In response to the increase in the prevalence of chronic kidney disease (CKD) in Korea, the growth of patients requiring renal replacement therapy and the subsequent increase in medical costs, the rapid expansion of patients with end-stage kidney disease (ESKD), and the decrease in patients receiving home therapy, including peritoneal dialysis, the Korean Society of Nephrology has proclaimed the new policy, Kidney Health Plan 2033 (KHP 2033). KHP 2033 would serve as a milestone to bridge the current issues to a future solution by directing the prevention and progression of CKD and ESKD, particularly diabetic kidney disease, and increasing the proportion of home therapy, thereby reducing the socioeconomic burden of kidney disease and improving the quality of life. Here, we provide the background for the necessity of KHP 2033, as well as the contents of KHP 2033, and enlighten the Korean Society of Nephrology’s future goals. Together with patients, healthcare providers, academic societies, and national policymakers, we need to move forward with goal-oriented drive and leadership to achieve these goals.

Fabry disease (FD) is an X-linked lysosomal storage disorder caused by the deficient activity of α-galactosidase (α-Gal A), affecting multiple organs including kidney. In this study, we aimed to determine the prevalence of FD in patients with chronic kidney disease (CKD) including those on renal replacement therapy in Korea. Methods: This is a national, multicenter, observational study performed between August 24, 2017 and February 28, 2020. Patients with the presence of proteinuria or treated on dialysis were screened by measuring the α-Gal A enzyme activity using either dried blood spot or whole blood, and plasma globotriaosylsphingosine (lyso-GL3) concentration. A GLA gene analysis was performed in patients with low α-Gal A enzyme activity or increased plasma lyso-GL3 concentration. Results: Of 897 screened patients, 405 (45.2%) were male and 279 (31.1%) were on dialysis. The α-Gal A enzyme activity was measured in 891 patients (99.3%), and plasma lyso-GL3 concentration was measured in all patients. Ten patients were eligible for a GLA gene analysis: eight with low α-Gal A enzyme activity and two with increased plasma lyso-GL3 concentration. The GLA mutations were analyzed in nine patients and one patient was found with a pathogenic mutation. Therefore, one patient was identified with FD, giving a prevalence of 0.1% (1 of 897) in this CKD population. Conclusion: Although the prevalence of FD in the CKD population was low (0.1%), screening tests are crucial to detect potential diseases in patients with relatives who can benefit from early treatment.

Polycystic kidney disease (PCKD) is the most common life-threatening genetic disease that causes kidney failure worldwide. Patients with autosomal dominant PCKD notice an increase in abdominal size as the kidney cysts grow and present with gastrointestinal and pulmonary symptoms. Surgical therapy, percutaneous drainage, sclerotherapy, cyst decompression, and laparoscopic fenestration have been used to treat the symptoms, but the results are often unsatisfactory. We recruited five patients with PCKD. Each patient complained of severe abdominal discomfort, and had a poor quality of life. In these patients, we performed renal artery embolization. After the procedure, all of the patients were discharged without severe complications. Follow-up abdominal computed tomography was performed 3-6 months after the procedure, and we were able to confirm a reduction in the size of both kidneys. In addition, the clinical symptoms improved in all five patients.
Decompression ; Drainage ; Follow-Up Studies ; Humans ; Kidney ; Polycystic Kidney Diseases* ; Quality of Life ; Renal Artery* ; Renal Insufficiency ; Sclerotherapy

A 69-year-old man was admitted to the hospital because of flu-like symptoms and fatigue for 2 weeks. Computed tomography revealed ground glass opacity and consolidation in both the lungs as well as pleural effusion. The patient was diagnosed with pneumonia and was hospitalized. At the time of hospitalization, he complained of shortness of breath and coughed-up blood-tinged sputum. Two days after admission, he died suddenly. An autopsy was performed; cardiomegaly was noted, and further examination revealed that the aortic valve had been destroyed by multiple, irregular vegetations. Herein, we report an autopsy case of infective endocarditis with a review of the relevant literatures.
Aged ; Aortic Valve ; Autopsy* ; Cardiomegaly ; Dyspnea ; Endocarditis* ; Fatigue ; Glass ; Hospitalization ; Humans ; Lung ; Pleural Effusion ; Pneumonia ; Sputum

Both death certificates and postmortem examination certificates are used as proof of death. These certificates sometimes contain erroneous information but how frequently they do so is unknown. In particular, only a few studies have measured the accuracy of the cause and manner of death on Korea death documents. In this study, we compared the cause and manner of death on both kinds of certificates with those on autopsy reports to determine the frequency of errors, and to identify way to improve the accuracy of these certificates. In 2012, 528 autopsies were requested of out institute, and certificates were submitted in 241 of the cases. The manner of death was classified as natural, unnatural, or unknown. The cause of death in the autopsy report matched that on the death certificate in 37 of 63 cases (58.7%), and the manner of death matched in 40 of 63 cases (63.5%). The cause of death in the autopsy report matched that on the postmortem examination certificate in 62 of 178 cases (34.8%), and the manner of death matched in 74 of 178 cases (41.6%). Death certificates and postmortem examination certificates are important documents. We identified many incorrect reports of causes and manners of death on both kinds of documents, especially the postmortem death certificates. These inaccuracies are presumably due to a lack of forensic information and education, as well as lack of interest on the part of medical doctors.
Autopsy* ; Cause of Death ; Death Certificates* ; Education ; Korea

PURPOSE: Cinacalcet is effective for treating refractory secondary hyperparathyroidism (SHPT), but little is known about the response rates and clinical factors influencing the response. MATERIALS AND METHODS: A prospective, single-arm, multi-center study was performed for 24 weeks. Cinacalcet was administered to patients with intact parathyroid hormone (iPTH) level greater than 300 pg/mL. Cinacalcet was started at a dose of 25 mg daily and titrated until 100 mg to achieve a serum iPTH level <300 pg/mL (primary end point). Early response to cinacalcet was defined as a decrease of iPTH more than 50% within one month. RESULTS: Fifty-seven patients were examined. Based on the magnitude of iPTH decrease, patients were divided into responder (n=47, 82.5%) and non-responder (n=10, 17.5%) groups. Among the responders, 38 achieved the primary end point, whereas 9 patients showed a reduction in serum iPTH of 30% or more, but did not reach the primary end point. Compared to non-responders, responders were significantly older (p=0.026), female (p=0.041), and diabetics (p<0.001). Additionally, early response was observed more frequently in the responders (30/47, 63.8%), of whom the majority (27/30, 90.0%) achieved the primary end point. Multivariate analysis showed that lower baseline iPTH levels [odds ratio (OR) 0.96, 95% confidence interval (CI) 0.93-0.99], the presence of diabetes (OR 46.45, CI 1.92-1125.6) and early response (OR 21.54, CI 2.94-157.7) were significant clinical factors affecting achievement of iPTH target. CONCLUSION: Cinacalcet was effective in most hemodialysis patients with refractory SHPT. The presence of an early response was closely associated with the achievement of target levels of iPTH.
Adult ; Aged ; Biomarkers, Pharmacological/blood ; Calcium/blood ; Female ; Humans ; Hyperparathyroidism, Secondary/*drug therapy ; Male ; Middle Aged ; Naphthalenes/adverse effects/*therapeutic use ; Parathyroid Hormone/*blood ; *Renal Dialysis ; Treatment Outcome

Most of the previous studies on immune dysregulation in end-stage renal disease (ESRD) have focused on T cell immunity. We investigated B cell subpopulations in ESRD patients and the effect of hemodialysis (HD) on B cell-associated immune profiles in these patients. Forty-four ESRD [maintenance HD patients (n = 27) and pre-dialysis patients (n = 17)] and 27 healthy volunteers were included in this study. We determined the percentage of B cell subtypes, such as mature and immature B cells, memory B cells, and interleukin (IL)-10+ cells, as well as B cell-producing cytokines (IL-10, IL-4 and IL-21) by florescent activated cell sorting (FACS). B cell-associated gene expression was examined using real-time PCR and B cell producing cytokines (IL-10, IL-4 and IL-21) were determined using an enzyme-linked immunosorbent assay (ELISA). The percentage of total B cells and mature B cells did not differ significantly among the three groups. The percentages of memory B cells were significantly higher in the pre-dialysis group than in the HD group (P < 0.01), but the percentage of immature B cells was significantly lower in the pre-dialysis group than in the other groups. The percentages of IL-10-expressing cells that were CD19+ or immature B cells did not differ significantly (P > 0.05) between the two subgroups within the ESRD group, but the serum IL-10 concentration was significantly lower in the pre-dialysis group (P < 0.01). The results of this study demonstrate significantly altered B cell-associated immunity. Specifically, an imbalance of immature and memory B cells in ESRD patients was observed, with this finding predominating in pre-dialysis patients.
Adaptor Proteins, Signal Transducing/genetics ; Adult ; Antigens, CD19/metabolism ; B-Lymphocyte Subsets/immunology/metabolism ; B-Lymphocytes/*immunology/metabolism ; Cytokines/biosynthesis ; Female ; Humans ; Immunophenotyping ; Interleukin-10/metabolism ; Kidney Failure, Chronic/*immunology/metabolism ; Leukocytes, Mononuclear/metabolism ; Male ; Middle Aged ; Proto-Oncogene Proteins/genetics ; T-Lymphocytes, Regulatory/immunology/metabolism

With the increasing use of magnets in toys, magnet ingestion is becoming a serious problem in children. Two or more magnets may attract across the gastrointestinal tract leading to pressure necrosis, perforation, fistula, volvulus or obstruction. We report a case of a 12-year-old boy with autism who presented with vomiting during seven days due to ingestion of 14 magnetic rods. Under general anesthesia, 5 of 14 magnets were removed from the second portion of the duodenum using a magnetic probe during endoscopy. The remaining magnets were not visible in the duodenum. A plain radiograph taken the next day revealed that the remaining magnets were impacted in the descending-sigmoid junction. One magnet passed spontaneously. However the other 8 magnets did not pass through the junction for 7 days. Five of 8 impacted magnets were removed by a colonoscopic procedure. After 2 hours of colonoscopy, one by one, the remaining three magnets spontaneously passed.
Anesthesia, General ; Autistic Disorder ; Child ; Colonoscopy ; Duodenum ; Eating ; Endoscopy ; Endoscopy, Gastrointestinal ; Fistula ; Foreign Bodies ; Gastrointestinal Tract ; Humans ; Intestinal Volvulus ; Magnetics ; Magnets ; Necrosis ; Play and Playthings ; Vomiting

PURPOSE: The clinical characteristics and associated anomalies in children with solitary kidney (SK) were analyzed retrospectively. METHODS: Total 38 children diagnosed to have SK at our hospital between December 1989 and December 2009 were recruited, and the clinical records including imaging studies were retrospectively reviewed. SK was defined as unilateral renal agenesis by imaging studies only, and patients with regression of unilateral dysplastic kidney were excluded. RESULTS: Among total 38 patients, 12 were male. The median age at the diagnosis of SK was 6.5 months (at birth-13 years). SK was detected by prenatal ultrasonography in 14 patients and during work-up for renal or urinary tract diseases in 13 (including urinary tract infection in 7). In 10 patients, SK was detected incidentally. Anomalies in the SK were noted in 17 patients including vesicoureteral reflux in 11. Other anomalies in the genitourinary tract were present in 16 patients, and multi-organ-involving syndromes or chromosomal anomalies were detected in 9. The mean duration of follow-up was 9 years (9 months-20 years). Two patients developed chronic renal failure during follow-up, and the median serum creatinine concentration of the remaining 36 at their last follow-up was 0.6 mg/dL. CONCLUSION: SK may be isolated and clinically asymptomatic; it is frequently accompanied by other anomalies in genitourinary tract and other organs, some of which can induce progressive renal dysfunction. Early recognition of associated anomalies with SK and regular follow-up is recommended to reduce long-term risk.
Child ; Congenital Abnormalities ; Creatinine ; Follow-Up Studies ; Humans ; Kidney ; Kidney Diseases ; Kidney Failure, Chronic ; Male ; Retrospective Studies ; Ultrasonography, Prenatal ; Urinary Tract Infections ; Urogenital Abnormalities ; Urologic Diseases ; Vesico-Ureteral Reflux

PURPOSE: Specimen adequacy and safety of percutaneous ultrasound-guided native kidney biopsies performed by short-term trained nephrology fellows were evaluated. METHODS: The differences in specimen adequacy and safety between nephrology fellow-performed (NP, n=67) and radiologist-performed (RP, n=82) percutaneous ultrasound-guided native kidney biopsies were retrospectively evaluated. RESULTS: The mean age of the patients was 35+/-15 years old, and the M:F ratio was 1.2:1. There were no differences in age, sex, anemia, platelet count and glomerular filtration rate between NP and RP patients. The mean glomerular count was 15.9+/-8.4 in light microscopy and 9.9+/-7.2 in immunofluorescent microscopy. Ninety five percent of biopsy specimens were adequate for pathological diagnosis. Between NP and RP kidney biopsies, there were no differences in the glomerular count in light and immunofluorescent microscopy, percentage of presence of glomeruli in electron microscopy, and the specimen adequacy for the pathological diagnosis. The rates of major and minor complications were 1.5% and 6%, respectively, in NP kidney biopsies. On the other hand, the rate of major complications was 9.8% in RP kidney biopsies, which was significantly higher than that in NP kidney biopsies. The rate of decrease in hemoglobin and hematocrit levels after biopsies was significantly higher in RP biopsies than in NP biopsies. CONCLUSION: Short-term trained nephrology fellows perform percutaneous ultrasound-guided kidney biopsy at a level equal to or superior to radiologists.
Anemia ; Biopsy ; Glomerular Filtration Rate ; Hand ; Hematocrit ; Hemoglobins ; Humans ; Kidney ; Light ; Microscopy ; Microscopy, Electron ; Nephrology ; Platelet Count ; Retrospective Studies