scholarly journals Csontanyagcsere felnőtt phenylketonuriás pácienseknél – hazai adatok

2017 ◽  
Vol 158 (47) ◽  
pp. 1868-1872 ◽  
Author(s):  
András Gellért Barta ◽  
Csaba Sumánszki ◽  
Péter Reismann

Abstract: Introduction: Patients with phenylketonuria have lower bone mineral density compared to healthy people, however, the ethiology of these alterations is not clear. Hungarian data were missing in this topic. Aim: The main aim of our study was to survey the correlation between metabolic control and change of bone mineral density in early treated Hungarian adult patients with phenylketonuria. Method: In this monocentric study bone mineral density of 59 adult PKU patients have been repeatedly measured in a 4-year interval using dual-energy X-ray absorptiometry. Two subgroups have been established based on average blood phenylalanine levels. The correlation between the change in bone mineral density and average phenylalanine, tyrosine concentrations have been determined while initial bone mineral density and change have also been examined in the subgroups. Results: Mean phenylalanine concentration was 614 (182–1222) micromol/L, whereas mean tyrosine concentration was 49 (24–99) micromol/L and the calculated ratio was 16 (4,5–35). Three patients have had severely decreased bone mineral density in either localisation while 22 have had mild decrease. Low bone mineral density compared to cronological age has been found by 9 patient. The mean change was +0.0380 (–0.1550–0.7800) g/cm2 in femur, and +0.0120 (–0.57300–0.3130) g/cm2 in the lumbar spine. There was a correlation in the change in Z-score neither with mean phenylalanine nor with tyrosine concentration. Conclusions: Bone mineral density was not changed and hardly influenced by the metabolic control in early-treated young adult phenylketonuria patients in a few years interval. Orv Hetil. 2017; 158(47): 1868–1872.

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1340.1-1340
Author(s):  
E. Kirilova ◽  
N. Kirilov ◽  
S. Vladeva

Background:Radiofrequency Echographic Multi-Spectrometry (REMS) is a non-ionizing innovative approach for the assessment of REMS-based bone mineral density (BMD) of the axial skeleton. The principle of the REMS technology is based on the analysis of native raw unfiltered ultrasound signals during an echographic scan of the lumbar spine or the femoral neck [1]. Several studies demonstrated the high concordance with dual energy X-ray absorptiometry (DXA) in terms of measured BMD with this novel technology [2,3]. In previous published literature it was envisaged to apply this technology for the examination of the axial bone density in pregnant women. Pregnancy-associated bone loss has been demonstrated as decreased bone mineral density (BMD) in previous studies [4].Objectives:The aim of the current study is to compare the BMD values of both femora between pregnant and non-pregnant women matched for age and BMI using the novel REMS technology.Methods:Of total fifty women twenty pregnant women (40%) and thirty non-pregnant women (60%) were included in the study. The mean age of the pregnant women was 32 years ± 5 standard deviations (SD), (range 25-41 years) and the mean age of the non-pregnant women was 30 years ± 6 standard deviations (SD), (range 24-39 years). REMS approach was used to assess REMS-based BMD and REMS-based Z-score values of the femoral neck. Furthermore, body mass index (BMI) and gestational age in weeks were evaluated.Results:The mean BMI of the pregnant women was 26 kg/m2 ± 7 kg/m2 (range 14 kg/m2-42 kg/m2) and those of the non-pregnant women was 25 kg/m2 ± 5 kg/m2 (range 16 kg/m2-35 kg/m2). The mean gestational age was 20 weeks’ gestation ± 5 weeks’ gestation (range 13-27 weeks’ gestation). REMS-based mean BMD of the left femoral neck of the pregnant women was 0.793 g/cm2 ± 0.167 g/cm2 (range 0.563 g/cm2-1.154 g/cm2). REMS-based mean BMD of the right femoral neck of the pregnant women was 0.828 g/cm2 ± 0.153 g/cm2 (range 0.570 g/cm2-1.161 g/cm2). After comparing left femoral neck BMD with the right femoral neck BMD of the pregnant women, we found a linear correlation (R=0.764). Left femoral neck BMD value (0.793 g/cm2) of the pregnant women was significantly lower than those of the non-pregnant women (0.854 g/cm2), p=0.002. The mean left femoral Z-score of the pregnant women (-0.1 SD with range -2.5 SD-2.9 SD) was also significantly lower compared to those of the non-pregnant women (1.2 SD with range -1.5 SD-3.1 SD), p=0.003.Conclusion:This is the first study which provides data about BMD and Z-score values of both femora in pregnant women assessed with the radiation-free REMS technology. Pregnant women demonstrated significantly lower femoral neck BMD values and Z-scores compared to those of the non-pregnant women. Innovative REMS method could be very helpful for making decision about the treatment of pregnant women who are at risk of lower BMD due to concomitant diseases and/or treatment associated with osteoporosis.References:[1]Casciaro S, Conversano F, Pisani P, Muratore M. New perspectives in echographic diagnosis of osteoporosis on hip and spine. Clin Cases Miner Bone Metab. 2015; 12(2):142-150.[2]Nikolov M, Nikolov N. AB0908 Assessment of the impact of the lean mass with body composition by dual-energy x-ray absorptiometry on the bone mineral density. Annals of the Rheumatic Diseases 2020; 79:1756.[3]Chakova M., Chernev D., Kashukeeva P., Krustev P., Abedinov F. Lumbar Sympathectomy - Literature Review. International Journal of Science and Research (IJSR) Volume 7 Issue 8, August 2018 ISSN (Online): 2319-7064.[4]Degennaro, V. A.; Cagninelli, G.; Lombardi, F. A. “VP34.12: First assessment of maternal status during pregnancy by means of radiofrequency echographic multi-spectrometry technology”. Ultrasound in Obstetrics & Gynecology. 2020, 56 (S1): 199.Disclosure of Interests:None declared.


2016 ◽  
Vol 69 (suppl. 1) ◽  
pp. 85-88
Author(s):  
Radmila Matijevic ◽  
Vladimir Harhaji ◽  
Srdjan Ninkovic ◽  
Zoran Gojkovic ◽  
Predrag Rasovic ◽  
...  

Introduction. Osteoporosis is a metabolic skeletal disease characterized by bone mineral density reduction, which may lead to an increased risk of bone fractures. Obesity is a condition of excessive body fat that causes or aggravates many public health problems. As it is easy to be measured, body mass index is widely used as an index of the degree of obesity. Material and Methods. The study included 1.372 female orthopedic patients between the ages of 30 to 79 years who visited the Clinical Centre of Vojvodina in Novi Sad to have a dual-energy x-ray absorptiometry (DEXA) examination in the period from March, 2010 to June, 2013. The following anthropometric data were collected: body mass index, body weight, height, dual-energy x-ray absorptiometry T-score and bone mineral density (BMD), as well as some other data. Results. The mean age was 62.08 years, the mean weight was 73.59 kg and the mean height was 1.6 m. There were 392 participants in the group of normal body mass index, 14 participants were underweight, and 966 were overweight and obese. In the overweight and obese group, 25.25% participants had osteoporosis, 35.4% had osteopenia and 39.33% had the normal T-score. In the normal body mass index group, 42.34% of the participants had osteoporosis, 29.3% had osteopenia and 28.31 had the normal T-score. In the underweight group, 57.14% of the participants had osteoporosis, 21.42% had osteopenia and 21.42% had the normal T-score. Conclusion. No strong correlation between body mass index and bone mineral density was found in our study, but it is obvious that there was a stronger correlation between body mass index and bone mineral density of the total hip than between body mass index and bone mineral density of the lumbar spine.


2019 ◽  
Vol 22 (1) ◽  
pp. 41-46
Author(s):  
Madhuchhanda Hazra Mou ◽  
Sadia Sultana ◽  
Pupree Mutsuddy ◽  
Suraya Sarmin ◽  
Naheed Khan ◽  
...  

Objective: Worldwide, osteoporosis in postmenopausal women is a common public health problem. The measurement of bone mineral density (BMD) of spine and hip using central dual energy X-ray absorptiometry (DXA) is the most standard technique to diagnose osteoporosis. But in many circumstances, measurement of spine or hip BMD is quite difficult to carry out where wrist BMD can be used as an alternative. Moreover, wrist BMD can be done with smaller, cheaper, portable peripheral devices for screening osteoporosis at primary health care level. The objective of the study was to explore the agreement of wrist BMD with spine and hip BMD in postmenopausal women. Patients and Methods: This observational, cross sectional study was carried out at National Institute of Nuclear Medicine & Allied Sciences (NINMAS), Bangabandhu Sheikh Mujib Medical University (BSMMU) campus, from July 2017 to June 2018. A total of 110 postmenopausal women referred to NINMAS for BMD measurement were enrolled in this study after fulfilling selection criteria.  BMD of spine, right and left hip and wrist of enrolled subjects were measured by dual energy X-ray absorptiometry (DXA) scan by Stratos DR Bone densitometer. Results:  Agreement analysis done by kappa statistics revealed kappa value of 0.930 (p<0.05), 0.782 (p<0.05) and 0.635 (p<0.05) between wrist and spine BMD, wrist and right hip BMD and wrist and left hip BMD, respectively. In Bland-Altman analysis, the mean difference of T-score measured by wrist and spine was -0.007 ± 1.361, wrist and right hip was -0.645 ± 1.271 and wrist and left hip was -0.450 ± 1.259 with 95% of differences were found in between -2.674 and 2.660, -3.136 and 1.846 and -2.917 and 2.017, respectively. Positive significant Pearson’s correlation was observed between wrist BMD and spine BMD (r=0.664; p=0.001), wrist BMD and right hip BMD (r=0.719; p=0.001) and wrist BMD and left hip BMD (r=0.727; p=0.001). Conclusion: This study results showed an excellent kappa agreement between wrist and spine BMD with good kappa agreement of wrist with right and left hip BMD as well as in Bland-Altman analysis, the mean differences of T-score between wrist andspine, wrist and right hip and wrist and left hip were small. The biases between the methods were considered not significant suggesting that these sites can be used interchangeably for measurement of BMD. Hence, wrist BMD can be used with regular skeletal sites (spine and hip) as an effective method of diagnosing osteopenia and osteoporosis by DXA where spine or hip BMD is difficult to carry out as well as it can be used for osteoporosis screening at primary health care level by portable peripheral DXA device to initiate early treatment to reduce fracture risks. Bangladesh J. Nuclear Med. 22(1): 41-46, Jan 2019  


2002 ◽  
Vol 120 (1) ◽  
pp. 09-12 ◽  
Author(s):  
Lúcia Costa Paiva ◽  
Silvana Filardi ◽  
Aarão Mendes Pinto-Neto ◽  
Adil Samara ◽  
João Francisco Marques Neto

CONTEXT: Measurements of bone density taken by dual-energy x-ray absorptiometry are the most accurate procedure for the diagnosis of osteoporosis. This procedure has the disadvantage of measuring the density of all mineral components, including osteophytes, vascular and extra vertebral calcifications. These alterations can influence bone density results and densitometry interpretation. OBJECTIVE: To correlate radiography and densitometry findings from women with osteoporosis, analyzing the influence of degenerative processes and vertebral fractures on the evaluation of bone density. DESIGN: Retrospective study. SETTING: Osteoporosis outpatients' clinic at Hospital das Clínicas, Universidade Estadual de Campinas. PARTICIPANTS: Ninety-six postmenopausal women presenting osteoporosis diagnosed by bone density. MAIN MEASUREMENTS: Bone mineral density of the lumbar spine and femoral neck were measured by the technique of dual-energy x-ray absorptiometry, using a LUNAR-DPX densitometer. Fractures, osteophytes and aortic calcifications were evaluated by simple x-rays of the thoracic and lumbar spine. RESULTS: The x-rays confirmed vertebral fractures in 41.6%, osteophytes in 33.3% and calcifications of the aorta in 30.2%. The prevalence of fractures and aortic calcifications increased with age. The mean bone mineral density was 0.783g/cm² and the mean T-score was --3.47 DP. Neither fractures nor aortic calcifications had significant influence on bone mineral density (P = 0.36 and P = 0.09, respectively), despite the fractured vertebrae having greater bone mineral density (P < 0.02). Patients with lumbar spine osteophytes showed greater bone mineral density (P = 0.04). Osteophytosis was associated with lumbar spine bone mineral density after adjustment for fractures and aortic calcifications by multiple regression (P = 0.01). CONCLUSION: Osteophytes and lumbar spine fractures can overestimate bone density interpretation. The interpretation of densitometry results should be carried out together with the interpretation of a simple lumbar spine x-ray in elderly women.


1996 ◽  
Vol 14 (1) ◽  
pp. 78-84 ◽  
Author(s):  
T J Powles ◽  
T Hickish ◽  
J A Kanis ◽  
A Tidy ◽  
S Ashley

PURPOSE Tamoxifen is an effective treatment for metastatic and primary breast cancer and is now being evaluated as a chemoprevention agent in healthy women. Any long-term effects on estrogen-sensitive tissues such as bone may have important therapeutic implications. METHODS We measured bone mineral density (BMD) in the lumbar spine and hip using dual-energy x-ray absorptiometry (DXA) in premenopausal and postmenopausal healthy women who participated in our placebo-controlled tamoxifen chemoprevention of breast cancer trial. RESULTS BMD data are now available from 179 women for this analysis. In premenopausal women, BMD decreased progressively in the lumbar spine (P < .001) and in the hip (P < .05) for women on tamoxifen, but not those on placebo. The mean annual loss in lumbar BMD per year over the 3-year study period in tamoxifen-treated compliant women who remained premenopausal throughout the study period was 1.44% (1.88% calculated on an intent-to-treat basis) compared with a small gain of 0.24% per annum for women on placebo (P < .001). Tamoxifen had the opposite effect in postmenopausal women. The mean annual increase in BMD for women on tamoxifen was 1.17% in the spine (P < .005) and 1.71% in the hip (P < .001) compared with a noninsignificant loss for women on placebo. CONCLUSION These results indicate that tamoxifen treatment is associated with a significant loss of BMD in premenopausal women, whereas it prevents bone loss in postmenopausal women. These adverse and beneficial effects of tamoxifen should be considered in the assessment of the therapeutic benefits for both the adjuvant treatment and the chemoprevention of breast cancer.


Author(s):  
Gabriella Martino ◽  
Federica Bellone ◽  
Carmelo M. Vicario ◽  
Agostino Gaudio ◽  
Andrea Caputo ◽  
...  

Clinical psychological factors may predict medical diseases. Anxiety level has been associated with osteoporosis, but its role on bone mineral density (BMD) change is still unknown. This study aimed to investigate the association between anxiety levels and both adherence and treatment response to oral bisphosphonates (BPs) in postmenopausal osteoporosis. BMD and anxiety levels were evaluated trough dual-energy X-ray absorptiometry and the Hamilton Anxiety Rating Scale (HAM-A), respectively. Participants received weekly medication with alendronate or risedronate and were grouped according to the HAM-A scores into tertiles (HAM-A 3 > HAM-A 2 > HAM-A 1). After 24 months, BMD changes were different among the HAM-A tertiles. The median lumbar BMD change was significantly greater in both the HAM-A 2 and HAM-A 3 in comparison with the HAM-A 1. The same trend was observed for femoral BMD change. Adherence to BPs was >75% in 68% of patients in the HAM-A 1, 79% of patients in the HAM-A 2, and 89% of patients in the HAM-A 3 (p = 0.0014). After correcting for age, body mass index, depressive symptoms, and the 10-yr. probability of osteoporotic fractures, anxiety levels independently predicted lumbar BMD change (β = 0.3417, SE 0.145, p = 0.02). In conclusion, women with higher anxiety levels reported greater BMD improvement, highlighting that anxiety was associated with adherence and response to osteoporosis medical treatment, although further research on this topic is needed.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1188.1-1188
Author(s):  
C. Daldoul ◽  
N. El Amri ◽  
K. Baccouche ◽  
H. Zeglaoui ◽  
E. Bouajina

Background:Inflammatory bowel disease (IBD), including ulcerative colitis (UC) and Crohn’s disease (CD), is considered as a risk factor of low bone mineral density (BMD). In fact, the prevalence of osteoporosis ranges from 17% to 41% in IBD patients. The possible contributing factors may include malabsorption, glucocorticoid treatment and coexisting comorbiditiesObjectives:The purpose of our work was to determine the frequency and the determinants of osteoporosis in patients with IBD and to assess whether there is a difference in BMD status between UC and CD.Methods:This is a retrospective study, over a period of 5 years (from January 2014 to December 2018) and including patients followed for IBD who had a measurement of BMD by DEXA. Clinical, anthropometric and densitometric data (BMD at the femoral and vertebral site) were recorded. The WHO criteria for the definition of osteoporosis and osteopenia were applied.Results:One hundred and five patients were collected; among them 45 were men and 60 were women. The average age was 45.89 years old. The average body mass index (BMI) was 25.81 kg/m2 [16.44-44.15]. CD and UC were diagnosed in respectively 57.1% and 42.9%. A personal history of fragility fracture was noted in 4.8%. Hypothyroidism was associated in one case. Early menopause was recorded in 7.6%. 46.8% patients were treated with corticosteroids. The mean BMD at the vertebral site was 1.023 g/cm3 [0.569-1.489 g/cm3]. Mean BMD at the femoral site was 0.920g/cm3 [0.553-1.286g / cm3]. The mean T-score at the femoral site and the vertebral site were -1.04 SD and -1.27 SD, respectively. Osteoporosis was found in 25.7% and osteopenia in 37.1%. Osteoporosis among CD and UC patients was found in respectively 63% and 37%. The age of the osteoporotic patients was significantly higher compared to those who were not osteoporotic (52.23 vs 43.67 years, p = 0.01). We found a significantly higher percentage of osteoporosis among men compared to women (35.6% vs 18.3%, p=0.046). The BMI was significantly lower in the osteoporotic patients (23.87 vs 26.48 kg/m2, p=0.035) and we found a significant correlation between BMI and BMD at the femoral site (p=0.01). No increase in the frequency of osteoporosis was noted in patients treated with corticosteroids (27.9% vs 21.6%, p=0.479). Comparing the UC and CD patients, no difference was found in baseline characteristics, use of steroids or history of fracture. No statistically significant difference was found between UC and CD patients for osteoporosis(p=0.478), BMD at the femoral site (p=0.529) and at the vertebral site (p=0.568).Conclusion:Osteoporosis was found in 25.7% of IBD patients without any difference between CD and UC. This decline does not seem to be related to the treatment with corticosteroids but rather to the disease itself. Hence the interest of an early screening of this silent disease.Disclosure of Interests:None declared


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 227.2-228
Author(s):  
D. Claire ◽  
M. Geoffroy ◽  
L. Kanagaratnam ◽  
C. Isabelle ◽  
A. Hittinger ◽  
...  

Background:Dual energy X-ray absoprtiometry is the reference method to mesure bone mineral density (1). Loss of bone mineral density is significant if it exceeds the least significant change. The threshold value used in general population is 0,03 g/cm2 (2). Patients with obesity are known for having a higher bone mineral density due to metabolism and physiopathology characteristics (3,4).Objectives:The aim of our study was to determine the least significant change in bone densitometry in patients with obesity.Methods:We conducted an interventionnal study in 120 patients with obesity who performed a bone densitometry. We measured twice the bone mineral density at the lumbar spine, the femoral neck and the total hip in the same time (5,6). We determined the least significant change in bone densitometry from each pair of measurements, using the Bland and Altman method. We also determined the least significant change in bone densitometry according to each stage of obesity.Results:The least significant change in bone densitometry in patients with obesity is 0,046g/cm2 at the lumbar spine, 0.069 g/cm2 at the femoral neck and 0.06 g/cm2 at the total hip.Conclusion:The least significant change in bone densitometry in patients with obesity is higher than in general population. These results may improve DXA interpretation in this specific population, and may personnalize their medical care.References:[1]Lees B, Stevenson JC. An evaluation of dual-energy X-ray absorptiometry and comparison with dual-photon absorptiometry. Osteoporos Int. mai 1992;2(3):146-52.[2]Briot K, Roux C, Thomas T, Blain H, Buchon D, Chapurlat R, et al. Actualisation 2018 des recommandations françaises du traitement de l’ostéoporose post-ménopausique. Rev Rhum. oct 2018;85(5):428-40.[3]Shapses SA, Pop LC, Wang Y. Obesity is a concern for bone health with aging. Nutr Res N Y N. mars 2017;39:1-13.[4]Savvidis C, Tournis S, Dede AD. Obesity and bone metabolism. Hormones. juin 2018;17(2):205-17.[5]Roux C, Garnero P, Thomas T, Sabatier J-P, Orcel P, Audran M, et al. Recommendations for monitoring antiresorptive therapies in postmenopausal osteoporosis. Jt Bone Spine Rev Rhum. janv 2005;72(1):26-31.[6]Ravaud P, Reny JL, Giraudeau B, Porcher R, Dougados M, Roux C. Individual smallest detectable difference in bone mineral density measurements. J Bone Miner Res. août 1999;14(8):1449-56.Disclosure of Interests:None declared.


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