Severe arterial hypertension (HTN) in pediatrics is mainly due to secondary causes. Here we describe the case of a 14-year-old female adolescent with severe HTN, metabolic alkalosis, and hypokalemia, secondary to a renin-secreting juxtaglomerular cell tumor diagnosed after 2 years of HTN progression.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.5546/aap.2022-02835.eng | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Background: Juxtaglomerular (JG) cells are sensors that control blood pressure and fluid-electrolyte homeostasis. In response to a decrease in perfusion pressure or changes in the composition and/or volume of the extracellular fluid, JG cells release renin, which initiates an enzymatic cascade that culminates in the production of angiotensin II (Ang II), a potent vasoconstrictor that restores blood pressure and fluid homeostasis. In turn, Ang II exerts a negative feedback on renin release, thus preventing excess circulating renin and the development of hypertension.
View Article and Find Full Text PDFRenal Juxtaglomerular Cell Tumors Exhibit Distinct Genomic and Epigenomic Features and Lack Recurrent Gene Fusions: Comprehensive Molecular Analysis of a Multi-institutional Series.
Am J Surg Pathol
December 2024
Department of Pathology, Indiana University School of Medicine, Indianapolis, IN.
Juxtaglomerular cell tumor (JxGCT) is a rare type of renal neoplasm demonstrating morphologic overlap with some mesenchymal tumors such as glomus tumor (GT) and solitary fibrous tumor (SFT). Its oncogenic drivers remain elusive, and only a few cases have been analyzed with modern molecular techniques. In prior studies, loss of chromosomes 9 and 11 appeared to be recurrent.
View Article and Find Full Text PDFTcf21 as a founder transcription factor in specifying Foxd1 cells to the juxtaglomerular cell lineage.
Am J Physiol Renal Physiol
January 2025
Division of Pediatric Nephrology, Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois, United States.
Renin is crucial for blood pressure regulation and electrolyte balance, and its expressing cells arise from Forkhead box D1-positive (Foxd1) stromal progenitors. However, factors guiding these progenitors toward renin-secreting cell fate remain unclear. Tcf21, a basic helix-loop-helix (bHLH) transcription factor, is essential in kidney development.
View Article and Find Full Text PDFJuxtaglomerular Cell Tumor With Cytohistological Correlation: A Case Report.
Diagn Cytopathol
February 2025
University of Virginia School of Medicine Inova Campus, Falls Church, Virginia, USA.
Juxtaglomerular cell tumors (JCT) are uncommon renin-secreting tumors of the kidney with cytologic findings of JCT rarely reported. We describe a case of JCT in a 37-year-old man with uncontrolled hypertension that was cured by removal of the tumor via partial nephrectomy. Cytology material was prepared by scraping of the freshly sectioned tumor mass and stained with Diff-Quik and Papanicolaou stains.
View Article and Find Full Text PDFPiezo channels in JG cells do not regulate renin expression or renin release to the circulation.
Clin Sci (Lond)
December 2024
Department of Pediatrics, University of Virginia School of Medicine, Charlottesville, VA, U.S.A.
Article Synopsis
- Renin-expressing juxtaglomerular (JG) cells have a mechanism that senses pressure and regulates renin release based on changes in blood flow.
- The study investigates whether Piezo2 receptors, known for their role in detecting touch, play a role in controlling renin synthesis and release in JG cells.
- Results show that Piezo2 channels are not necessary for renin release or synthesis in JG cells under normal or stressed conditions, suggesting that other mechanisms need to be identified.
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!