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Clinical Implication of Tolvaptan in Patients with Autosomal Dominant Polycystic Kidney Disease

Cho S, Paek JH, Park WY, Jin K, Kim DK, Han S, Kim Y

Tolvaptan, a non-peptide arginine vasopressin V2 receptor antagonist, is a newly developed drug to reduce kidney volume and preserve kidney function in autosomal dominant polycystic kidney disease (ADPKD) patients. We...
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The Natural Course of Total Kidney Volume in Patients with Autosomal Dominant Polycystic Kidney Disease undergoing Hemodialysis

Kim YN, Jung Y, Shin HS, Rim H, Park JG, Lee DY, Kim JK

Objectives: The natural course of native kidneys after hemodialysis initiation in patients with autosomal dominant polycystic kidney disease (ADPKD) remains poorly understood. Methods: We measured the total volumes of native kidneys...
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Clinical and genetic characteristics of Korean autosomal dominant polycystic kidney disease patients

Oh YK, Park HC, Ryu H, Kim YC, Oh KH

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....
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Comparison of chronic kidney disease trial designs and analysis strategies

Lawrence J

Background: Despite the large burden of chronic kidney disease (CKD), it is challenging to conduct adequately powered clinical trials in this setting. Sound and efficient trials are needed to advance...
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Expert-level segmentation using deep learning for volumetry of polycystic kidney and liver

Shin TY, Kim H, Lee JH, Choi JS, Min HS, Cho H, Kim K, Kang G, Kim J, Yoon S, Park H, Hwang YU, Kim HJ, Han M, Bae E, Yoon JW, Rha KH, Lee YS

Purpose: Volumetry is used in polycystic kidney and liver diseases (PKLDs), including autosomal dominant polycystic kidney disease (ADPKD), to assess disease progression and drug efficiency. However, since no rapid and...
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Importance of family segregation in the American College of Medical Genetics and Genomics and Association of Molecular Pathology guidelines: Case of a Korean family with autosomal dominant polycystic disease

Kwon WK, Kim S, Jang JH, Kim JW

Since the American College of Medical Genetics and Genomics and Association of Molecular Pathology published their guidelines in 2015, most interpretations of genetic tests have followed them. However, all variants...
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Urinary Angiotensinogen in addition to Imaging Classification in the Prediction of Renal Outcome in Autosomal Dominant Polycystic Kidney Disease

Park HC, Kim J, Cho A, Kim DH, Lee YK, Ryu H, Kim H, Oh KH, Oh YK, Hwang YH, Lee KB, Kim SW, Kim YH, Lee J, Ahn C, KNOW-CKD Investigators Group

Background: Intrarenal renin-angiotensin system (RAS) is known to play the major role in the development of hypertension and renal progression in autosomal dominant polycystic kidney disease (ADPKD). Urinary angiotensinogen to...
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Ca2+/calmodulin-dependent regulation of polycystic kidney disease 2-like-1 by binding at C-terminal domain

Baik JY, Park EYJ, So I

Polycystic kidney disease 2-like-1 (PKD2L1), also known as polycystin- L or TRPP3, is a non-selective cation channel that regulates intracellular calcium concentration. Calmodulin (CaM) is a calcium binding protein, consisting...
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The role of calmodulin in regulating calcium-permeable PKD2L1 channel activity

Park EY, Baik JY, Kwak M, So I

Polycystic kidney disease 2-like-1 (PKD2L1), polycystin-L or transient receptor potential polycystin 3 (TRPP3) is a TRP superfamily member. It is a calcium-permeable non-selective cation channel that regulates intracellular calcium concentration...
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Long-term Tolvaptan Treatment of Autosomal Dominant Polycystic Kidney Disease in Korea

Kim HY, Lee SJ, Kim BK, Kim M, Bae EH, Ma SK, Kim SW

A 22-year-old male patient was diagnosed with autosomal dominant polycystic kidney disease (ADPKD). He received conservative treatment with an angiotensin-converting enzyme inhibitor. Two years later, oral therapy, consisting of 60...
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Predictors and outcomes of laparoscopic nephrectomy in autosomal dominant polycystic kidney disease

Chen K, Tan YG, Tan D, Pek G, Huang HH, Sim SP

PURPOSE: Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease, and 20% of patients eventually require nephrectomies due to compressive symptoms or renal-related complications. Traditionally, nephrectomies...
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Femoral Neuropathy Secondary to Autosomal Dominant Polycystic Kidney Disease: A Case Report

Yoo J, Lim KB, Lee HJ, Kim J, You EC, Kang J

Compressive femoral neuropathy is a disabling condition accompanied by difficulty in hip flexion and knee extension. It may result from retroperitoneal hematoma or bleeding, or from complications associated with pelvic,...
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Esophageal Artery Pseudoaneurysm and Takayasu Arteritis in a Patient with Autosomal Dominant Polycystic Kidney Disease

Kim H, Yu Y, Shim KE, Kim JE, Koh J, Yoon JW, Ahn C, Oh YK

A 47-year-old female previously diagnosed with ADPKD visited the hospital due to sudden pain in her upper abdomen and back. Esophagogastroduodenoscopy, contrast-enhanced abdominal computed tomography (CT), and CT angiography identified...
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Incidental Detection of Peri-Renal Hemorrhage on F-18 FDG PET/CT Imaging in a Patient with Polycystic Kidney Disease

Kim MH, Kim DW

A 72-year-old male patient with a history of polycystic kidney disease and lung malignancy underwent F-18 fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT) for the evaluation of tumor recurrence. The...
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Autosomal Dominant Polycystic Kidney Desease Coexisting with Renal Dysplasia. First Case Described and Followed Since Prenatal Period

Truyols C

Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent hereditary renal disease and causes terminal chronic renal failure. ADPKD is characterized by bilateral multiple renal cysts, which are produced...
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Xp11.2 translocation renal cell carcinoma in the autosomal dominant polycystic kidney disease patient with preserved renal function

Huh H, Jo HA, Yi Y, Kim SH, Moon KC, Ahn C, Park HC

No abstract available.
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Baseline Cardiovascular Characteristics of Adult Patients with Chronic Kidney Disease from the KoreaN Cohort Study for Outcomes in Patients With Chronic Kidney Disease (KNOW-CKD)

Kim H, Yoo TH, Choi KH, Oh KH, Lee J, Kim SW, Kim TH, Sung S, Han SH, KNOW-CKD Group

Cardiovascular disease (CVD) is the most common cause of death in patients with chronic kidney disease (CKD). We report the baseline cardiovascular characteristics of 2,238 participants by using the data...
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Baseline General Characteristics of the Korean Chronic Kidney Disease: Report from the KoreaN Cohort Study for Outcomes in Patients With Chronic Kidney Disease (KNOW-CKD)

Kang E, Han M, Kim H, Park SK, Lee J, Hyun YY, Kim YS, Chung W, Kim HJ, Oh YK, Ahn C, Oh KH

The KoreaN Cohort Study for Outcomes in Patients With Chronic Kidney Disease (KNOW-CKD) was developed to investigate various clinical courses and risk factors for progression of Korean chronic kidney disease...
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Hypokalemic Hypertension Leading to a Diagnosis of Autosomal Dominant Polycystic Kidney Disease

Vutthikraivit W, Assanatham M, Sriphrapradang C

Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited renal disease. Hypertension is common and occurs before decline in renal function. However, the coexistence of hypertension and hypokalemia...
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Two cases of TSC2/PKD1 contiguous gene deletion syndrome

You J, Kang E, Kim Y, Lee BH, Ko TS, Kim GH, Choi JH, Yoo HW

Tuberous sclerosis complex (TSC, MIM#191100) is an autosomal dominant neurocutaneous syndrome caused by mutation or deletion of TSC1 encoding hamartin or TSC2 encoding tuberin and characterized by seizure, mental retardation,...
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