Solitary Band Acro-osteolysis in Hajdu-Cheney Syndrome.

Hajdu-Cheney syndrome (HCS) is a rare autosomal dominant manifestation of a genetic disorder caused by a mutation in the NOTCH2 gene, resulting in acro-osteolysis and generalized osteoporosis, accompanied by many developmental skeletal disorders and multiple clinical and radiological manifestations.1Radiographs of the hands of an 11-year-old boy with shorter left thumb and lower back pain show solitary band acro-osteolysis and shortening of the distal phalange of the left thumb (Figure 1A, with Figure 1B showing enlarged images of both thumbs).

Shift in Calcium From Peripheral Bone to Axial Bone After Tumor Resection in Patients With Tumor-Induced Osteomalacia.

CONTEXT: Tumor-induced osteomalacia (TIO) is a rare paraneoplastic syndrome caused by excessive production of fibroblast growth factor 23 (FGF23) by a tumor. After successful tumor resection, patients can recover from hypophosphatemia quicky. However, data on the changes in bone mineral density (BMD) and microstructure in the short term after surgery remained unclear. OBJECTIVE: This work aimed to investigate the postoperative changes in BMD and microstructure both in peripheral and axial bone in TIO patients. METHODS: We evaluated BMD and microarchitecture in 22 TIO patients using high-resolution peripheral quantitative computed tomography (HR-pQCT) and dual-energy x-ray absorptiometry (DXA) before and 3 months after surgery in this retrospective study. RESULTS: In this study, a total of 22 TIO patients who had recovered serum phosphate levels postoperatively were enrolled. After surgery, areal BMD (aBMD) increased by 21.6% in the femoral neck, by 18.9% in the total hip, and by 29.5% in the lumbar spine. Moreover, TBS increased by 14.1% (all P < .001). In contrast, trabecular or cortical volumetric BMD (vBMD), and microstructure of trabecular bone (trabecular number, separation and bone volume ratio) and cortical bone (cortical thickness and porosity) at the distal radius or tibia were further deteriorated. Correlation analyses found that changes in femoral neck and total hip aBMD were both conversely associated with changes in trabecular vBMD and bone volume ratio, while positively correlated with change in trabecular separation at the distal radius. CONCLUSION: Although aBMD and microstructure in the axial bone were improved, vBMD and microstructure in the peripheral bone were further impaired shortly after surgery. Correlation of improvement of aBMD in the total hip and femoral neck with deterioration of vBMD and microstructure at the distal radius indicated a shift in calcium from the peripheral bone to the axial bone in the short term after tumor resection in TIO patients.

[Age-Based Grouping Criteria in Medicine].

Age,a basic marker of the population,is an indispensable demographic characteristic in medicine.However,the grouping in medicine according to age have problems such as inconsistent grouping criteria and ambiguous definition of age-related terms.Therefore,this article reviews the age-based grouping criteria and the application of related terms in medicine.

Effect of different antiretroviral therapy on muscle mass, bone mineral density, and trabecular bone score in Chinese HIV-infected males.

This is the first study to report both greater BMD loss and muscle loss in Chinese HIV-infected males with lamivudine (3TC)-tenofovir disoproxil fumarate (TDF)-efavirenz (EFV) regimen, which highlights the importance of closely monitoring muscle mass and bone mineral density in patients treated with 3TC-TDF-EFV regimen and provides a foundation for the clinical intervention of sarcopenia and osteoporosis. PURPOSE: To compare the effect initiating different antiretroviral therapy (ART) regimens have on muscle mass, bone mineral density (BMD), and trabecular bone score (TBS). METHODS: We designed a retrospective study of ART-naive Chinese males with HIV (MWH) undergoing two different regimens at 1-year follow-up. All subjects underwent dual-energy absorptiometry (DXA) for BMD and muscle mass prior to ART initiation, and again 1 year later. TBS iNsight software was used for TBS. We analyzed differences in muscle mass, BMD, and TBS after different treatment arms and associations between ART regimens and changes in them. RESULTS: A total of 76 men were included (mean age 31.83 ± 8.75 years). Mean absolute muscle mass decreased significantly from baseline to follow-up after initiation of lamivudine (3TC)-tenofovir disoproxil fumarate (TDF)-efavirenz (EFV), whereas increased significantly after initiation of 3TC-zidovudine(AZT)/Stavudine(d4T)-Nevirapine(NVP). Assignment to 3TC-TDF-EFV resulted in greater percentage loss in BMD at lumbar spine (LS) and total hip (TH) compared to 3TC-AZT/d4T-NVP, but this difference was not statistically significant at the femoral neck BMD and TBS. In the multivariable logistic regression model adjusted for covariates, the 3TC-TDF-EFV regimen was associated with higher odds of decreased appendicular and total muscle mass, LS and TH BMD. CONCLUSIONS: This is the first study to report not only greater BMD loss but also muscle loss in Chinese MWH with 3TC-TDF-EFV regimen. Our work highlights the importance of closely monitoring muscle mass and BMD in patients treated with 3TC-TDF-EFV regimen and provides a foundation for the clinical intervention of sarcopenia and osteoporosis in them.

Association of hepatic/pancreatic iron overload evaluated by quantitative T2* MRI with bone mineral density and trabecular bone score.

BACKGROUND: Iron-overloaded patients are recognized as presenting an increased risk of osteoporosis. However, studies on the correlation between osteoporosis and organ iron overload are controversial or scarce. The aim of this study is to assess bone mineral density (BMD) and trabecular bone score (TBS) in correlation with hepatic and pancreatic iron overload. METHODS: Forty-one patients diagnosed with hemoglobinopathies, were studied. BMDs of the lumbar spine (LS), femoral neck (FN), and total hip (TH) were analyzed by Dual-energy X-ray absorptiometry (DXA) scan. LS bone quality was derived from each spine DXA examination using the TBS analysis. Hepatic and pancreatic iron overload were obtained with a multi-echo gradient echo T2* technique. RESULTS: Abnormal microarchitecture and abnormal bone mass were observed in 19/41 (46.3%) and 9/41 (22.0%) patients, respectively. For 26 males, BMD, T-score and Z-score of LS were significantly lower among subjects with moderate-severe hepatic iron-overload than their counterparts, as it is between no- and pancreatic iron-overload groups. For 15 females, patients with moderate-severe hepatic iron-overload had significantly lower BMD and T-score of FN and TH, and patients with pancreatic iron-overload had significantly lower BMD, T-score of FN, and lower BMD, T-score and Z-score of TH than their counterparts. Moreover, pancreatic T2*-value was positively correlated with BMD and T-score at all analyzed sites and Z-score at TH. CONCLUSION: These data showed lower bone mass in patients with organ iron overload, particularly for LS in males, FN and TH in females. TBS may well represent a complementary tool for the evaluation of bone quality and the risk of fracture in iron-overloaded patients.

Atypical skeletal involvement in patients with Erdheim-Chester disease: CT imaging findings.

OBJECTIVES: To review retrospectively atypical bone findings from computed tomographic (CT) imaging in patients with Erdheim-Chester disease. METHODS: All 28 patients with Erdheim-Chester disease (13 men and 15 women; mean age, 45 years; range, 7-63 years) underwent chest-abdomen-pelvis CT. CT images were reviewed and analyzed for the various features of atypical bone lesions by two radiologists in consensus. RESULTS: Twenty-one patients had atypical bone involvement. Radiologically, these atypical osseous lesions were categorized into three types: diffuse, nodular and patchy. Eleven (52%) of the 21 patients had spinal lesions, of which four (36%) had the diffuse type, eight (73%) had the nodular pattern, and six (55%) had the patchy pattern. Sixteen (76%) of the 21 patients had pelvic involvement, of which two (13%) were diffuse, nine (56%) were nodular and 11 were (69%) patchy. Ribs were involved in seven (33%) of the 21 patients, with the nodular pattern in one (14%) patient and the patchy type in six (86%) patients. Clavicle involvement was seen in nine (43%) of the 21 patients, of which the diffuse type was found in only one (11%) patient, the nodular type in six (67%) patients, the solitary patchy type in four (44%) patients. Sternum involvement was seen in 10 (48%) of the 21 patients and all were nodular. CONCLUSIONS: This series provides a detailed description of atypical bone involvement in Erdheim-Chester disease which on CT displays three major patterns. Understanding these patterns may help increase the accuracy of diagnosis of this disease.

Changes in trabecular bone score and bone mineral density in Chinese HIV-Infected individuals after one year of antiretroviral therapy.

OBJECTIVE: Human immunodeficiency virus (HIV) infection and antiretroviral therapy (ART) have been associated with reduced bone mineral density (BMD) in persons with HIV (PWH). BMD provides information only about bone mineral quantity. Trabecular bone score (TBS) is a noninvasive tool that estimates bone microarchitecture. The aim of this study is to measure BMD and TBS of Chinese PWH after one-year ART. METHODS: We designed a retrospective study of adult Chinese PWH. Patients with a dual-energy X-ray absorptiometry (DXA) scan prior to ART initiation, and again 48 weeks later were included. Information regarding demographic and clinical history, HIV treatment history, BMD and TBS were collected. We analyzed differences in BMD and TBS over 48 weeks and associations between key risk factors and changes in BMD and TBS. RESULTS: Our study included 233 â€‹PWH (mean age â€‹= â€‹36.6 â€‹± â€‹11.1 years). Before ART initiation, 19.3% of PWH had normal BMD but abnormal TBS. Both BMD and TBS decreased after one-year ART. TDF and LPV/r-containing regimens were associated with greater declines in BMD at different site. Traditional risk factors such as old age, low BMI and female sex were associated with lower baseline TBS. Greater change in TBS over one year was associated with lower BMI and lower baseline CD4+ cell count, but unlike BMD measures, it was not correlated with treatment with TDF and LPV/r in our study population. CONCLUSIONS: We present the first longitudinal analysis of change in TBS over 48 weeks compared with BMD among Asian PWH receiving ART. Before ART initiation, approximately 20% of PWH with impaired bone microarchitecture would not have been identified if DXA were used alone to assess for bone damage. Both BMD and TBS decreased after one-year ART. Change in TBS was not associated with different antiretroviral agents. THE TRANSLATIONAL POTENTIAL OF THIS ARTICLE: The trabecular microarchitecture measured indirectly by TBS may provide clinicians additional information about bone damage in PWH.

Long-term trabecular bone score and bone mineral density changes in Chinese antiretroviral-treated HIV-infected individuals.

This is the first 5-year analysis among persons with HIV (PWH) that reports both trabecular bone score (TBS), which is a novel index that estimates bone microarchitecture, and bone mineral density (BMD) over time to evaluate the long-term impact of antiretroviral therapy (ART) on bone health. PURPOSE: HIV infection and antiretroviral therapy (ART) have been associated with decreased bone mineral density (BMD). This study aims to evaluate long-term changes in trabecular bone score (TBS), a novel index that estimates bone microarchitecture, and BMD among Chinese persons with HIV (PWH) treated with ART. METHODS: We conducted a retrospective chart review of adult PWH at a large tertiary care hospital in China. Patients who had a DXA scan prior to ART and at least one follow-up DXA after ART initiation were included. Subgroup analyses examined the TBS and BMD changes in patients who switch from a non-TDF-containing regimen to one containing TDF, as compared to those who did not switch. RESULTS: Four hundred fifty-nine PWH were included. Among 68 patients ≥ 50 years, 13 patients (19.1%) had a normal BMD but partially degraded or degraded TBS. The mean percent decrease in lumbar spine (LS) BMD nadired at 48 weeks after ART initiation and then gradually improved. Percent decrease in femoral neck (FN) and total hip (TH) BMD nadired at 96 weeks and remained stably low thereafter. After switch to a TDF-containing regimen, only percent change in TH BMD was significant (-3.2%, p = 0.006). In the regression analyses, switch to a TDF-containing regimen was not associated with long-term change in TBS or BMD. CONCLUSION: This is the first study among PWH to evaluate the long-term impact of ART on TBS and BMD. At baseline, approximately 20% of patients had a normal BMD but impaired bone microstructure based upon TBS. For patients with 5 years of exposure to ART, there is a stabilization of TBS and BMD after initial nadir in the first 144 weeks. However, FN BMD, TH BMD, and TBS remained low at 5 years relative to baseline.

Comparison of Differences in Bone Mineral Density Measurement With 3 Hologic Dual-Energy X-Ray Absorptiometry Scan Modes.

Bone mineral density (BMD) measurement using dual-energy X-ray absorptiometry (DXA) is considered a diagnostic parameter for osteoporosis by the World Health Organization (WHO). DXA densitometers have different scanning modes for BMD measurements, although the specific scanning modes vary based upon the manufacturer. For DXA machines manufactured by Hologic, which are used globally, a range of scanning modes exist, including but not limited to (in order of decreasing spatial resolution) Array, Fast Array, and Express Array. Only a handful of prior studies have compared the reproducibility of BMD measurements across scan modes. The present study aimed to add to this body of literature by investigating the differences in BMD measured between 3 scanning modes in Hologic DXA machines at 19 different health centers. As part of cross-calibration activities for two multi-center studies in China measuring BMD, the European spine phantom (ESP, 1.000 g/cm2) was scanned on 19 different Hologic DXA machines. To measure differences in BMD between the 3 scan modes most commonly found on the Hologic models available (i.e., Array, Fast Array, Express Array), the ESP measurement was performed 10 times for each scan mode on each Hologic DXA machine. One-sample t test was used to compare the average difference between the measured ESP results of the 3 scanning modes at each hospital and reference ESP values. Single factor analysis of variance was performed to compare the average differences between the pairs of scanning modes using the reference ESP. Statistically significant differences between the measured ESP results with reference ESP values were found with each scanning mode at 19 hospitals (all p values <0.05). Consistent with this finding, differences in average BMD between the Array mode and Fast Array mode were invariably the smallest compared to differences seen between the other two pairs of scan modes. Significant differences were observed between average ESP BMD for the Array and Express Array scan modes (0.971 ± 0.013 vs 0.935 ± 0.027, p < 0.001), and between Fast Array and Express Array scan modes (0.972 ± 0.012 vs 0.935 ± 0.027, p < 0.001). However, no significant difference in average ESP BMD was observed between the Array and Fast Array scan modes (0.971 ± 0.013 vs 0.972 ± 0.012, p = 0.997). The selection of ideal scanning mode requires a balance of scanning time, radiation exposure, and measurement accuracy. In this ex vivo study, the Fast Array scanning mode appeared to be a reasonable choice compared with Array and Express Array for BMD measurements by Hologic DXA. Future in vivo studies can help guide the clinical application of these findings.

Pretherapeutic Assessment of Pancreatic Cancer: Comparison of FDG PET/CT Plus Delayed PET/MR and Contrast-Enhanced CT/MR.

PURPOSE: This study aims to determine the diagnostic performance of whole-body FDG PET/CT plus delayed abdomen PET/MR imaging in the pretherapeutic assessment of pancreatic cancer in comparison with that of contrast-enhanced (CE)-CT/MR imaging. MATERIALS AND METHODS: Forty patients with pancreatic cancer underwent nonenhanced whole-body FDG PET/CT, delayed abdomen PET/MR imaging, and CE-CT/MR imaging. Two nuclear medicine physicians independently reviewed these images and discussed to reach a consensus, determining tumor resectability according to a 5-point scale, N stage (N0 or N positive), and M stage (M0 or M1). With use of clinical-surgical-pathologic findings as the reference standard, diagnostic performances of the two imaging sets were compared by using the McNemar test. RESULTS: The diagnostic performance of FDG PET/CT plus delayed PET/MR imaging was not significantly different from that of CE-CT/MR imaging in the assessment of tumor resectability [area under the receiver operating characteristic curve: 0.927 vs. 0.925 (p = 0.975)], N stage (accuracy: 80% (16 of 20 patients) vs. 55% (11 of 20 patients), p = 0.125), and M stage (accuracy: 100% (40 of 40 patients) vs. 93% (37 of 40 patients), p = 0.250). Moreover, 14 of 40 patients had liver metastases. The number of liver metastases detected by CE-CT/MR imaging, PET/CT, and PET/MR imaging were 33, 18, and 61, respectively. Compared with CE-CT/MR imaging, PET/MR imaging resulted in additional findings of more liver metastases in 9/14 patients, of which 3 patients were upstaged. Compared with PET/CT, PET/MR imaging resulted in additional findings of more liver metastases in 12/14 patients, of which 6 patients were upstaged. CONCLUSIONS: Although FDG PET/CT plus delayed PET/MR imaging showed a diagnostic performance similar to that of CE-CT/MR imaging in the pretherapeutic assessment of the resectability and staging of pancreatic tumors, it still has potential as the more efficient and reasonable work-up approach for the additional value of metastatic information provided by delayed PET/MR imaging.

Thoracic paravertebral involvement in patients with IgG4-related disease: CT and MR imaging findings.

OBJECTIVES: To retrospectively evaluate CT and magnetic resonance (MR) imaging thoracic paravertebral findings at baseline and follow-up in patients with IgG4-related disease. METHODS: The study consisted of 36 patients with IgG4-related disease involving thoracic paravertebral regions (32 men and four women; mean age, 58 years; range, 25-78 years). A total of 36 patients underwent CT or MR imaging at baseline; 20 patients underwent follow-up. CT and MR images were reviewed and analysed in consensus by two radiologists for the various features of thoracic paravertebral lesions. RESULTS: All lesions were located around two or more thoracic vertebrae, particularly the lower thoracic vertebrae (n = 36). The right side of vertebrae was predominantly affected in all cases (n = 36). Radiologically, IgG4-related thoracic paravertebral lesions were categorized into three types: solitary or multiple saddle-like masses type (32 patients); multiple nodules type (three patients); and invasively irregular mass type (three patients). All the types showed soft-tissue density on CT images, isointense on T1- and T2-weighted images, and homogeneous enhancement with penetration of small arteries in the lesions on contrast-enhanced CT and MR images. Steroid therapy administered to 20 patients dramatically diminished the mean maximum thickness in 18 patients. One patient with T7-12 vertebrae involved improved after steroid therapy. CONCLUSION: IgG4-related paravertebral lesions occur mainly around the right side of the lower thoracic vertebrae and manifest as three major patterns of CT and MR imaging findings. Recognition of these diagnostic features will assist in the diagnosis and treatment of IgG4-related disease.

Lumbar Vertebrae Morphological Analysis and an Additional Approach for Vertebrae Identification in Lumbar Spine DXA Images.

INTRODUCTION: Bone mineral density measured by dual-energy X-ray absorptiometry (DXA) is the gold standard for diagnosis of osteoporosis. However, DXA interpretation can be influenced by analytic errors which can, in turn, interfere with diagnostic accuracy. One such source of potential error is the accurate identification of specific lumbar vertebrae on lumbar spine DXA images. Although the International Society for Clinical Densitometry has introduced several approaches to aid with this process, there remain individuals whose lumbar vertebrae cannot be labeled with certainty using these approaches. We designed the present study to systematically evaluate lumbar spine DXA images among a large cohort of Chinese patients and present an additional simple strategy for identifying L5 named the "em dash"-shaped L5 or "-"-shaped L5, based upon the two-dimensional relationship between the bilateral pedicles with the central spinous process on DXA images. METHODOLOGY: Lumbar spine posteroanterior DXA images from adult patients receiving care at a large tertiary hospital in Beijing, China from May to August 2016 were retrospectively reviewed. For each patient, data were collected regarding key anatomic features seen on DXA (positions of the most superior portions of the iliac crests, the lowest vertebra with ribs, and the longest transverse processes) and the proportion of patients presenting with "H"-shaped L4 and "-"-shaped L5. Chi-squared analyses were used to compare proportions across age strata. RESULTS: DXA images from 1125 patients (79.6% female) were evaluated. The mean age of patients was 52.5 ± 14.8 yr (range: 19-90 yr). A horizontal line drawn across the superior-most portions of the iliac crests crossed the disk space between L4 and L5 among 78.3% of patients. The lowest ribs were most frequently (83.9%) observed at T12. Almost 80% of individuals had the longest transverse processes at L3. L4 was predominantly "H"-shaped (73.3%), however we found that the proportion of individuals with "H"-shaped L4 decreased steadily after 50 yr of age (p < 0.001). By contrast we observed that L5 was predominantly "-"-shaped (80.3%), with no significant differences in proportions across all age strata (p = 0.063). CONCLUSIONS: The "-"-shaped L5 can be incorporated as an additional reference tool for lumbar spine DXA image analysis and may be particularly helpful for lumbar vertebrae identification among patients over 50 yr of age.

Impact of reference point selection on DXA-based measurement of forearm bone mineral density.

Few studies have systematically evaluated the technical aspects of forearm bone mineral density (BMD) measurement. We found that BMD remained stable regardless of the reference point; however, the ROI identified was not always consistent. Our study highlights the importance of using the same reference point for serial measurements of forearm BMD. BACKGROUND: Forearm fractures are clinically important outcomes from the perspective of morbidity, health care costs, and interruption of work. BMD of the forearm, as derived by dual-energy x-ray absorptiometry (DXA), is a better predictor of fracture at the forearm compared with BMD measured at other sites. However, very few studies have evaluated the technical aspects of selecting the ROI for forearm BMD measurement. This study aimed to compare the BMD values measured at the 1/3 radius site using three different reference points: the ulnar styloid process, the radial endplate, and the bifurcation of the ulna and radius. METHODOLOGY: Healthy Chinese patients participating in the control group of an ongoing study at Zhejiang Provincial People's Hospital were recruited for this study. For each patient, a DXA scan (GE Lunar Prodigy) of the forearm was performed and BMD values were separately calculated using each of the three reference points to identify the ROI. Pearson correlation coefficients were calculated to examine the correlation between the BMD measures derived from each reference point. The F test and independent t test were applied for more robust analysis of the differences in the variances and means. RESULTS: Sixty-eight healthy Chinese volunteers agreed to participate in this study. The root mean square standard deviation (RMS-SD) percentages of BMD values measured at the 1/3 radius site were 2.19%, 2.23%, and 2.20% when using the ulnar styloid process, radial endplate, and the bifurcation of the ulna and radius as the reference points, respectively. Pearson's correlation coefficients for all pairwise comparisons among these three groups were greater than 0.99. F tests and independent t tests showed p values ranging from 0.92 to 0.99. However, we observed that among 10% of patients, choosing an ROI at the ulnar styloid process led to an inability to accurately determine the BMD at the ultra-distal radius. CONCLUSIONS: Given equal ability to determine BMD at the 1/3 radius accurately, the radial endplate or the bifurcation of the ulna and radius should be preferentially selected as the reference point for routine forearm BMD measurements in order to avoid situations in which the ultra-distal radius BMD cannot be determined.

Ring sign: an imaging sign for osteochondromyxoma in Carney complex.

BACKGROUND: To introduce the concept of "ring sign" and to evaluate its role in the diagnostic imaging of osteochondromyxoma in patients with Carney complex (CNC). METHODS: Three patients presenting osteochondromyxoma with CNC were included for evaluation of the ring sign on different imaging modalities, including radiographs, computed tomography, and magnetic resonance imaging. The literature was reviewed for further discussion. RESULTS: The ring sign in osteochondromyxoma could be seen on radiographs, computed tomography, and magnetic resonance images in all three patients at each of the involved sites. The morphological patterns and the number of ring signs varied by case, site, and imaging method. Some previous reports have shown images that contain ring signs, but do not identify them as such. CONCLUSIONS: Ring sign can be considered as a relatively specific sign, helpful for the imaging diagnosis of osteochondromyxoma in the CNC.