scholarly journals Bone mineral density and explanatory factors in children and adults with juvenile dermatomyositis at long term follow-up; a cross sectional study

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Henriette Schermacher Marstein ◽  
Kristin Godang ◽  
Berit Flatø ◽  
Ivar Sjaastad ◽  
Jens Bollerslev ◽  
...  

Abstract Background Juvenile dermatomyositis (JDM) is the most common idiopathic inflammatory myopathy in children and adolescents. Both the disease and its treatment with glucocorticoids may negatively impact bone formation. In this study we compare BMD in patients (children/adolescence and adults) with long-standing JDM with matched controls; and in patients, explore how general/disease characteristics and bone turnover markers are associated with BMD. Methods JDM patients (n = 59) were examined median 16.8y (range 6.6–27.0y) after disease onset and compared with 59 age/sex-matched controls. Dual-energy X-ray absorptiometry (DXA) was used to measure BMD of the whole body and lumbar spine (spine) in all participants, and of ultra-distal radius, forearm and total hip in participants ≥20y only. Markers of bone turnover were analysed, and associations with outcomes explored. Results Reduced BMD Z-scores (<−1SD) were found in 19 and 29% of patients and 7 and 9% of controls in whole body and spine, respectively (p-values < 0.05). BMD and BMD Z-scores for whole body and spine were lower in all patients and for < 20y compared with their respective controls. In participants ≥20y, only BMD and BMD Z-score of forearm were lower in the patients versus controls. In patients, BMD Z-scores for whole body and/or spine were found to correlate negatively with prednisolone use at follow-up (yes/no) (age < 20y), inflammatory markers (age ≥ 20y) and levels of interferon gamma-induced protein 10 (IP-10) (both age groups). In all patients, prednisolone use at follow-up (yes/no) and age ≥ 20y were independent correlates of lower BMD Z-scores for whole body and spine, respectively. Conclusion In long-term JDM, children have more impairment of BMD than adults in spine and whole-body. Associations with BMD were found for both prednisolone and inflammatory markers, and a novel association was discovered with the biomarker of JDM activity, IP-10.

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Juraj Payer ◽  
Martin Kužma ◽  
Daša Stojkovičová ◽  
Juraj Smaha ◽  
Peter Jackuliak ◽  
...  

Abstract Introduction: Adult growth hormone deficiency (AGHD) is associated with lower bone mass and likely with increased risk of fragility fractures. GH replacement leads to increase in bone mineral density (BMD).However, only few studies longer than 2 years exist. Aim: To assess long-term effect of recombinant GH replacement on BMD and bone turnover markers duringperiod of 8 years. Patients & Methods: Prospective follow-up of all (N=63) AGHD patients at one single center. All patients with adult GHD followed at single center. All participants were replaced with daily injection of recombinant human (rh) GH in IGF-1 normalizing regimen according to Endocrine Society Guidelines. Every 2 years, lumbar spine (L-spine) and total hip (TH) BMD using dual X-ray absorptiometry on Hologic Discovery device, was assessed. All patients were assessed for bone turnover markers; carboxy-terminal collagen crosslinks (CTx) and osteocalcin (OC), and 25(OH)D levels. Deficiencies of other pituitary axes were treated if necessary. All patients were supplemented with 800 IU /day of cholecalciferol and 1000-1200mg/day of calcium as recommended by International Osteoporosis Foundation. Results: Study group consisted of 38 males and 25 females (35 with adult onset (AO) /28 with childhood onset (CO); mean age at diagnosis 25,1 yrs) AGHD patients. All patients ended 8 years follow-up period without any treatment discontinuation during this period. Treatment was well tolerated, without any serious adverse event. IGF-1 has reached the normal ranges during first 6 months and remains normal during whole study period documenting good adherence to treatment (average dose of rhGH=0,4 mg/day). Both, L-spine and TH BMD increased significantly after 8 years of GH replacement (+8 % for L-spine BMD, +7,7% for TH BMD, both p&lt;0,01). The highest peak of BMD was observed after 6 years of treatment. CTx increased by 35% (p&lt;0,05) and remain stable, and no significant change in OC was observed during study period. Levels of 25(OH)D increased by 32% (p&lt;0,05) from baseline. No clinical fractures were observed. Conclusion: Long-term GH replacement in adult GHD together with sufficient levels of vitamin D levels led to increase in BMD and CTx. This study supported fact that GH has sustained effect on bone mass and bone turnover and is safe and well -tolerated for the long time period.


2011 ◽  
Vol 164 (4) ◽  
pp. 475-483 ◽  
Author(s):  
M J E Wassenaar ◽  
N R Biermasz ◽  
N A T Hamdy ◽  
M C Zillikens ◽  
J B J van Meurs ◽  
...  

ObjectiveTo establish the prevalence of osteoporosis, vertebral fractures (VFs), and non-VFs in acromegaly patients with long-term controlled disease and factors potentially influencing fracture risk.DesignCase–control study.Patients and measurementsEighty-nine patients (46% male, mean age: 58 years) were included. We studied VFs and non-VFs, bone mineral density (BMD), and markers of bone turnover. In 48 patients, BMD assessment was also obtained 7 years prior to the current study. To compare VF prevalence, data from a sample of the Dutch population (n=3469) were used.ResultsVF prevalence was 59% (men 64% and women 54%), significantly increased when compared with controls (odds ratio up to 6.5), and independent of the duration of disease control, BMD, markers of bone turnover, and acromegalic disease characteristics. Mean number of VFs per patient was 3.4±0.3 (range 1–8). There was no relationship between the number and severity of fractures, parameters of bone turnover, and follow-up BMD measurements. BMD did not change during prolongation of follow-up by 7 years of controlled acromegaly.ConclusionThere is a very high prevalence of VFs in acromegaly patients with long-term controlled disease, independently of BMD. In view of the significant morbidity and mortality associated with VFs in general and the inability of BMD to predict fracture risk in acromegalic patients, we propose to include VF assessment, for example by lateral conventional radiographs of the spine in the screening of patients with acromegaly, both at diagnosis and during follow-up after establishment of disease control.


Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 4472-4472
Author(s):  
Amy Garee ◽  
Micah Skeens ◽  
Sasigarn Bowden ◽  
Manmohan Kamboj ◽  
Sally Wildman ◽  
...  

Abstract Abstract 4472 Introduction: Children undergoing hematopoietic stem cell transplantation (HSCT) are at risk of developing vitamin D deficiency (VDD). However, data on vitamin D status and its correlation with bone mineral density (BMD) in the long term survivors after childhood HSCT is limited. The aim of this study was to determine the prevalence of VDD among long term survivors after HSCT in childhood, and to evaluate the correlations between vitamin D and BMD. Methods: A retrospective study was carried out in patients seen in Long Term follow-up Clinic (LTFC) at our institution from January 2011 to July 2012. Vitamin D deficiency (VDD) and insufficiency (VDI) were defined as serum 25-hydroxyvitamin D (25-OHD) <15 ng/mL and 15–30 ng/mL, respectively. BMD was measured using dual-energy radiograph absorptiometry (Hologic Delphi). Lumbar, total body, and hip BMD Z scores were determined using manufacturer's normative data based on age. Spearman's correlation was performed to assess correlation between serum 25-OHD levels and different BMD variables. Results: Ninety eight patients underwent 103 HSCTs between 1990 and 2010. Fifty two (53%) patients were > 5 years out of transplant. A total of 114 vitamin D levels were recorded for the 98 patients, the median 25-OHD level was 26 (range 7 – 68 ng/mL). In 68/114 (60%) observations the 25-OHD levels were less than < 30ng/mL. Of these, 10 (9%) patients had VDD (levels < 15ng/mL, while 58 (51%) had VDI. There were no significant correlations between 25-OHD levels and age at HSCT, gender, underlying diagnosis, type of transplant, or development of acute or chronic GVHD (Table 2). There was a trend towards lower 25-OHD levels after non-TBI based conditioning regimen (p = 0.047). BMD was performed in 83 patients (85%). Low BMD was found in nearly one-third to half of patients tested: 29%, 54%, and 33% of the patients had BMDlumbar, BMDhip and BMDWB Z scores of < −1.0, respectively, while 5%, 9% and 5% of the patients had BMDlumbar, BMDhip and BMDWB Z scores < −2.5, respectively. The median Z scores of the BMDlumbar, BMDhip, and the BMDWB were −0.3 (range −4.2 to 2.4), −1.1 (range −3.3 to 1.9), and −0.4 (range −5.4 to 2.7) respectively. In patients with BMD < −2.5 and < −1.0, the corresponding median 25-OHD was 26 (range 7 – 62 ng/mL) and there was no significant association. Spearman correlation between 25-OHD D level, BMDWB and BMDlumbar showed a correlation coefficient of −0.24 (p value: 0.0409) and −0.22 (p value: 0.0546) respectively. There was no correlation between normal vitamin D levels, VDI and VDD with BMD of the hip, lumbar spine and whole body. Discussion: Low 25-OHD (<30ng/mL) was common (60%) in long term survivors after HSCT during childhood. Similar to other reports, VDD and VDI was seen in 9%, and 51% of the patients respectively. There was only a weak correlation of the 25-OHD levels with BMD of whole body and the lumbar spine, suggesting that factors other than hypovitaminosis D might have contributed to low BMD. There was a small trend of lower 25-OHD levels after non-TBI based conditioning. Disclosures: No relevant conflicts of interest to declare.


2014 ◽  
Author(s):  
Mingo Dominguez Maria Luisa de ◽  
Sonsoles Guadalix Iglesias ◽  
Maria Begona Lopez Alvarez ◽  
Guillermo Martinez Diaz-Guerra ◽  
Federico Hawkins Carranza

Endocrine ◽  
2020 ◽  
Vol 70 (2) ◽  
pp. 280-291
Author(s):  
Alfredo Campennì ◽  
Daniele Barbaro ◽  
Marco Guzzo ◽  
Francesca Capoccetti ◽  
Luca Giovanella

Abstract Purpose The standard of care for differentiated thyroid carcinoma (DTC) includes surgery, risk-adapted postoperative radioiodine therapy (RaIT), individualized thyroid hormone therapy, and follow-up for detection of patients with persistent or recurrent disease. In 2019, the nine Martinique Principles for managing thyroid cancer were developed by the American Thyroid Association, European Association of Nuclear Medicine, Society of Nuclear Medicine and Molecular Imaging, and European Thyroid Association. In this review, we present our clinical practice recommendations with regard to implementing these principles in the diagnosis, treatment, and long-term follow-up of patients with DTC. Methods A multidisciplinary panel of five thyroid cancer experts addressed the implementation of the Martinique Principles in routine clinical practice based on clinical experience and evidence from the literature. Results We provide a suggested approach for the assessment and diagnosis of DTC in routine clinical practice, including the use of neck ultrasound, measurement of serum thyroid-stimulating hormone and calcitonin, fine-needle aspiration, cytology, and molecular imaging. Recommendations for the use of surgery (lobectomy vs. total thyroidectomy) and postoperative RaIT are also provided. Long-term follow-up with neck ultrasound and measurement of serum anti-thyroglobulin antibody and basal/stimulated thyroglobulin is standard, with 123/131I radioiodine diagnostic whole-body scans and 18F-fluoro-2-deoxyglucose positron emission tomography/computed tomography suggested in selected patients. Management of metastatic DTC should involve a multidisciplinary team. Conclusions In routine clinical practice, the Martinique Principles should be implemented in order to optimize clinical management/outcomes of patients with DTC.


BMJ Open ◽  
2018 ◽  
Vol 8 (9) ◽  
pp. e018120
Author(s):  
Inge M Krul ◽  
Annemieke W J Opstal-van Winden ◽  
Josée M Zijlstra ◽  
Yolande Appelman ◽  
Sanne B Schagen ◽  
...  

IntroductionHodgkin’s lymphoma (HL) has become the prototype of a curable disease. However, many young survivors suffer from late adverse effects of treatment. Both chemotherapy (CT) and radiotherapy (RT) may induce primary ovarian insufficiency (POI), which has been associated with reduced bone mineral density (BMD), neurocognitive dysfunction and possibly cardiovascular disease (CVD). While the general assumption is that POI increases CVD risk, other hypotheses postulate reverse causality, suggesting that cardiovascular risk factors determine menopausal age or that biological ageing underlies both POI and CVD risk. None of these hypotheses are supported by convincing evidence. Furthermore, most studies on POI-associated conditions have been conducted in women with early natural or surgery-induced menopause with short follow-up times. In this study, we will examine the long-term effects of CT-induced and/or RT-induced POI on BMD, cardiovascular status, neurocognitive function and quality of life in female HL survivors.Methods and analysisThis study will be performed within an existing Dutch cohort of HL survivors. Eligible women were treated for HL at ages 15–39 years in three large hospitals since 1965 and survived for ≥8 years after their diagnosis. Women visiting a survivorship care outpatient clinic will be invited for a neurocognitive, cardiovascular and BMD assessment, and asked to complete several questionnaires and to provide a blood sample. Using multivariable regression analyses, we will compare the outcomes of HL survivors who developed POI with those who did not. Cardiovascular status will also be compared with women with natural POI.Ethics and disseminationThis study has been approved by the Institutional Review Board of the Netherlands Cancer Institute and has been registered at ‘Toetsingonline’ from the Dutch Central Committee on Research involving Human Subjects (file no. NL44714.031.13). Results will be disseminated through peer-reviewed publications and will be incorporated in follow-up guidelines for HL survivors.


Cancers ◽  
2021 ◽  
Vol 13 (22) ◽  
pp. 5640
Author(s):  
Michael Oertel ◽  
Jonas Martel ◽  
Jan-Henrik Mikesch ◽  
Sergiu Scobioala ◽  
Christian Reicherts ◽  
...  

Total body irradiation is an effective conditioning modality before autologous or allogeneic stem cell transplantation. With the whole body being the radiation target volume, a diverse spectrum of toxicities has been reported. This fact prompted us to investigate the long-term sequelae of this treatment concept in a large patient cohort. Overall, 322 patients with acute leukemia or myelodysplastic syndrome with a minimum follow-up of one year were included (the median follow-up in this study was 68 months). Pulmonary, cardiac, ocular, neurological and renal toxicities were observed in 23.9%, 14.0%, 23.6%, 23.9% and 20.2% of all patients, respectively. The majority of these side effects were grades 1 and 2 (64.9–89.2% of all toxicities in the respective categories). The use of 12 Gray total body irradiation resulted in a significant increase in ocular toxicities (p = 0.013) and severe mucositis (p < 0.001). Renal toxicities were influenced by the age at transplantation (relative risk: 1.06, p < 0.001) and disease entity. In summary, total body irradiation triggers a multifaceted, but manageable, toxicity profile. Except for ocular toxicities and mucositis, a 12 Gray regimen did not lead to an increase in long-term side effects.


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