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 |
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| 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 |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_pubme</sourceid><recordid>TN_cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_10584709</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>2878549477</sourcerecordid><originalsourceid>FETCH-LOGICAL-c475t-f6ef4d9a8761b47b57c18c3e4686a0a9ac151e1b93d49b6111a8f97887bd52943</originalsourceid><addsrcrecordid>eNp9ksuO1DAQRS0EYpqGH2CBLLFhE7ATJ7ZXCLV4SSOxAWl2lmNXOh4Su7GdYfqP-Ezck2F4LFh5UafudVVdhJ5S8pISwl8lQljHK1I3FelIK6rre2hDWVNXVIqL-2hDZEMrwujFGXqU0iUhRLSie4jOGs5oS1i9QT92I6SMTfCD2y9RZxc8dh6b0U02gsfaW6xtmCAZ8Dnh7y6PBRi0z24C7OEwxnDQeXQGm2PKzofkUhGcDzqCXfmQR4hFMwZfsK_Oejhi6xLoBLjIuuwg3Vi5YmEmVzA9YQsZ4ux88UqP0YNBTwme3L5b9OXd28-7D9X5p_cfd2_OK8N4m6uhg4FZqQXvaM9433JDhWmAdaLTREttaEuB9rKxTPYdpVSLQXIheG_bWrJmi16vuoeln8Geho56UofoZh2PKmin_q54N6p9uFK0XIDxsvItenGrEMO3pWxXza4sb5q0h7AkVYumrXkt-cns-T_oZViiL_MViouWScZ5oeqVMjGkFGG4-w0l6pQEtSZBlSSomySo69L07M857lp-nb4AzQqkUvJ7iL-9_yP7E2KHxTY</addsrcrecordid><sourcetype>Open Access Repository</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>2878549477</pqid></control><display><type>article</type><title>Chest configuration in children and adolescents with infantile nephropathic cystinosis compared with other chronic kidney disease entities and its clinical determinants</title><source>SpringerNature Journals</source><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</creator><creatorcontrib>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</creatorcontrib><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</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 & 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 (> 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><subject>Body measurements</subject><subject>Chest</subject><subject>Children</subject><subject>Childrens health</subject><subject>Cystinosis</subject><subject>Fanconi syndrome</subject><subject>Kidney diseases</subject><subject>Lysosomal storage diseases</subject><subject>Malnutrition</subject><subject>Medicine</subject><subject>Medicine & Public Health</subject><subject>Myopathy</subject><subject>Nephrology</subject><subject>Original</subject><subject>Original Article</subject><subject>Patients</subject><subject>Pediatrics</subject><subject>Rickets</subject><subject>Thorax</subject><subject>Urology</subject><issn>0931-041X</issn><issn>1432-198X</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2023</creationdate><recordtype>article</recordtype><sourceid>C6C</sourceid><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>AZQEC</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><recordid>eNp9ksuO1DAQRS0EYpqGH2CBLLFhE7ATJ7ZXCLV4SSOxAWl2lmNXOh4Su7GdYfqP-Ezck2F4LFh5UafudVVdhJ5S8pISwl8lQljHK1I3FelIK6rre2hDWVNXVIqL-2hDZEMrwujFGXqU0iUhRLSie4jOGs5oS1i9QT92I6SMTfCD2y9RZxc8dh6b0U02gsfaW6xtmCAZ8Dnh7y6PBRi0z24C7OEwxnDQeXQGm2PKzofkUhGcDzqCXfmQR4hFMwZfsK_Oejhi6xLoBLjIuuwg3Vi5YmEmVzA9YQsZ4ux88UqP0YNBTwme3L5b9OXd28-7D9X5p_cfd2_OK8N4m6uhg4FZqQXvaM9433JDhWmAdaLTREttaEuB9rKxTPYdpVSLQXIheG_bWrJmi16vuoeln8Geho56UofoZh2PKmin_q54N6p9uFK0XIDxsvItenGrEMO3pWxXza4sb5q0h7AkVYumrXkt-cns-T_oZViiL_MViouWScZ5oeqVMjGkFGG4-w0l6pQEtSZBlSSomySo69L07M857lp-nb4AzQqkUvJ7iL-9_yP7E2KHxTY</recordid><startdate>20231201</startdate><enddate>20231201</enddate><creator>Müller, Sophia</creator><creator>Kluck, Rika</creator><creator>Jagodzinski, Celina</creator><creator>Brügelmann, Malina</creator><creator>Hohenfellner, Katharina</creator><creator>Büscher, Anja</creator><creator>Kemper, Markus J.</creator><creator>Fröde, Kerstin</creator><creator>Oh, Jun</creator><creator>Billing, Heiko</creator><creator>Thumfart, Julia</creator><creator>Weber, Lutz T.</creator><creator>Acham-Roschitz, Birgit</creator><creator>Arbeiter, Klaus</creator><creator>Tönshoff, Burkhard</creator><creator>Hagenberg, Martina</creator><creator>Pavičić, Leo</creator><creator>Haffner, Dieter</creator><creator>Zivicnjak, Miroslav</creator><general>Springer Berlin Heidelberg</general><general>Springer Nature B.V</general><scope>C6C</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>3V.</scope><scope>7QP</scope><scope>7RV</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8AO</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>AZQEC</scope><scope>BENPR</scope><scope>CCPQU</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>K9-</scope><scope>K9.</scope><scope>KB0</scope><scope>M0R</scope><scope>M0S</scope><scope>M1P</scope><scope>NAPCQ</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>7X8</scope><scope>5PM</scope><orcidid>https://orcid.org/0000-0001-9302-712X</orcidid></search><sort><creationdate>20231201</creationdate><title>Chest configuration in children and adolescents with infantile nephropathic cystinosis compared with other chronic kidney disease entities and its clinical determinants</title><author>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</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c475t-f6ef4d9a8761b47b57c18c3e4686a0a9ac151e1b93d49b6111a8f97887bd52943</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2023</creationdate><topic>Body measurements</topic><topic>Chest</topic><topic>Children</topic><topic>Childrens health</topic><topic>Cystinosis</topic><topic>Fanconi syndrome</topic><topic>Kidney diseases</topic><topic>Lysosomal storage diseases</topic><topic>Malnutrition</topic><topic>Medicine</topic><topic>Medicine & Public Health</topic><topic>Myopathy</topic><topic>Nephrology</topic><topic>Original</topic><topic>Original Article</topic><topic>Patients</topic><topic>Pediatrics</topic><topic>Rickets</topic><topic>Thorax</topic><topic>Urology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><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><collection>Springer Nature OA/Free Journals</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Calcium & Calcified Tissue Abstracts</collection><collection>Nursing & Allied Health Database</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>ProQuest Pharma Collection</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central 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titles)</collection><jtitle>Pediatric nephrology (Berlin, West)</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Müller, Sophia</au><au>Kluck, Rika</au><au>Jagodzinski, Celina</au><au>Brügelmann, Malina</au><au>Hohenfellner, Katharina</au><au>Büscher, Anja</au><au>Kemper, Markus J.</au><au>Fröde, Kerstin</au><au>Oh, Jun</au><au>Billing, Heiko</au><au>Thumfart, Julia</au><au>Weber, Lutz T.</au><au>Acham-Roschitz, Birgit</au><au>Arbeiter, Klaus</au><au>Tönshoff, Burkhard</au><au>Hagenberg, Martina</au><au>Pavičić, Leo</au><au>Haffner, Dieter</au><au>Zivicnjak, Miroslav</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Chest configuration in children and adolescents with infantile nephropathic cystinosis compared with other chronic kidney disease entities and its clinical determinants</atitle><jtitle>Pediatric nephrology (Berlin, West)</jtitle><stitle>Pediatr Nephrol</stitle><addtitle>Pediatr Nephrol</addtitle><date>2023-12-01</date><risdate>2023</risdate><volume>38</volume><issue>12</issue><spage>3989</spage><epage>3999</epage><pages>3989-3999</pages><issn>0931-041X</issn><eissn>1432-198X</eissn><abstract>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</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> |
| fulltext | fulltext |
| identifier | ISSN: 0931-041X |
| ispartof | Pediatric nephrology (Berlin, West), 2023-12, Vol.38 (12), p.3989-3999 |
| issn | 0931-041X 1432-198X |
| language | eng |
| recordid | cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_10584709 |
| 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 |
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