Identifying genetic mutations in individuals with inherited cystic kidney disease is necessary for precise treatment. We aimed to elucidate the genetic characteristics of cystic kidney disease in the Korean population. Methods: We conducted a 3-year prospective, multicenter cohort study at eight hospitals from May 2019 to May 2022. Patients with more than three renal cysts were enrolled and classified into two categories, typical autosomal dominant polycystic kidney disease (ADPKD) and atypical PKD. We identified the clinical characteristics and performed a genetic analysis using a targeted gene panel. Results: A total of 725 adult patients were included in the study, of which 560 (77.2%) were diagnosed with typical ADPKD and 165 (22.8%) had atypical PKD. Among the typical ADPKD cases, the Mayo imaging classification was as follows: 1A (55, 9.9%), 1B (149, 26.6%), 1C (198, 35.8%), 1D (90, 16.3%), and 1E (61, 11.0%). The atypical PKD cases were classified as bilateral cystic with bilateral atrophic (31, 37.3%), lopsided (27, 32.5%), unilateral (nine, 10.8%), segmental (eight, 9.6%), bilateral cystic with unilateral atrophic (seven, 8.4%), and asymmetric (one, 1.2%). Pathogenic variants were found in 64.3% of the patients using the ciliopathy-related targeted gene panel. The typical ADPKD group demonstrated a higher discovery rate (62.3%) than the atypical PKD group (41.8%). Conclusion: We present a nationwide genetic cohort’s baseline clinical and genetic characteristics for Korean cystic kidney disease.

OBJECTIVES: We estimated the population prevalence of antibodies to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), including unreported infections, through a Korea Seroprevalence Study of Monitoring of SARS-CoV-2 Antibody Retention and Transmission (K-SEROSMART) in 258 communities throughout Korea. METHODS: In August 2022, a survey was conducted among 10,000 household members aged 5 years and older, in households selected through two stage probability random sampling. During face-to-face household interviews, participants self-reported their health status, COVID-19 diagnosis and vaccination history, and general characteristics. Subsequently, participants visited a community health center or medical clinic for blood sampling. Blood samples were analyzed for the presence of antibodies to spike proteins (anti-S) and antibodies to nucleocapsid proteins (anti-N) SARS-CoV-2 proteins using an electrochemiluminescence immunoassay. To estimate the population prevalence, the PROC SURVEYMEANS statistical procedure was employed, with weighting to reflect demographic data from July 2022. RESULTS: In total, 9,945 individuals from 5,041 households were surveyed across 258 communities, representing all basic local governments in Korea. The overall population-adjusted prevalence rates of anti-S and anti-N were 97.6% and 57.1%, respectively. Since the Korea Disease Control and Prevention Agency has reported a cumulative incidence of confirmed cases of 37.8% through July 31, 2022, the proportion of unreported infections among all COVID-19 infection was suggested to be 33.9%. CONCLUSIONS The K-SEROSMART represents the first nationwide, community-based seroepidemiologic survey of COVID-19, confirming that most individuals possess antibodies to SARS-CoV-2 and that a significant number of unreported cases existed. Furthermore, this study lays the foundation for a surveillance system to continuously monitor transmission at the community level and the response to COVID-19.

Hyperuricemia is associated with metabolic syndrome as well as gout, and the prevalence of hyperuricemia is increasing in Korea. This study aimed to compare the nutrient intake and diet quality between hyperuricemia subjects and controls. Of the 28,589 people who participated in a health examination between 2008 and 2011, 9,010 subjects were selected whose 3-day food records were available. Clinical and laboratory data were collected from electronic medical records. Diet quality was evaluated using the food habit score (FHS), nutrient adequacy ratio (NAR), and mean adequacy ratio (MAR). The prevalence of hyperuricemia was 13.8% (27.1%, men; 5.2%, women). Body mass index, waist circumference, triglycerides, total cholesterol, and low-density lipoprotein cholesterol were significantly higher (p < 0.0001), while high-density cholesterol (p < 0.001) was significantly lower in the hyperuricemia subjects than in the controls. The hyperuricemia subjects had a lower intake of vitamin A (p < 0.004), vitamin C, folate, fiber, and calcium than the controls (p < 0.0001). Intake of vegetables and dairy products was significantly lower, whereas alcohol intake was significantly higher in the hyperuricemia subjects than in the controls ( p < 0.0001). The FHS (p < 0.0001), MAR (p < 0.0001), and NARs for vitamin A (p = 0.01), vitamin B2, vitamin C, folate, and calcium (p < 0.0001) were significantly lower in the hyperuricemia subjects than in the controls. In conclusion, the hyperuricemia subjects reported poorer diet quality than the controls, including higher alcohol intake and lower vegetable and dairy product intake.
Ascorbic Acid ; Body Mass Index ; Calcium ; Cholesterol ; Dairy Products ; Diet Records ; Diet* ; Electronic Health Records ; Folic Acid ; Food Habits ; Gout ; Humans ; Hyperuricemia* ; Korea* ; Lipoproteins ; Male ; Nutritive Value ; Prevalence ; Riboflavin ; Triglycerides ; Uric Acid ; Vegetables ; Vitamin A ; Waist Circumference

BACKGROUND: Autologous peripheral blood-stem cell transplantation (autoPBSCT) is the treatment of choice for hematologic malignancy, because the technique requires neither general anesthesia nor surgical intervention, amongst many other advantages. Despite these benefits, the risk of hematologic malignancy, as well as the effect of patient age and sex on the prediction of successful collection of autoPBSCT are still unclear. The purpose of this study was to examine whether the hematologic diagnosis of the disease, and age or sex affect the mobilization of CD34+ cells and mononuclear cells. METHODS: We retrospectively investigated 30 (6 multiple myeloma, 11 diffuse large B-cell lymphoma, 8 acute myeloid leukemia, 2 acute lymphoid leukemia, and 3 T-cell lymphoma) patients who underwent autoPBSCT between 2008 and 2011 at Daegu Catholic University Hospital. RESULTS: Patients with multiple myeloma had the highest average of both mononuclear cell (MNC) (2.07+/-0.67x10(8) cells/kg) and CD34+ cell (1.28+/-0.58x10(6) cells/kg) counts. Patients with T-cell lymphoma had both the lowest MNC (1.23+/-0.49x10(8) cells/kg) and CD34+ cell (0.20+/-0.6x10(6) cells/kg) counts. Male patients showed greater collected CD34+ cell counts (0.96+/-1.38x10(6) cells/kg) and MNC counts (1.71+/-0.76x10(8) cells/kg) than the female patients. Patients under the age of 44 had higher collected CD34+ cell counts (0.96+/-1.37x10(6) cells/kg) but lower counts of MNC (1.49+/-0.74x10(8) cells/kg). CONCLUSIONS: The collected MNC and CD34+ cell counts varied between the types of malignancies, and with respect to sex and age. However, only collected MNC counts were significantly different (P<0.05) among the different types of malignancies.
Anesthesia, General ; Autografts* ; Cell Count ; Cell Transplantation ; Daegu ; Diagnosis ; Female ; Hematologic Neoplasms* ; Humans ; Leukemia, Myeloid, Acute ; Lymphoma, B-Cell ; Lymphoma, T-Cell ; Male ; Multiple Myeloma ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Retrospective Studies ; Stem Cells* ; T-Lymphocytes ; Transplantation, Autologous* ; Transplants

Paroxysmal nocturnal hemoglobinuria(PNH) is an acquired hematologic disorder characterized by intravascular hemolysis, nocturnal hemoglobinuria, thrombotic events and bone marrow failure. It rarely occurs in children and can be complicated by acute renal failure(ARF). Here, we present two cases of ARF complicating PNH which has not been reported yet in Korean children. We suggest that PNH should be considered in differential diagnosis of ARF in children associated with intravascular hemolysis.
Acute Kidney Injury ; Bone Marrow ; Child ; Diagnosis, Differential ; Hemoglobinuria ; Hemoglobinuria, Paroxysmal ; Hemolysis ; Humans

PURPOSE: Voiding cystourethrography(VCUG) is required to detect vesicoureteral reflux(VUR), which may manifest as urinary tract infection(UTI) in children. It is well known that VCUG can cause UTI(post-VCUG UTI). In this study, risk factors for post-VCUG UTI and the preventive effect of antibiotics against this complication of VCUG were explored. METHODS: Medical records of 284 patients who underwent VCUG at our hospital in 2007 were reviewed retrospectively. The incidence of post-VCUG UTI and risk factors for post-VCUG UTI, and the impact of antibiotic use on prevention of post-VCUG UTI were evaluated. According to antibiotics usage, we divided the enrolled patients into 4 groups of noantibiotics group, prophylactic antibiotics group(prophylactic antibiotics having been used before), antibiotics-for-VCUG group(antibiotics added for VCUG) and antibiotics-for-treatment group(treatment dose of antibiotics). RESULTS: Seven of 284 children(2.5%) developed UTI after they underwent VCUG. High-grade(grade> or =III) VUR was the only statistically significant risk factor(odds ratio[OR] 6.266, P=0.026) for post-VCUG UTI, while sex, age, and other anomalies of urinary system were not significant. Five post-VCUG UTI cases belonged to prophylactic antibiotics group. Antibiotics use (three groups using antibiotics vs. no-antibiotics group) or addition of antibiotics for VCUG (antibiotics-for-VCUG vs. other groups) did not have any effect on prevention of post-VCUG UTI. CONCLUSION: The risk factor for post-VCUG UTI was high-grade VUR. Antibiotics use did not prevent post-VCUG UTI in this study.
Anti-Bacterial Agents ; Child ; Humans ; Incidence ; Medical Records ; Retrospective Studies ; Risk Factors ; Urinary Tract ; Urinary Tract Infections

Primary pulmonary amyloidosis is a rare condition that can be classified into the tracheobronchial, diffuse alveolar septal, and nodular parenchymal type. Tracheobronchial amyloidosis is characterized by deposition of fib rilar proteins in the tracheobronchial tree, and it can be subdivided into diffuse and focal varieties. In this report, a case of diffuse tracheobronchial amyloidosis confirmed by flexible fiberoptic bronchoscopic biopsy is presented. The patient was a 43-year old male with a chief complaint of cough and sputum for 20 days and dyspnea for one day. The chest CT scan showed diffusely thickened walls of both the main and lobar bronchi with calcification. The bronchoscopic findings showed nodular lesions of the trachea, a diffuse bronchial stenosis of both the main bronchi and a pinpoint narrowing of the left upper and right middle lobar bronchus. The biopsy showed submucosal deposits of homogenous eosinophilic amyloid materials and an apple-green birefringence under polarizing microscopy following the Congo-red stain.
Adult ; Amyloid ; Amyloidosis* ; Biopsy ; Birefringence ; Bronchi ; Bronchoscopy ; Constriction, Pathologic ; Cough ; Dyspnea ; Eosinophils ; Humans ; Male ; Microscopy ; Sputum ; Tomography, X-Ray Computed ; Trachea

OBJECTIVE: To compare the clinical outcomes between Day 2 and Day 3 embryo transfer(ET) groups in in vitro fertilization and embryo transfer(IVF-ET) with intracytoplasmic sperm injection(ICSI). METHODS: From May, 1997 to December, 1998, 174 cycles of IVF-ET with ICSI were performed and classified into two groups: Day 2 ET group(n=134) and Day 3 ET group (n=40). In Day 3 ET group, embryos fertilized after ICSI were cultured in vitro for further 24 hours in M3 media. RESULTS: There were no significant differences in the age and BMI of patients, basal serum FSH level, protocol of controlled ovarian hyperstimulation(COH), indication of ICSI, and source of sperm for ICSI between two groups. Only the number of the previous failed IVF-ET cycles was significantly higher in Day 3 ET group(p<0.05). Serum E2 level on hCG day, the numbers of oocytes retrieved after COH, oocytes fertilized after ICSI, and embryos transferred, and the rates of fertilization, cleavage, and implantation showed no significant differences. However, cumulative embryo score(CES) was significantly higher in Day 3 ET group(p<0.05). Although there were no significant differences in the rates of pregnancy per ET, spontaneous abortion, and live birth, the rates of biochemical and multiple pregnancy were significantly higher in Day 3 ET group(p<0.05). CONCLUSIONS: In IVF-ET with ICSI, the relatively higher CES may contribute to the higher risk of multiple pregnancy in Day 3 ET group, compared with the conventional Day 2 ET group.
Abortion, Spontaneous ; Embryo Transfer* ; Embryonic Structures* ; Female ; Fertilization ; Fertilization in Vitro* ; Humans ; Live Birth ; Oocytes ; Pregnancy Rate* ; Pregnancy* ; Pregnancy, Multiple ; Sperm Injections, Intracytoplasmic* ; Spermatozoa

Tracheo-innominate artery fistula is a rare, but it is one of the gravest complications next to tracheostromy. Early recognition and prompt surgical management is mandatory. The patient was a 66 year old women with MCA infarct who maintained tracheostomy for lyear. She had respiratory arrest due to the excessive bleeding through the tracheostomy site. We report an successful experience for control of bleeding by an innominate artery fistula division and primay suture closure under direct digital compression.
Aged ; Arteries* ; Brachiocephalic Trunk ; Female ; Fistula* ; Hemorrhage ; Humans ; Sutures ; Tracheostomy

BACKGROUND: Onlay vein patch coronary angioplasty has been known to be an option for coronary artery stenosis in the selective iesions MATERIAL AND METHOD: During the period between July 1997 and August 1999, coronary angioplasty using autologous saphenous vein was done on 16 sites in 14 cases for the stenotic lesion at the bifurcation area and significantly stenosis distal to anastomosis. RESULT: Early patency of the angioplasty site was 85.7% at postoperative day 7. There was no statistically difference in graft patency, operative parameters, and complications compared to conventional anatomosis(p>0.05). CONCLUSIONS: Despite the small number of cases, the patency rate of the coronary patch angioplasty was comparable to the conventional CABG. Coronary artery only patch angioplasty could be performed in highly selected coronary arteries.
Angioplasty* ; Constriction, Pathologic ; Coronary Artery Bypass ; Coronary Stenosis ; Coronary Vessels ; Inlays* ; Saphenous Vein* ; Transplants ; Veins