ABSTRACT
Background/Aims@#In Korea, medications are available by prescription from a physician, or can be purchased over-the-counter (OTC) without a prescription. Education regarding both prescribed and OTC drugs is important to minimize side effects and avoid drug abuse. The risk of side effects due to polypharmacy is increasing due to the growing number of elderly patients with comorbidities. @*Methods@#There are various clinical guidelines for physicians, but it is difficult for patients and their caregivers to find published guidelines regarding drug use. In this regard, experts from nine subspecialties of internal medicine, geriatric medicine, and guideline development methodology formed a working group to develop guidelines for safe drug use under the Clinical Practice Guidelines Committee of the Korean Association of Internal Medicine. @*Results@#The main contents of this guideline are 1) safe and effective drug administration, 2) the proper use of analgesics (acetaminophen and nonsteroidal anti-inflammatory drugs), 3) the proper use of tranquilizers and sleeping pills to prevent drug abuse, 4) points to be aware of when taking multiple medications. @*Conclusions@#The guidelines were developed for patients and their caregivers to understand the general principles and precautions for drug use, including commonly used painkillers, mood stabilizers, sleeping pills, and polypharmacy. These guidelines could also be used as educational materials for physicians, nurses, and healthcare workers to educate patients and their caregivers.
ABSTRACT
Background/Aims@#In Korea, medications are available by prescription from a physician, or can be purchased over-the-counter (OTC) without a prescription. Education regarding both prescribed and OTC drugs is important to minimize side effects and avoid drug abuse. The risk of side effects due to polypharmacy is increasing due to the growing number of elderly patients with comorbidities. @*Methods@#There are various clinical guidelines for physicians, but it is difficult for patients and their caregivers to find published guidelines regarding drug use. In this regard, experts from nine subspecialties of internal medicine, geriatric medicine, and guideline development methodology formed a working group to develop guidelines for safe drug use under the Clinical Practice Guidelines Committee of the Korean Association of Internal Medicine. @*Results@#The main contents of this guideline are 1) safe and effective drug administration, 2) the proper use of analgesics (acetaminophen and nonsteroidal anti-inflammatory drugs), 3) the proper use of tranquilizers and sleeping pills to prevent drug abuse, 4) points to be aware of when taking multiple medications. @*Conclusions@#The guidelines were developed for patients and their caregivers to understand the general principles and precautions for drug use, including commonly used painkillers, mood stabilizers, sleeping pills, and polypharmacy. These guidelines could also be used as educational materials for physicians, nurses, and healthcare workers to educate patients and their caregivers.
ABSTRACT
BACKGROUND: Obstructive airway disease patients with increased variability of airflow and incompletely reversible airflow obstruction are often categorized as having asthma–chronic obstructive pulmonary disease (COPD) overlap syndrome (ACOS). ACOS is heterogeneous with two sub-phenotypes: asthma-ACOS and COPD-ACOS. The objective of this study was to determine the difference in risk of exacerbation between the two sub-phenotypes of ACOS. METHODS: A total of 223 patients exhibiting incompletely reversible airflow obstruction with increased variability (spirometrically defined ACOS) were enrolled. These patients were divided into asthma-ACOS and COPD-ACOS according to their physician's diagnosis and smoking history of 10 pack-years. Within-group comparisons were made for asthma-ACOS versus COPD-ACOS and light smokers versus heavy smokers. RESULTS: Compared to patients with COPD-ACOS, patients with asthma-ACOS experienced exacerbation more often despite their younger age, history of light smoking, and better lung function. While the light-smoking group showed better lung function, they made unscheduled outpatient clinic visits more frequently. On multivariate analysis, asthma-ACOS and poor inhaler compliance were significantly associated with more than two unscheduled clinic visits during the previous year. CONCLUSION: Spirometrically defined ACOS includes heterogeneous subgroups with different clinical features. Phenotyping of ACOS by physician's diagnosis could be significant in predicting future risk of exacerbation.
Subject(s)
Humans , Ambulatory Care , Ambulatory Care Facilities , Asthma , Compliance , Diagnosis , Lung , Lung Diseases, Obstructive , Multivariate Analysis , Nebulizers and Vaporizers , Phenotype , Pulmonary Disease, Chronic Obstructive , Smoke , SmokingABSTRACT
BACKGROUND: The purpose of this study was to assess the effect of our new video-assisted asthma education program on patients' knowledge regarding asthma and asthma control. METHODS: Adult asthmatics who were diagnosed by primary care physicians and followed for at least 1 year were educated via smart devices and pamphlets. The education sessions were carried out three times at 2-week intervals. Each education period lasted at most 5 minutes. The effectiveness was then evaluated using questionnaires and an asthma control test (ACT). RESULTS: The study enrolled 144 patients (mean age, 56.7±16.7 years). Half of the patients had not been taught how to use their inhalers. After participating in the education program, the participants' understanding of asthma improved significantly across all six items of a questionnaire assessing their general knowledge of asthma. The proportion of patients who made errors while manipulating their inhalers was reduced to less than 10%. The ACT score increased from 16.6±4.6 to 20.0±3.9 (p<0.001). The number of asthmatics whose ACT score was at least 20 increased from 45 (33.3%) to 93 (65.3%) (p<0.001). The magnitude of improvement in the ACT score did not differ between patients who received an education session at least three times within 1 year and those who had not. The majority of patients agreed to the need for an education program (95.8%) and showed a willingness to pay an additional cost for the education (81.9%). CONCLUSION: This study indicated that our newly developed education program would become an effective component of asthma management in primary care clinics.
Subject(s)
Adult , Humans , Asthma , Education , Nebulizers and Vaporizers , Pamphlets , Physicians, Primary Care , Primary Health CareABSTRACT
Nontuberculous mycobacteria (NTM) are emerging pathogens that affect both immunocompromised and immunocompetent patients. The incidence and prevalence of NTM lung disease are increasing worldwide and rapidly becoming a major public health problem. For the diagnosis of NTM lung disease, patients suspected to have NTM lung disease are required to meet all clinical and microbiologic criteria. The development of molecular methods allows the characterization of new species and NTM identification at a subspecies level. Even after the identification of NTM species from respiratory specimens, clinicians should consider the clinical significance of such findings. Besides the limited options, treatment is lengthy and varies by species, and therefore a challenge. Treatment may be complicated by potential toxicity with discouraging outcomes. The decision to start treatment for NTM lung disease is not easy and requires careful individualized analysis of risks and benefits. Clinicians should be alert to those unique aspects of NTM lung disease concerning diagnosis with advanced molecular methods and treatment with limited options. Current recommendations and recent advances for diagnosis and treatment of NTM lung disease are summarized in this article.
Subject(s)
Humans , Diagnosis , Incidence , Lung Diseases , Lung , Mycobacterium , Mycobacterium avium Complex , Mycobacterium kansasii , Nontuberculous Mycobacteria , Prevalence , Public Health , Risk AssessmentABSTRACT
Pulmonary tuberculosis (TB) persists as a great public health problem in Korea. Increases in the overall age of the population and the rise of drug-resistant TB have reinforced the need for rapid diagnostic improvements and new modalities to detect TB and drug-resistant TB, as well as to improve TB control. Standard guidelines and recent advances for diagnosing pulmonary TB are summarized in this article. An early and accurate diagnosis of pulmonary TB should be established using chest X-ray, sputum microscopy, culture in both liquid and solid media, and nucleic acid amplification. Chest computed tomography, histopathological examination of biopsy samples, and new molecular diagnostic tests can be used for earlier and improved diagnoses, especially in patients with smear-negative pulmonary TB or clinically-diagnosed TB and drug-resistant TB.
Subject(s)
Humans , Biopsy , Diagnosis , Korea , Lung , Microscopy , Pathology, Molecular , Public Health , Sputum , Thorax , Tuberculosis , Tuberculosis, PulmonaryABSTRACT
BACKGROUND: Postoperative admission to the surgical intensive care unit (S-ICU) is commonly planned to prevent and treat complications, unnecessary admission to the S-ICU increases medical costs and length of hospital stay. This study aimed evaluated outcome and the predictive factors for mortality in patients admitted to the S-ICU after abdominal surgery. METHODS: The 168 patients admitted to the S-ICU immediately after abdominal surgery were reviewed retrospectively from January to December 2011. RESULTS: The mortality rate of patients admitted to the S-ICU after abdominal surgery was 8.9% (15 of 168). Two preoperative factors (body mass index [BMI] < 18.5 kg/m2 [p < 0.001] and serum albumin < 3.0 g/dL [p = 0.018]), two operative factors (the need for transfusion [p = 0.008] or vasopressors [p = 0.013] during surgery), and three postoperative variables (mechanical ventilation immediately following surgery [p < 0.001], sequential organ failure assessment [p = 0.001] and SAPS II [p = 0.001] score) were associated with mortality in univariate analysis. After adjusting for age, gender, and SAPS II by a Cox regression, which revealed that BMI < 18.5 kg/m2 (p < 0.001, hazard ratio [HR] 9.690, 95% confidence interval [CI] 2.990-25.258) and the use of mechanical ventilation on admission to S-ICU (p < 0.001, HR 34.671, 95% CI 6.440-186.649) were independent prognostic factors. CONCLUSIONS: In patients in S-ICU after abdominal surgery, low BMI and postsurgical mechanical ventilation should be considered important predictors of mortality.
Subject(s)
Humans , Abdomen , Body Mass Index , Critical Care , Intensive Care Units , Length of Stay , Mortality , Postoperative Complications , Prognosis , Respiration, Artificial , Retrospective Studies , Serum Albumin , VentilationABSTRACT
The sandwich sign is used to describe mesenteric lymphoma in which mesenteric vessels and fat are enveloped by enlarged mesenteric lymph nodes. We present two cases of primary pleural lymphoma demonstrating the "pleural sandwich sign". Contrast-enhanced computed tomography showed conglomerated parietal pleural and extrapleural masses encasing the intercostal arteries. Histopathological examinations confirmed low grade marginal zone B-cell lymphoma in an 80-year-old man and diffuse large B-cell lymphoma in a 68-year-old man. The pleural sandwich sign may suggest the diagnosis of primary pleural lymphoma.
Subject(s)
Aged , Aged, 80 and over , Humans , Male , Antigens, CD20/metabolism , Antineoplastic Agents/therapeutic use , Lymphoma, B-Cell, Marginal Zone/diagnosis , Lymphoma, Large B-Cell, Diffuse/diagnosis , Pleura/pathology , Pleural Neoplasms/diagnosis , Positron-Emission Tomography , Tomography, X-Ray ComputedABSTRACT
BACKGROUND: Postoperative admission to the surgical intensive care unit (S-ICU) is commonly planned to prevent and treat complications, unnecessary admission to the S-ICU increases medical costs and length of hospital stay. This study aimed evaluated outcome and the predictive factors for mortality in patients admitted to the S-ICU after abdominal surgery. METHODS: The 168 patients admitted to the S-ICU immediately after abdominal surgery were reviewed retrospectively from January to December 2011. RESULTS: The mortality rate of patients admitted to the S-ICU after abdominal surgery was 8.9% (15 of 168). Two preoperative factors (body mass index [BMI] < 18.5 kg/m2 [p < 0.001] and serum albumin < 3.0 g/dL [p = 0.018]), two operative factors (the need for transfusion [p = 0.008] or vasopressors [p = 0.013] during surgery), and three postoperative variables (mechanical ventilation immediately following surgery [p < 0.001], sequential organ failure assessment [p = 0.001] and SAPS II [p = 0.001] score) were associated with mortality in univariate analysis. After adjusting for age, gender, and SAPS II by a Cox regression, which revealed that BMI < 18.5 kg/m2 (p < 0.001, hazard ratio [HR] 9.690, 95% confidence interval [CI] 2.990-25.258) and the use of mechanical ventilation on admission to S-ICU (p < 0.001, HR 34.671, 95% CI 6.440-186.649) were independent prognostic factors. CONCLUSIONS: In patients in S-ICU after abdominal surgery, low BMI and postsurgical mechanical ventilation should be considered important predictors of mortality.
Subject(s)
Humans , Abdomen , Body Mass Index , Critical Care , Intensive Care Units , Length of Stay , Mortality , Postoperative Complications , Prognosis , Respiration, Artificial , Retrospective Studies , Serum Albumin , VentilationABSTRACT
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.
Subject(s)
Humans , Middle Aged , Joints , Lung , Radiography, Thoracic , Spondylitis, Ankylosing , TuberculosisABSTRACT
Pulmonary tuberculosis is still serious, one of the great public health problems in Korea. Recently, the increase in the aged population, human immunodeficiency virus coinfection, and drug-resistant tuberculosis have reinforced the need for improved rapid diagnostics and better treatment strategies. The basic principles of care for persons with, or suspected of having, pulmonary tuberculosis are the same worldwide. The standard guidelines and recent advances in diagnosis and treatment are summarized in this article. Prompt, accurate diagnosis of pulmonary tuberculosis should be established using chest radiography, sputum microscopy, and culture in liquid and solid medium. The further evaluation of chest imaging, histopathological examination of biopsy samples, nucleic acid amplification tests, immunological evaluation, and new molecular diagnostic tests supplement earlier, improved diagnosis, especially in patients with smear-negative pulmonary tuberculosis. Standardized treatment regimens of proven efficacy should be used with appropriate patient education and treatment support. The response to treatment and the presence of side effects of antituberculosis drugs should be monitored regularly. In addition, essential public health responsibilities and public-private collaboration must be carried out for effective patient care and pulmonary tuberculosis control.
Subject(s)
Humans , Biopsy , Coinfection , Cooperative Behavior , Diagnosis , HIV , Korea , Microscopy , Nucleic Acid Amplification Techniques , Pathology, Molecular , Patient Care , Patient Education as Topic , Public Health , Radiography , Sputum , Thorax , Tuberculosis , Tuberculosis, Multidrug-Resistant , Tuberculosis, PulmonaryABSTRACT
Gefitinib is regarded as a relatively safe agent for the treatment of an advanced non-small cell lung cancer (NSCLC). Pulmonary toxicity such as interstitial lung disease associated with gefitinib is uncommon with an estimated all time incidence around 1% worldwide. Moreover, a case of gefitinib associated with pulmonary cystic changes has not been reported yet. In this report we present a case of progressive multiple air cystic changes in both lungs in a patient with NSCLC and intrapulmonary metastases who underwent a gefitinib therapy.
Subject(s)
Female , Humans , Middle Aged , Antineoplastic Agents/adverse effects , Brain Neoplasms/secondary , Carcinoma, Non-Small-Cell Lung/drug therapy , Cysts/chemically induced , Lung/pathology , Lung Diseases/chemically induced , Lung Diseases, Interstitial , Lung Neoplasms/drug therapy , Quinazolines/adverse effectsABSTRACT
BACKGROUND/AIMS: Gastroesophageal reflux disease is one of the most common causes of chronic cough and is a potential risk factor for the exacerbation of chronic obstructive pulmonary disease (COPD). The aim of this study was to investigate the prevalence and risk factors for reflux esophagitis (RE) in COPD patients. METHODS: From our hospital database, between September 2006 and April 2010, we searched for subjects who were 40 years old or older and had undergone both postbronchodilator spirometry and esophagogastroduodenoscopy (EGD). COPD was defined as having a ratio of forced expiratory volume in 1 second to forced vital capacity < 0.7 in postbronchodilator spirometry and no abnormality causing airway obstruction, except emphysematous changes, on a chest X-ray. The diagnosis of RE was based on a mucosal break surrounding the distal esophageal sphincter through EGD. RESULTS: In total, 253 patients with COPD were enrolled. The prevalence of RE in COPD was 30% (76/253). Multiple logistic regression analyses revealed that age (odds ratio [OR], 0.950; 95% confidence interval [CI], 0.918 to 0.983; p = 0.003), smoking pack-years (OR, 1.015; 95% CI, 1.004 to 1.025; p = 0.006), and inhaled anticholinergics (OR, 0.516; 95% CI, 0.271 to 0.982; p = 0.044) were independently associated with RE in COPD patients. CONCLUSIONS: The prevalence of RE in our COPD patients was higher than that reported previously in the Korean general population. In COPD, smoking increased the risk of RE, whereas inhaled anticholinergics may be associated with a reduced risk of RE.
Subject(s)
Aged , Female , Humans , Male , Middle Aged , Administration, Inhalation , Chi-Square Distribution , Cholinergic Antagonists/administration & dosage , Comorbidity , Databases, Factual , Endoscopy, Gastrointestinal , Esophagitis, Peptic/diagnosis , Logistic Models , Multivariate Analysis , Odds Ratio , Prevalence , Protective Factors , Pulmonary Disease, Chronic Obstructive/diagnosis , Republic of Korea/epidemiology , Retrospective Studies , Risk Factors , Smoking/adverse effects , SpirometryABSTRACT
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.
Subject(s)
Humans , Age Factors , Carcinoma, Non-Small-Cell Lung , Drug Therapy , Mortality , Multivariate Analysis , Retrospective Studies , Smoke , SmokingABSTRACT
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.
Subject(s)
Humans , Age Factors , Carcinoma, Non-Small-Cell Lung , Drug Therapy , Mortality , Multivariate Analysis , Retrospective Studies , Smoke , SmokingABSTRACT
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.
Subject(s)
Female , Humans , Young Adult , Adrenal Cortex Hormones , Bronchoalveolar Lavage Fluid , Cough , Dyspnea , Eosinophilia , Fever , Pulmonary Eosinophilia , Smoke , Smoking , Tobacco Products , Tobacco Smoke PollutionABSTRACT
The data regarding risk factors for death during tuberculosis (TB) treatment are inconsistent, and few studies examined this issue in Korea. The purpose of this study was to evaluate baseline prognostic factors for death during treatment of adult patients with pulmonary TB in Korea. A multicenter retrospective cohort study of 2,481 patients who received TB treatment at eight hospitals from January 2009 to December 2010 was performed. Successful treatment included cure (1,129, 45.5%) and treatment completion (1,204, 48.5%) in 2,333 patients (94.0%). Unsuccessful treatment included death (85, 3.4%) and treatment failure (63, 2.5%) occurred in 148 patients (6.0%). In multivariate analysis, male sex, anemia, dyspnea, chronic heart disease, malignancy, and intensive care unit (ICU) admission were significant risk factors for death during TB treatment. Therefore, male sex, anemia, dyspnea, chronic heart disease, malignancy, and ICU admission could be baseline prognostic factors for death during treatment of adult patients with pulmonary TB in Korea.
Subject(s)
Adult , Aged , Female , Humans , Male , Middle Aged , Anemia/complications , Antitubercular Agents/therapeutic use , Cohort Studies , Dyspnea/complications , Heart Diseases/complications , Intensive Care Units , Multivariate Analysis , Neoplasms/complications , Prognosis , Republic of Korea , Retrospective Studies , Risk Factors , Sex Factors , Treatment Outcome , Tuberculosis/complicationsABSTRACT
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.
Subject(s)
Aged, 80 and over , Female , Humans , Abdominal Wall , Acute Kidney Injury , Hematoma , Heparin, Low-Molecular-Weight , Pulmonary Embolism , ShockABSTRACT
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.
Subject(s)
Aged , Humans , Male , Body Mass Index , Carcinoma, Non-Small-Cell Lung , Lung , Lung Neoplasms , Prognosis , Retrospective Studies , Smoking , Survival Rate , Tertiary Care CentersABSTRACT
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.