Chest configuration in children and adolescents with infantile nephropathic cystinosis compared with other chronic kidney disease entities and its clinical determinants

Background Infantile nephropathic cystinosis (INC) is a systemic lysosomal storage disease causing intracellular cystine accumulation, resulting in renal Fanconi syndrome, progressive kidney disease (CKD), rickets, malnutrition, and myopathy. An INC-specific disproportionately diminished trunk lengt...

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Veröffentlicht in:Pediatric nephrology (Berlin, West) West), 2023-12, Vol.38 (12), p.3989-3999
Hauptverfasser: Müller, Sophia, Kluck, Rika, Jagodzinski, Celina, Brügelmann, Malina, Hohenfellner, Katharina, Büscher, Anja, Kemper, Markus J., Fröde, Kerstin, Oh, Jun, Billing, Heiko, Thumfart, Julia, Weber, Lutz T., Acham-Roschitz, Birgit, Arbeiter, Klaus, Tönshoff, Burkhard, Hagenberg, Martina, Pavičić, Leo, Haffner, Dieter, Zivicnjak, Miroslav
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container_end_page 3999
container_issue 12
container_start_page 3989
container_title Pediatric nephrology (Berlin, West)
container_volume 38
creator Müller, Sophia
Kluck, Rika
Jagodzinski, Celina
Brügelmann, Malina
Hohenfellner, Katharina
Büscher, Anja
Kemper, Markus J.
Fröde, Kerstin
Oh, Jun
Billing, Heiko
Thumfart, Julia
Weber, Lutz T.
Acham-Roschitz, Birgit
Arbeiter, Klaus
Tönshoff, Burkhard
Hagenberg, Martina
Pavičić, Leo
Haffner, Dieter
Zivicnjak, Miroslav
description Background Infantile nephropathic cystinosis (INC) is a systemic lysosomal storage disease causing intracellular cystine accumulation, resulting in renal Fanconi syndrome, progressive kidney disease (CKD), rickets, malnutrition, and myopathy. An INC-specific disproportionately diminished trunk length compared to leg length poses questions regarding the functionality of the trunk. Methods Thus, we prospectively investigated thoracic dimensions and proportions, as well as their clinical determinants in 44 pediatric patients with INC with CKD stages 1–5 and 97 age-matched patients with CKD of other etiology between the ages of 2–17 years. A total of 92 and 221 annual measurements of patients with INC and CKD, respectively, were performed, and associations between anthropometric and clinical parameters were assessed using linear mixed-effects models. Results Patients with INC exhibited altered chest dimensions that were distinct from CKD controls, characterized by markedly increased chest depth to height and chest depth to chest width ratio z -scores (> 1.0), while those of patients with CKD were only mildly affected ( z -score within ± 1.0). Ratio z -scores differed significantly between both patient groups from 2–6 years of age onward. The degree of chest disproportion in INC patients was significantly associated with both the degree of CKD and tubular dysfunction (e.g., low serum phosphate and bicarbonate) across three different age groups (2–6, 7–12, and 13–17 years). Conclusion Our data show an INC-specific alteration in thoracic shape from early childhood onward, which is distinct from CKD of other etiologies, suggesting early childhood subclinical changes of the musculoskeletal unit of the thoracic cage, which are associated with kidney function. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information
doi_str_mv 10.1007/s00467-023-06058-x
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An INC-specific disproportionately diminished trunk length compared to leg length poses questions regarding the functionality of the trunk. Methods Thus, we prospectively investigated thoracic dimensions and proportions, as well as their clinical determinants in 44 pediatric patients with INC with CKD stages 1–5 and 97 age-matched patients with CKD of other etiology between the ages of 2–17 years. A total of 92 and 221 annual measurements of patients with INC and CKD, respectively, were performed, and associations between anthropometric and clinical parameters were assessed using linear mixed-effects models. Results Patients with INC exhibited altered chest dimensions that were distinct from CKD controls, characterized by markedly increased chest depth to height and chest depth to chest width ratio z -scores (&gt; 1.0), while those of patients with CKD were only mildly affected ( z -score within ± 1.0). Ratio z -scores differed significantly between both patient groups from 2–6 years of age onward. The degree of chest disproportion in INC patients was significantly associated with both the degree of CKD and tubular dysfunction (e.g., low serum phosphate and bicarbonate) across three different age groups (2–6, 7–12, and 13–17 years). Conclusion Our data show an INC-specific alteration in thoracic shape from early childhood onward, which is distinct from CKD of other etiologies, suggesting early childhood subclinical changes of the musculoskeletal unit of the thoracic cage, which are associated with kidney function. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information</description><identifier>ISSN: 0931-041X</identifier><identifier>EISSN: 1432-198X</identifier><identifier>DOI: 10.1007/s00467-023-06058-x</identifier><identifier>PMID: 37415042</identifier><language>eng</language><publisher>Berlin/Heidelberg: Springer Berlin Heidelberg</publisher><subject>Body measurements ; Chest ; Children ; Childrens health ; Cystinosis ; Fanconi syndrome ; Kidney diseases ; Lysosomal storage diseases ; Malnutrition ; Medicine ; Medicine &amp; Public Health ; Myopathy ; Nephrology ; Original ; Original Article ; Patients ; Pediatrics ; Rickets ; Thorax ; Urology</subject><ispartof>Pediatric nephrology (Berlin, West), 2023-12, Vol.38 (12), p.3989-3999</ispartof><rights>The Author(s) 2023</rights><rights>2023. The Author(s).</rights><rights>The Author(s) 2023. This work is published under http://creativecommons.org/licenses/by/4.0/ (the “License”). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c475t-f6ef4d9a8761b47b57c18c3e4686a0a9ac151e1b93d49b6111a8f97887bd52943</citedby><cites>FETCH-LOGICAL-c475t-f6ef4d9a8761b47b57c18c3e4686a0a9ac151e1b93d49b6111a8f97887bd52943</cites><orcidid>0000-0001-9302-712X</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://link.springer.com/content/pdf/10.1007/s00467-023-06058-x$$EPDF$$P50$$Gspringer$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://link.springer.com/10.1007/s00467-023-06058-x$$EHTML$$P50$$Gspringer$$Hfree_for_read</linktohtml><link.rule.ids>230,315,782,786,887,27931,27932,41495,42564,51326</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/37415042$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Müller, Sophia</creatorcontrib><creatorcontrib>Kluck, Rika</creatorcontrib><creatorcontrib>Jagodzinski, Celina</creatorcontrib><creatorcontrib>Brügelmann, Malina</creatorcontrib><creatorcontrib>Hohenfellner, Katharina</creatorcontrib><creatorcontrib>Büscher, Anja</creatorcontrib><creatorcontrib>Kemper, Markus J.</creatorcontrib><creatorcontrib>Fröde, Kerstin</creatorcontrib><creatorcontrib>Oh, Jun</creatorcontrib><creatorcontrib>Billing, Heiko</creatorcontrib><creatorcontrib>Thumfart, Julia</creatorcontrib><creatorcontrib>Weber, Lutz T.</creatorcontrib><creatorcontrib>Acham-Roschitz, Birgit</creatorcontrib><creatorcontrib>Arbeiter, Klaus</creatorcontrib><creatorcontrib>Tönshoff, Burkhard</creatorcontrib><creatorcontrib>Hagenberg, Martina</creatorcontrib><creatorcontrib>Pavičić, Leo</creatorcontrib><creatorcontrib>Haffner, Dieter</creatorcontrib><creatorcontrib>Zivicnjak, Miroslav</creatorcontrib><title>Chest configuration in children and adolescents with infantile nephropathic cystinosis compared with other chronic kidney disease entities and its clinical determinants</title><title>Pediatric nephrology (Berlin, West)</title><addtitle>Pediatr Nephrol</addtitle><addtitle>Pediatr Nephrol</addtitle><description>Background Infantile nephropathic cystinosis (INC) is a systemic lysosomal storage disease causing intracellular cystine accumulation, resulting in renal Fanconi syndrome, progressive kidney disease (CKD), rickets, malnutrition, and myopathy. An INC-specific disproportionately diminished trunk length compared to leg length poses questions regarding the functionality of the trunk. Methods Thus, we prospectively investigated thoracic dimensions and proportions, as well as their clinical determinants in 44 pediatric patients with INC with CKD stages 1–5 and 97 age-matched patients with CKD of other etiology between the ages of 2–17 years. A total of 92 and 221 annual measurements of patients with INC and CKD, respectively, were performed, and associations between anthropometric and clinical parameters were assessed using linear mixed-effects models. Results Patients with INC exhibited altered chest dimensions that were distinct from CKD controls, characterized by markedly increased chest depth to height and chest depth to chest width ratio z -scores (&gt; 1.0), while those of patients with CKD were only mildly affected ( z -score within ± 1.0). Ratio z -scores differed significantly between both patient groups from 2–6 years of age onward. The degree of chest disproportion in INC patients was significantly associated with both the degree of CKD and tubular dysfunction (e.g., low serum phosphate and bicarbonate) across three different age groups (2–6, 7–12, and 13–17 years). Conclusion Our data show an INC-specific alteration in thoracic shape from early childhood onward, which is distinct from CKD of other etiologies, suggesting early childhood subclinical changes of the musculoskeletal unit of the thoracic cage, which are associated with kidney function. 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An INC-specific disproportionately diminished trunk length compared to leg length poses questions regarding the functionality of the trunk. Methods Thus, we prospectively investigated thoracic dimensions and proportions, as well as their clinical determinants in 44 pediatric patients with INC with CKD stages 1–5 and 97 age-matched patients with CKD of other etiology between the ages of 2–17 years. A total of 92 and 221 annual measurements of patients with INC and CKD, respectively, were performed, and associations between anthropometric and clinical parameters were assessed using linear mixed-effects models. Results Patients with INC exhibited altered chest dimensions that were distinct from CKD controls, characterized by markedly increased chest depth to height and chest depth to chest width ratio z -scores (&gt; 1.0), while those of patients with CKD were only mildly affected ( z -score within ± 1.0). Ratio z -scores differed significantly between both patient groups from 2–6 years of age onward. The degree of chest disproportion in INC patients was significantly associated with both the degree of CKD and tubular dysfunction (e.g., low serum phosphate and bicarbonate) across three different age groups (2–6, 7–12, and 13–17 years). Conclusion Our data show an INC-specific alteration in thoracic shape from early childhood onward, which is distinct from CKD of other etiologies, suggesting early childhood subclinical changes of the musculoskeletal unit of the thoracic cage, which are associated with kidney function. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information</abstract><cop>Berlin/Heidelberg</cop><pub>Springer Berlin Heidelberg</pub><pmid>37415042</pmid><doi>10.1007/s00467-023-06058-x</doi><tpages>11</tpages><orcidid>https://orcid.org/0000-0001-9302-712X</orcidid><oa>free_for_read</oa></addata></record>
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source SpringerNature Journals
subjects Body measurements
Chest
Children
Childrens health
Cystinosis
Fanconi syndrome
Kidney diseases
Lysosomal storage diseases
Malnutrition
Medicine
Medicine & Public Health
Myopathy
Nephrology
Original
Original Article
Patients
Pediatrics
Rickets
Thorax
Urology
title Chest configuration in children and adolescents with infantile nephropathic cystinosis compared with other chronic kidney disease entities and its clinical determinants
url https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2024-12-06T07%3A28%3A18IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_pubme&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Chest%20configuration%20in%20children%20and%20adolescents%20with%20infantile%20nephropathic%20cystinosis%20compared%20with%20other%20chronic%20kidney%20disease%20entities%20and%20its%20clinical%20determinants&rft.jtitle=Pediatric%20nephrology%20(Berlin,%20West)&rft.au=M%C3%BCller,%20Sophia&rft.date=2023-12-01&rft.volume=38&rft.issue=12&rft.spage=3989&rft.epage=3999&rft.pages=3989-3999&rft.issn=0931-041X&rft.eissn=1432-198X&rft_id=info:doi/10.1007/s00467-023-06058-x&rft_dat=%3Cproquest_pubme%3E2835272974%3C/proquest_pubme%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=2878549477&rft_id=info:pmid/37415042&rfr_iscdi=true