| dc.contributor.advisor | Gross, Oliver Prof. Dr. | |
| dc.contributor.author | Assmann, Angela | |
| dc.date.accessioned | 2015-01-20T10:15:08Z | |
| dc.date.available | 2015-01-28T23:50:12Z | |
| dc.date.issued | 2015-01-20 | |
| dc.identifier.uri | http://hdl.handle.net/11858/00-1735-0000-0022-5D88-7 | |
| dc.identifier.uri | http://dx.doi.org/10.53846/goediss-4850 | |
| dc.language.iso | deu | de |
| dc.rights.uri | http://creativecommons.org/licenses/by-nc-nd/3.0/ | |
| dc.subject.ddc | 610 | de |
| dc.title | Prognose von Patienten mit Alport-Syndrom unter Berücksichtigung einer medikamentösen Intervention und verschiedener Nierenersatzverfahren | de |
| dc.type | doctoralThesis | de |
| dc.title.translated | Prognosis of patients with aport syndrome considering a medical intervention and different renal replacement therapy | de |
| dc.contributor.referee | Lakomek, Max Prof. Dr. | |
| dc.date.examination | 2015-01-21 | |
| dc.description.abstracteng | Alport syndrome is a hereditary kidney disease which typically has a progressive course (Alport 1927). Several extrarenal symptoms such as inner ear hearing loss and a specific eye disease known as „Lenticonus“ can also occur in the course of Alport syndrome.
Mutations in either the α3-, α4- or α5- chain of collagen type IV are responsible for this disease. These mutations cause a dysfunctional structure of the glomerular basal membrane which leads to hematuria and proteinuria in an early clinical setting. At a later stage of this disease the kidneys become fibrotic which eventually ends up in renal failure (Gunwar et al. 1998). In order to postpone this event ACE inhibitors are recommended as treatment but not yet officially approved.
The aim of this study was to collect and analyze data from patients suffering from Alport syndrome (n=285) focussing on different prognostic parameters. The patient cohort was representative in the distribution of affected chromosomes (83% X-chromosomal, 17% autosomal). 178 of 285 patients received an ACE inhibitor treatment before starting with dialysis. 111 patients suffered from renal insufficiency when this study started. Additionally, 53 of these patients had received a kidney transplantation already.
A median survival probability could not be determined due to a survival of more than 50% of all patients in this cohort. However the life expectation of patients with Alport syndrome was significantly improved following ACE inhibitor treatment (p<0.05). The average life expectation of patients who passed away (n=6) and did not receive an ACE inhibitor treatment was 41 years. The median life expectation in this group was 55 years. It was impossible to calculate a median life expectation for the collective that has received ACE inhibitor treatment since no patient has died in this group.
The median age when starting with dialysis treatment could be singnificantly postponed following ACE inhibitor treatment. (p<0.0001). In more detail, it was 35 years in the group with ACE inhibitor treatment and 22 when not receiving ACE inhibitors. Therefore, ACE inhibitor treatment could extend the time to dialysis for 13 years.
In addition, this study demonstrates that patients getting ACE inhibitors prior to dialysis treatment showed a longer survival of the kidney transplant. The average age at transplantation was 25 years. The median transplant survival of Alport syndrome patients not receiving ACE inhibitors before dialysis was 19 years, 7 transplants failed in this group.
The transplant survival until failure ranged from 2,5 until 19 years. A median survival in the group of patients with ACE inhibitor treatment could not be determined because more than 50% of the transplants were still working when this study was carried out. In this group only one transplant failed in total. A significant difference in terms of transplant survival between patients reveiving ACE inhibitor treatment compared with no treatment could not be detected but a tendency towards a benefit for the treated group was present.
The results of this study base on the analysis of the European Alportregister. As demonstrated in previous studies, this study provides additional data towards a benefit for an ACE inhibitor treatment in Alport syndrome patients. Therefore, ACE inhibitors should be officially approved in Alport syndrome in the near future. | de |
| dc.contributor.coReferee | Schön, Margarete Prof. Dr. | |
| dc.subject.ger | ACE-Hemmer | de |
| dc.subject.ger | Alport-Syndrom | de |
| dc.subject.ger | Nierenersatztherapie | de |
| dc.subject.eng | alport syndrome | de |
| dc.subject.eng | renal replacement therapy | de |
| dc.subject.eng | angiotensin-converting enzyme inhibition | de |
| dc.identifier.urn | urn:nbn:de:gbv:7-11858/00-1735-0000-0022-5D88-7-4 | |
| dc.affiliation.institute | Medizinische Fakultät | de |
| dc.subject.gokfull | GOK-MEDIZIN | de |
| dc.description.embargoed | 2015-01-28 | |
| dc.identifier.ppn | 815825781 | |