorough examination of urine color and an assessment of muscle enzymes through blood tests can help diagnose and treat rhabdomyolysis, a condition that might otherwise be overlooked.

• Journal of Chest Surgery
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• v.57 no.1
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• pp.36-43
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• 2024

Background: Coronavirus disease 2019 (COVID-19) can lead to acute respiratory failure, which frequently necessitates invasive mechanical ventilation and extracorporeal membrane oxygenation (ECMO). However, the limited availability of ECMO resources poses challenges to patient selection and associated decision-making. Consequently, this retrospective single-center study was undertaken to evaluate the characteristics and clinical outcomes of patients with COVID-19 receiving ECMO. Methods: Between March 2020 and July 2022, 65 patients with COVID-19 were treated with ECMO and were subsequently reviewed. Patient demographics, laboratory data, and clinical outcomes were examined, and statistical analyses were performed to identify risk factors associated with mortality. Results: Of the patients studied, 15 (23.1%) survived and were discharged from the hospital, while 50 (76.9%) died during their hospitalization. The survival group had a significantly lower median age, at 52 years (interquartile range [IQR], 47.5-61.5 years), compared to 64 years (IQR, 60.0-68.0 years) among mortality group (p=0.016). However, no significant differences were observed in other underlying conditions or in factors related to intervention timing. Multivariable analysis revealed that the requirement of a change in ECMO mode (odds ratio [OR], 366.77; 95% confidence interval [CI], 1.92-69911.92; p=0.0275) and the initiation of continuous renal replacement therapy (CRRT) (OR, 139.15; 95% CI, 1.95-9,910.14; p=0.0233) were independent predictors of mortality. Conclusion: Changes in ECMO mode and the initiation of CRRT during management were associated with mortality in patients with COVID-19 who were supported by ECMO. Patients exhibiting these factors require careful monitoring due to the potential for adverse outcomes.

• Journal of Chest Surgery
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• v.57 no.2
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• pp.184-194
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• 2024

Background: Left ventricular assist devices (LVADs) are widely employed as a therapeutic option for end-stage heart failure. We evaluated the outcomes associated with centrifugal-flow LVAD implantation, comparing 2 device models: the Heartmate 3 (HM3) and the Heartware Ventricular Assist Device (HVAD). Methods: Data were collected from patients who underwent LVAD implantation between June 1, 2015 and December 31, 2022. We analyzed overall survival, first rehospitalization, and early, late, and LVAD-related complications. Results: In total, 74 patients underwent LVAD implantation, with 42 receiving the HM3 and 32 the HVAD. A mild Interagency Registry for Mechanically Assisted Circulatory Support score was more common among HM3 than HVAD recipients (p=0.006), and patients receiving the HM3 exhibited lower rates of preoperative ventilator use (p=0.010) and extracorporeal membrane oxygenation (p=0.039). The overall early mortality rate was 5.4% (4 of 74 patients), with no significant difference between groups. Regarding early right ventricular (RV) failure, HM3 implantation was associated with a lower rate (13 of 42 [31.0%]) than HVAD implantation (18 of 32 [56.2%], p=0.051). The median rehospitalization-free period was longer for HM3 recipients (16.9 months) than HVAD recipients (5.3 months, p=0.013). Furthermore, HM3 recipients displayed a lower incidence of late hemorrhagic stroke (p=0.016). In the multivariable analysis, preoperative use of continuous renal replacement therapy (odds ratio, 22.31; p=0.002) was the only significant predictor of postoperative RV failure. Conclusion: The LVAD models (HM3 and HVAD) demonstrated comparable overall survival rates. However, the HM3 was associated with a lower risk of late hemorrhagic stroke.