Severe Renal Osteodystrophy in a Pediatric Patient With End-Stage Renal Disease: Sagliker Syndrome?
Renal osteodystrophy (ROD) is a multifactorial disorder of bone metabolism in chronic kidney disease (CKD). As CKD progresses, ensuing abnormalities in vitamin D metabolism and parathyroid hormone (PTH) secretion result in distortions in trabecular microarchitecture, thinning of the cortical shell,...
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Veröffentlicht in: | Journal of renal nutrition 2013-07, Vol.23 (4), p.326-330 |
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container_title | Journal of renal nutrition |
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creator | Yavascan, Onder, MD Kose, Engin, MD Alparslan, Caner, MD Sirin Kose, Seda, MD Bal, Alkan, MD Kanik, Ali, MD Aksu, Nejat, MD |
description | Renal osteodystrophy (ROD) is a multifactorial disorder of bone metabolism in chronic kidney disease (CKD). As CKD progresses, ensuing abnormalities in vitamin D metabolism and parathyroid hormone (PTH) secretion result in distortions in trabecular microarchitecture, thinning of the cortical shell, and increased cortical porosity. The recently described Sagliker syndrome (SS) might be an exaggerated version of ROD and is a very striking and prominent feature of secondary hyperparathyroidism in patients with end-stage renal disease (ESRD). It includes a distorted facial appearance, short stature, extremely severe maxillary and mandibulary changes, soft tissue tumors in the mouth, teeth/dental abnormalities, fingertip changes, knee and scapula deformities, hearing abnormalities, and neurologic and psychological problems. We herein describe an affected 14-year-old girl with severe ROD resulting from ESRD, who had severe peripheral and central neurologic problems caused by bone deformities, mimicking the features of Sagliker syndrome. |
doi_str_mv | 10.1053/j.jrn.2012.07.002 |
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As CKD progresses, ensuing abnormalities in vitamin D metabolism and parathyroid hormone (PTH) secretion result in distortions in trabecular microarchitecture, thinning of the cortical shell, and increased cortical porosity. The recently described Sagliker syndrome (SS) might be an exaggerated version of ROD and is a very striking and prominent feature of secondary hyperparathyroidism in patients with end-stage renal disease (ESRD). It includes a distorted facial appearance, short stature, extremely severe maxillary and mandibulary changes, soft tissue tumors in the mouth, teeth/dental abnormalities, fingertip changes, knee and scapula deformities, hearing abnormalities, and neurologic and psychological problems. We herein describe an affected 14-year-old girl with severe ROD resulting from ESRD, who had severe peripheral and central neurologic problems caused by bone deformities, mimicking the features of Sagliker syndrome.</description><identifier>ISSN: 1051-2276</identifier><identifier>EISSN: 1532-8503</identifier><identifier>DOI: 10.1053/j.jrn.2012.07.002</identifier><identifier>PMID: 23026501</identifier><language>eng</language><publisher>United States: Elsevier Inc</publisher><subject>Adolescent ; Bone Diseases - complications ; Bone Diseases - pathology ; Chronic Kidney Disease-Mineral and Bone Disorder - complications ; Chronic Kidney Disease-Mineral and Bone Disorder - physiopathology ; Female ; Humans ; Hyperparathyroidism, Secondary - complications ; Hyperparathyroidism, Secondary - pathology ; Kidney Failure, Chronic - complications ; Kidney Failure, Chronic - physiopathology ; Nephrology ; Syndrome</subject><ispartof>Journal of renal nutrition, 2013-07, Vol.23 (4), p.326-330</ispartof><rights>National Kidney Foundation, Inc.</rights><rights>2013 National Kidney Foundation, Inc.</rights><rights>Copyright © 2013 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c408t-5d113945d560b20e5e87d73aa279e246446d59e972bcaeaa59dd46523b5f510c3</citedby><cites>FETCH-LOGICAL-c408t-5d113945d560b20e5e87d73aa279e246446d59e972bcaeaa59dd46523b5f510c3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1053/j.jrn.2012.07.002$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,780,784,3548,27922,27923,45993</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/23026501$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Yavascan, Onder, MD</creatorcontrib><creatorcontrib>Kose, Engin, MD</creatorcontrib><creatorcontrib>Alparslan, Caner, MD</creatorcontrib><creatorcontrib>Sirin Kose, Seda, MD</creatorcontrib><creatorcontrib>Bal, Alkan, MD</creatorcontrib><creatorcontrib>Kanik, Ali, MD</creatorcontrib><creatorcontrib>Aksu, Nejat, MD</creatorcontrib><title>Severe Renal Osteodystrophy in a Pediatric Patient With End-Stage Renal Disease: Sagliker Syndrome?</title><title>Journal of renal nutrition</title><addtitle>J Ren Nutr</addtitle><description>Renal osteodystrophy (ROD) is a multifactorial disorder of bone metabolism in chronic kidney disease (CKD). As CKD progresses, ensuing abnormalities in vitamin D metabolism and parathyroid hormone (PTH) secretion result in distortions in trabecular microarchitecture, thinning of the cortical shell, and increased cortical porosity. The recently described Sagliker syndrome (SS) might be an exaggerated version of ROD and is a very striking and prominent feature of secondary hyperparathyroidism in patients with end-stage renal disease (ESRD). It includes a distorted facial appearance, short stature, extremely severe maxillary and mandibulary changes, soft tissue tumors in the mouth, teeth/dental abnormalities, fingertip changes, knee and scapula deformities, hearing abnormalities, and neurologic and psychological problems. We herein describe an affected 14-year-old girl with severe ROD resulting from ESRD, who had severe peripheral and central neurologic problems caused by bone deformities, mimicking the features of Sagliker syndrome.</description><subject>Adolescent</subject><subject>Bone Diseases - complications</subject><subject>Bone Diseases - pathology</subject><subject>Chronic Kidney Disease-Mineral and Bone Disorder - complications</subject><subject>Chronic Kidney Disease-Mineral and Bone Disorder - physiopathology</subject><subject>Female</subject><subject>Humans</subject><subject>Hyperparathyroidism, Secondary - complications</subject><subject>Hyperparathyroidism, Secondary - pathology</subject><subject>Kidney Failure, Chronic - complications</subject><subject>Kidney Failure, Chronic - physiopathology</subject><subject>Nephrology</subject><subject>Syndrome</subject><issn>1051-2276</issn><issn>1532-8503</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2013</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kU1v1DAURS0EoqXwA9ggL9kkfbbz8gESCJUWkCq1IiCWlsd-0zrNJIPtqZR_j0fTsmDByl7cc6V3LmOvBZQCUJ0O5RCmUoKQJTQlgHzCjgUqWbQI6mn-A4pCyqY-Yi9iHACEwFY-Z0dSgawRxDGzPd1TIP6dJjPyq5hodktMYd7eLtxP3PBrct6k4C2_NsnTlPgvn275-eSKPpmbR_Szj2QiveO9uRn9HQXeL5ML84Y-vmTP1maM9OrhPWE_L85_nH0tLq--fDv7dFnYCtpUoBNCdRU6rGElgZDaxjXKGNl0JKu6qmqHHXWNXFlDxmDnXFWjVCtcowCrTtjbQ-82zL93FJPe-GhpHM1E8y5qoepOKmwqyFFxiNowxxhorbfBb0xYtAC9d6sHnd3qvVsNjc5uM_PmoX632pD7SzzKzIH3hwDlI-89BR1tFmazwEA2aTf7_9Z_-Ie2o5-8NeMdLRSHeRey53yFjpnR_X7c_bZC5l2xa9UfPj2drw</recordid><startdate>20130701</startdate><enddate>20130701</enddate><creator>Yavascan, Onder, MD</creator><creator>Kose, Engin, MD</creator><creator>Alparslan, Caner, MD</creator><creator>Sirin Kose, Seda, MD</creator><creator>Bal, Alkan, MD</creator><creator>Kanik, Ali, MD</creator><creator>Aksu, Nejat, MD</creator><general>Elsevier Inc</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>20130701</creationdate><title>Severe Renal Osteodystrophy in a Pediatric Patient With End-Stage Renal Disease: Sagliker Syndrome?</title><author>Yavascan, Onder, MD ; Kose, Engin, MD ; Alparslan, Caner, MD ; Sirin Kose, Seda, MD ; Bal, Alkan, MD ; Kanik, Ali, MD ; Aksu, Nejat, MD</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c408t-5d113945d560b20e5e87d73aa279e246446d59e972bcaeaa59dd46523b5f510c3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2013</creationdate><topic>Adolescent</topic><topic>Bone Diseases - complications</topic><topic>Bone Diseases - pathology</topic><topic>Chronic Kidney Disease-Mineral and Bone Disorder - complications</topic><topic>Chronic Kidney Disease-Mineral and Bone Disorder - physiopathology</topic><topic>Female</topic><topic>Humans</topic><topic>Hyperparathyroidism, Secondary - complications</topic><topic>Hyperparathyroidism, Secondary - pathology</topic><topic>Kidney Failure, Chronic - complications</topic><topic>Kidney Failure, Chronic - physiopathology</topic><topic>Nephrology</topic><topic>Syndrome</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Yavascan, Onder, MD</creatorcontrib><creatorcontrib>Kose, Engin, MD</creatorcontrib><creatorcontrib>Alparslan, Caner, MD</creatorcontrib><creatorcontrib>Sirin Kose, Seda, MD</creatorcontrib><creatorcontrib>Bal, Alkan, MD</creatorcontrib><creatorcontrib>Kanik, Ali, MD</creatorcontrib><creatorcontrib>Aksu, Nejat, MD</creatorcontrib><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>Journal of renal nutrition</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Yavascan, Onder, MD</au><au>Kose, Engin, MD</au><au>Alparslan, Caner, MD</au><au>Sirin Kose, Seda, MD</au><au>Bal, Alkan, MD</au><au>Kanik, Ali, MD</au><au>Aksu, Nejat, MD</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Severe Renal Osteodystrophy in a Pediatric Patient With End-Stage Renal Disease: Sagliker Syndrome?</atitle><jtitle>Journal of renal nutrition</jtitle><addtitle>J Ren Nutr</addtitle><date>2013-07-01</date><risdate>2013</risdate><volume>23</volume><issue>4</issue><spage>326</spage><epage>330</epage><pages>326-330</pages><issn>1051-2276</issn><eissn>1532-8503</eissn><abstract>Renal osteodystrophy (ROD) is a multifactorial disorder of bone metabolism in chronic kidney disease (CKD). As CKD progresses, ensuing abnormalities in vitamin D metabolism and parathyroid hormone (PTH) secretion result in distortions in trabecular microarchitecture, thinning of the cortical shell, and increased cortical porosity. The recently described Sagliker syndrome (SS) might be an exaggerated version of ROD and is a very striking and prominent feature of secondary hyperparathyroidism in patients with end-stage renal disease (ESRD). It includes a distorted facial appearance, short stature, extremely severe maxillary and mandibulary changes, soft tissue tumors in the mouth, teeth/dental abnormalities, fingertip changes, knee and scapula deformities, hearing abnormalities, and neurologic and psychological problems. We herein describe an affected 14-year-old girl with severe ROD resulting from ESRD, who had severe peripheral and central neurologic problems caused by bone deformities, mimicking the features of Sagliker syndrome.</abstract><cop>United States</cop><pub>Elsevier Inc</pub><pmid>23026501</pmid><doi>10.1053/j.jrn.2012.07.002</doi><tpages>5</tpages></addata></record> |
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subjects | Adolescent Bone Diseases - complications Bone Diseases - pathology Chronic Kidney Disease-Mineral and Bone Disorder - complications Chronic Kidney Disease-Mineral and Bone Disorder - physiopathology Female Humans Hyperparathyroidism, Secondary - complications Hyperparathyroidism, Secondary - pathology Kidney Failure, Chronic - complications Kidney Failure, Chronic - physiopathology Nephrology Syndrome |
title | Severe Renal Osteodystrophy in a Pediatric Patient With End-Stage Renal Disease: Sagliker Syndrome? |
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