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Osteoprotegerin Levels (osteoprotegerin + level)
Selected AbstractsJuvenile Paget's Disease: The Second Reported, Oldest Patient Is Homozygous for the TNFRSF11B "Balkan" Mutation (966_969delTGACinsCTT), Which Elevates Circulating Immunoreactive Osteoprotegerin Levels,,§¶JOURNAL OF BONE AND MINERAL RESEARCH, Issue 6 2007Michael P Whyte MD Abstract The oldest person (60 yr) with juvenile Paget's disease is homozygous for the TNFRSF11B mutation 966_969delTGACinsCTT. Elevated circulating levels of immunoreactive OPG and soluble RANKL accompany this genetic defect that truncates the OPG monomer, preventing formation of OPG homodimers. Introduction: Juvenile Paget's disease (JPD), a rare autosomal recessive disorder, features skeletal pain, fracture, and deformity from extremely rapid bone turnover. Deafness and sometimes retinopathy also occur. Most patients have diminished osteoprotegerin (OPG) inhibition of osteoclastogenesis caused by homozygous loss-of-function defects in TNFRSF11B, the gene that encodes OPG. Circulating immunoreactive OPG (iOPG) is undetectable with complete deletion of TNFRSF11B but normal with a 3-bp in-frame deletion. Materials and Methods: We summarize the clinical course of a 60-yr-old Greek man who is the second reported, oldest JPD patient, including his response to two decades of bisphosphonate therapy. Mutation analysis involved sequencing all exons and adjacent mRNA splice sites of TNFRSF11B. Over the past 4 yr, we used ELISAs to quantitate his serum iOPG and soluble RANKL (sRANKL) levels. Results: Our patient suffered progressive deafness and became legally blind, although elevated markers of bone turnover have been normal for 6 yr. He carries the same homozygous mutation in TNFRSF11B (966_969delTGACinsCTT) reported in a seemingly unrelated Greek boy and Croatian man who also have relatively mild JPD. This frame-shift deletes 79 carboxyterminal amino acids from the OPG monomer, including a cysteine residue necessary for homodimerization. Nevertheless, serum iOPG and sRANKL levels are persistently elevated. Conclusions: Homozygosity for the TNFRSF11B "Balkan" mutation (966_969delTGACinsCTT) causes JPD in the second reported, oldest patient. Elevated circulating iOPG and sRANKL levels complement evidence that this deletion/insertion omits a cysteine residue at the carboxyterminus needed for OPG homodimerization. [source] Evolution of Coronary Artery Calcifications Following Kidney Transplantation: Relationship with Osteoprotegerin LevelsAMERICAN JOURNAL OF TRANSPLANTATION, Issue 11 2009A.-S. Bargnoux We prospectively assessed the evolution of coronary artery calcification (CAC) and osteoprotegerin (OPG) levels after renal transplantation (RT). Eighty-three recipients were followed-up prospectively during 1 year. Blood was collected before (baseline) and after RT for determination of mineral metabolism parameters including OPG. CAC was measured by multidetector computed tomography at transplantation (baseline) and 1 year later. Progression of CAC was defined as a difference between the follow-up square-root transformed volume (SRV) and the baseline SRV , 2.5. By multivariate analysis, baseline OPG level, age and low LDL levels were significantly associated with baseline CAC. RT was accompanied by mineral metabolism improvement with a decrease of OPG from 955 [395,5652] to 527 [217,1818] pg/mL and parathyroid hormone from 94 [1,550] to 62 [16,410] pg/mL. Thirty-one percent of patients did not exhibit CAC at baseline. CAC diminished in 14.5%, stabilized in 59.2% and progressed in 26.3% of patients. Baseline CAC was associated with progression (OR 2.92 [1.02,8.36]). No significant association was found between OPG and CAC progression despite a higher baseline OPG level in progressors (1046 [456,3285]) vs. non-progressors (899 [396,5952] pg/mL). CAC at baseline, but not 1 year after RT, is independently associated with baseline OPG; posttransplant CAC progression is predicted by baseline CAC score. [source] Children with chronic kidney disease: a 3-year prospective study of growth, bone mass and bone turnoverACTA PAEDIATRICA, Issue 2 2009Diana Swolin-Eide Abstract Aim: Children with chronic kidney disease (CKD) are at risk of developing skeletal problems. This 3-year prospective study investigated the development of bone mass and bone turnover in children with CKD. Methods: Fifteen patients, 4,15 years, were included with a median glomerular filtration rate of 48 (range 8,94) mL/min/1.73 m2. Bone mineral density (BMD) and markers of bone and mineral metabolism were investigated over a 3-year period. Results: Growth was satisfactory but a delayed bone age was observed. Total body bone mineral density (TBBMD) Z-scores were below zero in five patients at start and after 3 years, but none had a Z-score below ,2.5. Lumbar spine BMD Z-scores were below zero in three patients at start and in five patients after 3 years. The median TBBMD and lumbar spine Z-scores did not change during the study period. Eleven CKD patients had increased PTH levels at baseline and 13 patients after 3 years. Most children had normal levels of leptin and vitamin D. Almost 50% of the patients had increased osteoprotegerin levels after 3 years. Conclusion: A normal BMD does not exclude mineral bone disorder in patients with CKD, yet the BMD Z-scores were well preserved and most markers of bone turnover were within the reference intervals. [source] The relationship between circulating osteoprotegerin levels and bone mineral metabolism in healthy womenCLINICAL ENDOCRINOLOGY, Issue 2 2004Ki Won Oh Summary objective, Osteoprotegerin (OPG) is a recently identified cytokine that acts as a decoy receptor for the RANK ligand. Moreover, OPG has been shown to be an important inhibitor of osteoclastogenesis in animal models. However, the relationship between circulating OPG levels and female bone status in human populations is unclear. In this study we undertook to investigate the relationship between circulating OPG levels and bone mineral metabolism in healthy women. patients and measurements, Our subjects were 287 women aged 37,73 years (mean age 51·5 years). The serum concentrations of OPG were determined by enzyme-linked immunosorbent assay (ELISA). The biochemical markers of bone turnover and FSH were measured using standard methods. Bone mineral densities at the lumbar spine and femoral neck were measured by dual-energy X-ray absorptiometry. results, Postmenopausal women had a significantly higher mean value of serum OPG than premenopausal women (1358·5 ± 32·5 pg/ml vs. 1228·8 ± 33·3 pg/ml, P < 0·01). Serum OPG levels were positively correlated with age (r = 0·169, P < 0·01), as were urine deoxypyridinoline levels (r = 0·133, P < 0·05) and serum FSH levels (r = 0·187, P < 0·01) in a bivariate correlation analyses. In a multiple regression analysis, only urine calcium excretion was identified as a significant predictor for serum OPG levels. conclusions, Circulating OPG levels were found to be associated with urine calcium excretion and menopause in healthy women. Our observations suggest that circulating OPG levels reflect an antiresorptive activity in bone, and they are related to endogenous oestrogen levels. [source] | ||||||||||