Growth charts for patients affected with Morquio A disease
Children with Morquio A disease grow poorly and become physically handicapped because of systemic bone disease. The purpose of this study was to describe observed growth patterns and their relationship with the physical condition of patients with Morquio A. In a one‐center study, questionnaire‐based...
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| Veröffentlicht in: | American journal of medical genetics. Part A 2008-05, Vol.146A (10), p.1286-1295 |
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| container_title | American journal of medical genetics. Part A |
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| creator | Montaño, Adriana M. Tomatsu, Shunji Brusius, Ana Smith, Mary Orii, Tadao |
| description | Children with Morquio A disease grow poorly and become physically handicapped because of systemic bone disease. The purpose of this study was to describe observed growth patterns and their relationship with the physical condition of patients with Morquio A. In a one‐center study, questionnaire‐based longitudinal and cross sectional data were used to develop growth curves, to assess physical activity and to determine the incidence of surgical procedures in 354 patients with Morquio A. Mean birth lengths of boys and girls were 52.6 and 52.1 cm, respectively. The mean final heights for males and females at 18 years and older were 122.4 ± 21.5 and 113.1 ± 22.6 cm, respectively. These results corresponded to −7.4 SD for males and −7.7 SD for females compared to the normal healthy controls. Mean birth weights for boys and girls were 3.59 ± 0.58 and 3.5 ± 0.7 kg, respectively. The mean body mass index for males and females at over 18 years of age was 24.7 ± 6.1 and 25.6 ± 5.4 kg/m2, respectively. The growth pattern in Morquio A patients was characterized by impaired growth velocity after 1 year of age. This is the first report providing growth charts for patients with Morquio A, which can help with monitoring the disease and assessing the clinical efficacy of treatments. © 2008 Wiley‐Liss, Inc. |
| doi_str_mv | 10.1002/ajmg.a.32281 |
| format | Article |
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The purpose of this study was to describe observed growth patterns and their relationship with the physical condition of patients with Morquio A. In a one‐center study, questionnaire‐based longitudinal and cross sectional data were used to develop growth curves, to assess physical activity and to determine the incidence of surgical procedures in 354 patients with Morquio A. Mean birth lengths of boys and girls were 52.6 and 52.1 cm, respectively. The mean final heights for males and females at 18 years and older were 122.4 ± 21.5 and 113.1 ± 22.6 cm, respectively. These results corresponded to −7.4 SD for males and −7.7 SD for females compared to the normal healthy controls. Mean birth weights for boys and girls were 3.59 ± 0.58 and 3.5 ± 0.7 kg, respectively. The mean body mass index for males and females at over 18 years of age was 24.7 ± 6.1 and 25.6 ± 5.4 kg/m2, respectively. The growth pattern in Morquio A patients was characterized by impaired growth velocity after 1 year of age. This is the first report providing growth charts for patients with Morquio A, which can help with monitoring the disease and assessing the clinical efficacy of treatments. © 2008 Wiley‐Liss, Inc.</description><identifier>ISSN: 1552-4825</identifier><identifier>EISSN: 1552-4833</identifier><identifier>DOI: 10.1002/ajmg.a.32281</identifier><identifier>PMID: 18412124</identifier><language>eng</language><publisher>Hoboken: Wiley Subscription Services, Inc., A Wiley Company</publisher><subject>Adolescent ; Biological and medical sciences ; Body Height ; Body Mass Index ; Body Weight ; Carbohydrates (enzymatic deficiencies). Glycogenosis ; Child ; Child, Preschool ; Disease Progression ; Errors of metabolism ; Female ; GALNS ; Growth ; Humans ; Infant ; Infant, Newborn ; Internationality ; Male ; Medical genetics ; Medical Records ; Medical sciences ; Metabolic diseases ; Morquio A disease ; MPS IVA ; Mucopolysaccharidosis IV - epidemiology ; Mucopolysaccharidosis IV - genetics ; Mucopolysaccharidosis IV - physiopathology ; Mucopolysaccharidosis IV - surgery ; Registries ; Surveys and Questionnaires</subject><ispartof>American journal of medical genetics. 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Part A</title><addtitle>Am. J. Med. Genet</addtitle><description>Children with Morquio A disease grow poorly and become physically handicapped because of systemic bone disease. The purpose of this study was to describe observed growth patterns and their relationship with the physical condition of patients with Morquio A. In a one‐center study, questionnaire‐based longitudinal and cross sectional data were used to develop growth curves, to assess physical activity and to determine the incidence of surgical procedures in 354 patients with Morquio A. Mean birth lengths of boys and girls were 52.6 and 52.1 cm, respectively. The mean final heights for males and females at 18 years and older were 122.4 ± 21.5 and 113.1 ± 22.6 cm, respectively. These results corresponded to −7.4 SD for males and −7.7 SD for females compared to the normal healthy controls. Mean birth weights for boys and girls were 3.59 ± 0.58 and 3.5 ± 0.7 kg, respectively. The mean body mass index for males and females at over 18 years of age was 24.7 ± 6.1 and 25.6 ± 5.4 kg/m2, respectively. The growth pattern in Morquio A patients was characterized by impaired growth velocity after 1 year of age. This is the first report providing growth charts for patients with Morquio A, which can help with monitoring the disease and assessing the clinical efficacy of treatments. © 2008 Wiley‐Liss, Inc.</description><subject>Adolescent</subject><subject>Biological and medical sciences</subject><subject>Body Height</subject><subject>Body Mass Index</subject><subject>Body Weight</subject><subject>Carbohydrates (enzymatic deficiencies). Glycogenosis</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Disease Progression</subject><subject>Errors of metabolism</subject><subject>Female</subject><subject>GALNS</subject><subject>Growth</subject><subject>Humans</subject><subject>Infant</subject><subject>Infant, Newborn</subject><subject>Internationality</subject><subject>Male</subject><subject>Medical genetics</subject><subject>Medical Records</subject><subject>Medical sciences</subject><subject>Metabolic diseases</subject><subject>Morquio A disease</subject><subject>MPS IVA</subject><subject>Mucopolysaccharidosis IV - epidemiology</subject><subject>Mucopolysaccharidosis IV - genetics</subject><subject>Mucopolysaccharidosis IV - physiopathology</subject><subject>Mucopolysaccharidosis IV - surgery</subject><subject>Registries</subject><subject>Surveys and Questionnaires</subject><issn>1552-4825</issn><issn>1552-4833</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2008</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqF0EtP6zAQBWALXcR7d9dX2VxWpPidhF2FoOXNAsTSmtgTMKRNsVMV_j0pLWUHK4-lb85Ih5C_jPYYpfwQnkePPegJznO2RraYUjyVuRB_VjNXm2Q7xmdKBVWZ3iCbLJeMMy63yNEgNLP2KbFPENqYVE1IJtB6HHcfqCq0Lbpk5jtx1YTXqW-SfuJ8RIi4S9YrqCPuLd8dcn96cnc8TC9vBmfH_cvUSsFZysrMFlYUUqCgZemszkDKHHOtCgXWuUKUqDUWoCQvKweFLBUXDJxDXXItdsj-IncSmtcpxtaMfLRY1zDGZhqNLpiSGS1-hZwyzmUuO3iwgDY0MQaszCT4EYR3w6iZl2rmpRown6V2_N8yd1qO0H3jZYsd-L8EEC3UVYCx9XHlOBWcSj0PEgs38zW-_3jU9M-vBl_n08WWjy2-rbYgvBidiUyZh-uBkae37OJ2eG2G4gOnap4x</recordid><startdate>20080515</startdate><enddate>20080515</enddate><creator>Montaño, Adriana M.</creator><creator>Tomatsu, Shunji</creator><creator>Brusius, Ana</creator><creator>Smith, Mary</creator><creator>Orii, Tadao</creator><general>Wiley Subscription Services, Inc., A Wiley Company</general><general>Wiley-Liss</general><scope>BSCLL</scope><scope>IQODW</scope><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>7QP</scope><scope>8FD</scope><scope>FR3</scope><scope>P64</scope><scope>RC3</scope><scope>7X8</scope></search><sort><creationdate>20080515</creationdate><title>Growth charts for patients affected with Morquio A disease</title><author>Montaño, Adriana M. ; Tomatsu, Shunji ; Brusius, Ana ; Smith, Mary ; Orii, Tadao</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c4321-1b7c9c3943e30bbdc67a448e86595acdd93be66e9a542bfda94b5231adde6b263</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2008</creationdate><topic>Adolescent</topic><topic>Biological and medical sciences</topic><topic>Body Height</topic><topic>Body Mass Index</topic><topic>Body Weight</topic><topic>Carbohydrates (enzymatic deficiencies). Glycogenosis</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Disease Progression</topic><topic>Errors of metabolism</topic><topic>Female</topic><topic>GALNS</topic><topic>Growth</topic><topic>Humans</topic><topic>Infant</topic><topic>Infant, Newborn</topic><topic>Internationality</topic><topic>Male</topic><topic>Medical genetics</topic><topic>Medical Records</topic><topic>Medical sciences</topic><topic>Metabolic diseases</topic><topic>Morquio A disease</topic><topic>MPS IVA</topic><topic>Mucopolysaccharidosis IV - epidemiology</topic><topic>Mucopolysaccharidosis IV - genetics</topic><topic>Mucopolysaccharidosis IV - physiopathology</topic><topic>Mucopolysaccharidosis IV - surgery</topic><topic>Registries</topic><topic>Surveys and Questionnaires</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Montaño, Adriana M.</creatorcontrib><creatorcontrib>Tomatsu, Shunji</creatorcontrib><creatorcontrib>Brusius, Ana</creatorcontrib><creatorcontrib>Smith, Mary</creatorcontrib><creatorcontrib>Orii, Tadao</creatorcontrib><collection>Istex</collection><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>Calcium & Calcified Tissue Abstracts</collection><collection>Technology Research Database</collection><collection>Engineering Research Database</collection><collection>Biotechnology and BioEngineering Abstracts</collection><collection>Genetics Abstracts</collection><collection>MEDLINE - Academic</collection><jtitle>American journal of medical genetics. Part A</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Montaño, Adriana M.</au><au>Tomatsu, Shunji</au><au>Brusius, Ana</au><au>Smith, Mary</au><au>Orii, Tadao</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Growth charts for patients affected with Morquio A disease</atitle><jtitle>American journal of medical genetics. Part A</jtitle><addtitle>Am. J. Med. Genet</addtitle><date>2008-05-15</date><risdate>2008</risdate><volume>146A</volume><issue>10</issue><spage>1286</spage><epage>1295</epage><pages>1286-1295</pages><issn>1552-4825</issn><eissn>1552-4833</eissn><abstract>Children with Morquio A disease grow poorly and become physically handicapped because of systemic bone disease. The purpose of this study was to describe observed growth patterns and their relationship with the physical condition of patients with Morquio A. In a one‐center study, questionnaire‐based longitudinal and cross sectional data were used to develop growth curves, to assess physical activity and to determine the incidence of surgical procedures in 354 patients with Morquio A. Mean birth lengths of boys and girls were 52.6 and 52.1 cm, respectively. The mean final heights for males and females at 18 years and older were 122.4 ± 21.5 and 113.1 ± 22.6 cm, respectively. These results corresponded to −7.4 SD for males and −7.7 SD for females compared to the normal healthy controls. Mean birth weights for boys and girls were 3.59 ± 0.58 and 3.5 ± 0.7 kg, respectively. The mean body mass index for males and females at over 18 years of age was 24.7 ± 6.1 and 25.6 ± 5.4 kg/m2, respectively. The growth pattern in Morquio A patients was characterized by impaired growth velocity after 1 year of age. This is the first report providing growth charts for patients with Morquio A, which can help with monitoring the disease and assessing the clinical efficacy of treatments. © 2008 Wiley‐Liss, Inc.</abstract><cop>Hoboken</cop><pub>Wiley Subscription Services, Inc., A Wiley Company</pub><pmid>18412124</pmid><doi>10.1002/ajmg.a.32281</doi><tpages>10</tpages></addata></record> |
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| subjects | Adolescent Biological and medical sciences Body Height Body Mass Index Body Weight Carbohydrates (enzymatic deficiencies). Glycogenosis Child Child, Preschool Disease Progression Errors of metabolism Female GALNS Growth Humans Infant Infant, Newborn Internationality Male Medical genetics Medical Records Medical sciences Metabolic diseases Morquio A disease MPS IVA Mucopolysaccharidosis IV - epidemiology Mucopolysaccharidosis IV - genetics Mucopolysaccharidosis IV - physiopathology Mucopolysaccharidosis IV - surgery Registries Surveys and Questionnaires |
| title | Growth charts for patients affected with Morquio A disease |
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