PURPOSE: Despite medical and surgical treatments, some cases of nasal polyps (NP) exhibit recidivism. However, the endotype of refractory chronic rhinosinusitis with NP (CRSwNP) remains unclear. Therefore, the objective of this study was to characterize the immunological profile of refractory CRSwNP. METHODS: The control (n =23), primary NP group (pNP, n =70) and refractory NP group (rNP, n =86) were enrolled in this study. Patients who underwent revision surgeries due to failed maximal medical treatment after primary surgery were defined as the rNP group. A total of 18 inflammatory markers were investigated in nasal tissues using multiplex cytokine assay or enzyme-linked immunosorbent assay. RESULTS: The clinical characteristics of rNP included more extensive disease and worse clinical course after surgery. Additionally, rNP subjects showed higher infection rate (mucopurulence and culture-positive rate), more frequent use of antibiotics and suffered from symptomatic bacterial infection, increased asthma morbidity compared to pNP. Cytokine profile analysis showed that levels of Th17-associated mediators (myeloperoxidase, interleukin (IL)-8, IL-17A and IL-23), B-cell activating factor (BAFF) and Th1 cytokine (interferon-γ) were up-regulated in rNP compared to controls and pNP. Human neutrophil elastase-positive cells were also enhanced in rNP compared with pNP. Upregulation of Th17/Th1mediators and BAFF were observed in rNP, regardless of tissue eosinophilia or asthmatic comorbidity. Interestingly, eosinophilic markers, such as eosinophil cationic protein and C-C motif chemokine ligand 24, were up-regulated in asthmatic rNP compared to pNP and controls. Levels of anti-dsDNA immunoglobulin (Ig) G and IgA were up-regulated in rNP and highest in asthmatic eosinophilic rNP among subtypes of rNP. CONCLUSIONS: Our results suggest that Th17/Th1-associated mediators and BAFF may play a role and be a potential therapeutic target in refractory CRSwNP. Additionally, eosinophilic markers and autoantibodies may contribute to refractoriness in asthmatic rNP.
Anti-Bacterial Agents ; Asthma ; Autoantibodies ; B-Cell Activating Factor ; Bacterial Infections ; Comorbidity ; Enzyme-Linked Immunosorbent Assay ; Eosinophil Cationic Protein ; Eosinophilia ; Eosinophils ; Humans ; Immunoglobulin A ; Immunoglobulins ; Interleukin-17 ; Interleukins ; Nasal Polyps ; Neutrophils ; Sinusitis ; Th17 Cells ; Up-Regulation

PURPOSE: Obtaining brain tissue is critical to definite diagnosis and to furthering understanding of neurodegenerative diseases. The present authors have maintained the National Neuropathology Reference and Diagnostic Laboratories for Dementia in South Korea since 2016. We have built a nationwide brain bank network and are collecting brain tissues from patients with neurodegenerative diseases. We are aiming to facilitate analyses of clinic-pathological and image-pathological correlations of neurodegenerative disease and to broaden understanding thereof. MATERIALS AND METHODS: We recruited participants through two routes: from memory clinics and the community. As a baseline evaluation, clinical interviews, a neurological examination, laboratory tests, neuropsychological tests, and MRI were undertaken. Some patients also underwent amyloid PET. RESULTS: We recruited 105 participants, 70 from clinics and 35 from the community. Among them, 11 died and were autopsied. The clinical diagnoses of the autopsied patients included four with Alzheimer's disease (AD), two with subcortical vascular dementia, two with non-fluent variant primary progressive aphasia, one with leukoencephalopathy, one with frontotemporal dementia (FTD), and one with Creutzfeldt-Jakob disease (CJD). Five patients underwent amyloid PET: two with AD, one with mixed dementia, one with FTD, and one with CJD. CONCLUSION: The clinical and neuropathological information to be obtained from this cohort in the future will provide a deeper understanding of the neuropathological mechanisms of cognitive impairment in Asia, especially Korea.
Alzheimer Disease ; Amyloid ; Aphasia, Primary Progressive ; Asia ; Brain* ; Cognition Disorders ; Cohort Studies ; Creutzfeldt-Jakob Syndrome ; Dementia ; Dementia, Vascular ; Diagnosis ; Frontotemporal Dementia ; Humans ; Korea* ; Leukoencephalopathies ; Magnetic Resonance Imaging ; Memory ; Neurodegenerative Diseases ; Neurologic Examination ; Neuropathology ; Neuropsychological Tests

Hemophagocytic lymphohistiocytosis (HLH) is characterized by fever, splenomegaly, jaundice, and pathologic findings of hemophagocytosis in bone marrow or other tissues such as the lymph nodes and liver. Pleocytosis, or the presence of elevated protein levels in cerebrospinal fluid, could be helpful in diagnosing HLH. However, the pathologic diagnosis of the brain is not included in the diagnostic criteria for this condition. In the present report, we describe the case of a patient diagnosed with HLH, in whom the brain pathology, but not the bone marrow pathology, showed hemophagocytosis. As the diagnosis of HLH is difficult in many cases, a high level of suspicion is required. Moreover, the pathologic diagnosis of organs other than the bone marrow, liver, and lymph nodes may be a useful alternative.
Biopsy* ; Bone Marrow ; Brain Diseases ; Brain* ; Central Nervous System ; Cerebrospinal Fluid ; Diagnosis ; Fever ; Humans ; Jaundice ; Leukocytosis ; Liver ; Lymph Nodes ; Lymphohistiocytosis, Hemophagocytic* ; Pathology ; Splenomegaly

PURPOSE: Although microalbuminuria is considered as an early marker of nephropathy in diabetic adults, available information in diabetic adolescents is limited. The aim of this study was to investigate prevalence and frequency of regression of microalbuminuria in type 1 (T1DM) and type 2 diabetes mellitus (T2DM) patients with childhood onset. METHODS: One hundred and nine adolescents (median, 18.9 years; interquartile range (IQR), 16.5-21.0 years) with T1DM and 18 T2DM adolescents (median, 17.9 years; IQR, 16.8-18.4 years) with repeated measurements of microalbuminuria (first morning urine microalbumin/creatinine ratios) were included. The median duration of diabetes was 10.1 (7.8-14.0) years and 5.0 (3.5-5.6) years, respectively, and follow-up period ranged 0.5-7.0 years. Growth parameters, estimated glomerular filtration rate, glycosylated hemoglobin (HbA1c) and lipid profiles were obtained after reviewing medical record in each subject. RESULTS: The prevalence of microalbuminuria at baseline and evaluation were 21.1% and 17.4% in T1DM, and 44.4% and 38.9% in T2DM. Regression of microalbuminuria was observed in 13 T1DM patients (56.5%) and 3 T2DM patients (37.5%), and progression rate was 10.5% and 20% in T1DM and T2DM respectively. In regression T1DM group, HbA1c at baseline and follow-up was lower, and C-peptide at baseline was higher compared to persistent or progression groups. In T2DM, higher triglyceride was observed in persistent group. CONCLUSION: Considerable regression of microalbuminuria more than progression in diabetes adolescents indicates elevated urinary microalbumin excretion in a single test does not imply irreversible diabetic nephropathy. Careful monitoring and adequate intervention should be emphasized in adolescents with microalbuminuria to prevent rapid progression toward diabetic nephropathy.
Adolescent* ; Adult ; Albuminuria ; C-Peptide ; Child ; Diabetes Mellitus* ; Diabetes Mellitus, Type 1 ; Diabetes Mellitus, Type 2 ; Diabetic Nephropathies ; Follow-Up Studies ; Glomerular Filtration Rate ; Hemoglobin A, Glycosylated ; Humans ; Medical Records ; Prevalence ; Triglycerides

PURPOSE: To evaluate the clinical characteristics and treatment outcomes of acute upper extremity thromboembolism. METHODS: From April 1997 to July 2012, nineteen patients (10 males, mean age 69.7 years) were treated for acute upper extremity thromboembolism. Iatrogenic or traumatic acute thromboembolisms were excluded. We retrospectively reviewed patient demographics, clinical characteristics (symptom, risk factor, involved artery, and duration from initial symptom onset to primary treatment) and treatment outcomes. RESULTS: Numbness or tingling sense was the most common symptom in patients (84.2%). Twelve patients (63.1%) had cardiac arrhythmia, of which 9 patients had atrial fibrillation (47.3%). Floating thrombus was detected on transesophageal echocardiography in 6 patients. Fourteen patients (73.7%) were treated within 24 hours from the symptom onset. Most thromboembolism was located in the brachial artery bifurcation with or without proximal or distal extension. Fogarty catheter thromboembolectomy was the primary treatment in 17 patients (89.5%), of whom 13 patients (72.2%) were operated under local anesthesia. All patients received anticoagulation or antithrombotic therapy after the procedure. Three patients had recurrent thrombosis on duplex scan; however, their symptoms were improved without further intervention. All other patients were symptom-free without recurrence during the mean follow-up of 17.1+/-21.3 months. CONCLUSION: Early diagnosis and Fogarty catheter thromboembolectomy under local anesthesia followed by proper anticoagulation is the most effective and useful treatment in patients with acute upper extremity thromboembolism.
Anesthesia, Local ; Arrhythmias, Cardiac ; Arteries ; Atrial Fibrillation ; Brachial Artery ; Catheters ; Demography ; Early Diagnosis ; Echocardiography, Transesophageal ; Follow-Up Studies ; Humans ; Hypesthesia ; Male ; Recurrence ; Retrospective Studies ; Risk Factors ; Thromboembolism ; Thrombosis ; Upper Extremity

BACKGROUND AND OBJECTIVES: Acute aortic syndrome (AAS) is a heterogeneous group of disorders that often present with severe chest or back pain. It includes acute aortic dissection (AD), intramural hematoma (IMH), dissecting aneurysm, and penetrating aortic ulcer (PAU). The clinical picture of AAS and its prognosis have not been studied in a large number of Korean patients. Therefore, we organized a multi-center registry to identify the clinical characteristics and treatment patterns, as well as long-term outcomes in Korean patients with AAS. SUBJECTS AND METHODS: Five-hundred twenty-eight patients, who had been diagnosed with AAS, were enrolled into this registry from 10 centers. On a retrospective basis, we collected demographic, laboratory, imaging data, as well as follow-up clinical outcomes by reviewing medical records from individual centers. All the data were collected in core lab and analyzed in detail. RESULTS: The mean patient age was 60.1+/-14.5 years; the male-to-female ratio was M : F=297 : 231. The prevalent risk factors for AAS included hypertension (361, 68.4%) and diabetes (52, 11.1%). The components of AAS that are included in this study are acute AD (446, 84.5%), IMH (57, 10.7%), and PAU (11, 2.1%). By type of AAS, patients diagnosed with Stanford A were 45.6% of enrolled patients, whereas those with Stanford B were 54.4% of enrolled patients. Among nearly half of the patients were treated with medicine (55.7%) alone, whereas 40.0% underwent surgery and 4.3% underwent endovascular treatment. Overall, the in-hospital event rate was 21.2% and the in-hospital death rate was 8.1%. The mean follow-up duration was 42.8 months and there showed 22.9% of total event and 10.1% of death during this period. CONCLUSION: By organizing a multi-center registry of AAS, we could identify the characteristics of AAS in real-world Korean patients. Further, prospective study is warranted with a larger number of patients.
Aneurysm, Dissecting ; Aortic Diseases ; Back Pain ; Follow-Up Studies ; Hematoma ; Humans ; Hypertension ; Medical Records ; Population Characteristics ; Prognosis ; Pyridines ; Retrospective Studies ; Risk Factors ; Thiazoles ; Thorax ; Ulcer

With rapid population aging, the socioeconomic burden caused by dementia care is snowballing. Although a few community-based studies of Alzheimer's disease (AD) have been performed in Korea, there has never been a nationwide hospital-based study thereof. We aimed to identify the demographics and clinical characteristics of mild-to-moderate AD patients from the Clinical Research Center for Dementia of Korea (CREDOS) registry. A total of 1,786 patients were consecutively included from September 2005 to June 2010. Each patient underwent comprehensive neurological examination, interview for caregivers, laboratory investigations, neuropsychological tests, and brain MRI. The mean age was 74.0 yr and the female percentage 67.0%. The mean period of education was 7.1 yr and the frequency of early-onset AD (< 65 yr old) was 18.8%. Among the vascular risk factors, hypertension (48.9%) and diabetes mellitus (22.3%) were the most frequent. The mean score of the Korean version of Mini-Mental State Examination (K-MMSE) was 19.2 and the mean sum of box scores of Clinical Dementia Rating (CDR-SB) 5.1. Based on the well-structured, nationwide, and hospital-based registry, this study provides the unique clinical characteristics of AD and emphasizes the importance of vascular factors in AD in Korea.
Aged ; Aged, 80 and over ; Alzheimer Disease/complications/*diagnosis ; Brain/radionuclide imaging ; Caregivers ; Dementia/diagnosis ; Demography ; Diabetes Mellitus, Type 2/etiology ; Female ; Hospitals ; Humans ; Hypertension/etiology ; Interviews as Topic ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Questionnaires ; *Registries ; Republic of Korea ; Risk Factors

Multiple endocrine neoplasia (MEN) 2B is charaterized by tumors of endocrine glands, consisting of medullary thyroid carcinoma (MTC), pheochromocytoma and mucosal neuromas of the tongue, lips and other sites. Especially, MTC is the main cause of death in patients who have not received early prophylactic treatment, and MTC in MEN 2B represents more aggressive progress than that of MEN 2A. We encountered two cases of multiple endocrine neoplasia type 2B. One was a 13 month old boy who had familial history of MEN 2B without any symptoms, and the other was a 6-year old boy who manifested multiple mucosal neuromas of the tongue which had been aggravated in four months. Their genetic analysis revealed a point mutation 918th cordon in the RET proto-oncogene. Both of them underwent an operation for prophylactic total thyroidectomy and the 6 year old boy's specimen turned out to be thyroid medullary carcinoma. We encountered two cases of MEN 2B with prophylactic thyroidectomy by early diagnosis of RET proto-oncogene, and report the cases with review of literature.

No abstract available.

OBJECTIVE: The aim of this study was to analyze the perinatal mortality rate (PMR) and to evaluate the risk factors of perinatal deaths such as birth weight, gestational age, maternal age, fetal sex, number of antenatal visits, and cause of deaths. METHODS: Review of electronic medical records of 4,910 cases of singleton deliveries from 1998 to 2003 at Yongdong Severance Hospital was done. The perinatal period was defined according to the WHO definition and the cause of mortality was determined according to Aberdeen Classification. RESULTS: Crude (corrected) PMR was 22.8 (13.4). Among 4862 deliveries excluding termination of pregnancy and severe congenital anomalies, (1) stillbirths accounted for 78% (51/65) of perinatal deaths. (2) Distribution of neonatal birth weights less than 1,000 g, 1,000~1,499 g, 1,500 g~2,499 g were 0.3%, 0.7%, 5.6% with survival rates of 73.7%, 86.1%, and 99.6% respectively. (3) Deaths of preterm births accounted for 86% (12/14) of total neonatal deaths. (4) PMR of 494 cases of advanced maternal age was higher (20.2) than those of other age groups without statistical significance (p=0.273). (5) PMR of male-to-female ratio was 173:100 with statistical significance (p= 0.031). (6) PMR of infants with less than 2 antenatal visits was 152.0, in comparison with the rate (7.3) of infants with more than 2 antenatal visits (p<0.001). Among 112 cases of crude perinatal deaths, the leading causes were congenital anomalies (31.3%), prematurity cause unknown (28.6%), and maternal disease (10.7%). CONCLUSIONS: The number of births is decreasing, but no significant decrease of PMR was observed. Therefore, intensive care of preterm infants and congenital anomalous babies should be improved.
Birth Weight ; Cause of Death ; Electronic Health Records ; Gestational Age ; Humans ; Infant ; Infant, Newborn ; Infant, Premature ; Critical Care ; Maternal Age ; Parturition ; Perinatal Mortality ; Pregnancy ; Premature Birth ; Risk Factors ; Stillbirth ; Survival Rate