dc.contributor.author | Savva, Isavella | en |
dc.contributor.author | Pierides, Alkis M. | en |
dc.contributor.author | Constantinou-Deltas, Constantinos D. | en |
dc.creator | Savva, Isavella | en |
dc.creator | Pierides, Alkis M. | en |
dc.creator | Constantinou-Deltas, Constantinos D. | en |
dc.date.accessioned | 2019-11-04T12:52:38Z | |
dc.date.available | 2019-11-04T12:52:38Z | |
dc.date.issued | 2016 | |
dc.identifier.issn | 1043-6618 | |
dc.identifier.uri | http://gnosis.library.ucy.ac.cy/handle/7/53369 | |
dc.description.abstract | Alport syndrome (AS) is a hereditary progressive glomerulonephritis with a high life-time risk for end-stage renal disease (ESRD). Most patients will reach ESRD before the age of 30 years, while a subset of them with milder mutations will do so at older ages, even after 50 years. Frequent extrarenal manifestations are hearing loss and ocular abnormalities. AS is a genetically heterogeneous collagen IV nephropathy, with 85% of the cases caused by mutations in the X-linked COL4A5 gene and the rest by homozygous or compound heterozygous mutations in either the COL4A3 or the COL4A4 gene on chromosome 2q36-37. There is no radical cure for the disease and attempts to use various stem cell therapies in animal models have been met with ambiguous success. However, effective treatment has been accomplished with pharmacological intervention at the renin-angiotensin-aldosterone system (RAAS), first in animal models of AS and more recently in humans. Angiotensin converting enzyme inhibitors (ACEis) and angiotensin receptor blockers (ARBs) have been shown to significantly delay the progression of chronic kidney disease and the onset of ESRD. Also, renin inhibitors and aldosterone blockade were used with positive results, while the combination of ACEis and ARBs was met with mixed success. An important study, the EARLY-PROTECT, aims at evaluating the efficacy of ACEis when administered very early on in children with AS. Novel therapies are also tested experimentally or are under design in animal models by several groups, including the use of amniotic fluid stem cells and synthetic chaperones. | en |
dc.source | Pharmacological Research | en |
dc.source.uri | https://www.scopus.com/inward/record.uri?eid=2-s2.0-84962252473&doi=10.1016%2fj.phrs.2016.03.017&partnerID=40&md5=2a040448beba02fc703f8b457e760b6f | |
dc.subject | Review | en |
dc.subject | human | en |
dc.subject | Humans | en |
dc.subject | priority journal | en |
dc.subject | drug efficacy | en |
dc.subject | drug safety | en |
dc.subject | fatigue | en |
dc.subject | drug tolerability | en |
dc.subject | hypotension | en |
dc.subject | placebo | en |
dc.subject | nonhuman | en |
dc.subject | weight reduction | en |
dc.subject | protein | en |
dc.subject | kidney disease | en |
dc.subject | proteinuria | en |
dc.subject | Animals | en |
dc.subject | animal | en |
dc.subject | genetics | en |
dc.subject | gene mutation | en |
dc.subject | heterozygote | en |
dc.subject | collagen type 4 | en |
dc.subject | creatinine clearance | en |
dc.subject | transforming growth factor beta1 | en |
dc.subject | drug effects | en |
dc.subject | coughing | en |
dc.subject | dipeptidyl carboxypeptidase inhibitor | en |
dc.subject | glomerulus filtration rate | en |
dc.subject | Collagen Type IV | en |
dc.subject | orthostatic hypotension | en |
dc.subject | candesartan | en |
dc.subject | lisinopril | en |
dc.subject | losartan | en |
dc.subject | angiotensin receptor antagonist | en |
dc.subject | Alport syndrome | en |
dc.subject | Nephritis, Hereditary | en |
dc.subject | cyclosporin | en |
dc.subject | Angiotensin Receptor Antagonists | en |
dc.subject | renin angiotensin aldosterone system | en |
dc.subject | renin inhibitor | en |
dc.subject | Renin-Angiotensin System | en |
dc.subject | gene therapy | en |
dc.subject | lifespan | en |
dc.subject | Abbreviations RAAS renin angiotensin aldosterone system | en |
dc.subject | ACEi(s) angiotensin converting enzyme inhibitor(s) | en |
dc.subject | aldosterone blockade | en |
dc.subject | aliskiren | en |
dc.subject | amlodipine | en |
dc.subject | amniotic fluid stem cell | en |
dc.subject | angioneurotic edema | en |
dc.subject | Angiotensin-Converting Enzyme Inhibitors | en |
dc.subject | ARAS autosomal recessive AS | en |
dc.subject | ARB(s) angiotensin receptor blocker(s) | en |
dc.subject | AS Alport syndrome | en |
dc.subject | CKD chronic kidney disease | en |
dc.subject | enalapril | en |
dc.subject | ESRD end-stage renal disease | en |
dc.subject | estimated glomerulus filtration rate | en |
dc.subject | gynecomastia | en |
dc.subject | hyperkalemia | en |
dc.subject | pharmacological blocking | en |
dc.subject | protein urine level | en |
dc.subject | ramipril | en |
dc.subject | renal protection | en |
dc.subject | SP spironolactone | en |
dc.subject | spironolactone | en |
dc.subject | TBMN thin basement membrane nephropathy | en |
dc.subject | urea | en |
dc.subject | urea blood level | en |
dc.subject | XLAS X-linked AS | en |
dc.title | RAAS inhibition and the course of Alport syndrome | en |
dc.type | info:eu-repo/semantics/article | |
dc.identifier.doi | 10.1016/j.phrs.2016.03.017 | |
dc.description.volume | 107 | |
dc.description.startingpage | 205 | |
dc.description.endingpage | 210 | |
dc.author.faculty | Σχολή Θετικών και Εφαρμοσμένων Επιστημών / Faculty of Pure and Applied Sciences | |
dc.author.department | Τμήμα Βιολογικών Επιστημών / Department of Biological Sciences | |
dc.type.uhtype | Article | en |
dc.description.notes | <p>Cited By :3</p> | en |
dc.source.abbreviation | Pharmacol.Res. | en |
dc.contributor.orcid | Constantinou-Deltas, Constantinos D. [0000-0001-5549-9169] | |
dc.gnosis.orcid | 0000-0001-5549-9169 | |