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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.


Metallic prostheses used in bilateral hip replacements artificially elevate BMD on WB DXA. Every patient had artificial hips, invalidating WB DXA results. BMD, bone mineral density; WB DXA, whole body dual-energy X-ray absorptiometry.
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f0010: Metallic prostheses used in bilateral hip replacements artificially elevate BMD on WB DXA. Every patient had artificial hips, invalidating WB DXA results. BMD, bone mineral density; WB DXA, whole body dual-energy X-ray absorptiometry.

Mentions: The presence of metallic artifact from prosthetic hips on every WB scan precluded valid assessment of the results (Fig.2). Metal is interpreted as bone on DXA and therefore the presence of metal artificially elevates BMD. Two of the three patients had vertebral overlap at T12 and L1, invalidating LS scan results. The one technically valid LS scan resulted in a normal BMD Z-score of − 0.8, but wedging of L3, which can elevate LS BMD DXA results, was noted [27] (Fig. 3). The LDF yielded technically valid results for all patients, and Z-scores were low at all three regions of interest with average Z-scores of − 3.1, − 2.3, and − 2.1 at R1–R3, respectively (Fig. 4). Every region of interest for all measurements (both femurs) was consistently below normal.


Bone mineral density in mucopolysaccharidosis IVB
Metallic prostheses used in bilateral hip replacements artificially elevate BMD on WB DXA. Every patient had artificial hips, invalidating WB DXA results. BMD, bone mineral density; WB DXA, whole body dual-energy X-ray absorptiometry.
© Copyright Policy - CC BY-NC-ND
Related In: Results  -  Collection

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

f0010: Metallic prostheses used in bilateral hip replacements artificially elevate BMD on WB DXA. Every patient had artificial hips, invalidating WB DXA results. BMD, bone mineral density; WB DXA, whole body dual-energy X-ray absorptiometry.
Mentions: The presence of metallic artifact from prosthetic hips on every WB scan precluded valid assessment of the results (Fig.2). Metal is interpreted as bone on DXA and therefore the presence of metal artificially elevates BMD. Two of the three patients had vertebral overlap at T12 and L1, invalidating LS scan results. The one technically valid LS scan resulted in a normal BMD Z-score of − 0.8, but wedging of L3, which can elevate LS BMD DXA results, was noted [27] (Fig. 3). The LDF yielded technically valid results for all patients, and Z-scores were low at all three regions of interest with average Z-scores of − 3.1, − 2.3, and − 2.1 at R1–R3, respectively (Fig. 4). Every region of interest for all measurements (both femurs) was consistently below normal.

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.