Pulmonary arterial hypertension (PAH) is characterized by remodeling of the distal pulmonary arteries resulting in high pulmonary vascular resistance and, eventually, right ventricular heart failure. Although current advances in PAH therapy have improved outcomes, poor survival remains a reality worldwide, including Korea. One of the most important issues in PAH is the late diagnosis, since screening or diagnostic efforts are often overlooked due to the rarity of disease. Data from Korean registries and observational cohorts show that delayed detection leads to increased morbidity. Additionally, low percentages of Korean patients are committed to intensive PAH-targeted therapy. Current Korean health insurance policies' lack of coverage for new PAH-targeted drugs and upfront combination therapy may also hamper the improvement of treatment outcomes. Understanding individual variability in response to therapeutics through deep phenotyping is a novel strategy that should be considered when treating PAH. Overall, early detection of PAH by proactive screening together with early, intensive, individualized PAH therapy using deep phenotyping is crucial for improving prognoses for PAH patients in Korea.

Mucormycosis is a highly aggressive and fatal fungal infection. Due to its rapid progression, combination of early wide surgical debridement with administration of antifungal agent is extremely crucial. European Society for Clinical Microbiology and Infectious Diseases, and European Confedration of Medical Mycology recommended amphotericin B as the first-line of treatment and posaconazole as the salvage agent for mucormycosis. We report a case of rhino-orbital-cerebral mucormycosis successfully treated by changing antifungal agent from the first-line amphotericin B to the salvage agent posaconazole. A 57-years-old female with uncontrolled type 2 diabetes mellitus was presented with foot abscess and rhino-orbital-cerebral mucormycosis. The infection was intractable despite orbital orbital exenteration with surgical debridement and 2 months of antibiotics treatment including amphotericin B. Changing amphotericin B to posaconazole resulted in clinical improvement within the first week. Conclusively the newly administered antifungal agent, posaconazole is expected to be an effective salvage treatment option for mucormycosis after failure of initial treatment of amphotericin B with surgical management.
Abscess ; Amphotericin B ; Anti-Bacterial Agents ; Communicable Diseases ; Debridement ; Diabetes Mellitus, Type 2 ; Female ; Foot ; Humans ; Mucormycosis* ; Mycology ; Orbit

BACKGROUND: Fungal infections are frequent encountered conditions presented in various clinical forms. The prevalence of fungal infection is gradually growing because of the increase in aged population and immune-compromised host which is susceptible factor of fungal infection. Broad array of studies of fungal infection have been published in Korea. However, epidemiologic study of fungal infection in admitted patients via emergency department has not been conducted. OBJECTIVE: The purpose of this study is to generate background data for emergency department visits in relation to fungal infection for developing a subsequent management and control system. METHODS: The data was collected from National Emergency Department Information System (NEDIS) in Korea from 2011 to 2013. Data analysis was done to evaluate the age distribution of admission, regional variance and sex of the patients with fungal infection. RESULTS: The patients were divided into five groups according to their primary diagnosis which were skin fungal infection, subcutaneous mycoses, opportunistic mycoses, systemic mycoses and other mycoses. The annual admission ratio of general fungal infections increased from 2.15 persons per 100,000 in 2011 to 2.46 persons per 100,000 in 2013. The highest admission ratio was seen in opportunistic mycoses group followed by cutaneous fungal infections, other mycoses, subcutaneous mycoses and systemic mycoses groups. CONCLUSION: This study provides a basic set of data pertaining to fungal infections in emergency departments. Therefore, more systemically approached researches are required to implement sounder management and control systems of fungal infections presented in emergency departments.
Age Distribution ; Diagnosis ; Emergencies* ; Emergency Service, Hospital* ; Epidemiologic Studies ; Epidemiology* ; Humans ; Information Systems ; Korea* ; Mycoses ; Prevalence ; Skin ; Statistics as Topic

OBJECTIVE: Efficacy or long-term result of metastasectomy for recurrent or metastatic biliary tract carcinoma (BTC) is not well established. We conducted a retrospective review of the outcomes of metastasectomy for recurrent or metastatic BTCs. METHODS: The clinicopathological features and outcomes of consecutive patients with BTCs who underwent surgical resection for primary and metastatic disease at a tertiary referral hospital from 2003 to 2013 were reviewed retrospectively. RESULTS: We found 19 eligible patients. Median age of patients was 57 years old (range, 27 to 68 years old), and 11 patients (58%) were female. Primary sites were gallbladder cancer (seven patients, 37%), intrahepatic cholangiocarcinoma (five patients, 26%), distal common bile duct cancer (three patients, 16%), proximal common bile duct cancer (two patients, 11%), and ampulla of Vater cancer (two patients, 11%). Eight patients (42%) had synchronous metastasis, while 11 (58%) had metachronous metastasis. The most common metastatic site was liver (nine patients, 47%), lymph node (nine patients, 47%), and peritoneum (three patients, 16%). Nine patients (47%) achieved R0 resection, while four (21%) and six (32%) patients had R1 and R2 resection, respectively. With a median follow-up period of 26.7 months, the estimated median overall survival (OS) was 18.2 months (95% confidence interval [CI], 13.6 to 22.9 months). Lower Eastern Cooperative Oncology Group performance status (P=0.023), metachronous metastasis (P=0.04), absence of lymph node metastasis (P=0.009), lower numbers of metastatic organs (P<0.001), normal postoperative carbohydrate antigen 19-9 level (P=0.034), and time from diagnosis to metastasectomy more than one year (P=0.019) were identified as prognostic factors for a longer OS after metastasectomy. CONCLUSION: For recurrent or metastatic BTCs, metastasectomy can be a viable option for selected patients.
Ampulla of Vater ; Biliary Tract Neoplasms* ; Biliary Tract* ; Cholangiocarcinoma ; Common Bile Duct ; Diagnosis ; Female ; Follow-Up Studies ; Gallbladder Neoplasms ; Humans ; Liver ; Lymph Nodes ; Metastasectomy* ; Neoplasm Metastasis ; Peritoneum ; Prognosis ; Retrospective Studies ; Tertiary Care Centers

Eosinophilic gastroenteritis is a rare disease in which the symptoms are associated with eosinophilic infiltration in various layers of the gastrointestinal tract. A 56-year-old man complained of severe abdominal pain after eating yellow tail fish and oyster. There was no peripheral blood eosinophilia in the initial laboratory test. Abdominal computed tomography demonstrated circumferential wall thickening and dilatation of small intestine with ascites. An emergency laparotomy accompanied by segmental resection of the ileum and end-to-end anastomosis was performed. Histologically, there was a dense infiltration of eosinophils throughout the entire layers of ileal wall, through which this case could be diagnosed as eosinophilic enteritis. We did not prescribe systemic glucocorticosteroid, but asked him to avoid fish and oyster. He did not complain of recurrent gastrointestinal symptoms anymore after discharge. This is the case of eosinophilic gastroenteritis with intestinal obstruction requiring emergency surgery, which was developed or aggravated after ingestion of yellow tail fish and oyster that were suspected to be culprit foods. In patients with eosinophilic gastroenteritis, foods which are related to this abnormal condition should be identified and avoided to control this disease and prevent from aggravation or flare-up.
Abdominal Pain ; Ascites ; Dilatation ; Eating ; Emergencies ; Enteritis ; Eosinophilia ; Eosinophils* ; Food Hypersensitivity ; Gastroenteritis* ; Gastrointestinal Tract ; Humans ; Ileum ; Intestinal Obstruction* ; Intestine, Small ; Laparotomy ; Middle Aged ; Ostreidae* ; Rare Diseases ; Tail*

Background and Objectives: Pulmonary arterial hypertension (PAH) is a rare but fatal disease. Recent advances in PAH-specific drugs have improved its outcomes, although the healthcare burden of novel therapeutics may lead to a discrepancy in outcomes between developing and developed countries. We analyzed how the epidemiology and clinical features of PAH has changed through the rapidly advancing healthcare infrastructure in South Korea. Methods: PAH was defined according to a newly devised 3-component algorithm. Using a nationwide health insurance claims database, we delineated annual trends in the prevalence, incidence, medication prescription pattern, and 5-year survival of PAH in Korea. Cumulative survival and potential predictors of mortality were also assessed among 2,151 incident PAH cases. Results: Between 2002 or 2004 and 2018, the prevalence and incidence of PAH increased 75-fold (0.4 to 29.9 per million people) and 12-fold (0.5 to 6.3 per million person-years), respectively. The proportion of patients on combination PAH-specific drug therapy has also steadily increased up to 29.0% in 2018. Among 2,151 incident PAH cases (median [interquartile range] age, 50 [37–62] years; 67.2% female), the 5-year survival rate and median survival duration were 71.8% and 13.1 years, respectively. Independent predictors of mortality were age, sex, etiology of PAH, diabetes, dyslipidemia, and chronic kidney disease. Conclusions This nationwide study delineated that the prevalence and incidence of PAH have grown rapidly in Korea since the early 2000s. The use of combination therapy has also increased, and the 5-year survival rate of PAH in Korea was similar to those in western countries.

Pulmonary hypertension is a rare and progressive illness with a devastating prognosis. Promising research efforts have advanced the understanding and recognition of the pathobiology of pulmonary hypertension. Despite remarkable achievements in terms of improving the survival rate, reducing disease progression, and enhancing quality of life, pulmonary arterial hypertension (PAH) is not completely curable. Therefore, an effective treatment strategy is still needed. Recently, many studies of the underlying molecular mechanisms and technological developments have led to new approaches and paradigms for PAH treatment. Management based on stem cells and related paracrine effects, epigenetic drugs and gene therapies has yielded prospective results for PAH treatment in preclinical research. Further trials are ongoing to optimize these important insights into clinical circumstances.

Purpose: Heart failure (HF) poses significant morbidity and mortality. Recently, the ventriculo-vascular coupling index (VVI) was introduced as an independent prognostic factor reflective of the overall cardiovascular performance index in HF. We aimed to determine the effectiveness of force-titration of valsartan on VVI values in HF patients. Materials and Methods: In this multicenter and prospective observational trial, the effect of valsartan was stratified according to dosages [non-ceiling dose (NCD) vs. ceiling dose (CD)] in HF patients with left ventricular ejection fraction (LVEF) <55%. Biochemical studies, including N-terminal pro-B-type natriuretic peptide (NT-proBNP), echocardiography with VVI, the treadmill test, and the activity scale index were assessed at baseline and after 24 weeks of treatment. Results: One-hundred thirty-eight patients were force-titrated to either a CD group (n=81) or a NCD group (n=57). The mean age of the study participants was 59 years and 66% were male. After 6 months of follow up, left ventricular mass index (LVMI) values had significantly improved in the CD group but not in the NCD group. Intriguingly, in HF patients with a reduced ejection fraction (HFrEF) (n=52, LVEF <40%), a significant improvement in VVI was only observed in the CD group (from 2.4±0.6 to 1.8±0.5, p<0.001). Conclusion CDs of valsartan for 6 months showed better improvement in VVI, as well as LVMI, in patients with HFrEF, compared with NCDs.

Purpose: Heart failure (HF) poses significant morbidity and mortality. Recently, the ventriculo-vascular coupling index (VVI) was introduced as an independent prognostic factor reflective of the overall cardiovascular performance index in HF. We aimed to determine the effectiveness of force-titration of valsartan on VVI values in HF patients. Materials and Methods: In this multicenter and prospective observational trial, the effect of valsartan was stratified according to dosages [non-ceiling dose (NCD) vs. ceiling dose (CD)] in HF patients with left ventricular ejection fraction (LVEF) <55%. Biochemical studies, including N-terminal pro-B-type natriuretic peptide (NT-proBNP), echocardiography with VVI, the treadmill test, and the activity scale index were assessed at baseline and after 24 weeks of treatment. Results: One-hundred thirty-eight patients were force-titrated to either a CD group (n=81) or a NCD group (n=57). The mean age of the study participants was 59 years and 66% were male. After 6 months of follow up, left ventricular mass index (LVMI) values had significantly improved in the CD group but not in the NCD group. Intriguingly, in HF patients with a reduced ejection fraction (HFrEF) (n=52, LVEF <40%), a significant improvement in VVI was only observed in the CD group (from 2.4±0.6 to 1.8±0.5, p<0.001). Conclusion CDs of valsartan for 6 months showed better improvement in VVI, as well as LVMI, in patients with HFrEF, compared with NCDs.