Complications and prognosis of patients diagnosed with autosomal recessive polycystic kidney disease in neonatal period
AbstractThere are no clinical guidelines for performing nephrectomy in patients with autosomal recessive polycystic kidney disease (ARPKD). Few reports have described the clinical course of ARPKD diagnosed in the neonatal period in detail. Here, we report seven patients diagnosed with ARPKD and treated at our center during the neonatal period. Two died within 48 h of life due to pulmonary hypoplasia. Of the remaining five patients, three had anuria and required for kidney replacement therapy (KRT) within one week after birth, whereas two with a milder phenotype survived without KRT. All three patients who received KRT un...
Source: CEN Case Reports - October 25, 2023 Category: Urology & Nephrology Source Type: research
Genes, Vol. 14, Pages 1983: Double Heterozygous Pathogenic Variants in the LOX and PKD1 Genes in a 5-Year-Old Patient with Thoracic Aortic Aneurysm and Polycystic Kidney Disease
We present a case report of a 5.5-year-old girl with an aortic aneurysm and concomitant polycystic kidney disease. Whole exome sequencing was performed, followed by family screening by amplicon deep sequencing and diagnostic imaging studies. In the proband, two pathogenic variants were identified: p.Tyr257Ter in the LOX gene inherited from her mother, and p.Thr2977Ile in the PKD1 gene inherited from her father. All adult carriers of either of these variants showed symptoms of aortic disease. We conclude that the coexistence of two independent genetic variants in the proband may be the reason for an early onset of disease. (Source: Genes)
Source: Genes - October 24, 2023 Category: Genetics & Stem Cells Authors: Joanna Kinga Poni ńska Weronika Pelczar-P łachta Agnieszka Pollak Katarzyna Jo ńczyk-Potoczna Gra żyna Truszkowska Ilona Micha łowska Emilia Szafran Zofia T. Bili ńska Waldemar Bobkowski Rafa ł Płoski Tags: Case Report Source Type: research
Simultaneous Echinococcal superinfection in a patient with polycystic liver disease
This article aims to explore both clinical conditions and highlight the therapeutic considerations for their management. Moreover, we discuss the significance of this unique case, emphasizing the possibility of the coexistence of these two pathologies. (Source: Clinical Case Reports)
Source: Clinical Case Reports - October 20, 2023 Category: General Medicine Authors: Ottavia Cicerone,
Raffaella Lissandrin,
Enrico Brunetti,
Marcello Maestri Tags: CASE REPORT Source Type: research
Suppression of dengue virus replication by the French maritime pine extract Pycnogenol ®
Virus Res. 2023 Oct 11:199244. doi: 10.1016/j.virusres.2023.199244. Online ahead of print.ABSTRACTDengue virus (DENV) is mainly found in the tropics and infects approximately 400 million people annually. However, no clinically available therapeutic agents specific to dengue have been developed. Here, we examined the potential antiviral effects of the French maritime pine extract Pycnogenol® (PYC) against DENV because we previously found that the extract exerts antiviral effects on hepatitis C virus, which belongs to the Flavivirus family. First, we examined the efficacy of PYC against DENV1, 2, 3, and 4 serotypes and dete...
Source: Virus Research - October 13, 2023 Category: Virology Authors: Kazi Anowar Hossain Rupaly Akhter Md Haroon Or Rashid Lipi Akter Masashi Utsunomiya Bouchra Kitab NgweTun Mm Takayuki Hishiki Michinori Kohara Kouichi Morita Kyoko Tsukiyama-Kohara Source Type: research
Suppression of dengue virus replication by the French maritime pine extract Pycnogenol ®
Virus Res. 2023 Oct 11:199244. doi: 10.1016/j.virusres.2023.199244. Online ahead of print.ABSTRACTDengue virus (DENV) is mainly found in the tropics and infects approximately 400 million people annually. However, no clinically available therapeutic agents specific to dengue have been developed. Here, we examined the potential antiviral effects of the French maritime pine extract Pycnogenol® (PYC) against DENV because we previously found that the extract exerts antiviral effects on hepatitis C virus, which belongs to the Flavivirus family. First, we examined the efficacy of PYC against DENV1, 2, 3, and 4 serotypes and dete...
Source: Cell Research - October 13, 2023 Category: Cytology Authors: Kazi Anowar Hossain Rupaly Akhter Md Haroon Or Rashid Lipi Akter Masashi Utsunomiya Bouchra Kitab NgweTun Mm Takayuki Hishiki Michinori Kohara Kouichi Morita Kyoko Tsukiyama-Kohara Source Type: research
Suppression of dengue virus replication by the French maritime pine extract Pycnogenol ®
Virus Res. 2023 Oct 11:199244. doi: 10.1016/j.virusres.2023.199244. Online ahead of print.ABSTRACTDengue virus (DENV) is mainly found in the tropics and infects approximately 400 million people annually. However, no clinically available therapeutic agents specific to dengue have been developed. Here, we examined the potential antiviral effects of the French maritime pine extract Pycnogenol® (PYC) against DENV because we previously found that the extract exerts antiviral effects on hepatitis C virus, which belongs to the Flavivirus family. First, we examined the efficacy of PYC against DENV1, 2, 3, and 4 serotypes and dete...
Source: Virus Research - October 13, 2023 Category: Virology Authors: Kazi Anowar Hossain Rupaly Akhter Md Haroon Or Rashid Lipi Akter Masashi Utsunomiya Bouchra Kitab NgweTun Mm Takayuki Hishiki Michinori Kohara Kouichi Morita Kyoko Tsukiyama-Kohara Source Type: research
Vascular polycystin proteins in health and disease
AbstractPKD1 (polycystin 1) and PKD2 (polycystin 2) are expressed in a variety of different cell types, including arterial smooth muscle and endothelial cells. PKD1 is a transmembrane domain protein with a large extracellular N-terminus that is proposed to act as a mechanosensor and receptor. PKD2 is a member of the transient receptor potential (TRP) channel superfamily which is also termed TRPP1. Mutations in the genes which encode PKD1 and PKD2 lead to autosomal polycystic kidney disease (ADPKD). ADPKD is one of the most prevalent monogenic disorders in humans and is associated with extrarenal and vascular complications,...
Source: Microcirculation - October 13, 2023 Category: Research Authors: Ulrich C. Mbiakop,
Jonathan H. Jaggar Tags: REVIEW Source Type: research
Ouabain enhances renal cyst growth in a slowly progressive mouse model of autosomal dominant polycystic kidney disease
Am J Physiol Renal Physiol. 2023 Oct 12. doi: 10.1152/ajprenal.00056.2023. Online ahead of print.ABSTRACTRenal cyst progression in autosomal dominant polycystic kidney disease (ADPKD) is highly dependent on agents circulating in blood. We have previously shown, using different in vitro models, that one of these agents is the hormone ouabain. By binding to Na,K-ATPase (NKA), ouabain triggers a cascade of signal transduction events which enhance ADPKD cyst progression by stimulating cell proliferation, fluid secretion, and dedifferentiation of the renal tubular epithelial cells. Here, we determined the effects of ouabain in ...
Source: American Journal of Physiology. Renal Physiology - October 12, 2023 Category: Physiology Authors: Jordan Trant Gladis Sanchez Jeffrey McDermott Gustavo Blanco Source Type: research
Ouabain enhances renal cyst growth in a slowly progressive mouse model of autosomal dominant polycystic kidney disease
Am J Physiol Renal Physiol. 2023 Oct 12. doi: 10.1152/ajprenal.00056.2023. Online ahead of print.ABSTRACTRenal cyst progression in autosomal dominant polycystic kidney disease (ADPKD) is highly dependent on agents circulating in blood. We have previously shown, using different in vitro models, that one of these agents is the hormone ouabain. By binding to Na,K-ATPase (NKA), ouabain triggers a cascade of signal transduction events which enhance ADPKD cyst progression by stimulating cell proliferation, fluid secretion, and dedifferentiation of the renal tubular epithelial cells. Here, we determined the effects of ouabain in ...
Source: Am J Physiol Renal P... - October 12, 2023 Category: Urology & Nephrology Authors: Jordan Trant Gladis Sanchez Jeffrey McDermott Gustavo Blanco Source Type: research
Ouabain enhances renal cyst growth in a slowly progressive mouse model of autosomal dominant polycystic kidney disease
Am J Physiol Renal Physiol. 2023 Oct 12. doi: 10.1152/ajprenal.00056.2023. Online ahead of print.ABSTRACTRenal cyst progression in autosomal dominant polycystic kidney disease (ADPKD) is highly dependent on agents circulating in blood. We have previously shown, using different in vitro models, that one of these agents is the hormone ouabain. By binding to Na,K-ATPase (NKA), ouabain triggers a cascade of signal transduction events which enhance ADPKD cyst progression by stimulating cell proliferation, fluid secretion, and dedifferentiation of the renal tubular epithelial cells. Here, we determined the effects of ouabain in ...
Source: American Journal of Physiology. Renal Physiology - October 12, 2023 Category: Physiology Authors: Jordan Trant Gladis Sanchez Jeffrey McDermott Gustavo Blanco Source Type: research
Ouabain enhances renal cyst growth in a slowly progressive mouse model of autosomal dominant polycystic kidney disease
Am J Physiol Renal Physiol. 2023 Oct 12. doi: 10.1152/ajprenal.00056.2023. Online ahead of print.ABSTRACTRenal cyst progression in autosomal dominant polycystic kidney disease (ADPKD) is highly dependent on agents circulating in blood. We have previously shown, using different in vitro models, that one of these agents is the hormone ouabain. By binding to Na,K-ATPase (NKA), ouabain triggers a cascade of signal transduction events which enhance ADPKD cyst progression by stimulating cell proliferation, fluid secretion, and dedifferentiation of the renal tubular epithelial cells. Here, we determined the effects of ouabain in ...
Source: Am J Physiol Renal P... - October 12, 2023 Category: Urology & Nephrology Authors: Jordan Trant Gladis Sanchez Jeffrey McDermott Gustavo Blanco Source Type: research
