Background and Objectives: The recent developments in chronic thromboembolic pulmonary hypertension (CTEPH) are emphasizing the multidisciplinary team. We report on the changes in clinical practice following the development of a multidisciplinary team, based on our 7 years of experience. Methods: Multidisciplinary team was established in 2015 offering both balloon pulmonary angioplasty (BPA) and pulmonary endarterectomy (PEA) with technical upgrades by internal and external expertise. For operable cases, PEA was recommended as the primary treatment modality, followed by pulmonary angiography and right heart catheterization after 6 months to evaluate treatment effect and identify patients requiring further BPA. For patients with inoperable anatomy or high surgical risk, BPA was recommended as the initial treatment modality. Patient data and clinical outcomes were closely monitored. Results: The number of CTEPH treatments rapidly increased and postoperative survival improved after team development. Before the team, 38 patients were treated by PEA for 18 years; however, 125 patients were treated by PEA or BPA after the team for 7 years. The number of PEA performed was 64 and that of BPA 342 sessions. World Health Organization functional class I or II was achieved in 93% of patients. The patients treated with PEA was younger, male dominant, higher pulmonary artery pressure, and smaller cardiac index, than BPA-only patients. In-hospital death after PEA was only 1 case and none after BPA. Conclusions The balanced development of BPA and PEA through a multidisciplinary team approach proved synergistic in increasing the number of actively treated CTEPH patients and improving clinical outcomes.

Sjögren's syndrome is a systemic autoimmune disease characterized by sicca symptoms and extraglandular manifestations. Anemia, leukopenia, thrombocytopenia and lymphoproliferative disorders are well-known extraglandular, hematological complications of Sjögren's syndrome. These hematologic alterations are usually mild and respond well with steroid therapy. We report a case of a 52-year-old female patient who was initially presented with thrombocytopenia. The patient was then diagnosed with primary Sjögren's syndrome and initially treated with steroid. The patient's platelet count was decreased when steroid was tapered. The dose of steroid could be effectively reduced after combined medication with hydroxychloroquine.
Anemia ; Autoimmune Diseases ; Female ; Humans ; Hydroxychloroquine ; Leukopenia ; Lymphoproliferative Disorders ; Middle Aged ; Platelet Count ; Thrombocytopenia*

Common atrium is a rare congenital heart disease characterized by complete absence of the interatrial septum, and is commonly accompanied by malformation of the atrioventricular valve. Most patients with common atrium experience symptoms during childhood. Here, we describe a patient with common atrium who experienced his first obvious symptom at 48 years of age.
Adult ; Heart Atria ; Heart Defects, Congenital ; Humans

Bordetella bronchiseptica is a common cause of respiratory disease in animals but is a rare cause of human infection. Furthermore, most patients with Bordetella bronchiseptica infections are immunocompromised. The Bordetella bronchiseptica organism can cause pneumonia, septicemia, and peritonitis in humans with impaired immune systems. Additionally, it can lead to a life-threatening infection patients who have an underlying debilitation or impaired immunity. The respiratory tract is the most common site of infection. Sixty-two human cases of Bordetella bronchiseptica have been published in the English literature, and 84 % hadof the cases were associated with pneumonia or bronchitis. However, only one case of Bordetella bronchiseptica has been reported in South Korea, and it was associated with peritonitis. In the current study, we report a case of Bordetella bronchiseptica pneumonia diagnosed in an immunocompromised patient.
Animals ; Bordetella bronchiseptica* ; Bordetella* ; Bronchitis ; Humans ; Immune System ; Immunocompromised Host ; Korea ; Lung Neoplasms ; Peritonitis ; Pneumonia ; Respiratory System ; Sepsis

Intra-articular hyaluronic acid injections for symptomatic treatment of osteoarthritis are widely used but can result in complications, such as infectious arthritis. Staphylococcus lugdunensis is a common normal skin flora but can cause severe infectious disease, such as infective endocarditis. We present the first report of infectious arthritis caused by methicillin-sensitive S. lugdunensis after intra-articular hyaluronic acid injection in an immunocompromised patient in Korea.
Arthritis, Infectious* ; Arthritis, Rheumatoid* ; Communicable Diseases ; Diabetes Mellitus* ; Endocarditis ; Humans ; Hyaluronic Acid* ; Immunocompromised Host ; Korea ; Osteoarthritis ; Skin ; Staphylococcus lugdunensis* ; Staphylococcus*

Patients with massive pulmonary embolism may present with severe dyspnea at rest, syncope, or cardiac arrest. Early diagnosis and treatment are essential to reduce mortality; however, patient-specific factors can influence the hemodynamic effect of pulmonary embolism. Here, we present a case of massive pulmonary embolism masked by a ventricular septal defect in a 73-year-old female.
Aged ; Dyspnea ; Early Diagnosis ; Female ; Heart Arrest ; Heart Septal Defects, Ventricular* ; Hemodynamics ; Humans ; Masks* ; Mortality ; Pulmonary Embolism* ; Syncope

PURPOSE:In many countries, patients and dialysis unit physicians attempt to address issues regarding withholding and withdrawing dialysis through advance directives and clinical guidelines for dialysis utilization. However, there are only a few reports of withholding and withdrawing dialysis in Korea. This study was developed to investigate the attitudes of dialysis unit physicians regarding withholding and withdrawing dialysis in Korea. METHODS:A questionnaire survey was conducted among 45 dialysis unit physicians from January, 2006 to January, 2008. Physicians were asked about their decision making process to withhold and withdraw dialysis, and their opinions regarding the necessities of advance directives and guidelines for withholding and withdrawing dialysis. RESULTS:Analysis of surveys revealed that physicians agreed more about whether to withhold and withdraw dialysis in vegetative patients compared to patients with dementia ( p<0.001, p<0.001). There were more agreements about whether to withhold dialysis than whether to withdraw dialysis in patients with unimpaired cognition ( p<0.037). However, there were no differences in agreements between withholding and withdrawing dialysis in patients with severe neurological impairments. There appeared to be a general consensus regarding the need for guidelines addressing withholding and withdrawing dialysis (84.4%). However, opinions supporting the necessity for advance directives were not strongly favored (33.3%). CONCLUSION:Conflicting opinions are present among dialysis unit physicians regarding whether to withhold and withdraw dialysis according to a patient's neurological impairments. However, most dialysis unit physicians were of one accord regarding the need of guidelines for withholding and withdrawing dialysis.
Advance Directives ; Cognition ; Consensus ; Decision Making ; Dementia ; Dialysis ; Humans ; Korea

PURPOSE:In many countries, patients and dialysis unit physicians attempt to address issues regarding withholding and withdrawing dialysis through advance directives and clinical guidelines for dialysis utilization. However, there are only a few reports of withholding and withdrawing dialysis in Korea. This study was developed to investigate the attitudes of dialysis unit physicians regarding withholding and withdrawing dialysis in Korea. METHODS:A questionnaire survey was conducted among 45 dialysis unit physicians from January, 2006 to January, 2008. Physicians were asked about their decision making process to withhold and withdraw dialysis, and their opinions regarding the necessities of advance directives and guidelines for withholding and withdrawing dialysis. RESULTS:Analysis of surveys revealed that physicians agreed more about whether to withhold and withdraw dialysis in vegetative patients compared to patients with dementia ( p<0.001, p<0.001). There were more agreements about whether to withhold dialysis than whether to withdraw dialysis in patients with unimpaired cognition ( p<0.037). However, there were no differences in agreements between withholding and withdrawing dialysis in patients with severe neurological impairments. There appeared to be a general consensus regarding the need for guidelines addressing withholding and withdrawing dialysis (84.4%). However, opinions supporting the necessity for advance directives were not strongly favored (33.3%). CONCLUSION:Conflicting opinions are present among dialysis unit physicians regarding whether to withhold and withdraw dialysis according to a patient's neurological impairments. However, most dialysis unit physicians were of one accord regarding the need of guidelines for withholding and withdrawing dialysis.
Advance Directives ; Cognition ; Consensus ; Decision Making ; Dementia ; Dialysis ; Humans ; Korea

PURPOSE: Recently, obesity with metabolic syndrome is considered as an important risk factor in the development and progression of chronic kidney disease (CKD). Glomerulomegaly and focal segmental glomerulosclerosis (FSGS) are found in the obese patients, suggesting that investigation of structural- functional relationship in the obesity-related glomerulopathy (ORG) is needed to prevent CKD. Thus, we report here clinical and pathologic characteristics of ORG and its association with other clinical variables. METHODS: Obesity was defined by body mass index >25 kg/m2 and ORG morphologically by FSGS and glomerulomegaly or glomerulomegaly alone. Clinicopathologic findings and glomerular sizes of ORG (14 cases) were compared with age-matched controls with thin basement membrane disease. Multiple variable analysis was performed between glomerular size and clinical variables. RESULTS: There was no nephrotic syndrome or pretibial pitting edema in all obese patients. Mean glomerular diameter was increased in obese patients compared to controls (240+/-21 micrometer vs 197+/-21 micrometer, p=0.001). Seven cases had lesions with FSGS with glomerulomegaly and seven cases glomerulomegaly alone. Mild tubular atrophy, interstitial fibrosis and arteriolosclerosis were observed in more than half of patients. In obese patients, seven patients with FSGS had more elevated systolic blood pressure and tubular interstitial fibrosis compared to patients with glomerulomegaly only. Patients' systolic blood pressure and waist circumference were independent risk factors influencing the glomerular size in obese patients. CONCLUSION: FSGS or glomerulomegaly are prominent even in the mild obesity with insignificant clinical symptoms. This indicates that the clinical attention to glomerular disease is needed in obese patients.
Arteriolosclerosis ; Atrophy ; Basement Membrane ; Blood Pressure ; Body Mass Index ; Edema ; Fibrosis ; Glomerulosclerosis, Focal Segmental ; Humans ; Nephrotic Syndrome ; Obesity ; Renal Insufficiency, Chronic ; Risk Factors ; Waist Circumference

Aprotinin is a nonspecific serine protease inhibitor and antifibrinolytic agent. It has been used to control bleeding and reduce the amounts of transfusion during the perioperative period. There are few reports on adverse effects following aprotinin use. However, several reports have been recently published, suggesting an increased risk for renal events or deaths in patients given aprotinin. We report two cases of ARF associated with aprotinin. To reduce perioperative blood loss, aprotinin was administered to two patients who underwent obstetrical surgeries in which ARF subsequently developed. Renal biopsies displayed microthrombi within the arterioles and small arteries, causing infarctions and collapses of glomeruli. Although renal functions were not completely recovered, the two patients are now being followed up without dialysis
Acute Kidney Injury ; Aprotinin ; Arteries ; Arterioles ; Biopsy ; Female ; Hemorrhage ; Humans ; Infarction ; Obstetric Surgical Procedures ; Perioperative Period ; Serine Proteases