Представлены оценки максимальных температур источников как внешней, так и собственной засветки, при которых будут иметь место искажения изображений, формируемых посредствам активных оптических систем. Показана возможность использования высокочастотного CuBr-лазера в качестве усилителя яркости лазерного монитора

Azurmendi, Pablo J.

Bacher, Janina

Burchardi, Christian

Eng, Charis

Gläsker, Sven

Hoffmann, Michael M.

Ivanovas, Vera

Jilg, Cordula

Krämer-Guth, Annette

Mader, Irina

Malinoc, Angelica

Martin, Rodolfo S.

Nabulsi, Zinaida

Neumann, Hartmut P. H.

Ortiz Bruechle, Nadine

Riegler, Peter

Schaeffner, Elke

Zerres, Klaus

Вестник Науки Сибири

PubMed

Background Patients who harbor intracranial aneurysms (IAs) run a risk for aneurysm rupture and subsequent subarachnoid hemorrhage which frequently results in permanent deficits or death. Prophylactic treatment of unruptured aneurysms is possible and recommended depending on the size and location of the aneurysm as well as patient age and condition. IAs are major manifestations of autosomal dominant polycystic kidney disease (ADPKD). Current guidelines do not suggest surveillance of IAs in ADPKD except in the setting of family history if IA was known in any relative with ADPKD. Management of IAs in ADPKD is problematic because limited data exist from large studies. Methods We established the Else Kröner-Fresenius Registry for ADPKD in Germany. Clinical data were assessed for age at diagnosis of IAs, stage of renal insufficiency, and number, location and size of IAs as well as family history of cerebral events. Patients with symptomatic or asymptomatic IAs were included. All patients with ADPKD-related IAs were offered mutation scanning of the susceptibility genes for ADPKD, the PKD1 and PKD2 genes. Results Of 463 eligible ADPKD patients from the population base of Germany, 32 (7%) were found to have IAs, diagnosed at the age of 2?71 years, 19 females and 13 males. Twenty (63%) of these 32 patients were symptomatic, whereas IAs were detected in an asymptomatic stage in 12 patients. IAs were multifocal in 12 and unifocal in 20 patients. In 26 patients (81%), IAs were diagnosed before end-stage renal failure. Twenty-five out of 27 unrelated index cases (93%) had no IAs or cerebral events documented in their relatives with ADPKD. In 16 unrelated index patients and 3 relatives, we detected germline mutations. The mutations were randomly distributed across the PKD1 gene in 14 and the PKD2 gene in 2 index cases. Questionnaires answered for 320/441 ADPKD patients without IAs revealed that only 45/320 (14%) had MR angiography. Conclusion In ADPKD, rupture of IAs occurs frequently before the start of dialysis, is only infrequently associated with a family history of IAs or subarachnoid hemorrhage, and is associated with mutations either of the PKD1 or the PKD2 gene of any type. Screening for IAs is widely insufficiently performed, should not be restricted to families with a history of cerebral events and should be started before end-stage renal failure.Fil: Neumann, Hartmut P. H.. No especifíca;Fil: Malinoc, Angelica. No especifíca;Fil: Bacher, Janina. No especifíca;Fil: Nabulsi, Zinaida. No especifíca;Fil: Ivanovas, Vera. No especifíca;Fil: Ortiz Bruechle, Nadine. No especifíca;Fil: Mader, Irina. No especifíca;Fil: Hoffmann, Michael M.. No especifíca;Fil: Riegler, Peter. No especifíca;Fil: Kraemer Guth, Annette. No especifíca;Fil: Burchard, Christian. No especifíca;Fil: Schaeffner, Elke. No especifíca;Fil: Martin, Rodolfo Santiago. Consejo Nacional de Investigaciones Científicas y Técnicas; ArgentinaFil: Azurmendi, Pablo Javier. Consejo Nacional de Investigaciones Científicas y Técnicas; ArgentinaFil: Zerres, Klaus. No especifíca;Fil: Jilg, Cordula. No especifíca;Fil: Eng, Charis. No especifíca;Fil: Gläsker, Sven. No especifíca

Kraemer Guth, Annette

Burchard, Christian

Martin, Rodolfo Santiago

Azurmendi, Pablo Javier

LAReferencia - Red Federada de Repositorios Institucionales de Publicaciones Científicas Latinoamericanas

Cerebrovascular Diseases

English

Characteristics of intracranial aneurysms in the else kröner-fresenius registry of autosomal dominant polycystic kidney disease

CONICET Digital

Торгаев, Станислав Николаевич

Чертихина, Дарья Сергеевна

Шаклунов, Александр Александрович

Electronic archive of Tomsk Polytechnic University

Возможность использования высокочастотного CuBr-лазера для создания скоростного лазерного монитора

RWTH Publications

Characteristics of intracranial aneurysms in the else Kröner-Fresenius registry of autosomal dominant polycystic kidney disease

Publikationsserver der RWTH Aachen University

Background: Patients who harbor intracranial aneurysms (IAs) run a risk for aneurysm rupture and subsequent subarachnoid hemorrhage which frequently results in permanent deficits or death. Prophylactic treatment of unruptured aneurysms is possible and recommended depending on the size and location of the aneurysm as well as patient age and condition. IAs are major manifestations of autosomal dominant polycystic kidney disease (ADPKD). Current guidelines do not suggest surveillance of IAs in ADPKD except in the setting of family history if IA was known in any relative with ADPKD. Management of IAs in ADPKD is problematic because limited data exist from large studies. Methods: We established the Else Kröner-Fresenius Registry for ADPKD in Germany. Clinical data were assessed for age at diagnosis of IAs, stage of renal insufficiency, and number, location and size of IAs as well as family history of cerebral events. Patients with symptomatic or asymptomatic IAs were included. All patients with ADPKD-related IAs were offered mutation scanning of the susceptibility genes for ADPKD, the PKD1 and PKD2 genes. Results: Of 463 eligible ADPKD patients from the population base of Germany, 32 (7%) were found to have IAs, diagnosed at the age of 2–71 years, 19 females and 13 males. Twenty (63%) of these 32 patients were symptomatic, whereas IAs were detected in an asymptomatic stage in 12 patients. IAs were multifocal in 12 and unifocal in 20 patients. In 26 patients (81%), IAs were diagnosed before end-stage renal failure. Twenty-five out of 27 unrelated index cases (93%) had no IAs or cerebral events documented in their relatives with ADPKD. In 16 unrelated index patients and 3 relatives, we detected germline mutations. The mutations were randomly distributed across the PKD1 gene in 14 and the PKD2 gene in 2 index cases. Questionnaires answered for 320/441 ADPKD patients without IAs revealed that only 45/320 (14%) had MR angiography. Conclusion: In ADPKD, rupture of IAs occurs frequently before the start of dialysis, is only infrequently associated with a family history of IAs or subarachnoid hemorrhage, and is associated with mutations either of the PKD1 or the PKD2 gene of any type. Screening for IAs is widely insufficiently performed, should not be restricted to families with a history of cerebral events and should be started before end-stage renal failure

Hartmut P.H. Neumann

Angelica Malinoc

Janina Bacher

Zinaida Nabulsi

Vera Ivanovas

Nadine Ortiz Bruechle

Irina Mader

Michael M. Hoffmann

Peter Riegler

Annette Kraemer-Guth

Christian Burchardi

Elke Schaeffner

Rodolfo S. Martin

Pablo J. Azurmendi

Klaus Zerres

Cordula Jilg

Charis Eng

Sven Gläsker

Directory of Open Access Journals

Cerebrovascular Diseases Extra

Characteristics of Intracranial Aneurysms in the Else Kröner-Fresenius Registry of Autosomal Dominant Polycystic Kidney Disease

&lt;b&gt;&lt;i&gt;Background:&lt;/i&gt;&lt;/b&gt; Patients who harbor intracranial aneurysms (IAs) run a risk for aneurysm rupture and subsequent subarachnoid hemorrhage which frequently results in permanent deficits or death. Prophylactic treatment of unruptured aneurysms is possible and recommended depending on the size and location of the aneurysm as well as patient age and condition. IAs are major manifestations of autosomal dominant polycystic kidney disease (ADPKD). Current guidelines do not suggest surveillance of IAs in ADPKD except in the setting of family history if IA was known in any relative with ADPKD. Management of IAs in ADPKD is problematic because limited data exist from large studies. &lt;b&gt;&lt;i&gt;Methods:&lt;/i&gt;&lt;/b&gt; We established the Else Kröner-Fresenius Registry for ADPKD in Germany. Clinical data were assessed for age at diagnosis of IAs, stage of renal insufficiency, and number, location and size of IAs as well as family history of cerebral events. Patients with symptomatic or asymptomatic IAs were included. All patients with ADPKD-related IAs were offered mutation scanning of the susceptibility genes for ADPKD, the &lt;i&gt;PKD1&lt;/i&gt; and &lt;i&gt;PKD2 &lt;/i&gt;genes. &lt;b&gt;&lt;i&gt;Results:&lt;/i&gt;&lt;/b&gt; Of 463 eligible ADPKD patients from the population base of Germany, 32 (7%) were found to have IAs, diagnosed at the age of 2–71 years, 19 females and 13 males. Twenty (63%) of these 32 patients were symptomatic, whereas IAs were detected in an asymptomatic stage in 12 patients. IAs were multifocal in 12 and unifocal in 20 patients. In 26 patients (81%), IAs were diagnosed before end-stage renal failure. Twenty-five out of 27 unrelated index cases (93%) had no IAs or cerebral events documented in their relatives with ADPKD. In 16 unrelated index patients and 3 relatives, we detected germline mutations. The mutations were randomly distributed across the &lt;i&gt;PKD1&lt;/i&gt; gene in 14 and the &lt;i&gt;PKD2&lt;/i&gt; gene in 2 index cases. Questionnaires answered for 320/441 ADPKD patients without IAs revealed that only 45/320 (14%) had MR angiography. &lt;b&gt;&lt;i&gt;Conclusion:&lt;/i&gt;&lt;/b&gt; In ADPKD, rupture of IAs occurs frequently before the start of dialysis, is only infrequently associated with a family history of IAs or subarachnoid hemorrhage, and is associated with mutations either of the &lt;i&gt;PKD1&lt;/i&gt; or the &lt;i&gt;PKD2&lt;/i&gt; gene of any type. Screening for IAs is widely insufficiently performed, should not be restricted to families with a history of cerebral events and should be started before end-stage renal failure.</jats:p

Crossref

AA: ASEdb: a database of alanine mutations and their effects on the free energy of binding in protein interactions. Bioinformatics

Amino acid difference formula to help explain protein evolution. Science

Autosomal dominant polycystic kidney disease.

Autosomal dominant polycystic kidney disease. Lancet

CS: Location and size of ruptured intracranial aneurysm and serious clinical outcomes early after subarachnoid hemorrhage: a population-based study in Australasia. Cerebrovasc Dis

GJ: Optimal screening strategy for familial intracranial aneurysms: a cost-effectiveness analysis. Neurology

Henikoff S: Predicting the effects of amino acid substitutions on protein function. Annu Rev Genomics Hum Genet

Jilg C: Adult patients with sporadic polycystic kidney disease: the importance of screening for mutations in the PKD1 and PKD2 genes. Int Urol Nephrol 2012, E-pub ahead of print.

MH: Screening for intracranial aneurysm in 355 patients with autosomaldominant polycystic kidney disease. Stroke

MutationTaster evaluates disease-causing potential of sequence alterations.

Natural history, epidemiology and screening of unruptured intracranial aneurysms. Rev Neurol (Paris)

PC: A complete mutation screen of the ADPKD genes by DHPLC. Kidney Int

Polycystic kidney disease, autosomal dominant; in

Practical decision-making in the treatment of unruptured cerebral aneurysm in Japan: the U-CARE study. Cerebrovasc Dis

Rinkel GJ: Risk of aneurysm rupture at intracranial arterial bifurcations. Cerebrovasc Dis

Sunyaev S: Human non-synonymous SNPs: server and survey.

Surgical risk as related to time of intervention in the repair of intracranial aneurysms.

Unruptured intracranial aneurysms – risk of rupture and risks of surgical intervention.

VE: Extended follow-up of unruptured intracranial aneurysms detected by presymptomatic screening in patients with autosomal dominant polycystic kidney disease.

Zerres K: Multiple intracranial aneurysms in a patient with autosomal recessive polycystic kidney disease. Nephrol Dial Transplant

Возможность использования высокочастотного CuBr-лазера для создания скоростного лазерного монитора

Abstract

Similar works

Full text

Available Versions