Decreased bone mineral density and content in neurofibromatosis. type 1: Lowest local values are located in the load-carrying parts of the body
Neurofibromatosis type 1 (NF1) is a dominantly inherited disease. Skeletal ailments such as short stature, kyphoscoliosis, tibial bowing and pseudarthrosis are common osseous manifestations of NF1. Previously, a correlation with scoliosis and decreased bone mineral density (BMD) of the lumbar spine...
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| Veröffentlicht in: | Osteoporosis international 2005-08, Vol.16 (8), p.928-936 |
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| description | Neurofibromatosis type 1 (NF1) is a dominantly inherited disease. Skeletal ailments such as short stature, kyphoscoliosis, tibial bowing and pseudarthrosis are common osseous manifestations of NF1. Previously, a correlation with scoliosis and decreased bone mineral density (BMD) of the lumbar spine has been reported in 12 NF1 patients. A total of 35 NF1 patients and 26 healthy controls were included in the present study. Of the participants over 20 years of age (26 NF1 patients and all controls) 14 were male and 12 were female, seven of whom were premenopausal. The controls were matched for age, sex and body mass index (BMI). Physical activity and medical history of NF1 patients were evaluated to screen the fractures and osseous manifestations of the disease and to rule out the factors that effect BMD. BMD and bone mineral content (BMC) were measured with DXA, using a total body program. The present study detected a lowered bone mineral density (p =0.028) and content (p |
| doi_str_mv | 10.1007/s00198-004-1801-4 |
| format | Article |
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Skeletal ailments such as short stature, kyphoscoliosis, tibial bowing and pseudarthrosis are common osseous manifestations of NF1. Previously, a correlation with scoliosis and decreased bone mineral density (BMD) of the lumbar spine has been reported in 12 NF1 patients. A total of 35 NF1 patients and 26 healthy controls were included in the present study. Of the participants over 20 years of age (26 NF1 patients and all controls) 14 were male and 12 were female, seven of whom were premenopausal. The controls were matched for age, sex and body mass index (BMI). Physical activity and medical history of NF1 patients were evaluated to screen the fractures and osseous manifestations of the disease and to rule out the factors that effect BMD. BMD and bone mineral content (BMC) were measured with DXA, using a total body program. The present study detected a lowered bone mineral density (p =0.028) and content (p <0.001) in NF1 patients of both sexes. The results of the present study also show that NF1 patients have an increased risk for osteoporosis. Among NF1 patients seven cases of osteoporosis and 13 cases of osteopenia were detected. In controls, one case of osteoporosis and 13 cases of osteopenia were detected. The location of the lowest local BMD was clustered to the load-carrying parts of the body in NF1 patients. Physical activity and the medical history of the NF1 patients did not explain the decreased BMD and BMC. The findings of the present and previous studies suggest that the pathogenesis of the osseous manifestations in NF1 may involve impaired development of the skeletal system and impaired maintenance of bone structure.</description><identifier>ISSN: 0937-941X</identifier><identifier>EISSN: 1433-2965</identifier><identifier>DOI: 10.1007/s00198-004-1801-4</identifier><identifier>PMID: 15551055</identifier><language>eng</language><publisher>London: Springer</publisher><subject>Absorptiometry, Photon ; Adult ; Age ; Aged ; Arm Bones - physiopathology ; Biological and medical sciences ; Body Composition - physiology ; Body mass index ; Bone density ; Bone Density - physiology ; Bone Diseases, Metabolic - physiopathology ; Case-Control Studies ; Diseases of the osteoarticular system ; Exercise ; Exercise - physiology ; Female ; Humans ; Investigative techniques, diagnostic techniques (general aspects) ; Leg Bones - physiopathology ; Male ; Medical sciences ; Middle Aged ; Neurofibromatosis 1 - physiopathology ; Osteoarticular system. Muscles ; Osteoporosis ; Osteoporosis - physiopathology ; Osteoporosis. Osteomalacia. Paget disease ; Radiodiagnosis. Nmr imagery. 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Skeletal ailments such as short stature, kyphoscoliosis, tibial bowing and pseudarthrosis are common osseous manifestations of NF1. Previously, a correlation with scoliosis and decreased bone mineral density (BMD) of the lumbar spine has been reported in 12 NF1 patients. A total of 35 NF1 patients and 26 healthy controls were included in the present study. Of the participants over 20 years of age (26 NF1 patients and all controls) 14 were male and 12 were female, seven of whom were premenopausal. The controls were matched for age, sex and body mass index (BMI). Physical activity and medical history of NF1 patients were evaluated to screen the fractures and osseous manifestations of the disease and to rule out the factors that effect BMD. BMD and bone mineral content (BMC) were measured with DXA, using a total body program. The present study detected a lowered bone mineral density (p =0.028) and content (p <0.001) in NF1 patients of both sexes. The results of the present study also show that NF1 patients have an increased risk for osteoporosis. Among NF1 patients seven cases of osteoporosis and 13 cases of osteopenia were detected. In controls, one case of osteoporosis and 13 cases of osteopenia were detected. The location of the lowest local BMD was clustered to the load-carrying parts of the body in NF1 patients. Physical activity and the medical history of the NF1 patients did not explain the decreased BMD and BMC. The findings of the present and previous studies suggest that the pathogenesis of the osseous manifestations in NF1 may involve impaired development of the skeletal system and impaired maintenance of bone structure.</description><subject>Absorptiometry, Photon</subject><subject>Adult</subject><subject>Age</subject><subject>Aged</subject><subject>Arm Bones - physiopathology</subject><subject>Biological and medical sciences</subject><subject>Body Composition - physiology</subject><subject>Body mass index</subject><subject>Bone density</subject><subject>Bone Density - physiology</subject><subject>Bone Diseases, Metabolic - physiopathology</subject><subject>Case-Control Studies</subject><subject>Diseases of the osteoarticular system</subject><subject>Exercise</subject><subject>Exercise - physiology</subject><subject>Female</subject><subject>Humans</subject><subject>Investigative techniques, diagnostic techniques (general aspects)</subject><subject>Leg Bones - physiopathology</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Middle Aged</subject><subject>Neurofibromatosis 1 - physiopathology</subject><subject>Osteoarticular system. Muscles</subject><subject>Osteoporosis</subject><subject>Osteoporosis - physiopathology</subject><subject>Osteoporosis. Osteomalacia. Paget disease</subject><subject>Radiodiagnosis. Nmr imagery. 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Muscles</topic><topic>Osteoporosis</topic><topic>Osteoporosis - physiopathology</topic><topic>Osteoporosis. Osteomalacia. Paget disease</topic><topic>Radiodiagnosis. Nmr imagery. Nmr spectrometry</topic><topic>Scoliosis</topic><topic>Scoliosis - physiopathology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>KUORILEHTO, T</creatorcontrib><creatorcontrib>PÖYHÖNEN, M</creatorcontrib><creatorcontrib>BLOIGU, R</creatorcontrib><creatorcontrib>HEIKKINEN, J</creatorcontrib><creatorcontrib>VÄÄNÄNEN, K</creatorcontrib><creatorcontrib>PELTONEN, Juha</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>ProQuest Central (Corporate)</collection><collection>Calcium & Calcified Tissue Abstracts</collection><collection>Nursing & Allied Health Database</collection><collection>Physical Education Index</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>Public Health Database</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 UK/Ireland</collection><collection>ProQuest Central</collection><collection>ProQuest One Community College</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Nursing & Allied Health Database (Alumni Edition)</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>Nursing & Allied Health Premium</collection><collection>ProQuest Central (New)</collection><collection>ProQuest One Academic (New)</collection><collection>ProQuest Health & Medical Research Collection</collection><collection>ProQuest One Academic Middle East (New)</collection><collection>ProQuest One Health & Nursing</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</collection><collection>MEDLINE - Academic</collection><jtitle>Osteoporosis international</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>KUORILEHTO, T</au><au>PÖYHÖNEN, M</au><au>BLOIGU, R</au><au>HEIKKINEN, J</au><au>VÄÄNÄNEN, K</au><au>PELTONEN, Juha</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Decreased bone mineral density and content in neurofibromatosis. type 1: Lowest local values are located in the load-carrying parts of the body</atitle><jtitle>Osteoporosis international</jtitle><addtitle>Osteoporos Int</addtitle><date>2005-08-01</date><risdate>2005</risdate><volume>16</volume><issue>8</issue><spage>928</spage><epage>936</epage><pages>928-936</pages><issn>0937-941X</issn><eissn>1433-2965</eissn><abstract>Neurofibromatosis type 1 (NF1) is a dominantly inherited disease. Skeletal ailments such as short stature, kyphoscoliosis, tibial bowing and pseudarthrosis are common osseous manifestations of NF1. Previously, a correlation with scoliosis and decreased bone mineral density (BMD) of the lumbar spine has been reported in 12 NF1 patients. A total of 35 NF1 patients and 26 healthy controls were included in the present study. Of the participants over 20 years of age (26 NF1 patients and all controls) 14 were male and 12 were female, seven of whom were premenopausal. The controls were matched for age, sex and body mass index (BMI). Physical activity and medical history of NF1 patients were evaluated to screen the fractures and osseous manifestations of the disease and to rule out the factors that effect BMD. BMD and bone mineral content (BMC) were measured with DXA, using a total body program. The present study detected a lowered bone mineral density (p =0.028) and content (p <0.001) in NF1 patients of both sexes. The results of the present study also show that NF1 patients have an increased risk for osteoporosis. Among NF1 patients seven cases of osteoporosis and 13 cases of osteopenia were detected. In controls, one case of osteoporosis and 13 cases of osteopenia were detected. The location of the lowest local BMD was clustered to the load-carrying parts of the body in NF1 patients. Physical activity and the medical history of the NF1 patients did not explain the decreased BMD and BMC. The findings of the present and previous studies suggest that the pathogenesis of the osseous manifestations in NF1 may involve impaired development of the skeletal system and impaired maintenance of bone structure.</abstract><cop>London</cop><pub>Springer</pub><pmid>15551055</pmid><doi>10.1007/s00198-004-1801-4</doi><tpages>9</tpages></addata></record> |
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| subjects | Absorptiometry, Photon Adult Age Aged Arm Bones - physiopathology Biological and medical sciences Body Composition - physiology Body mass index Bone density Bone Density - physiology Bone Diseases, Metabolic - physiopathology Case-Control Studies Diseases of the osteoarticular system Exercise Exercise - physiology Female Humans Investigative techniques, diagnostic techniques (general aspects) Leg Bones - physiopathology Male Medical sciences Middle Aged Neurofibromatosis 1 - physiopathology Osteoarticular system. Muscles Osteoporosis Osteoporosis - physiopathology Osteoporosis. Osteomalacia. Paget disease Radiodiagnosis. Nmr imagery. Nmr spectrometry Scoliosis Scoliosis - physiopathology |
| title | Decreased bone mineral density and content in neurofibromatosis. type 1: Lowest local values are located in the load-carrying parts of the body |
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