The Profile of Early Sedation Depth and Clinical Outcomes of Mechanically Ventilated Patients in Korea

Background@#Current international guidelines recommend against deep sedation as it is associated with worse outcomes in the intensive care unit (ICU). However, in Korea the prevalence of deep sedation and its impact on patients in the ICU are not well known. @*Methods@#From April 2020 to July 2021, a multicenter, prospective, longitudinal, noninterventional cohort study was performed in 20 Korean ICUs. Sedation depth extent was divided into light and deep using a mean Richmond Agitation–Sedation Scale value within the first 48 hours. Propensity score matching was used to balance covariables; the outcomes were compared between the two groups. @*Results@#Overall, 631 patients (418 [66.2%] and 213 [33.8%] in the deep and light sedation groups, respectively) were included. Mortality rates were 14.1% and 8.4% in the deep and light sedation groups (P = 0.039), respectively. Kaplan-Meier estimates showed that time to extubation (P < 0.001), ICU length of stay (P = 0.005), and death P = 0.041) differed between the groups. After adjusting for confounders, early deep sedation was only associated with delayed time to extubation (hazard ratio [HR], 0.66; 95% confidence inter val [CI], 0.55– 0.80; P < 0.001). In the matched cohort, deep sedation remained significantly associated with delayed time to extubation (HR, 0.68; 95% 0.56–0.83; P < 0.001) but was not associated with ICU length of stay (HR, 0.94; 95% CI, 0.79–1.13; P = 0.500) and in-hospital mortality (HR, 1.19; 95% CI, 0.65–2.17; P = 0.582). @*Conclusion@#In many Korean ICUs, early deep sedation was highly prevalent in mechanically ventilated patients and was associated with delayed extubation, but not prolonged ICU stay or in-hospital death.

Safety and Utility of Rush Immunotherapy with Aqueous Allergen Extracts for Treatment of Respiratory Allergies

Background@#Generally, allergen immunotherapy must be administered for three to five years. Meanwhile, rush immunotherapy (RIT) shortens the required duration for the build-up phase, thereby improving the therapy's convenience compared with conventional immunotherapy (CIT). However, RIT is often performed with modified allergens. Therefore, this study aimed to investigate the safety and utility of RIT with aqueous allergens. @*Methods@#Medical records of 98 patients sensitized with at least one inhalant allergen who had received subcutaneous immunotherapy for allergic rhinitis with or without asthma were retrospectively reviewed. All patients were classified into three groups: depot-RIT (n = 25), receiving RIT with depot allergen; aqueous-RIT (n = 48), receiving RIT with aqueous allergen; and aqueous-CIT (n = 25), receiving CIT with aqueous allergen. Patients who had received immunotherapy targeting only house dust mites were excluded. @*Results@#The proportions of patients presenting with a systemic reaction to depot-RIT, aqueous-RIT, or aqueous-CIT were 80.0%, 85.4%, and 48.0%, respectively (P = 0.002). The proportions of patients experiencing severe systemic reaction were 4.0%, 16.7%, and 8.0% in depot-RIT, aqueous-RIT and aqueous-CIT, respectively (P = 0.223). The proportions of depotRIT and aqueous-RIT patients presenting with systemic reaction or severe systemic reaction did not differ significantly (P = 0.553 and P = 0.118, respectively). Significantly fewer depotRIT (1.0 ± 0.2) and aqueous-RIT patients (2.0 ± 1.3) required outpatient clinical visits during the build-up phase, compared to those administered aqueous-CIT (13.6 ± 1.9; P < 0.001).Moreover, the build-up phase decreased to 17.4 ± 1.8 days in depot-RIT and 23.7 ± 10.9 days in aqueous-RIT, compared to 92.0 ± 12.5 days in aqueous-CIT (P < 0.001). @*Conclusion@#RIT with aqueous allergen reduced the build-up phase duration and frequency of hospital visits, with acceptable safety levels. RIT with aqueous allergen may, therefore, be suitable for broad application to patients with respiratory allergies.

Prevalence and Clinical Features of Drug Reactions With Eosinophilia and Systemic Symptoms Syndrome Caused by Antituberculosis Drugs: A Retrospective Cohort Study

PURPOSE: Although there have been reported cases of drug reactions with eosinophilia and systemic symptoms (DRESS) syndrome caused by antituberculosis drugs, there has been no research to examine its prevalence. This study assessed the prevalence and clinical characteristics of DRESS syndrome caused by antituberculosis drugs. METHODS: The electronic medical records of a cohort consisting of adult patients diagnosed with tuberculosis between July 2006 and June 2010 were reviewed and retrospectively inspected. We searched the surveillance system for adverse drug reactions and the electronic medical records to identify patients who reported severe cutaneous adverse reactions to antituberculosis drugs. These patients were then re-assessed using a European Registry of Severe Cutaneous Adverse Reactions to Drugs and Collection of Biological Samples (RegiSCAR) scoring system. Clinical characteristics, including the symptoms and latency of DRESS syndrome, the therapeutic dosage and period of steroids, and the final duration of tuberculosis therapy, were examined. RESULTS: Of the 1,253 adult patients with tuberculosis receiving antituberculosis drugs, 15 were identified as potential cases of DRESS syndrome (prevalence of 1.2%). Ethambutol was the most frequently used drug (53.5%), followed by rifampicin (26.7%), pyrazinamide (20.0%), streptomycin (13.3%), and isoniazid (6.7%). The median latency after day 1 of antituberculosis medication was 42 days. The median daily dose of steroids, expressed in prednisone-equivalent units, was 33-mg/day, and the median dosing period was 14 days. The duration of tuberculosis treatment was 76 days longer than the standard treatment period of 180 days. There was a significant difference in the peak eosinophil counts of DRESS syndrome patients according to RegiSCAR scores. Moreover, there was a significant quantitative correlation between the RegiSCAR score and peak eosinophil count. A negative correlation was also found between the RegiSCAR score and latency. CONCLUSIONS: This study confirmed the prevalence of DRESS syndrome in a cohort of adult patients with tuberculosis.

Usefulness of delta neutrophil index for early prediction of overt disseminated intravascular coagulopathy in patients with venomous snakebite

OBJECTIVE: Clinically, consumptive coagulopathy, such as disseminated intravascular coagulopathy (DIC), is the most important among the common venomous snakebite complications owing to the serious hemorrhage risk associated with this condition. We evaluated the predictive value of the delta neutrophil index (DNI)—a new indicator for immature granulocytes—for DIC diagnosis. METHODS: This retrospective observational study consecutively assessed adult patients with venomous snakebites for over 51 months. Patients were categorized into the no DIC and DIC groups. DNI values were measured within 24 hours after snakebite. RESULTS: Thirty patients (26.3%) developed DIC. The DIC group had significantly higher median initial DNI than the no DIC group (0% vs. 0.2%, P < 0.001). When the DIC group was divided into early and late groups (within and over 24 hours after snakebite, respectively), the DNI of the former was significantly higher than that of the latter and no DIC group. The late DIC group had significantly higher DNI than the no DIC group. Furthermore, DNI positively correlated with the DIC score (r=0.548, P < 0.001). The initial DNI (odds ratio, 4.449; 95% confidence interval, 1.738 to 11.388; P=0.002) was an early DIC predictor. The area under the curve based on the initial DNI’s receiver operating characteristic curve was 0.724. CONCLUSION: DNI values were significantly higher in the DIC group. Additionally, DNI was an early predictor of DIC development in patients with venomous snakebites in the emergency department.

Rapid Response Systems Reduce In-Hospital Cardiopulmonary Arrest: A Pilot Study and Motivation for a Nationwide Survey / 대한구급학회지

BACKGROUND: Early recognition of the signs and symptoms of clinical deterioration could diminish the incidence of cardiopulmonary arrest. The present study investigates outcomes with respect to cardiopulmonary arrest rates in institutions with and without rapid response systems (RRSs) and the current level of cardiopulmonary arrest rate in tertiary hospitals. METHODS: This was a retrospective study based on data from 14 tertiary hospitals. Cardiopulmonary resuscitation (CPR) rate reports were obtained from each hospital to include the number of cardiopulmonary arrest events in adult patients in the general ward, the annual adult admission statistics, and the structure of the RRS if present. RESULTS: Hospitals with RRSs showed a statistically significant reduction of the CPR rate between 2013 and 2015 (odds ratio [OR], 0.731; 95% confidence interval [CI], 0.577 to 0.927; P = 0.009). Nevertheless, CPR rates of 2013 and 2015 did not change in hospitals without RRS (OR, 0.988; 95% CI, 0.868 to 1.124; P = 0.854). National university-affiliated hospitals showed less cardiopulmonary arrest rate than private university-affiliated in 2015 (1.92 vs. 2.40; OR, 0.800; 95% CI, 0.702 to 0.912; P = 0.001). High-volume hospitals showed lower cardiopulmonary arrest rates compared with medium-volume hospitals in 2013 (1.76 vs. 2.63; OR, 0.667; 95% CI, 0.577 to 0.772; P < 0.001) and in 2015 (1.55 vs. 3.20; OR, 0.485; 95% CI, 0.428 to 0.550; P < 0.001). CONCLUSIONS: RRSs may be a feasible option to reduce the CPR rate. The discrepancy in cardiopulmonary arrest rates suggests further research should include a nationwide survey to tease out factors involved in in-hospital cardiopulmonary arrest and differences in outcomes based on hospital characteristics.

Rapid Response Systems Reduce In-Hospital Cardiopulmonary Arrest: A Pilot Study and Motivation for a Nationwide Survey / 대한중환자의학회지

BACKGROUND: Early recognition of the signs and symptoms of clinical deterioration could diminish the incidence of cardiopulmonary arrest. The present study investigates outcomes with respect to cardiopulmonary arrest rates in institutions with and without rapid response systems (RRSs) and the current level of cardiopulmonary arrest rate in tertiary hospitals. METHODS: This was a retrospective study based on data from 14 tertiary hospitals. Cardiopulmonary resuscitation (CPR) rate reports were obtained from each hospital to include the number of cardiopulmonary arrest events in adult patients in the general ward, the annual adult admission statistics, and the structure of the RRS if present. RESULTS: Hospitals with RRSs showed a statistically significant reduction of the CPR rate between 2013 and 2015 (odds ratio [OR], 0.731; 95% confidence interval [CI], 0.577 to 0.927; P = 0.009). Nevertheless, CPR rates of 2013 and 2015 did not change in hospitals without RRS (OR, 0.988; 95% CI, 0.868 to 1.124; P = 0.854). National university-affiliated hospitals showed less cardiopulmonary arrest rate than private university-affiliated in 2015 (1.92 vs. 2.40; OR, 0.800; 95% CI, 0.702 to 0.912; P = 0.001). High-volume hospitals showed lower cardiopulmonary arrest rates compared with medium-volume hospitals in 2013 (1.76 vs. 2.63; OR, 0.667; 95% CI, 0.577 to 0.772; P < 0.001) and in 2015 (1.55 vs. 3.20; OR, 0.485; 95% CI, 0.428 to 0.550; P < 0.001). CONCLUSIONS: RRSs may be a feasible option to reduce the CPR rate. The discrepancy in cardiopulmonary arrest rates suggests further research should include a nationwide survey to tease out factors involved in in-hospital cardiopulmonary arrest and differences in outcomes based on hospital characteristics.

Clinical Practice Guideline of Acute Respiratory Distress Syndrome / 대한구급학회지

There is no well-stated practical guideline for mechanically ventilated patients with or without acute respiratory distress syndrome (ARDS). We generate strong (1) and weak (2) grade of recommendations based on high (A), moderate (B) and low (C) grade in the quality of evidence. In patients with ARDS, we recommend low tidal volume ventilation (1A) and prone position if it is not contraindicated (1B) to reduce their mortality. However, we did not support high-frequency oscillatory ventilation (1B) and inhaled nitric oxide (1A) as a standard treatment. We also suggest high positive end-expiratory pressure (2B), extracorporeal membrane oxygenation as a rescue therapy (2C), and neuromuscular blockage for 48 hours after starting mechanical ventilation (2B). The application of recruitment maneuver may reduce mortality (2B), however, the use of systemic steroids cannot reduce mortality (2B). In mechanically ventilated patients, we recommend light sedation (1B) and low tidal volume even without ARDS (1B) and suggest lung protective ventilation strategy during the operation to lower the incidence of lung complications including ARDS (2B). Early tracheostomy in mechanically ventilated patients can be performed only in limited patients (2A). In conclusion, of 12 recommendations, nine were in the management of ARDS, and three for mechanically ventilated patients.

Clinical Practice Guideline of Acute Respiratory Distress Syndrome / 결핵및호흡기질환

There is no well-stated practical guideline for mechanically ventilated patients with or without acute respiratory distress syndrome (ARDS). We generate strong (1) and weak (2) grade of recommendations based on high (A), moderate (B) and low (C) grade in the quality of evidence. In patients with ARDS, we recommend low tidal volume ventilation (1A) and prone position if it is not contraindicated (1B) to reduce their mortality. However, we did not support high-frequency oscillatory ventilation (1B) and inhaled nitric oxide (1A) as a standard treatment. We also suggest high positive end-expiratory pressure (2B), extracorporeal membrane oxygenation as a rescue therapy (2C), and neuromuscular blockage for 48 hours after starting mechanical ventilation (2B). The application of recruitment maneuver may reduce mortality (2B), however, the use of systemic steroids cannot reduce mortality (2B). In mechanically ventilated patients, we recommend light sedation (1B) and low tidal volume even without ARDS (1B) and suggest lung protective ventilation strategy during the operation to lower the incidence of lung complications including ARDS (2B). Early tracheostomy in mechanically ventilated patients can be performed only in limited patients (2A). In conclusion, of 12 recommendations, nine were in the management of ARDS, and three for mechanically ventilated patients.

Clinical Practice Guideline of Acute Respiratory Distress Syndrome / 대한중환자의학회지

There is no well-stated practical guideline for mechanically ventilated patients with or without acute respiratory distress syndrome (ARDS). We generate strong (1) and weak (2) grade of recommendations based on high (A), moderate (B) and low (C) grade in the quality of evidence. In patients with ARDS, we recommend low tidal volume ventilation (1A) and prone position if it is not contraindicated (1B) to reduce their mortality. However, we did not support high-frequency oscillatory ventilation (1B) and inhaled nitric oxide (1A) as a standard treatment. We also suggest high positive end-expiratory pressure (2B), extracorporeal membrane oxygenation as a rescue therapy (2C), and neuromuscular blockage for 48 hours after starting mechanical ventilation (2B). The application of recruitment maneuver may reduce mortality (2B), however, the use of systemic steroids cannot reduce mortality (2B). In mechanically ventilated patients, we recommend light sedation (1B) and low tidal volume even without ARDS (1B) and suggest lung protective ventilation strategy during the operation to lower the incidence of lung complications including ARDS (2B). Early tracheostomy in mechanically ventilated patients can be performed only in limited patients (2A). In conclusion, of 12 recommendations, nine were in the management of ARDS, and three for mechanically ventilated patients.

The Effectiveness of Real-Time PCR Assay, Compared with Microbiologic Results for the Diagnosis of Pulmonary Tuberculosis / 결핵및호흡기질환

BACKGROUND: The incidence of tuberculosis (TB) in Korea is relatively high compared to the other Organisation for Economic Co-operation and Development (OECD) countries, with a prevalence of 71 per 100,000 in 2012, although the incidence is declining. Real-time polymerase chain reaction (PCR) has been introduced for the rapid diagnosis of TB. Recently, its advantage lies in higher sensitivity and specificity for the diagnosis of TB. This study evaluated the clinical accuracy of real-time PCR using respiratory specimens in a clinical setting. METHODS: Real-time PCR assays using sputum specimens and/or bronchoscopic aspirates from 2,877 subjects were reviewed retrospectively; 2,859 subjects were enrolled. The diagnosis of TB was determined by positive microbiology, pathological findings of TB in the lung and pleura, or clinical suspicion of active TB following anti-TB medication for more than 6 months with a favorable response. RESULTS: Sensitivity, specificity, and accuracy were 44%, 99%, and 86% from sputum, and 65%, 97%, and 87% from bronchoscopic aspirates, respectively. For overall respiratory specimens, sensitivity was 59%, specificity was 98%, and accuracy increased to 89%. CONCLUSION: Positivity in real-time PCR using any respiratory specimens suggests the possibility of active TB in clinically suspected cases, guiding to start anti-TB medication. Real-time PCR from selective bronchoscopic aspirates enhances the diagnostic yield much more when added to sputum examination.

Pulmonary Mucormycosis Treated Successfully with Posaconazole as Salvage Therapy / 계명의대학술지

Mucormycosis (formerly known as zygomycosis) is a life-threatening opportunistic mycosis that infects a broad range of hosts with qualitative or quantitative defects in innate immunity. The overall mortality rate of pulmonary mucormycosis is above 70%. The prognosis and outcome of pulmonary mucormycosis have not improved significantly over the last decade, mainly because of difficulty in early diagnosis and the limited activity of current antifungal agents against members of the order Mucorales. We report a case of pulmonary mucormycosis treated successfully with posaconazole as salvage therapy. We suggest that posaconazole may be considered as an alternative therapeutic approach in patients with invasive pulmonary mucormycosis who are unable to tolerate surgical treatment.

Progressive Pulmonary Fibrocystic Changes of Both Upper Lungs in a Patient with Ankylosing Spondylitis / 결핵및호흡기질환

Ankylosing spondylitis is a chronic inflammatory multisystem disease that primarily affects the axial joints. Pleuropulmonary involvement is an uncommon extra-articular manifestation of ankylosing spondylitis. There is a wide spectrum of pulmonary parenchymal changes in ankylosing spondylitis, beginning in the early stages of the disease and increasing over time. The lesions are usually asymptomatic, and not visible on chest radiographs in early stages. We reported a case of advanced ankylosing spondylitis in a 56-year-old man with progressive pulmonary bullous fibrocystic changes on both upper lobes that were misdiagnosed as tuberculosis in the early stages of the disease.

Pulmonary Langerhans Cell Histiocytosis in an Adult Male Presenting with Central Diabetes Insipidus and Diabetes Mellitus: A Case Report / 결핵및호흡기질환

Pulmonary Langerhans cell histiocytosis is an uncommon diffuse cystic lung disease in adults. In rare cases, it can involve extrapulmonary organs and lead to endocrine abnormalities such as central diabetes insipidus. A 42-year-old man presented with polyphagia and polydipsia, as well as a dry cough and dyspnea on exertion. Magnetic resonance imaging of the hypothalamic-pituitary system failed to show the posterior pituitary, which is a typical finding in patients with central diabetes insipidus. This condition was confirmed by a water deprivation test, and the patient was also found to have type 2 diabetes mellitus. Computed tomographic scanning of the lungs revealed multiple, irregularly shaped cystic lesions and small nodules bilaterally, with sparing of the costophrenic angles. Lung biopsy through video-assisted thoracoscopic surgery revealed pulmonary Langerhans cell histiocytosis. On a follow-up visit, only 1 year after the patient had quit smoking, clinical and radiological improvement was significant. Here, we report an uncommon case of pulmonary Langerhans cell histiocytosis that simultaneously presented with diabetes insipidus and diabetes mellitus.

Micropapillary Lung Adenocarcinoma with Aerogenous Spread / 계명의대학술지

We experienced a case of micropapillary lung adenocarcinoma with aerogenous spread in a patient who was suspected of having interstitial pneumonia. To our knowledge, our case has not been described in the Korean literature. Our case indicates that clinicians cannot rule out the possibility of micropapillary lung adenocarcinoma with aerogenous spread in patients with a persistent presence of lesions in the lower left lung.

Outcomes of Second-Line Chemotherapy for Advanced Non-Small Cell Lung Cancer in One Institution / 결핵및호흡기질환

BACKGROUND: This study analyzed the negative prognostic factors in patients who received second-line chemotherapy for advanced inoperable non-small cell lung cancer (NSCLC). METHODS: We retrospectively reviewed the records of 137 patients with inoperable stage III-IV NSCLC who received second-line chemotherapy. The effects of clinical parameters on survival were analyzed and the hazard ratios (HR) for mortality were identified by a Cox regression analysis. RESULTS: Sex, age older than 65 years, smoking history, cell type, T-stage, best response to first-line chemotherapy and first-line chemotherapy regimen were significant negative predictors in univariate analysis. The multivariate analysis showed that patients older than 65 years (HR, 1.530; 95% confidence interval [CI], 1.020-2.297), advanced T stage (T4 vs. T1; HR, 2.273; 95% CI, 1.010-5.114) and non-responders who showed progression with first-line chemotherapy (HR, 1.530; 95% CI, 1.063-2.203) had higher HR for death. CONCLUSION: The age factor, T stage and responsiveness to first-line chemotherapy were important factors in predicting the outcome of patients with advanced NSCLC who received second-line chemotherapy. The results may help to predict outcomes for these patients in the future.

Outcomes of Second-Line Chemotherapy for Advanced Non-Small Cell Lung Cancer in One Institution / 결핵및호흡기질환

BACKGROUND: This study analyzed the negative prognostic factors in patients who received second-line chemotherapy for advanced inoperable non-small cell lung cancer (NSCLC). METHODS: We retrospectively reviewed the records of 137 patients with inoperable stage III-IV NSCLC who received second-line chemotherapy. The effects of clinical parameters on survival were analyzed and the hazard ratios (HR) for mortality were identified by a Cox regression analysis. RESULTS: Sex, age older than 65 years, smoking history, cell type, T-stage, best response to first-line chemotherapy and first-line chemotherapy regimen were significant negative predictors in univariate analysis. The multivariate analysis showed that patients older than 65 years (HR, 1.530; 95% confidence interval [CI], 1.020-2.297), advanced T stage (T4 vs. T1; HR, 2.273; 95% CI, 1.010-5.114) and non-responders who showed progression with first-line chemotherapy (HR, 1.530; 95% CI, 1.063-2.203) had higher HR for death. CONCLUSION: The age factor, T stage and responsiveness to first-line chemotherapy were important factors in predicting the outcome of patients with advanced NSCLC who received second-line chemotherapy. The results may help to predict outcomes for these patients in the future.

A Fatal Case of a Large Abdominal Wall Muscle Hematoma Secondary to Low-Molecular-Weight Heparin Injections / 대한구급학회지

The use of low-molecular-weight heparin (LMWH) can lead to major life threatening complications, including hematomas. Abdominal wall muscle hematomas are rarely fatal, and encompass a wide spectrum of severity depending on size, etiology, and associated complications; but because of their rarity may be misdiagnosed clinically. We report a fatal case of an 80-year-old female who received LMWH after an episode of pulmonary thromboembolism and was subsequently diagnosed with a large right abdominal wall hematoma complicated with hypovolemic shock and acute kidney injury.

Acute Eosinophilic Pneumonia Following Secondhand Cigarette Smoke Exposure / 결핵및호흡기질환

Acute eosinophilic pneumonia (AEP) is a disease characterized by an acute febrile onset, eosinophilia in bronchoalveolar lavage fluid, and a dramatic response to corticosteroids. Although many studies have reported a close relationship between direct cigarette smoking and AEP, few studies have identified an association between passive smoking and AEP. Here, we report a case of AEP in a 19-year-old female with cough, fever, and dyspnea after 4 weeks of intense exposure to secondhand smoke for 6 to 8 hours a day in an enclosed area.

Clinical Features and Prognostic Factors in Elderly Koreans with Advanced Non-Small-Cell Lung Cancer in a Tertiary Referral Hospital / 결핵및호흡기질환

BACKGROUND: More than half of cases for advanced non-small-cell lung cancer (NSCLC) occur in elderly patients with a median age at diagnosis of 70 years. The aim of our study was to examine the clinical features and prognostic factors contributing to mortality in elderly patients with advanced NSCLC. METHODS: Following a retrospective review of clinical data, 122 patients aged 70 years and over with a histopathological diagnosis of locally advanced (stage IIIB, n=32) and metastatic (stage IV, n=90) NSCLC between 2005 and 2011 were enrolled. RESULTS: The median age was 76 years (interquartile range, [IQR], 72-80 years), and 85 (70%) patients were male. Fifty-seven (46%) patients had never smoked, and 17 (19%) were in a malnourished state with a body mass index (BMI) of <18.5 kg/m2. The initial treatments included chemotherapy (40%) and radiotherapy (7%), but 57% of the patients received supportive care only. The 1-year survival rate was 32%, and the 3-year survival rate was 4%, with a median survival duration of 6.2 months (IQR, 2.5-15.3 months). Male gender (hazard ratio [HR], 2.2; 95% confidence interval [CI], 1.3-3.9; p=0.005), low BMI (HR, 2.3; 95% CI, 1.3-3.9; p=0.004), and supportive care only (HR, 1.9; 95% CI, 1.2-2.9; p=0.007) were independent predictors of shorter survival based on a Cox proportional hazards model. CONCLUSION: Elderly patients with advanced NSCLC had a poor prognosis, particularly male patients, those with a low BMI, and those who received supportive care only.

A Case of Patient with Eisenmenger Syndrome Admitted to Intensive Care Unit Due to Septic Shock Complicated with Newlydiagnosed Down Syndrome and Moyamoya Disease / 대한구급학회지

Eisenmenger syndrome is a severe form of pulmonary arterial hypertension related to congenital cardiac defects. Many patients die at a young age from such complications. The treatment of primary pulmonary hypertension is being applied to Eisenmenger syndrome such as endothelin receptor antagonists, phosphodiesterase-5 blockers, and prostacyclin. We experienced a case of 29-year female with ventricular septal defect-related Eisenmenger syndrome complicated with Down syndrome and Moyamoya disease, who was admitted to intensive care unit due to enteritis-associated septic shock. After the combination treatment with iloprost and sildenafil within the intensive care unit, the patient was able to wean mechanical ventilation without further applications of invasive rescue therapy such as extracorporeal membrane oxygenator. She was later discharged with bosentan. She maintained bosentan therapy for 34 months continuously without aggravations of symptom but eventually died with intracranial hemorrhage, a complication of Moyamoya disease. To our knowledge, this is the first case report of Eisenmenger syndrome accompanied by mosaic Down syndrome and Moyamoya disease.