Search Results
Search for other papers by Athanasios D Anastasilakis in
Google Scholar
PubMed
Search for other papers by Marina Tsoli in
Google Scholar
PubMed
Search for other papers by Gregory Kaltsas in
Google Scholar
PubMed
Search for other papers by Polyzois Makras in
Google Scholar
PubMed
Langerhans cell histiocytosis (LCH) is a rare disease of not well-defined etiology that involves immune cell activation and frequently affects the skeleton. Bone involvement in LCH usually presents in the form of osteolytic lesions along with low bone mineral density. Various molecules involved in bone metabolism are implicated in the pathogenesis of LCH or may be affected during the course of the disease, including interleukins (ILs), tumor necrosis factor α, receptor activator of NF-κB (RANK) and its soluble ligand RANKL, osteoprotegerin (OPG), periostin and sclerostin. Among them IL-17A, periostin and RANKL have been proposed as potential serum biomarkers for LCH, particularly as the interaction between RANK, RANKL and OPG not only regulates bone homeostasis through its effects on the osteoclasts but also affects the activation and survival of immune cells. Significant changes in circulating and lesional RANKL levels have been observed in LCH patients irrespective of bone involvement. Standard LCH management includes local or systematic administration of corticosteroids and chemotherapy. Given the implication of RANK, RANKL and OPG in the pathogenesis of the disease and the osteolytic nature of bone lesions, agents aiming at inhibiting the RANKL pathway and/or osteoclastic activation, such as bisphosphonates and denosumab, may have a role in the therapeutic approach of LCH although further clinical investigation is warranted.
Beijing Key Laboratory of Diabetes Research and Care, Beijing, China
Search for other papers by Rong Xu in
Google Scholar
PubMed
Beijing Key Laboratory of Diabetes Research and Care, Beijing, China
Search for other papers by Difei Lian in
Google Scholar
PubMed
Beijing Key Laboratory of Diabetes Research and Care, Beijing, China
Search for other papers by Yan Xie in
Google Scholar
PubMed
Beijing Key Laboratory of Diabetes Research and Care, Beijing, China
Search for other papers by Lin Mu in
Google Scholar
PubMed
Beijing Key Laboratory of Diabetes Research and Care, Beijing, China
Search for other papers by Yali Wu in
Google Scholar
PubMed
Beijing Key Laboratory of Diabetes Research and Care, Beijing, China
Search for other papers by Zhilei Chen in
Google Scholar
PubMed
Beijing Key Laboratory of Diabetes Research and Care, Beijing, China
Search for other papers by Baoyu Zhang in
Google Scholar
PubMed
Osteoporosis (OP) is a systemic bone disease in which bone density and quality decrease and bone fragility increases due to a variety of causes, making it prone to fractures. The development of OP is closely related to oxidative stress. Uric acid (UA) is the end product of purine metabolism in the human body. Extracellular UA has antioxidant properties and is thought to have a protective effect on bone metabolism. However, the process of UA degradation can lead to intracellular oxidative stress, which together with UA-induced inflammatory factors, leads to increased bone destruction. In addition, UA can inhibit vitamin D production, resulting in secondary hyperparathyroidism and further exacerbating UA-associated bone loss. This review summarizes the relationship between serum UA levels and bone mineral density, bone turnover markers, and so on, in the hope of providing new insights into the pathogenesis and treatment of OP.
Search for other papers by Anna Gorbacheva in
Google Scholar
PubMed
Search for other papers by Anna Eremkina in
Google Scholar
PubMed
Search for other papers by Daria Goliusova in
Google Scholar
PubMed
Search for other papers by Julia Krupinova in
Google Scholar
PubMed
Search for other papers by Natalia Mokrysheva in
Google Scholar
PubMed
Multiple endocrine neoplasia type 1 (MEN1) is the most common cause of hereditary primary hyperparathyroidism (PHPT). Bone disorders are considered one of the key symptoms in PHPT present with the significant reduction in bone mineral density and low-energy fractures. Previously, these bone disorders were believed to be caused solely by the increase in the level of parathyroid hormone and its subsequent effect on bone resorption. The current paradigm, however, states that the mutations in the menin gene, which cause the development of MEN1, can also affect the metabolism of the cells of the osteoid lineage. This review analyzes both the proven and the potential intracellular mechanisms through which menin can affect bone metabolism.
Search for other papers by Huda M Elsharkasi in
Google Scholar
PubMed
Search for other papers by Suet C Chen in
Google Scholar
PubMed
Search for other papers by Lewis Steell in
Google Scholar
PubMed
Paediatric Neurosciences Research Group, Royal Hospital for Children, NHS Greater Glasgow & Clyde, Glasgow, UK
Search for other papers by Shuko Joseph in
Google Scholar
PubMed
Search for other papers by Naiemh Abdalrahaman in
Google Scholar
PubMed
Search for other papers by Christie McComb in
Google Scholar
PubMed
Search for other papers by Blair Johnston in
Google Scholar
PubMed
Search for other papers by John Foster in
Google Scholar
PubMed
Search for other papers by Sze Choong Wong in
Google Scholar
PubMed
Search for other papers by S Faisal Ahmed in
Google Scholar
PubMed
Objective
The aim of this study is to investigate the role of 3T-MRI in assessing musculoskeletal health in children and young people.
Design
Bone, muscle and bone marrow imaging was performed in 161 healthy participants with a median age of 15.0 years (range, 8.0, 30.0).
Methods
Detailed assessment of bone microarchitecture (constructive interference in the steady state (CISS) sequence, voxel size 0.2 × 0.2 × 0.4 mm3), bone geometry (T1-weighted turbo spin echo (TSE) sequence, voxel size 0.4 × 0.4 × 2 mm3) and bone marrow (1H-MRS, point resolved spectroscopy sequence (PRESS) (single voxel size 20 × 20 × 20 mm3) size and muscle adiposity (Dixon, voxel size 1.1 × 1.1 × 2 mm3).
Results
There was an inverse association of apparent bone volume/total volume (appBV/TV) with age (r = −0.5, P < 0.0005). Cortical area, endosteal and periosteal circumferences and muscle cross-sectional area showed a positive association to age (r > 0.49, P < 0.0001). In those over 17 years of age, these parameters were also higher in males than females (P < 0.05). This sex difference was also evident for appBV/TV and bone marrow adiposity (BMA) in the older participants (P < 0.05). AppBV/TV showed a negative correlation with BMA (r = −0.22, P = 0.01) which also showed an association with muscle adiposity (r = 0.24, P = 0.04). Cortical geometric parameters were highly correlated with muscle area (r > 0.57, P < 0.01).
Conclusions
In addition to providing deep insight into the normal relationships between bone, fat and muscle in young people, these novel data emphasize the role of MRI as a non-invasive method for performing a comprehensive and integrated assessment of musculoskeletal health in the growing skeleton.
Search for other papers by Cristina Lamas in
Google Scholar
PubMed
Search for other papers by Elena Navarro in
Google Scholar
PubMed
Search for other papers by Anna Casterás in
Google Scholar
PubMed
Search for other papers by Paloma Portillo in
Google Scholar
PubMed
Search for other papers by Victoria Alcázar in
Google Scholar
PubMed
Search for other papers by María Calatayud in
Google Scholar
PubMed
Search for other papers by Cristina Álvarez-Escolá in
Google Scholar
PubMed
Search for other papers by Julia Sastre in
Google Scholar
PubMed
Search for other papers by Evangelina Boix in
Google Scholar
PubMed
Search for other papers by Lluis Forga in
Google Scholar
PubMed
Search for other papers by Almudena Vicente in
Google Scholar
PubMed
Search for other papers by Josep Oriola in
Google Scholar
PubMed
Search for other papers by Jordi Mesa in
Google Scholar
PubMed
Search for other papers by Nuria Valdés in
Google Scholar
PubMed
Primary hyperparathyroidism is the most frequent manifestation of multiple endocrine neoplasia type 1 (MEN1) syndrome. Bone and renal complications are common. Surgery is the treatment of choice, but the best timing for surgery is controversial and predictors of persistence and recurrence are not well known. Our study describes the clinical characteristics and the surgical outcomes, after surgery and in the long term, of the patients with MEN1 and primary hyperparathyroidism included in the Spanish Registry of Multiple Endocrine Neoplasia, Pheochromocytomas and Paragangliomas (REGMEN). Eighty-nine patients (49 men and 40 women, 34.2 ± 13 years old) were included. Sixty-four out of the 89 underwent surgery: a total parathyroidectomy was done in 13 patients, a subtotal parathyroidectomy in 34 and a less than subtotal parathyroidectomy in 15. Remission rates were higher after a total or a subtotal parathyroidectomy than after a less than subtotal (3/4 and 20/22 vs 7/12, P < 0.05), without significant differences in permanent hypoparathyroidism (1/5, 9/23 and 0/11, N.S.). After a median follow-up of 111 months, 20 of the 41 operated patients with long-term follow-up had persistent or recurrent hyperparathyroidism. We did not find differences in disease-free survival rates between different techniques, patients with or without permanent hypoparathyroidism and patients with different mutated exons, but a second surgery was more frequent after a less than subtotal parathyroidectomy.
Search for other papers by Ann-Kristin Picke in
Google Scholar
PubMed
Search for other papers by Graeme Campbell in
Google Scholar
PubMed
Division of Bone and Mineral Diseases, Washington University in St Louis, St Louis, Missouri, USA
Search for other papers by Nicola Napoli in
Google Scholar
PubMed
Search for other papers by Lorenz C Hofbauer in
Google Scholar
PubMed
Search for other papers by Martina Rauner in
Google Scholar
PubMed
The prevalence of type 2 diabetes mellitus (T2DM) is increasing worldwide, especially as a result of our aging society, high caloric intake and sedentary lifestyle. Besides the well-known complications of T2DM on the cardiovascular system, the eyes, kidneys and nerves, bone strength is also impaired in diabetic patients. Patients with T2DM have a 40–70% increased risk for fractures, despite having a normal to increased bone mineral density, suggesting that other factors besides bone quantity must account for increased bone fragility. This review summarizes the current knowledge on the complex effects of T2DM on bone including effects on bone cells, bone material properties and other endocrine systems that subsequently affect bone, discusses the effects of T2DM medications on bone and concludes with a model identifying factors that may contribute to poor bone quality and increased bone fragility in T2DM.
University of Ljubljana, Faculty of Medicine, Ljubljana, Slovenia
Search for other papers by Mojca Zerjav Tansek in
Google Scholar
PubMed
Search for other papers by Ana Bertoncel in
Google Scholar
PubMed
Search for other papers by Brina Sebez in
Google Scholar
PubMed
Search for other papers by Janez Zibert in
Google Scholar
PubMed
University of Ljubljana, Faculty of Medicine, Ljubljana, Slovenia
Search for other papers by Urh Groselj in
Google Scholar
PubMed
University of Ljubljana, Faculty of Medicine, Ljubljana, Slovenia
Search for other papers by Tadej Battelino in
Google Scholar
PubMed
University of Ljubljana, Faculty of Medicine, Ljubljana, Slovenia
Search for other papers by Magdalena Avbelj Stefanija in
Google Scholar
PubMed
Despite recent improvements in the composition of the diet, lower mineral bone density and overweight tendencies are incoherently described in patients with phenylketonuria (PKU). The impact of dietary factors and plasma phenylalanine levels on growth, BMI, body composition, and bone mineral density was investigated in our cohort of patients with hyperphenylalaninemia (HPA) with or without dietary treatment. The anthropometric, metabolic, BMI and other nutritional indicators and bone mineral density were compared between the group of 96 treated patients with PKU (58 classic PKU (cPKU) and 38 patients with moderate-mild PKU defined as non-classic PKU (non-cPKU)) and the untreated group of 62 patients with benign HPA. Having compared the treated and untreated groups, there were normal outcomes and no statistically significant differences in BMI, body composition, and bone mineral density. Lower body height standard deviation scores were observed in the treated as compared to the untreated group (P < 0.001), but the difference was not significant when analyzing patients older than 18 years; however, cPKU adults were shorter compared to non-cPKU treated adults (P = 0.012). Interestingly, the whole-body fat was statistically higher in non-cPKU as compared to cPKU patients. In conclusion, the dietary treatment ensured adequate nutrition without significant consequences in BMI, body composition, and bone mineral density. A low protein diet may have delayed the growth in childhood, but the treated patients gained a normal final height. Mild untreated hyperphenylalaninemia characteristic for benign HPA had no negative physiological effect on bone mineral density.
Department of Endocrinology, Jiading Branch of Shanghai General Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China
Search for other papers by Lizhi Zhang in
Google Scholar
PubMed
Search for other papers by Jinwei He in
Google Scholar
PubMed
Search for other papers by Xiang Sun in
Google Scholar
PubMed
Search for other papers by Dongyue Pang in
Google Scholar
PubMed
Search for other papers by Jingjing Hu in
Google Scholar
PubMed
Search for other papers by Bo Feng in
Google Scholar
PubMed
We demonstrated previously that there is a correlation between glucagon-like peptide-1 (GLP-1) single-nucleotide polymorphism (SNP) and bone mineral density in postmenopausal women. Both GLP-1 and glucose-dependent insulinotropic peptide are incretins. The glucose-dependent insulinotropic peptide receptor (GIPR) SNP rs10423928 has been extensively studied. However, it is not clear whether GIPR gene mutations affect bone metabolism. The aim of this study was to investigate the association between rs10423928 and bone mineral density in postmenopausal women in Shanghai. rs10423928 was detected in 884 postmenopausal women in Shanghai, and the correlation between the GIPR SNP and bone mineral density was assessed. The dominant T/T genotype of rs10423928 was found to be related to the bone mineral density of the femoral neck (P = 0.035). Overall, our findings indicate that the dominant T/T genotype of rs10423928 in postmenopausal women is significantly associated with a higher bone mineral density and that the T/T genotype exerts a bone-protective effect.
Search for other papers by Martine Cohen-Solal in
Google Scholar
PubMed
Search for other papers by Thomas Funck-Brentano in
Google Scholar
PubMed
Department of Renal Physiology, Necker Hospital, Université de Paris, Paris, France
Search for other papers by Pablo Ureña Torres in
Google Scholar
PubMed
Mineral and bone diseases (MBD) are predominant in patients with chronic kidney disease (CKD) and lead to several bone manifestations, from pain to skeletal fractures. Cumulative traditional clinical risk factors, such as age and gender, in addition to those related to CKD, enhance the risk of comorbidity and mortality related to fractures. Despite great advances in understanding MBD in CKD, clinical and biological targets are lacking, which leads to under-management of fractures. Optimal PTH control results in a net improvement in defining the levels of bone remodeling. In addition, circulating biomarkers such as bone-specific alkaline phosphatase and cross-linked collagen type I peptide will also provide additional information about remodeling rate, bone mineralization and the evaluation of fracture risk. Imaging techniques identify patients at risk by measurement of bone mineral density by DEXA or by high peripheral QCT, which allow the discrimination of trabecular and cortical bone. Here, we have reviewed the literature related to epidemiology and the pathophysiological role of mineral and biochemical factors involved in CKD-MBD with a special focus on fracture risk. We also provide an algorithm that could be used for the management of bone diseases and to guide treatment decisions. Finally, the combined expertise of clinicians from various disciplines is crucial for the best prevention of fractures.
College of Medicine, Chang Gung University, Taoyuan, Taiwan
Search for other papers by Heng Yeh in
Google Scholar
PubMed
Department of Nephrology, Clinical Poison Center, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan
Search for other papers by Hsuan Yeh in
Google Scholar
PubMed
Department of Gastroenterology and Hepatology, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan
Search for other papers by Chun-Cheng Chiang in
Google Scholar
PubMed
Search for other papers by Ju-Ching Yen in
Google Scholar
PubMed
Department of Nephrology, China Medical University Hospital, Taichung, Taiwan
Search for other papers by I-Kuan Wang in
Google Scholar
PubMed
Department of Nephrology, Clinical Poison Center, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan
Search for other papers by Shou-Hsuan Liu in
Google Scholar
PubMed
Department of Nephrology, Clinical Poison Center, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan
Search for other papers by Cheng-Chia Lee in
Google Scholar
PubMed
Department of Nephrology, Clinical Poison Center, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan
Search for other papers by Cheng-Hao Weng in
Google Scholar
PubMed
Department of Nephrology, Clinical Poison Center, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan
Search for other papers by Wen-Hung Huang in
Google Scholar
PubMed
Department of Nephrology, Clinical Poison Center, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan
Search for other papers by Ching-Wei Hsu in
Google Scholar
PubMed
Department of Nephrology, Clinical Poison Center, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan
Search for other papers by Tzung-Hai Yen in
Google Scholar
PubMed
Secondary hyperparathyroidism (SHPT) is a common complication of end-stage kidney disease (ESKD). Hungry bone syndrome (HBS) occurs frequently in patients on maintenance dialysis receiving parathyroidectomy for refractory SHPT. However, there is scanty study investigating the clinical risk factors that predict postoperative HBS, and its outcome in peritoneal dialysis (PD) patients. We conducted a single-center retrospective study to analyze 66 PD patients who had undergone parathyroidectomy for secondary hyperparathyroidism at Chang Gung Memorial Hospital between 2009 and 2019. The patients were stratified into two groups based on the presence (n=47) or absence (n=19) of HBS after parathyroidectomy. Subtotal parathyroidectomy was the most common surgery performed (74.2%), followed by total parathyroidectomy with autoimplantation (25.8%). Pathological examination of all surgical specimens revealed parathyroid hyperplasia (100%). Patients with HBS had lower levels of postoperative nadir corrected calcium, higher alkaline phosphate (ALP), and higher potassium levels compared with patients without HBS (all P<0.05). A multivariate logistic regression model confirmed that lower preoperative serum calcium level (OR 0.354, 95% CI 0.133–0.940, P=0.037), higher ALP (OR 1.026, 95% CI 1.008–1.044, P=0.004), and higher potassium level (OR 6.894, 95% CI 1.806–26.317, P=0.005) were associated with HBS after parathyroidectomy. Patients were followed for 58.2±30.8 months after the surgery. There was no significant difference between HBS and non-HBS groups in persistence (P=0.496) or recurrence (P=1.000) of hyperparathyroidism. The overall mortality rate was 10.6% with no significant difference found between both groups (P=0.099). We concluded that HBS is a common complication (71.2%) of parathyroidectomy for SHPT and should be managed appropriately.
