Images in cardiovascular medicine. A swelling of the right neck and sudden death.
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Aneurysm↗
Biomedical subjects
Gert Hein
Publications and source records attributed to Gert Hein.
The potential of digital X-ray radiogrammetry (DXR) in the assessment of osteopenia in children with chronic inflammatory bowel disease.
BACKGROUND: Loss of bone mass is a known complication of chronic inflammatory bowel disease (IBD) in children. The gold standard in the evaluation of bone mineral density (BMD) is dual energy X-ray absorptiometry (DXA). OBJECTIVE: In this preliminary study we evaluated digital X-ray radiogrammetry (DXR) which estimates BMD (DXR-BMD) from hand radiographs in children with IBD. MATERIALS AND METHODS: A total of 26 children with IBD (10 girls, 16 boys; age range 10-18 years) underwent DXR for the calculation of DXR-BMD and metacarpal index (DXR-MCI) using the Pronosco X-posure system. The results were compared with a local reference database and correlated with the results of DXA. RESULTS: DXR-BMD was 0.36-0.56 g/cm(2) (median 0.46 g/cm(2)) in Crohn disease patients and 0.38-0.63 g/cm(2) (median 0.48 g/cm(2)) in ulcerative colitis patients. DXR-MCI was 0.29-0.49 in Crohn disease patients and 0.28-0.53 in ulcerative colitis patients. The Z-scores were reduced to <-1 SD in five Crohn disease patients and in six ulcerative colitis patients. The coefficients (r) for the correlations between DXR-BMD and DXA-BMD were 0.78 for the lumbar spine and 0.61 for the proximal femur (P<0.01), and between DXR-MCI and DXA-BMD were 0.78 for the lumbar spine and 0.51 for the proximal femur (P<0.01). CONCLUSIONS: DXR seems to be able to estimate cortical osteopenia in children with chronic IBD. The DXR results showed a positive correlation with DXA results.
Absorptiometry, Photon↗
Hypercalcemia in rheumatoid arthritis: relationship with disease activity and bone metabolism.
To investigate the relationship between ionized calcium and disease activity, parameters of bone metabolism and bone mineral density (BMD) at the lumbar spine (BMD-LS) and the femoral neck (BMD-FN) measured by dual X-ray absorptiometry in rheumatoid arthritis (RA). In 146 patients with RA, the following parameters were investigated: serum levels of ionized calcium, total calcium, vitamin D metabolites 25-hydroxyvitamin D3 (25D3) and 1,25-dihydroxyvitamin D3 (1,25D3), intact parathyroid hormone (iPTH), interleukin-6, osteocalcin, erythrocyte sedimentation rate (ESR) and c-reactive protein (CRP); renal excretion of pyridinolin (PYD)- and desoxypyridinolin (DPD)-crosslinks. A total of 30.1% of the patients were hypercalcemic (ionized calcium >1.30 mmol/l). In comparison with normocalcemic patients, those with hypercalcemia had significantly higher ESR (P<0.01) and CRP values (P<0.05) and significantly lower serum levels of both iPTH (P<0.01) and 1,25D3 (P<0.05) and a significantly lower BMD-LS (P<0.05). The results indicate that a substantial part of RA patients is hypercalcemic. Hypercalcemia is associated with high disease activity and may contribute to suppression of PTH secretion and vitamin D hormone synthesis. High levels of ionized calcium may be a reflection of disease-activity-related systemic bone loss, and could be a predictor of BMD at the lumbar spine in RA.
Absorptiometry, Photon↗
Computerized digital imaging techniques provided by digital X-ray radiogrammetry as new diagnostic tool in rheumatoid arthritis.
PURPOSE: Our study evaluates digital x-ray radiogrammetry (DXR) and Radiogrammetry Kit (RK) as a new diagnostic method for the measurement of disease-related osteoporosis including quantification of joint space narrowing dependent on the severity of rheumatoid arthritis (RA). MATERIALS AND METHODS: A total of 172 unselected patients with RA underwent computerized measurements of bone mineral density (BMD) and metacarpal index (MCI) by DXR, as well as a semiautomated measurement of joint space distances at the metacarpal-phalangeal articulation (JSD-MCP 2-5), both were analyzed from plain radiographs of the nondominant hand. RESULTS: Correlations between DXR-BMD and DXR-MCI vs. parameters of RK were all significant (0.34 < R < 0.61; p < 0.01). An expected negative association was observed between RK parameters and the different scoring methods (-0.27 < R < -0.59). The maximum relative decrease in BMD vs. MCI as measured by DXR between the highest and lowest RA severity group was -27.7% vs. -27.5% (p < 0.01) for the modified Larsen Score, whereas the minimal value of relative DXR-BMD and DXR-MCI reduction could be documented for the Sharp Erosion Score (-20.8% vs. -26.8%; p < 0.01). The relative reduction of mean JSD-MCP using RK significantly varied from -25.0% (Sharp Erosion Score) to -41.2% (modified Larsen Score). In addition, an excellent reproducibility of DXR and RK could be verified. CONCLUSION: DXR in combination with RK could be a promising, widely available diagnostic tool to supplement the different scoring methods of RA with quantitative data, allowing an earlier and improved diagnosis and more precision in determining disease progression.
Adolescent↗
Influence of image-capturing parameters on digital X-ray radiogrammetry.
The purpose of this study was to evaluate the importance of different image-capturing conditions, which might influence the characteristics of radiographs and, consequently, impact calculations of bone mineral density (BMD) and Metacarpal Index (MCI) using digital X-ray radiogrammetry (DXR). Radiographs of the left hand of deceased males were acquired three times using systematically varied parameters: 4-8 miliamp seconds (mA); 40-52 kV; film-focus distance (FFD); 90-130 cm; film sensitivity, 200/400; and different image modalities (conventional vs original digital radiographs as well as digital printouts). Furthermore, the interradiograph reproducibility using both conventional equipment and printouts vs originals of digital images and the intraradiograph reproducibility (either conventional or digital printouts) were evaluated. All BMD and MCI measurements were obtained with the DXR technology. The interradiograph reproducibility of DXR-BMD using conventional images under standardized conditions (6 mAs; 42 kV; 1 m FFD; film sensitivity of 200) was calculated to be coefficient of variation (CV) = 0.49% for Agfa Curix film and CV = 0.33% for Kodak T-MAT-Plus film, whereas reproducibility error using digital images ranged from CV = 0.57% (digital printouts; Philips) to CV = 1.50% (original digital images; Siemens). The intraradiograph reproducibility error was observed to be CV = 0.13% (conventional; Kodak film) vs CV = 0.27% (digital printouts; Philips). The BMD calculation was not noticeably affected by changes of FFD, exposure level, or film sensitivity/film brand, but was influenced by tube voltage (CV = 0.99% for Kodak film to CV = 2.05% for Siemens digital printouts). No significant differences were observed between the BMD and MCI data. DXR provides measurements of MCI and BMD with high precision and reproducibility. The measurements are unaffected by all tested image-capturing conditions, with the exception of tube voltage. In addition, different digital image devices clearly have an effect on DXR reproducibility.
Bone Density↗
Identification of the receptor for advanced glycation end products in synovial tissue of patients with rheumatoid arthritis.
OBJECTIVES: Generation of advanced glycation end products (AGE) is an inevitable process in vivo and can be accelerated under pathologic conditions such as oxidative stress, e.g. in rheumatoid arthritis (RA). This process is mediated by the AGE-specific receptor (RAGE). In this study we analysed the presence of RAGE in RA and osteoarthritic (OA) synovial tissue using immunohistology. METHODS: Frozen synovial tissue samples from 11 RA patients and 12 OA patients were treated with goat anti-RAGE immunoglobulin G (IgG) and rabbit antigoat IgG. Immunostaining was visualised with streptavidin horse radish peroxidase (chromogen amino-ethyl-carbazole). Cell differentiation was performed with antibodies against CD68, CD45RO, and CD20. RESULTS: In 9/11 RA and 8/12 OA synovial specimens, RAGE was detected in synovial lining, sublining, and stroma. In RA, many T cells (CD45RO(+)) and some macrophages (CD68(+)) showed positive immunostaining for RAGE, whereas B cells were mostly negative. We found no difference in staining patterns between the RA and OA samples. CONCLUSIONS: We detected RAGE in RA and OA synovial tissue. The presence of RAGE on macrophages, T cells, and some B cells suggests its role in the pathogenesis of inflammatory joint disease.
Aged↗
Orbital inflammatory pseudotumor due to hypersensitivity vasculitis and mononeuritis multiplex in a patient with atypical, cANCA-positive Wegener's granulomatosis.
OBJECTIVE: We report on a 60-year-old woman with a retro-orbital pseudotumor and polyneuropathy. The retro-orbital inflammation was histologically diagnosed as hypersensitivity vasculitis (HV). As cytoplasmatic antineutrophilic cytoplasmatic antibody (cANCA) and anti-proteinase-3 antibody were detected, the differential diagnosis also included atypical Wegener's granulomatosis. Hypersensitivity vasculitis is defined as small-vessel vasculitis mediated by the deposition of immune complexes (Arthus reaction) after exposure to various agents such as drugs, toxins, and infections. Since an inflammatory retro-orbital pseudotumor due to HV has not previously been reported, the following case is presented. METHODS AND MAIN OUTCOME MEASURES: Magnetic resonance imaging (MRI) revealed retro-orbital infiltrate without granuloma. Histology from an orbital biopsy confirmed HV. Electromyography was used for the diagnosis of polyneuropathy. Serum investigation indicated erythrocyte sedimentation rate (ESR) >100 mm/h, C-reactive protein (CRP) 223 mg/l, antinuclear antibodies 1:80, and cANCA 100 U/ml. RESULTS: The bilateral orbital pseudotumor, polyneuropathy, and serum levels of inflammation reactants (ESR and CRP) improved from therapy with corticosteroids (1 g of methylprednisolone initially) and azathioprine (150 mg/day). CONCLUSIONS: Because of cANCA and anti-proteinase-3 antibody positivity, this case can be viewed more as an atypical Wegener's granulomatosis than a systemic HV. The causal variety of inflammatory orbital pseudotumor, including HV and different therapeutic consequences, requires histological differentiation from usual orbital pseudotumors.
Antibodies, Antineutrophil Cytoplasmic↗
[Hypersensitivity vasculitis].
BACKGROUND: Hypersensitivity vasculitis (leukocytoclastic vasculitis) is defined as small-vessel vasculitis mediated by deposition of immune complexes (Arthus reaction) after exposition to various agents, such as drugs, toxins and infections. Due to a wide spectrum of precipitating agents and symptoms, classification systems and synonyms, understanding of the disease, its diagnosis and therapeutic strategy are difficult. METHODS: Based on an extensive analysis of the literature, causal agents, etiopathogenesis as well as symptoms and their frequencies are summarized. The widespread differential diagnoses and the development of a diagnostic strategy for practical management of the disease are discussed. RESULTS AND CONCLUSIONS: The course of disease is generally benign, and spontaneous remission is often observed. Severe organ manifestations and chronic courses have, however, been described. Clinical diagnosis must be confirmed histopathologically. Once the diagnosis is made, search for etiologic agents (successful in only 50% of the cases) and an intensive organ diagnostics should be performed. Subsequently, treatment with steroids, antihistamines and slow-acting antirheumatic drugs such as cytotoxic substances and immunosuppressive drugs is planned according to severity and prognosis.
Adrenal Cortex Hormones↗
Synovial fibroblasts and synovial macrophages from patients with rheumatoid arthritis and other inflammatory joint diseases show chromosomal aberrations.
Chromosomal aberrations were investigated in nuclei extracted from synovial tissue and first-passage synovial fibroblasts (P-1 SFB, 98% enrichment) or macrophages (P-1 Mphi) from patients with rheumatoid arthritis (n=10). The findings were compared with those in other rheumatic diseases (osteoarthritis, n=14; reactive arthritis, n=1), as well as with those in chronic obstructive pulmonary disease (n=8). Controls were paired peripheral blood lymphocytes from arthritic patients, synovial tissue or SFB/Mphi from joint trauma/normals (n=9), and peripheral blood monocytes from normal donors (n=10). GTG banding of metaphase chromosomes and interphase fluorescence in situ hybridization with centromere-specific probes were used. Comparable chromosomal aberrations were observed in synovial tissue and P-1 SFB of patients with rheumatoid arthritis, osteoarthritis, and reactive arthritis (polysomy 7 and aneusomies of chromosomes 4, 8, 9, 12, and 18). Notably, aneusomies of chromosomes 4, 6, 7, 8, 9, 11, 12, and/or X were also detected in P-1 synovial Mphi from rheumatoid arthritis (90% of the cases), osteoarthritis (93%), and reactive arthritis (1/1), as well as bronchial Mphi from chronic obstructive pulmonary disease (25%). No aberrations were detected in paired peripheral blood lymphocytes (except for one osteoarthritis case with a karyotype 45,X[10]/46,XX[17]), or in peripheral blood monocytes and synovial tissue of normals/joint trauma. Because Mphi aberrations were common to chronic joint and pulmonary disease, chronic inflammatory stress may induce chromosomal aberrations with potential functional relevance in local mesenchymal cells and infiltrating leukocytes in an organ-independent fashion.
Adult↗
Cytokines, osteoprotegerin, and RANKL in vitro and histomorphometric indices of bone turnover in patients with different bone diseases.
Cytokines are supposed to play an essential role in the regulation of the bone metabolic unit. However, information on cytokine production of primary human osteoblasts from patients with metabolic bone disease is scarce, and few attempts have been made to correlate such data to histomorphometric parameters of individual patients. We investigated 11 patients with metabolic bone disease referred to our outpatient department for bone biopsy and analyzed interleukin (IL)-1, IL-6, and TNF-alpha protein release and gene expression in primary osteoblast cultures. Compared with four controls, five patients showed normal cytokine protein release, whereas six patients showed much higher levels of interleukin-6 (26-fold) and TNF-alpha (84-fold). All three cytokines were strongly correlated concerning gene expression and/or protein levels (r = 0.72-0.96). Histomorphometric analysis of the bone samples showed that eroded surface (ES/BS) as a parameter of bone resorption was significantly associated with TNF-a. In addition, RANKL gene expression was positively associated with ES/BS and osteoclast surface (Oc.S/BS). Finally, the formation parameters osteoid volume and osteoid surface were negatively associated with TNF-alpha. In conclusion, in an in vitro-ex vivo model of bone cells obtained from a group of 11 patients with different forms of metabolic bone disease, cytokine release in conditioned medium was significantly associated with bone resorption and bone formation, as quantified by histomorphometry. TNF-alpha seemed to be the more important cytokine; its effect on bone resorption could be mediated by RANKL.
Adult↗
Relation between body mass index and radiological progression in patients with rheumatoid arthritis.
OBJECTIVE: To determine if there is an influence of body mass index (BMI) on the radiological progression in early and longer duration rheumatoid arthritis (RA). METHODS: Fifty-four patients with RA were observed in a progressive 2 year followup for radiological progression of joint damage. At the beginning of study, 27 (50%) patients had a duration of complaints less than 6 months, grouped as early RA. BMI at the beginning and end of the study were monitored, together with HLA-DRB1 alleles, initial joint erosions, duration of disease, age, sex, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP). Outcome was defined as radiographic damage according to yearly increase of Larsen score. RESULTS: Increased radiographic joint damage of patients was significantly correlated with lower BMI at the beginning of the study (r = 0.363, p < 0.05), the presence of initial joint erosions (r = 0.341, p < 0.01), ESR (r = 0.315, p < 0.05), and CRP at study entry (r = 0.427, p < 0.01). Patients with an increase of Larsen score > or = 5.8/year were found to have a lower weight at the beginning of their complaints (BMI 24.8 +/- 4.7 vs 27.8 +/- 3.8; p < 0.05) as well as after the time of observation (BMI 24.6 +/- 3.7 vs 27.6 +/- 4.9; p < 0.05). Stepwise logistic regression analysis revealed a BMI < 27 at the beginning of disease (beta = 2.04, p = 0.003, odds ratio = 7.69), the presence of HLA-DR4 shared epitope (beta = 1.76, p = 0.015, OR 5.82), and joint erosions at study entry (beta = 1.56, p = 0.044, OR 4.78) as significant predictors for rapid joint damage. CONCLUSION: Together with the presence of HLA-DR4 shared epitope and erosive disease at study entry, a low BMI at the beginning of RA was found in association with higher radiographic progression in RA. Accordingly, BMI could be of interest as a sensitive and inflammation-independent predictor for radiological outcome of RA.
Adult↗
Validated capillary gas chromatographic-mass spectrometric assay to determine 2-methylcitric acid I and II levels in human serum by using a pulsed splitless injection procedure.
BACKGROUND: Despite some clinical applications of 2-methylcitric acid (2-MCA) determination in urine and amniotic fluid, a diagnostic use of 2-MCA levels in serum is not common practice. This could be related to the complexity of the assay, or possibly to unawareness of other feasible clinical applications. METHODS: The levels of the diastereomers 2-MCA I and II in human serum were determined by GC-MS based on a method using a pulsed splitless injection technique. A stable isotope dilution principle was modified considering the diastereomer ratio and impurities of the internal standard. Precision parameters as well as recovery rates of the assay were determined. Reference intervals for 2-MCA(total), 2-MCA I and II levels were obtained in 52 healthy volunteers (31 female, 21 male, mean age 41.7+/-14.4 years). RESULTS: 2-MCA was readily detected in each sample of serum, as well as in urine, cerebrospinal fluid and amniotic fluid. The limit of detection was 10 nmol/l for 2-MCA(total). The internal standard showed a diastereomer ratio of 2-MCA II-d3 to 2-MCA I-d3 of 0.83+/-0.05, its chemical purity had to be corrected to 90.5+/-0.5%. In concentrations of 446, 750 and 1256 nmol/l 2-MCA(total), recovery rates of 98.5, 93.7 and 88% with a mean intra-assay RSD of 1.5% were determined. The day-to-day precision was 10% RSD (SD 40 nmol/l) for 2-MCA(total) obtained with a pooled serum sample at a concentration of 401 nmol/l 2-MCA(total) over a period of 5 months (n=17). The normal range for 2-MCA(total) in human serum was calculated as 81-266 nmol/l confirming previous findings. CONCLUSIONS: The GC-MS assay using a pulsed splitless injection procedure ensures a good response to differing concentrations of 2-MCA in various specimens. Considering exact determination of the diastereomer ratio as well as the purity of the internal standard, the assay offers good precision and recovery for 2-MCA I and II levels in serum.
Adult↗
Lupus erythematosus: uncommon presentations.
Lupus erythematosus is the great imitator of other diseases. There is a broad range of cutaneous symptoms induced by lupus or by its various treatments. This article provides a short overview of uncommon presentations of cutaneous symptoms in the lupus spectrum.
Humans↗
Normative data for digital X-ray radiogrammetry from a female and male German cohort.
This study presents German reference data for digital X-ray radiogrammetry (DXR) differentiated by males as well as females, and quantifies for gender-specific and age-related differences including all DXR parameters. This study also documents the effects of different X-ray settings (e.g., radiographs of the wrist or the hand) on DXR measurements. There were 2085 patients who were prospectively enrolled (954 females and 1131 males) from a data pool of 11,915 patients with radiographs of the nondominant hand or wrist. All patients underwent measurements of bone mineral density (BMD), cortical thickness, bone width, and the metacarpal index (MCI) using DXR technology. These data showed a continuous age-related increase of the DXR parameters to the point of peak bone mass, then a continuous decline beyond the peak bone mass with accentuated age-related cortical bone loss in women. Peak bone mass is reached at approximately 30-34 yr for women and 45-49 yr for men. In addition, men had a significantly higher DXR BMD (mean: +12.8%) compared with woman in all age groups. Regarding the impact of various X-ray settings (e.g., X-ray(wrist) vs. X-ray(hand)), no significant difference was observed between both groups, men as well as women. The development of digital imaging technology has enabled more precise measurements of several radio-geometric features. The present study estimated normative reference values for DXR in German Caucasian women and men. Based on this reference data, a valid and reliable quantification of disease-related demineralization based on measurements of DXR BMD and MCI is now available for the Caucasian ethnic group.
Absorptiometry, Photon↗