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Bone mineral density in mucopolysaccharidosis IVB

View Article: PubMed Central - PubMed

ABSTRACT

To date, the only published reports of bone mineral density (BMD) in MPS IV involve patients with MPS IVA; no reports exist describing BMD for MPS IVB. In this prospective study of BMD in three patients with MPS IVB, BMD was acquired by dual-energy X-ray absorptiometry (DXA) at whole body (WB), lumbar spine (LS), and lateral distal femur (LDF). Functional abilities, ambulatory status, medical history, and height z-score were evaluated. Three patients with MPS IVB (two females), aged 17.7, 31.4 and 31.7 years, were evaluated. Every patient was ambulatory and one sustained two fractures caused by trauma. Whole body and hip DXA scans were technically invalid in every patient due to the presence of prosthetic hip hardware. Lumbar spine was valid in only 1 patient due skeletal abnormalities, and was normal (Z-score of − 0.8). The LDF was valid in every patient and was low at all three regions of interest: average LDF z-scores were − 3.1 (range, − 2.9 to − 3.6), − 2.3 (range, − 2.0 to − 2.5), and − 2.1 (range, − 2.0 to − 2.3) for region 1–region 3, respectively. Patients with MPS IVB have low BMD of the lower extremities even with full-time ambulation. Routine body sites to measure by DXA were problematic; hip and WB were invalid due to artifact, and LS had limited utility. The LDF was the only body site consistently available on all patients. Patients did not experience low-energy fractures despite low BMD.

No MeSH data available.


The LDF DXA scan is analyzed for three regions of interest: Region 1 (anterior distal metaphysis) is essentially trabecular bone, region 2 (metadiaphysis) is composed of both trabecular and cortical bone, region 3 (diaphysis) is composed primarily of cortical bone. There has been proximal femoral surgery with the distal end of the metal prosthesis visible above region 3. LDF DXA, lateral distal femur dual-energy X-ray absorptiometry; R1, region 1; R2, region 2; R3, region 3.
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f0005: The LDF DXA scan is analyzed for three regions of interest: Region 1 (anterior distal metaphysis) is essentially trabecular bone, region 2 (metadiaphysis) is composed of both trabecular and cortical bone, region 3 (diaphysis) is composed primarily of cortical bone. There has been proximal femoral surgery with the distal end of the metal prosthesis visible above region 3. LDF DXA, lateral distal femur dual-energy X-ray absorptiometry; R1, region 1; R2, region 2; R3, region 3.

Mentions: The LDF scans were analyzed for three distinct regions of interest, described by Henderson et al., to assess bone density in different types of bone [24]. Region 1 (R1), the most distal region, is predominantly trabecular bone; region 2 (R2) is a mix of trabecular and cortical bone; and region 3 (R3), the most proximal region, is primarily cortical bone (Fig. 1). The LDF BMD was assessed bilaterally, left and right femur BMD values were averaged, and Z-scores were calculated. Abnormal DXA results were defined as more than two standard deviations (SD) below the normal mean, expressed as Z-score < − 2 [26]. Radiographs of the LS, including inter-vertebral assessment by DXA, were reviewed by a radiologist and were used to aid in correct region of interest placement on the LS DXA.


Bone mineral density in mucopolysaccharidosis IVB
The LDF DXA scan is analyzed for three regions of interest: Region 1 (anterior distal metaphysis) is essentially trabecular bone, region 2 (metadiaphysis) is composed of both trabecular and cortical bone, region 3 (diaphysis) is composed primarily of cortical bone. There has been proximal femoral surgery with the distal end of the metal prosthesis visible above region 3. LDF DXA, lateral distal femur dual-energy X-ray absorptiometry; R1, region 1; R2, region 2; R3, region 3.
© Copyright Policy - CC BY-NC-ND
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC5036531&req=5

f0005: The LDF DXA scan is analyzed for three regions of interest: Region 1 (anterior distal metaphysis) is essentially trabecular bone, region 2 (metadiaphysis) is composed of both trabecular and cortical bone, region 3 (diaphysis) is composed primarily of cortical bone. There has been proximal femoral surgery with the distal end of the metal prosthesis visible above region 3. LDF DXA, lateral distal femur dual-energy X-ray absorptiometry; R1, region 1; R2, region 2; R3, region 3.
Mentions: The LDF scans were analyzed for three distinct regions of interest, described by Henderson et al., to assess bone density in different types of bone [24]. Region 1 (R1), the most distal region, is predominantly trabecular bone; region 2 (R2) is a mix of trabecular and cortical bone; and region 3 (R3), the most proximal region, is primarily cortical bone (Fig. 1). The LDF BMD was assessed bilaterally, left and right femur BMD values were averaged, and Z-scores were calculated. Abnormal DXA results were defined as more than two standard deviations (SD) below the normal mean, expressed as Z-score < − 2 [26]. Radiographs of the LS, including inter-vertebral assessment by DXA, were reviewed by a radiologist and were used to aid in correct region of interest placement on the LS DXA.

View Article: PubMed Central - PubMed

ABSTRACT

To date, the only published reports of bone mineral density (BMD) in MPS IV involve patients with MPS IVA; no reports exist describing BMD for MPS IVB. In this prospective study of BMD in three patients with MPS IVB, BMD was acquired by dual-energy X-ray absorptiometry (DXA) at whole body (WB), lumbar spine (LS), and lateral distal femur (LDF). Functional abilities, ambulatory status, medical history, and height z-score were evaluated. Three patients with MPS IVB (two females), aged 17.7, 31.4 and 31.7&nbsp;years, were evaluated. Every patient was ambulatory and one sustained two fractures caused by trauma. Whole body and hip DXA scans were technically invalid in every patient due to the presence of prosthetic hip hardware. Lumbar spine was valid in only 1 patient due skeletal abnormalities, and was normal (Z-score of &minus;&nbsp;0.8). The LDF was valid in every patient and was low at all three regions of interest: average LDF z-scores were &minus;&nbsp;3.1 (range, &minus;&nbsp;2.9 to &minus;&nbsp;3.6), &minus;&nbsp;2.3 (range, &minus;&nbsp;2.0 to &minus;&nbsp;2.5), and &minus;&nbsp;2.1 (range, &minus;&nbsp;2.0 to &minus;&nbsp;2.3) for region 1&ndash;region 3, respectively. Patients with MPS IVB have low BMD of the lower extremities even with full-time ambulation. Routine body sites to measure by DXA were problematic; hip and WB were invalid due to artifact, and LS had limited utility. The LDF was the only body site consistently available on all patients. Patients did not experience low-energy fractures despite low BMD.

No MeSH data available.