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Clin J Am Soc Nephrol 2021 Jun 11;16(6):908-915. Epub 2021 Jun 11.

University of Colorado Anschutz Medical Campus, Aurora, Colorado.

Background And Objectives: On the basis of earlier observations, we evaluated the association between overweight and obesity and rapid progression of autosomal dominant polycystic kidney disease in participants in the Tolvaptan Efficacy and Safety in Management of Autosomal Dominant Polycystic Kidney Disease and Its Outcomes (TEMPO) 3:4 trial. More importantly, we also determined whether efficacy of tolvaptan was attenuated in individuals with baseline overweight or obesity.

Design, Setting, Participants, & Measurements: A total of 1312 study participants with relatively early-stage autosomal dominant polycystic kidney disease (mean eGFR 78±22 ml/min per 1. Read More

Clin J Am Soc Nephrol 2021 Jun;16(6):850-852

Division of Nephrology, Department of Internal Medicine, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands

Front Physiol 2021 25;12:623599. Epub 2021 May 25.

Department of Biomedical Sciences, Macquarie University, Sydney, NSW, Australia.

Carotid body feedback and hypoxia may serve to enhance respiratory-sympathetic nerve coupling (respSNA) and act as a driver of increased blood pressure. Using the Lewis polycystic kidney (LPK) rat model of chronic kidney disease, we examined respSNA in adult female rodents with CKD and their response to acute hypoxia or hypercapnia compared to Lewis control animals. Under urethane anesthesia, phrenic nerve activity, splanchnic sympathetic nerve activity (sSNA), and renal sympathetic nerve activity (rSNA) were recorded under baseline conditions and during mild hypoxic or hypercapnic challenges. Read More

J Transl Med 2021 Jun 9;19(1):253. Epub 2021 Jun 9.

Graduate Institute of Biomedical Sciences and School of Medicine, College of Medicine, China Medical University, No. 2, Yuh-Der Road, Taichung, 404, Taiwan.

Background: Polycystic kidney disease (PKD) is a common renal disorder affecting approximately 1 in 1000 live births. Tuberculosis (TB) is an infectious disease worldwide. This study investigated the risk of TB infection in patients with PKD. Read More

Clin Genet 2021 Jun 7. Epub 2021 Jun 7.

Department of Nephrology, Changzheng Hospital, Naval Medical University, Shanghai, China.

PKD2 gene variants account for 4.5% to 20% of patients with autosomal dominant polycystic kidney disease (ADPKD). Little is known about the clinical characteristics of PKD2 variants in Chinese patients with ADPKD. Read More

Radiology 2021 Jun 8:202302. Epub 2021 Jun 8.

From the Departments of Radiology (C.J.M., S.F., J.R.P., N.P., M.A.G., D.M., C.A.F., Y.C.), Genetics and Genome Sciences (M.L.D.), Pediatrics (M.L.D., K.M.D., C.A.F.), and Biomedical Engineering (M.A.G., D.M., C.A.F.), Case Western Reserve University, 11100 Euclid Ave, Bowell Building, Room B131, Cleveland, OH 44106; Departments of Radiology (M.M.) and Pediatrics (K.M., K.K.), University Hospitals Cleveland Medical Center, Cleveland, Ohio; and Center for Pediatric Nephrology, Cleveland Clinic Children's Hospital, Cleveland, Ohio (A.P., K.M.D.).

Background MR fingerprinting (MRF) provides rapid and simultaneous quantification of multiple tissue parameters in a single scan. Purpose To evaluate a rapid kidney MRF technique at 3.0 T in phantoms, healthy volunteers, and patients. Read More

Zhonghua Yi Xue Yi Chuan Xue Za Zhi 2021 Jun;38(6):585-588

Department of Ultrasonography, Zhumadian Central Hospital, Zhumadian, Henan 463000, China.

Objective: To explore the genetic basis for a fetus with renal abnormalities through whole exome sequencing and imaging examination.

Methods: Clinical data and result of medical imaging of the fetus was collected. Amniotic fluid sample was collected for the extraction of fetal DNA. Read More

Clin Exp Nephrol 2021 Jun 4. Epub 2021 Jun 4.

Department of Advanced Informatics for Genetic Disease, Juntendo University Graduate School of Medicine, Hongo 2-1-1, Bunkyo-ku, Tokyo, 113-8421, Japan.

Background: Autosomal dominant polycystic kidney disease (ADPKD) is a progressive condition that eventually leads to end-stage renal disease. A phase 3 trial of tolvaptan (TEMPO 3:4; NCT00428948) and its open-label extension (TEMPO Extension Japan: TEMPO-EXTJ; NCT01280721) were conducted in patients with ADPKD. In this post hoc analysis, effects on renal function and the safety profile of tolvaptan were assessed over a long-term period that included the 3-year TEMPO 3:4 and the approximately 3-year TEMPO-EXTJ trials. Read More

Orphanet J Rare Dis 2021 Jun 2;16(1):251. Epub 2021 Jun 2.

Department of Renal Medicine, University College London and Paediatric Nephrology Unit, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, UK.

Background: The European Rare Kidney Disease Reference Network (ERKNet) recently established ERKReg, a Web-based registry for all patients with rare kidney diseases. The main objectives of this core registry are to generate epidemiological information, identify current patient cohort for clinical research, explore diagnostic and therapeutic management practices, and monitor treatment performance and patient's outcomes. The registry has a modular design that allows to integrate comprehensive disease-specific registries as extensions to the core database. Read More

Dig Liver Dis 2021 Jun;53 Suppl 2:S49-S86

Multivisceral Transplant Unit, Gastroenterology, Department of Surgery, Oncology and Gastroenterology, University Hospital of Padua, Via Giustiniani 2, 35128, Padua, Italy. Electronic address:

Liver and kidney are strictly connected in a reciprocal manner, in both the physiological and pathological condition. The Italian Association for the Study of Liver, in collaboration with the Italian Society of Nephrology, with this position paper aims to provide an up-to-date overview on the principal relationships between these two important organs. A panel of well-recognized international expert hepatologists and nephrologists identified five relevant topics: 1) The diagnosis of kidney damage in patients with chronic liver disease; 2) Acute kidney injury in liver cirrhosis; 3) Association between chronic liver disease and chronic kidney disease; 4) Kidney damage according to different etiology of liver disease; 5) Polycystic kidney and liver disease. Read More

Int J Mol Sci 2021 May 4;22(9). Epub 2021 May 4.

Department of Biomedical Laboratory Science, Konyang University, Daejeon 35365, Korea.

Polycystic Kidney Disease () is a disorder that affects the kidneys and other organs, and its major forms are encoded by polycystin-1 () and polycystin-2 (), as and It is located sandwiched inside and outside cell membranes and interacts with other cells. This protein is most active in kidney cells before birth, and and work together to help regulate cell proliferation, cell migration, and interactions with other cells. The molecular relationship and the function between and cancer is well known, such as increased or decreased cell proliferation and promoting or suppressing cell migration depending on the cancer cell type specifically. Read More

Clin Exp Nephrol 2021 May 31. Epub 2021 May 31.

College of Medicine and Public Health, Ubon Ratchathani University, Sathonlamark Road, Warin Chamrap, Ubon Ratchathani, 34190, Thailand.

Background: Renal bilateral fluid filled-cyst in polycystic kidney disease (PKD) is associated with abnormal epithelial cell proliferation and transepithelial fluid secretion which leads to end-stage renal disease (ESRD). A chalcone derivative, isoliquiritigenin (ISLQ), has been shown to have various pharmacological properties. Since several studies have shown that ISLQ could inhibit CFTR channel activity, it is interesting to see whether it can inhibit renal cyst enlargement. Read More

Biomed Hub 2020 Sep-Dec;5(3):32-37. Epub 2020 Dec 3.

Department of Pediatrics, Marshall University, Joan C Edwards School of Medicine, Huntington, West Virginia, USA.

We present the case of an infant referred to our NICU born at 39 weeks' gestation with persistent hypoglycemia with elevated insulin levels (HI) requiring diazoxide to maintain normoglycemia. Additionally, polycystic kidney disease (PKD) was detected by ultrasound. Molecular genetic testing revealed pathogenic variants in the gene, i. Read More

Intern Med 2021 May 29. Epub 2021 May 29.

Department of Nephrology, Toranomon Hospital, Japan.

We herein report a 70-year-old man with malaise and muscle weakness that had developed within a month. The patient also had abdominal fullness due to polycystic kidney disease. Severe proximal skeletal muscle weakness and mild elevation of creatinine kinase to 301 IU/L were noted. Read More

J Am Soc Nephrol 2021 May 27. Epub 2021 May 27.

D Kohan, Division of Nephrology, University of Utah, Salt Lake City, United States

Background: Primary cilia regulation of renal function and blood pressure (BP) in health and disease is incompletely understood. The current study investigated the effect of nephron ciliary loss on renal physiology, blood pressure and ensuing cystogenesis.

Methods: Mice underwent doxycycline (DOX)-inducible nephron-specific knockout (KO) of the gene at 2 months of age using a Cre-LoxP strategy. Read More

PLoS One 2021 27;16(5):e0252479. Epub 2021 May 27.

Australasian Kidney Trials Network, University of Queensland, Brisbane, QLD, Australia.

Pain is a common symptom in people with autosomal dominant polycystic kidney disease (ADPKD), but it is assessed and reported inconsistently in research, and the validity of the measures remain uncertain. The aim of this study was to identify the characteristics, content, and psychometric properties of measures for pain used in ADPKD. We conducted a systematic review including all trials and observational studies that reported pain in people with ADPKD. Read More

Int J Stroke 2021 May 27:17474930211024584. Epub 2021 May 27.

University medical Center Utrecht, Neurology, Heidelberglaan 100, Utrecht, Netherlands.

Background: Subarachnoid hemorrhage from rupture of an intracranial aneurysm (aneurysmal subarachnoid hemorrhage, ASAH) is a devastating subset of stroke. Since brain damage from the initial hemorrhage is a major cause for the poor outcome after ASAH, prevention of ASAH has the highest potential to prevent poor outcome from ASAH.

Aim: In this review, we describe the groups at high risk of ASAH who may benefit from preventive screening for unruptured intracranial aneurysms (UIA) followed by preventive treatment of UIAs found. Read More

Andrologia 2021 May 27:e14124. Epub 2021 May 27.

Department of Animal Sciences, University of Illinois, Urbana-Champaign, IL, USA.

Polycystin-2, also known as transient receptor potential polycystin-2 (TRPP2), is a membrane protein that regulates calcium homeostasis in renal epithelial cells. Mutations in PKD2, the gene encoding human TRPP2, cause enlarged cystic kidneys and contribute to polycystic kidney disease (PKD). Male Drosophila melanogaster with mutations in amo, the homolog of PKD2, display a mild decrease in sperm motility but have a drastic reduction in fertility due to failed sperm migration and storage within the female tract. Read More

Indian J Nucl Med 2021 Jan-Mar;36(1):43-45. Epub 2021 Mar 4.

Department of Nuclear Medicine and PET-CT, Apollo Gleneagles Hospitals, Kolkata, West Bengal, India.

Fever of unknown origin (FUO) is a convoluted clinical dilemma. It can be caused by infective, inflammatory, malignant, and other pathologies. The identification of etiopathogenesis is essential for instituting definitive management. Read More

Langenbecks Arch Surg 2021 May 26. Epub 2021 May 26.

Department of General, Visceral and Vascular Surgery, University Hospital Jena, Am Klinikum 1, 07747, Jena, Germany.

Purpose: In patients suffering from autosomal dominant polycystic liver and kidney disease (ADPLKD), combined organ transplantation often poses a technical challenge due to the large volume of both organs. To simplify the transplantation procedure by improving the exposure of anatomical structures, we introduce a novel surgical technique of orthotopic liver and kidney transplantation.

Methods: The modified simultaneous liver and kidney transplantation technique via a right-sided L-incision included three steps: (1) right-sided nephrectomy in the recipient followed by (2) orthotopic liver transplantation in cava replacement technique and (3) the orthotopic kidney transplantation with arterial reconstruction to the right common iliac artery. Read More

Arch Argent Pediatr 2021 06;119(3):213

Sydney School of Public Health, The University of Sydney, Sydney, New South Wales, Australia.

Living with chronic kidney disease (CKD) is associated with hardships for patients and their care-partners. Empowering patients and their care-partners, including family members or friends involved in their care, may help minimize the burden and consequences of CKD related symptoms to enable life participation. There is a need to broaden the focus on living well with kidney disease and re-engagement in life, including an emphasis on patients being in control. Read More

J Nephrol 2021 May 25. Epub 2021 May 25.

Sydney School of Public Health, The University of Sydney, Sydney, NSW, Australia.

J Cell Mol Med 2021 May 25. Epub 2021 May 25.

Department of Ophthalmology, Xiang'an Hospital of Xiamen University, Fujian Provincial Key Laboratory of Ophthalmology and Visual Science, School of Medicine, Xiamen University, Fujian Engineering and Research Center of Eye Regenerative Medicine, Eye Institute of Xiamen University, Xiamen, China.

Polycystic kidney disease (PKD) is known to occur in three main forms, namely autosomal dominant PKD (ADPKD), autosomal recessive PKD (ARPKD) and syndromic PKD (SPKD), based on the clinical manifestations and genetic causes, which are diagnosable from the embryo stage to the later stages of life. Selection of the genetic test for the individuals with diagnostic imaging reports of cystic kidneys without a family history of the disease continues to be a challenge in clinical practice. With the objective of maintaining a limit on the time and medical cost of the procedure, a practical strategy for genotyping and targeted validation to resolve cystogene variations was developed in our clinical laboratory, which combined the techniques of whole-exome sequencing (WES), Long-range PCR (LR-PCR), Sanger sequencing and multiplex ligation-dependent probe amplification (MLPA) to work in a stepwise approach. Read More

BMC Nephrol 2021 May 24;22(1):194. Epub 2021 May 24.

Department of renal medicine, Salford Royal NHS Foundation Trust, Stott Lane, Salford, M6 8HD, UK.

Background: The Kidney Failure Risk Equation (KFRE) predicts the 2- and 5-year risk of end-stage renal disease (ESRD) in patients with chronic kidney disease (CKD) stages 3a-5. Its predictive performance in advanced CKD and in specific disease aetiologies requires further exploration. This study validates the 4- and 8-variable KFREs in an advanced CKD population in the United Kingdom by evaluating discrimination, calibration and clinical utility. Read More

Clin J Gastroenterol 2021 May 24. Epub 2021 May 24.

Department of Molecular Gastroenterology and Hepatology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, 465 Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, 602-8566, Japan.

A 60-year-old man with autosomal dominant polycystic kidney disease presented with malaise, melena, and epigastric discomfort. Esophagogastroduodenoscopy revealed a massive elevated gastric cancer lesion involving the cardia. Histopathological evaluation of a biopsy specimen showed poorly differentiated adenocarcinoma. Read More

Urol Case Rep 2021 Sep 3;38:101701. Epub 2021 May 3.

Department of Urology, University of Maryland School of Medicine, 655 W. Baltimore Street, Baltimore MD 21201, USA.

We present a very rare Case of a 53-year-old female with autosomal dominant polycystic kidney disease (ADPKD) who was incidentally found to have a reno-appendiceal fistula while undergoing open bilateral nephrectomy. The mid-portion of the appendix was fistulized to a cyst in the lower pole of the right kidney. The etiology was likely due to chronic inflammation. Read More

Patient Educ Couns 2021 May 5. Epub 2021 May 5.

Sydney School of Public Health, The University of Sydney, Sydney, New South Wales, Australia.

Biochem Biophys Res Commun 2021 Jul 18;561:73-79. Epub 2021 May 18.

Department of Biochemistry and Molecular Biology, School of Basic Medical Sciences, Tianjin Medical University, Tianjin, 300070, China. Electronic address:

Metabolic reprogramming is emerging as a key pathological contributor to the progression of autosomal dominant polycystic kidney disease (ADPKD), but the molecular mechanisms underlying dysregulated cellular metabolism remain elusive. Here we report that amino acid biosynthesis is reprogrammed in Pkd2-knockout mouse kidneys via a defective PERK-eIF2ɑ-ATF4 pathway. Transcriptomic analysis revealed that the amino acid biosynthesis pathways such as serine, arginine and cysteine were impaired, and associated critical enzymes were downregulated in Pkd2-knockout mouse kidneys. Read More

Kidney Int Rep 2021 May 27;6(5):1482-1483. Epub 2021 Mar 27.

Division of Nephrology and Hypertension and the Kidney Institute, University of Kansas Medical Center, Kansas City, Kansas, USA.

Kidney Int Rep 2021 May 27;6(5):1482. Epub 2021 Mar 27.

Division of Nephrology, Changzheng Hospital, Second Military Medical University, Shanghai, China.