Magnetic resonance imaging of renal involvement in genetically studied patients with tuberous sclerosis complex

Abstract Purpose To evaluate renal involvement in tuberous sclerosis complex (TSC). Materials and methods A series of 24 TSC patients (19 with genetically demonstrated disease), underwent abdominal 1.0-T MR imaging with axial/coronal T1- and T2-weighted scans, with/without fat saturation. We looked...

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Veröffentlicht in:European journal of radiology 2009-11, Vol.72 (2), p.335-341
Hauptverfasser: Pompili, Giovanni, Zirpoli, Salvatore, Sala, Chiara, Flor, Nicola, Alfano, Rosa Maria, Volpi, Angela, Brancaccio, Diego, Sardanelli, Francesco, Cornalba, Gianpaolo
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container_end_page 341
container_issue 2
container_start_page 335
container_title European journal of radiology
container_volume 72
creator Pompili, Giovanni
Zirpoli, Salvatore
Sala, Chiara
Flor, Nicola
Alfano, Rosa Maria
Volpi, Angela
Brancaccio, Diego
Sardanelli, Francesco
Cornalba, Gianpaolo
description Abstract Purpose To evaluate renal involvement in tuberous sclerosis complex (TSC). Materials and methods A series of 24 TSC patients (19 with genetically demonstrated disease), underwent abdominal 1.0-T MR imaging with axial/coronal T1- and T2-weighted scans, with/without fat saturation. We looked for angyomiolipomas (AMLs) and cysts in 47 kidneys of 24 patients. We evaluated the percentage of parenchymal involvement by manual contouring on the coronal scans in 39 kidneys of 20 patients. Results We detected AMLs in 15/24 (63%) patients and in 27/47 (57%) kidneys, cysts in 14/24 (58%) and in 26/47 (55%); respectively. AMLs were found in 2/4 TSC1 and in 11/15 TSC2 patients, cysts were found in 2/4 TSC1 and in 9/15 TSC2 patients. The global renal involvement ranged from 0 to 32% (median, 18%) in TSC1 and from 0 to 100% (median, 39%) in TSC2 patients. A fair correlation ( r = .464) was found between patient’s age and renal involvement, a good correlation ( r = 0.655) between renal involvement and creatinine clearance. Conclusion Renal parenchyma of TSC patients can be evaluated with MR imaging. A detectable renal involvement was found in 83% of patients, higher in TSC2 than in TSC1. Renal function seems to correlate with renal involvement quantified with MR imaging.
doi_str_mv 10.1016/j.ejrad.2008.07.046
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Materials and methods A series of 24 TSC patients (19 with genetically demonstrated disease), underwent abdominal 1.0-T MR imaging with axial/coronal T1- and T2-weighted scans, with/without fat saturation. We looked for angyomiolipomas (AMLs) and cysts in 47 kidneys of 24 patients. We evaluated the percentage of parenchymal involvement by manual contouring on the coronal scans in 39 kidneys of 20 patients. Results We detected AMLs in 15/24 (63%) patients and in 27/47 (57%) kidneys, cysts in 14/24 (58%) and in 26/47 (55%); respectively. AMLs were found in 2/4 TSC1 and in 11/15 TSC2 patients, cysts were found in 2/4 TSC1 and in 9/15 TSC2 patients. The global renal involvement ranged from 0 to 32% (median, 18%) in TSC1 and from 0 to 100% (median, 39%) in TSC2 patients. A fair correlation ( r = .464) was found between patient’s age and renal involvement, a good correlation ( r = 0.655) between renal involvement and creatinine clearance. Conclusion Renal parenchyma of TSC patients can be evaluated with MR imaging. A detectable renal involvement was found in 83% of patients, higher in TSC2 than in TSC1. Renal function seems to correlate with renal involvement quantified with MR imaging.</description><identifier>ISSN: 0720-048X</identifier><identifier>EISSN: 1872-7727</identifier><identifier>DOI: 10.1016/j.ejrad.2008.07.046</identifier><identifier>PMID: 18835118</identifier><identifier>CODEN: EJRADR</identifier><language>eng</language><publisher>Amsterdam: Elsevier Ireland Ltd</publisher><subject>Adolescent ; Adult ; Angiomiolipoma ; Angiomyolipoma - complications ; Angiomyolipoma - diagnosis ; Angiomyolipoma - genetics ; Biological and medical sciences ; Child ; Child, Preschool ; Female ; Humans ; Investigative techniques, diagnostic techniques (general aspects) ; Kidney ; Liver Neoplasms - complications ; Liver Neoplasms - diagnosis ; Liver Neoplasms - genetics ; Magnetic Resonance Imaging - methods ; Male ; Medical sciences ; Middle Aged ; MRI ; Neurology ; Radiology ; Tuberous sclerosis ; Tuberous Sclerosis - complications ; Tuberous Sclerosis - diagnosis ; Tuberous Sclerosis - genetics ; Tumors of the nervous system. 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Materials and methods A series of 24 TSC patients (19 with genetically demonstrated disease), underwent abdominal 1.0-T MR imaging with axial/coronal T1- and T2-weighted scans, with/without fat saturation. We looked for angyomiolipomas (AMLs) and cysts in 47 kidneys of 24 patients. We evaluated the percentage of parenchymal involvement by manual contouring on the coronal scans in 39 kidneys of 20 patients. Results We detected AMLs in 15/24 (63%) patients and in 27/47 (57%) kidneys, cysts in 14/24 (58%) and in 26/47 (55%); respectively. AMLs were found in 2/4 TSC1 and in 11/15 TSC2 patients, cysts were found in 2/4 TSC1 and in 9/15 TSC2 patients. The global renal involvement ranged from 0 to 32% (median, 18%) in TSC1 and from 0 to 100% (median, 39%) in TSC2 patients. A fair correlation ( r = .464) was found between patient’s age and renal involvement, a good correlation ( r = 0.655) between renal involvement and creatinine clearance. Conclusion Renal parenchyma of TSC patients can be evaluated with MR imaging. A detectable renal involvement was found in 83% of patients, higher in TSC2 than in TSC1. Renal function seems to correlate with renal involvement quantified with MR imaging.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Angiomiolipoma</subject><subject>Angiomyolipoma - complications</subject><subject>Angiomyolipoma - diagnosis</subject><subject>Angiomyolipoma - genetics</subject><subject>Biological and medical sciences</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Female</subject><subject>Humans</subject><subject>Investigative techniques, diagnostic techniques (general aspects)</subject><subject>Kidney</subject><subject>Liver Neoplasms - complications</subject><subject>Liver Neoplasms - diagnosis</subject><subject>Liver Neoplasms - genetics</subject><subject>Magnetic Resonance Imaging - methods</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Middle Aged</subject><subject>MRI</subject><subject>Neurology</subject><subject>Radiology</subject><subject>Tuberous sclerosis</subject><subject>Tuberous Sclerosis - complications</subject><subject>Tuberous Sclerosis - diagnosis</subject><subject>Tuberous Sclerosis - genetics</subject><subject>Tumors of the nervous system. 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Phacomatoses</topic><topic>Young Adult</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Pompili, Giovanni</creatorcontrib><creatorcontrib>Zirpoli, Salvatore</creatorcontrib><creatorcontrib>Sala, Chiara</creatorcontrib><creatorcontrib>Flor, Nicola</creatorcontrib><creatorcontrib>Alfano, Rosa Maria</creatorcontrib><creatorcontrib>Volpi, Angela</creatorcontrib><creatorcontrib>Brancaccio, Diego</creatorcontrib><creatorcontrib>Sardanelli, Francesco</creatorcontrib><creatorcontrib>Cornalba, Gianpaolo</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>European journal of radiology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Pompili, Giovanni</au><au>Zirpoli, Salvatore</au><au>Sala, Chiara</au><au>Flor, Nicola</au><au>Alfano, Rosa Maria</au><au>Volpi, Angela</au><au>Brancaccio, Diego</au><au>Sardanelli, Francesco</au><au>Cornalba, Gianpaolo</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Magnetic resonance imaging of renal involvement in genetically studied patients with tuberous sclerosis complex</atitle><jtitle>European journal of radiology</jtitle><addtitle>Eur J Radiol</addtitle><date>2009-11-01</date><risdate>2009</risdate><volume>72</volume><issue>2</issue><spage>335</spage><epage>341</epage><pages>335-341</pages><issn>0720-048X</issn><eissn>1872-7727</eissn><coden>EJRADR</coden><abstract>Abstract Purpose To evaluate renal involvement in tuberous sclerosis complex (TSC). Materials and methods A series of 24 TSC patients (19 with genetically demonstrated disease), underwent abdominal 1.0-T MR imaging with axial/coronal T1- and T2-weighted scans, with/without fat saturation. We looked for angyomiolipomas (AMLs) and cysts in 47 kidneys of 24 patients. We evaluated the percentage of parenchymal involvement by manual contouring on the coronal scans in 39 kidneys of 20 patients. Results We detected AMLs in 15/24 (63%) patients and in 27/47 (57%) kidneys, cysts in 14/24 (58%) and in 26/47 (55%); respectively. AMLs were found in 2/4 TSC1 and in 11/15 TSC2 patients, cysts were found in 2/4 TSC1 and in 9/15 TSC2 patients. The global renal involvement ranged from 0 to 32% (median, 18%) in TSC1 and from 0 to 100% (median, 39%) in TSC2 patients. A fair correlation ( r = .464) was found between patient’s age and renal involvement, a good correlation ( r = 0.655) between renal involvement and creatinine clearance. Conclusion Renal parenchyma of TSC patients can be evaluated with MR imaging. A detectable renal involvement was found in 83% of patients, higher in TSC2 than in TSC1. Renal function seems to correlate with renal involvement quantified with MR imaging.</abstract><cop>Amsterdam</cop><pub>Elsevier Ireland Ltd</pub><pmid>18835118</pmid><doi>10.1016/j.ejrad.2008.07.046</doi><tpages>7</tpages></addata></record>
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subjects Adolescent
Adult
Angiomiolipoma
Angiomyolipoma - complications
Angiomyolipoma - diagnosis
Angiomyolipoma - genetics
Biological and medical sciences
Child
Child, Preschool
Female
Humans
Investigative techniques, diagnostic techniques (general aspects)
Kidney
Liver Neoplasms - complications
Liver Neoplasms - diagnosis
Liver Neoplasms - genetics
Magnetic Resonance Imaging - methods
Male
Medical sciences
Middle Aged
MRI
Neurology
Radiology
Tuberous sclerosis
Tuberous Sclerosis - complications
Tuberous Sclerosis - diagnosis
Tuberous Sclerosis - genetics
Tumors of the nervous system. Phacomatoses
Young Adult
title Magnetic resonance imaging of renal involvement in genetically studied patients with tuberous sclerosis complex
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