Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease. It is characterized by the dysregulated growth of kidney cysts, resulting in end-stage kidney failure. By identifying the genes involved in ADPKD and detailing the molecular pathology of the disease, putative therapeutic agents have been developed. However, clinical trials of vasopressin receptor antagonists and somatostatin analogues have raised several concerns among researchers and clinicians. Questions regarding when and who to treat and what surrogate marker to use for describing endpoints have been raised. This review focuses on the current methods for managing ADPKD and describes recent findings from clinical trials. The main difficulties associated with implementing therapeutic agents in patients with ADPKD and considerations for clinical settings will also be discussed.
Biomarkers ; Humans ; Hypertension ; Kidney ; Kidney Diseases ; Pathology, Molecular ; Polycystic Kidney Diseases ; Polycystic Kidney, Autosomal Dominant* ; Receptors, Vasopressin ; Renal Insufficiency ; Renal Insufficiency, Chronic ; Somatostatin

BACKGROUND: An inactivated Hantaan virus vaccine (iHV) has been broadly used as a preventive strategy for hemorrhagic fever with renal syndrome (HFRS) by the South Korean Army. After the vaccination program was initiated, the overall incidence of HFRS cases was reduced in the military population. While there are about 400 HFRS cases annually, few studies have demonstrated the efficacy of the iHV in field settings. Therefore, this study aimed to evaluate the iHV efficacy on HFRS severity. METHODS: From 2009 to 2017, HFRS cases were collected in South Korean Army hospitals along with patients’ vaccination history. HFRS patients were classified retrospectively into two groups according to vaccination records: no history of iHV vaccination and valid vaccination. Vaccine efficacy on the severity of acute kidney injury (AKI) stage and dialysis events were investigated. RESULTS: The effects of the iHV on renal injury severity in between 18 valid vaccinated and 110 non-vaccinated patients were respectively evaluated. In the valid vaccination group, six of the 18 HFRS patients (33.3%) had stage 3 AKI, compared to 60 of the 110 (54.5%) patients in the non-vaccination group. The iHV efficacy against disease progression (VEp) was 58.1% (95% confidence interval, 31.3% to 88.0%). CONCLUSION: The iHV efficacy against the progression of HFRS failed to demonstrate statistically significant protection. However, different severity profiles were observed between the iHV and non-vaccination groups. Additional studies with larger populations are needed to demonstrate the effectiveness of the iHV in patients with HFRS.
Acute Kidney Injury ; Dialysis ; Disease Progression ; Hantaan virus ; Hantavirus* ; Hemorrhagic Fever with Renal Syndrome* ; Hospitals, Military ; Humans ; Incidence ; Military Personnel ; Preventive Medicine ; Retrospective Studies ; Vaccination

Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease. It is characterized by cyst growth in the kidneys, resulting in kidney enlargement and end-stage kidney disease. The polycystic kidney disease 1 (PKD1) and PKD2 have been identified as genes related to ADPKD and their significance in the molecular pathology of the disease has been studied. A disease-modifying drug has been approved; therefore, it has become important to identify patients at a high risk of kidney disease progression. Genetic tests, image analysis methods, and clinical factors for kidney disease progression prediction have been established. This review describes genetic and clinical characteristics, and discusses ongoing studies in Korean ADPKD patients.

Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease. It is characterized by cyst growth in the kidneys, resulting in kidney enlargement and end-stage kidney disease. The polycystic kidney disease 1 (PKD1) and PKD2 have been identified as genes related to ADPKD and their significance in the molecular pathology of the disease has been studied. A disease-modifying drug has been approved; therefore, it has become important to identify patients at a high risk of kidney disease progression. Genetic tests, image analysis methods, and clinical factors for kidney disease progression prediction have been established. This review describes genetic and clinical characteristics, and discusses ongoing studies in Korean ADPKD patients.

Encapsulating peritoneal sclerosis (EPS) is a rare but fatal complication of continuous ambulatory peritoneal dialysis (CAPD). There are some reports on the effect of immunosuppressant therapy including steroid, but the results have not always been promising. Recently, owing to the advance of surgical techniques, there are some reports of the EPS cases significantly improved after successful surgery. A 30-year old man developed EPS after 9 years of peritoneal dialysis, and switched to hemodialysis. In spite of repetitive conservative management and immunosuppressant therapy, there was no improvement. His body weight decreased from 50 kg to 40 kg (BMI 14.2 kg/m2) due to severe malnutrition, so we decided to perform surgery. Total intestinal enterolysis was done successfully without concomitant enterectomy, and his general condition improved dramatically. Four months after surgery, the serum albumin concentration increased from 3.1 g/dL to 4.3 g/dL, cholesterol from 92 mg/dL to 208 mg/dL, and hemoglobin from 9.2 g/dL to 12.5 g/dL. His body weight increased to 61 kg (BMI 21.6 kg/m2), and there was not any fluid collection or bowel obstruction seen on the abdominal CT scan. We experienced a case of EPS which was significantly improved after enterolysis. We report this case with review of the literature.
Body Weight ; Cholesterol ; Hemoglobins ; Malnutrition ; Peritoneal Dialysis ; Peritoneal Dialysis, Continuous Ambulatory ; Peritoneal Fibrosis ; Peritonitis ; Renal Dialysis ; Serum Albumin