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Moyamoya-like vasculopathy (MMV) is a rare, chronic, progressive cerebrovascular disorder characterized by stenosis or occlusion of the terminal portion of the bilateral internal carotid arteries and development of abnormal collateral vessels at the base of the brain. This disorder develops in association with various systemic diseases and conditions, including neurofibromatosis type 1, Down syndrome, thyroid disease, radiation therapy, and autoimmune disease. We report a case of a 51-year-old female patient with low-activity systemic lupus erythematosus (SLE) who had a sudden onset of global aphasia and right hemiplegia. Three months previous, she had been on antiplatelet medication due to a single transient ischemic attack. Brain magnetic resonance imaging demonstrated a massive infarct of the left middle cerebral artery territory. Conventional angiography showed complete occlusion of the left middle cerebral artery with poor development of basal collateral vessels. This case demonstrates that a patient with underlying autoimmune disease such as SLE accompanied by MMV should be considered vulnerable to ischemic stroke.

Small cell lung cancer (SCLC) is a unique tumor that has a distinct clinical behavior and dismal prognosis. If untreated, it can become aggressively malignant, and life expectancy could be limited to weeks. Spontaneous regression of lung cancer has rarely been reported, and among them SCLC is even rarer. The underlying mechanisms of spontaneous regression are poorly understood. Here, we report a case of complete spontaneous SCLC remission in an elderly patient., An 80‐year‐old man presented with hemoptysis and hoarseness and was diagnosed with small cell lung cancer. He refused treatment and consumed beet juice for 2 years. Complete spontaneous remission was confirmed in subsequent examinations.

OBJECTIVES To investigate the impact of normothermia on compliance with sepsis bundles and in-hospital mortality in patients with sepsis who present to emergency departments. DESIGN Retrospective multicenter observational study. PATIENTS Nineteen university-affiliated hospitals of the Korean Sepsis Alliance participated in this study. Data were collected regarding patients who visited emergency departments for sepsis during the 1-month period. The patients were divided into three groups based on their body temperature at the time of triage in the emergency department (i.e., hypothermia [ 38°C]). INTERVENTIONS None. MEASUREMENTS AND MAIN RESULTS Of 64,021 patients who visited emergency departments, 689 with community-acquired sepsis were analyzed (182 hyperthermic, 420 normothermic, and 87 hypothermic patients). The rate of compliance with the total hour-1 bundle was lowest in the normothermia group (6.0% vs 9.3% in hyperthermia vs 13.8% in hypothermia group; p = 0.032), the rate for lactate measurement was lowest in the normothermia group (62.1% vs 73.1% vs 75.9%; p = 0.005), and the blood culture rate was significantly lower in the normothermia than in the hyperthermia group (p < 0.001). The in-hospital mortality rates in the hyperthermia, normothermia, and hypothermia groups were 8.5%, 20.6%, and 30.8%, respectively (p < 0.001), but there was no significant association between compliance with sepsis bundles and in-hospital mortality. However, in a multivariate analysis, compared with hyperthermia, normothermia was significantly associated with an increased in-hospital mortality (odds ratio, 2.472; 95% CI, 1.005-6.080). This association remained significant even after stratifying patients by median lactate level. CONCLUSIONS Normothermia at emergency department triage was significantly associated with an increased risk of in-hospital mortality and a lower rate of compliance with the sepsis bundle. Despite several limitations, our findings suggest a need for new strategies to improve sepsis outcomes in this group of patients.

Severe fever with thrombocytopenia syndrome (SFTS) caused by the SFTS virus (SFTSV), a novel Phlebovirus, is endemic to South Korea, central and northeastern China, and western Japan. SFTS poses a threat to public health because of its high mortality and secondary transmission. Ticks and domestic animals are hosts for SFTSV in endemic areas. There is no specific treatment for SFTS, and avoiding tick bites is the best way to prevent infection. Early therapeutic plasma exchange (TPE) is a rescue therapy in patients with rapidly progressive SFTS. Here, we present a patient with SFTS who was improving on TPE but died suddenly due to acute lung injury after TPE.

BACKGROUND: The impact of mechanical ventilation parameters and management on outcomes of patients with coronavirus disease 2019 (COVID-19) ARDS is unclear. METHODS: This multi-center observational study enrolled consecutive mechanically ventilated patients with COVID-19 ARDS admitted to one of 7 Korean ICUs between February 1, 2020–February 28, 2021. Patients who were age < 17 y or had missing ventilation parameters for the first 4 d of mechanical ventilation were excluded. Multivariate logistic regression was used to identify which strategies or ventilation parameters that were independently associated with ICU mortality. RESULTS: Overall, 129 subjects (males, 60%) with a median (interquartile range) age of 69 (62–78) y were included. Neuromuscular blocker (NMB) use and prone positioning were applied to 76% and 16% of subjects, respectively. The ICU mortality rate was 37%. In the multivariate analysis, higher dynamic driving pressure (ΔP) values during the first 4 d of mechanical ventilation were associated with increased mortality (adjusted odds ratio 1.16 [95% CI 1.00–1.33], P = .046). NMB use was associated with decreased mortality (adjusted odds ratio 0.27 [95% CI 0.09–0.81], P = .02). The median tidal volume values during the first 4 d of mechanical ventilation and the ICU mortality rate were significantly lower in the NMB group than in the no NMB group. However, subjects who received NMB for ≥ 6 d (vs < 6 d) had higher ICU mortality rate. CONCLUSIONS: In subjects with COVID-19 ARDS receiving mechanical ventilation, ΔP during the first 4 d of mechanical ventilation was independently associated with mortality. The short-term use of NMB facilitated lung-protective ventilation and was independently associated with decreased mortality.

Old age is associated with disease severity and poor prognosis among coronavirus disease 2019 (COVID-19) cases; however, characteristics of elderly patients with severe COVID-19 are limited. We aimed to assess the clinical characteristics and outcomes of patients hospitalized with severe COVID-19 at tertiary care centers in South Korea. This retrospective multicenter study included patients with severe COVID-19 who were admitted at seven hospitals in South Korea from 2 February 2020 to 28 February 2021. The Cox regression analyses were performed to assess factors associated with the in-hospital mortality. Of 488 patients with severe COVID-19, 318 (65.2%) were elderly (≥65 years). The older patient group had more underlying diseases and a higher severity score than the younger patient group. The older patient group had a higher in-hospital mortality rate than the younger patient group (25.5% versus 4.7%, p-value < 0.001). The in-hospital mortality risk factors among patients with severe COVID-19 included age, acute physiology and chronic health evaluation II score, presence of diabetes and chronic obstructive lung disease, high white blood cell count, low neutrophil-lymphocyte ratio and platelet count, do-not-resuscitate order, and treatment with invasive mechanical ventilation. In addition to old age, disease severity and examination results must be considered in treatment decision-making.

Background Mortality rates associated with sepsis have increased progressively in Korea, but domestic epidemiologic data remain limited. The objective of this study was to investigate the characteristics, management and clinical outcomes of sepsis patients in Korea. Methods This study is a multicenter retrospective cohort study. A total of 64,021 adult patients who visited an emergency department (ED) within one of the 19 participating hospitals during a 1-month period were screened for eligibility. Among these, patients diagnosed with sepsis based on the third International Consensus Definitions for Sepsis and Septic Shock (Sepsis-3) were included in the study. Results Using the Sepsis-3 criteria, 977 sepsis patients were identified, among which 36.5% presented with septic shock. The respiratory system (61.8%) was the most common site of infection. The pathogen involved was identified in 444 patients (45.5%) and multi-drug resistance (MDR) pathogens were isolated in 171 patients. Empiric antibiotic therapy was appropriate in 68.6% of patients, but the appropriateness was significantly reduced in infections associated with MDR pathogens as compared with non-MDR pathogens (58.8% vs. 76.0%, P

Chronic thromboembolic pulmonary hypertension is potentially curable with a pulmonary endarterectomy. However, approximately 20% of patients have persistent pulmonary hypertension after pulmonary endarterectomy, which is a major risk factor for postoperative death. Here, we report a 34-year-old woman who suffered persistent severe pulmonary hypertension following a successful pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension. Extracorporeal membrane oxygenation (ECMO) and atrial septostomy were successfully performed as rescue treatments, and active rehabilitation during ECMO was prescribed to facilitate recovery.

Aims: Although the cases of macrolide-resistant Mycoplasma pneumoniae (MRMP) have been increasing substantially among children, there are very few studies that have focused on MRMP in adults. This ...

Due to rapid advances in our understanding of the molecular pathogenesis of non‐small cell lung cancer (NSCLC), the 2015 World Health Organization (WHO) classification has defined large cell carcinoma (LCC) as a subtype that is lacking glandular or squamous cells, and any neuroendocrine differentiation. Accordingly, LCC is one of the rarest subtypes of NSCLC. LCC usually presents as a large peripheral tumour with prominent necrosis and its specific characteristics are not well known. Here, we report a case of LCC identified during differential diagnosis of diffuse alveolar haemorrhage in a patient with haemoptysis.

Actinomycosis is a rare infectious bacterial disease typically involving cervicofacial, abdominopelvic, or thoracic regions, caused by Actinomyces species. However, involvement of the mediastinum as the primary site of origin is extremely rare. An elderly patient complained of left‐sided chest pain. The chest radiograph revealed pleural effusion, which revealed lymphocyte‐predominant exudates. Chest computed tomography showed a soft tissue mass in the left anterior mediastinum. Positron emission tomography revealed an increased uptake of 18F‐fluorodeoxyglucose in this lesion. To exclude malignancy and make a confirmative diagnosis, a mediastinal biopsy was performed through video‐assisted thoracic surgery. Finally, mediastinal actinomycosis was diagnosed. We report herein a case of mediastinal actinomycosis mimicking lung malignancy presented with recurrent lymphocyte‐predominant pleural fluid exudate. In patients with a recurrent or unexplained exudative pleural effusion, it may be worthwhile to consider a hidden foci of actinomycosis., Mediastinal actinomycosis is a rare infectious condition and often mimics malignancy. A high level of clinical suspicion is needed to diagnose and cure actinomycosis in patients with indolent, unresolving, or relapsing chronic inflammatory disease.

Nonsecretory multiple myeloma is a rare variant of multiple myeloma characterized by the proliferation of clonal plasma cells in the bone marrow. It is difficult to establish an early and accurate diagnosis of nonsecretory multiple myeloma because in nonsecretory myeloma cases, monoclonal immunoglobulin cannot be detected in the serum or urine via electrophoresis. In this report, we describe a case of nonsecretory multiple myeloma presenting as recurrent vertebral compression fractures in a 70-year-old male patient and suggest that nonsecretory multiple myeloma can be included in the differential diagnosis of multiple and recurrent vertebral compression fractures, despite the lack of detectable monoclonal immunoglobulin in the serum or urine by electrophoresis.

Coxiella burnetii infects humans and wild and domesticated animals. Although reported cases on Jeju Island, off the coast of South Korea, are rare, the region is considered to have a high potential for Q fever. We investigated the seroprevalence of antibodies to C. burnetii in 230 farmers living in ten rural areas on Jeju Island between January 2015 and December 2019. Blood samples were collected and examined for C. burnetii Phase I/II IgM and IgG antibodies. Trained researchers collected ticks from rural areas. Clone XCP-1 16S ribosomal RNA gene sequencing was performed to identify Coxiella species from the collected ticks. The overall seroprevalence of antibodies to C. burnetii in farmers was 35.7%. The seroprevalence was significantly higher in fruit farmers. Of the collected ticks, 5.4% (19/351) of the Haemaphysalis longicornis ticks harbored C. burnetti. A high seroprevalence of antibodies to C. burnetii was observed in this region of Jeju Island, confirming that C. burnetti is endemic. Physicians should thus consider Q fever in the differential diagnosis of patients that present with acute fever after participating in outdoor activities.

Severe fever with thrombocytopenia syndrome (SFTS) is associated with a high mortality caused by rapidly progressive multiple organ failure. SFTS virus induces immunosuppression, mediated by interleukin-10 production, reduction of CD3+ and CD4+ T cells, and cytokine storms, and this may lead to various complications in critical SFTS patients. Recently, there have been reports of cases of invasive pulmonary Aspergillosis (IPA) in patients with SFTS in the absence of predisposing factors of IPA. However, there is no known relationship between SFTS and mycosis. Here, we report a SFTS patient with a low CD4+ T-cell count and a high viral load, who developed possible IPA in the absence of common risk factors for mycosis. This case adds to the evidence that IPA may occur as a complication of SFTS.

Amniotic fluid embolism (AFE) is a rare but fatal obstetric emergency, which is characterized by a sudden cardiovascular collapse, respiratory failure, and disseminated intravascular coagulation (DIC). We report a case of sudden cardiac arrest due to an amniotic-fluid embolism which was successfully treated with veno-arterial extracorporeal membrane oxygenation (ECMO). A 32-year-old female at 39.1 weeks of gestation was scheduled for induction labor. During labor, a sudden generalized tonic-clonic seizure was developed followed by dyspnea and hypotension. An immediate vaginal delivery was performed, and the baby was healthy. Despite the appropriate cardiopulmonary resuscitation, she became hypoxemic and experienced recurrent cardiovascular collapse. ECMO was applied promptly, and the patient became stable rapidly and was discharged without any complications. ECMO seems to be a proper treatment option for catastrophic amniotic-fluid embolism.

Exogenous lipoid pneumonia is an uncommon medical condition resulting from aspiration or inhalation of oily material. Generally, lipoid pneumonia has nonspecific clinical and radiological presentations, and may be misdiagnosed as bacterial pneumonia or lung cancer. We describe an unusual case of exogenous lipoid pneumonia accompanied by peripheral blood and pulmonary eosino philia. A 63-year-old man was admitted with progressively worsening exertional dyspnea and productive cough for 5 days. A chest radiograph showed abnormalities in the lower lobe of the right lung, and a diagnosis of community-acquired pneumonia was made; intravenous antibiotics were administered. However, dyspnea and hypoxia gradually worsened and peripheral blood eosino philia developed. A bronchoscopy was performed and bronchoalveolar lavage fluid analysis showed markedly increased numbers of eosinophils (40%). Subsequently, a comprehensive review of history revealed that he fell asleep with camellia oil in his mouth for 2 weeks to relieve foreign body sensation of the throat. Sputum and bronchoalveolar lavage fluid cytology showed the presence of lipid-laden macrophages. He was diagnosed with lipoid pneumonia and acute eosinophilic pneumonia. Chest radiograph and symptom were rapidly improved after treatment with intravenous methylprednisolone. (Allergy Asthma Respir Dis 2019;7:57-60)

Propionibacterium acnes is one of the commensals living on the human skin and glands, implicated mainly in acnes, but seldom in deep infection. Pleural empyema is rarely complicated with closed thoracostomy. We experienced 1 case of empyema caused by P. acnes after pleural biopsy and closed thoracostomy through a percutaneous pigtail catheter. A 79-year-old man was admitted for cough, purulent sputum and shortness of breath. Three weeks ago, closed thoracostomy and pleural biopsy were performed to confirm a diagnosis for his recurrent pleural effusion. He had increased amount of right pleural effusion. Through the pigtail cathe ter, pleural effusion was removed. Gram-positive rods were observed in Gram stain, but not cultured. By 16S rRNA analysis, P. acnes was confirmed as the pathogen. His empyema was repeatedly treated with antibiotics, fibrolysis and irrigation. Pleural decortication was recommended. We report the first case of empyema with P. acnes in Korea, possibly complicated with closed thoracostomy pro cedures. (Allergy Asthma Respir Dis 2019;7:61-64)

Kikuchi-Fujimoto Disease (KFD) is a benign, self-limited disease characterized by tender regional lymphadenopathy with fever. KFD remains a poorly defined disease, and no clear diagnostic criteria are available. Here, we assess the clinical, laboratory, and histopathologic findings of KFD cases and report two unusual cases. Forty KFD patients that underwent lymph node (LN) biopsy and diagnosed by immunohistochemical staining, from January 2003 to November 2010, were enrolled in this retrospective study. The patients had a mean age of 29.3 years, and 29 (72.5 %) were women. Affected LNs were mainly located unilaterally in the cervical area. Mean LN size was 15.3 mm. Twenty-eight (70 %) patients had LN tenderness, and 25 (62.5 %) patients had fever. Leukopenia was observed in 18 of 35 evaluable patients. C-reactive protein and erythrocyte sedimentation rate were elevated in most patients. Anti-nuclear antibody was positive in four of 19 evaluable patients, but all had been diagnosed with concurrent systemic lupus erythematosus. Histologically, the 40 cases were classified into three types, that is, as proliferative (37.5 %), necrotizing (55.0 %), or xanthomatous (7.5 %). Interesting cases: Case 1 was a 35-year-old female with KFD and uveitis, retinal vasculitis, and superior sagittal sinus thrombosis. Case 2 was a 47-year-old male with KFD and bone marrow involvement and presented with severe bicytopenia. Although KFD is an uncommon self-limited benign disorder, it must be included in the differential diagnosis of lymphadenopathy with fever and cytopenia. It is important that the clinical features of KFD be understood to reach a correct diagnosis.

Air embolism is a rare but mostly iatrogenic complication of medical or surgical procedures and may have a serious outcome. On the removal of a central venous catheter (CVC), minor carelessness can lead to a venous air embolism sometimes accompanied by arterial embolism. We experienced the case of a 61-year-old male who suffered from a paradoxical systemic air embolism while we removed a CVC. Immediate resuscitation and venovenous extracorporeal membrane oxygenation support saved his life. Multiple end-organ damage related to the systemic air embolism was noted, including the kidney, liver, and brain. In echocardiography, multiple air bubbles and an atrial septal defect were observed. An air embolism is preventable with appropriate precautions and techniques. Therefore, it is important to identify errors and prevent occurrence.

We report an unusual case of Plasmodium vivax malaria that occurred in a 22-year-old ankylosing spondylitis patient after initiating adalimumab therapy. P. falciparum malaria was initially included as a possible differential diagnosis due to hyperparasitemia and similar features in the peripheral blood smear. The patient was successfully treated with conventional therapy for P. vivax malaria.

Kikuchi disease, also called histiocytic necrotizing lymphadenitis, is an uncommon, idiopathic and generally self-limited disease, characterized by cervical lymphadenopathy. It can present systemic symptoms and signs, but ocular involvement is unusual. We report a 35-year-old woman who presented sudden decreased visual acuity and a swollen lymph node on the left side of her neck. On laboratory findings, there were no evidences of infection, autoimmune disease and systemic vasculitis. She was diagnosed with Kikuchi disease and bilateral retinal vasculitis by histologic analysis of lymph node, fundoscopy and fluorescein angiography.