Präemptive Therapie mit Angiotensin-Converting-Enzyme-Inhibitoren verzögert Nierenersatztherapie bei heterozygoten Mutationsträgerinnen mit X-chromosomalem und autosomal-rezessivem Alport-Syndrom

Wüst, Catharina

dc.contributor.advisor	Gross, Oliver Prof. Dr.	de
dc.contributor.author	Wüst, Catharina	de
dc.date.accessioned	2013-02-08T09:18:42Z	de
dc.date.available	2013-03-05T23:50:04Z	de
dc.date.issued	2013-02-08	de
dc.identifier.uri	http://hdl.handle.net/11858/00-1735-0000-000D-FAC3-7	de
dc.identifier.uri	http://dx.doi.org/10.53846/goediss-3710
dc.identifier.uri	http://dx.doi.org/10.53846/goediss-3710
dc.language.iso	deu	de
dc.rights.uri	http://creativecommons.org/licenses/by-nc-nd/3.0/
dc.subject.ddc	610	de
dc.title	Präemptive Therapie mit Angiotensin-Converting-Enzyme-Inhibitoren verzögert Nierenersatztherapie bei heterozygoten Mutationsträgerinnen mit X-chromosomalem und autosomal-rezessivem Alport-Syndrom	de
dc.type	doctoralThesis	de
dc.title.translated	Pre-emptive treatment with angiotensin converting enzyme inhibitors delays renal replacement therapy in heterozygous carriers of X-chromosomal and autosomal recessive Alport mutations	de
dc.contributor.referee	Gross, Oliver Prof. Dr.	de
dc.date.examination	2013-02-25	de
dc.description.abstracteng	The Alport syndrome (AS) is a hereditary chronic kidney disease which is characterized by ultrastructural lesions of the glomerular basement membrane (GBM), hearing loss and ocular changes. The cause of AS are various mutations in the genes COL4A3/4/5 that code for the different Type-IV collagen α-chains. Therefore, the embryonic α1-α1-α2- collagen network in the basement membranes of the glomerulus, the lens capsule and the cochlea cannot be replaced by the mature α3-α4-α5-collagen network. This leads to an increased proteolytic degradation of the basement membranes and in case of the GBM to chronic inflammation and fibrosis of the kidney resulting in end stage renal disease. The renin angiotensin aldosteron system (RAAS) plays an important role in the development of chronic kidney diseases. In this non-interventional observational study we wanted to show the effect of ACE-inhibitors on the kidney function of female patients with X-chromosomal or autosomal recessive AS. Therefore, a standardized questionnaire was sent to various nephrologic wards. The questionnaire provided information about kidney function parameters, clinical symptoms such as haematuria or proteinuria, therapy with ACE-inhibitors or angiotensin-receptor antagonists, necessity of renal replacement therapy, kidney transplants and the age at the onset of end stage renal disease and renal replacement therapy. The results showed that there were significant differences regarding the age at the onset of end stage renal disease between the females that were treated with an ACE-inhibitor and and those who did not receive any therapy. Thus, it should be noted that ACE-inhibitors should be introduced as a standard therapy for female patients with AS not only because of the reduction of arterial hypertension as a cardiac risk factor but also because of their nephroprotective effect.	de
dc.contributor.coReferee	Lakomek, Max Prof. Dr.	de
dc.subject.ger	Alport-Syndrom	de
dc.subject.ger	ACE-Inhibitoren	de
dc.subject.eng	Alport syndrome	de
dc.subject.eng	angiotensin converting enzyme inhibitor	de
dc.identifier.urn	urn:nbn:de:gbv:7-11858/00-1735-0000-000D-FAC3-7-1	de
dc.affiliation.institute	Medizinische Fakultät	de
dc.subject.gokfull	Medizin (PPN619874732)	de
dc.subject.gokfull	Innere Medizin - Allgemein- und Gesamtdarstellungen (PPN619875747)	de
dc.description.embargoed	2013-03-05	de
dc.identifier.ppn	773355103

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