Amyloid A (AA) amyloidosis, a complication of chronic inflammatory conditions, develops when proteolytic fragments of serum amyloid A protein are deposited in tissues as amyloid fibrils. This ...placebo-controlled trial investigated the effect of eprodisate, a small molecule that inhibits amyloid fibril polymerization and tissue deposition in patients with renal AA amyloidosis. As compared with placebo, the drug slowed a decline in renal function. Eprodisate is a member of a new class of compounds that interfere with interactions between amyloidogenic proteins and glycosaminoglycans.
This trial investigated the effect of eprodisate, a small molecule that inhibits amyloid fibril polymerization and tissue deposition in patients with renal AA amyloidosis. As compared with placebo, the drug slowed a decline in renal function.
The amyloidoses constitute a group of diseases in which proteins are deposited extracellularly in the tissues as insoluble fibrils, causing progressive organ dysfunction and death.
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Amyloid A (AA) amyloidosis, also referred to as secondary amyloidosis, is a rare but serious complication of chronic inflammatory diseases and chronic infections. The amyloidogenic protein in AA amyloidosis is a proteolytic fragment of serum amyloid A protein (SAA), an acute-phase reactant produced by the liver. The kidney is the organ most frequently affected in AA amyloidosis.
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Ongoing deposition of amyloid in the kidney results in proteinuria and progressive loss of renal function. The gastrointestinal . . .
Vitamin D plays a critical role in skeletal development and maintenance, thus sufficiency is an important goal for public health programs. Given the absence of foods fortified in vitamin D in ...Armenia, we hypothesized that vitamin D insufficiency would be widespread.
We conducted a random modified cluster model survey of vitamin D status of women in the country. We measured 25-hydroxyvitamin D 25(OH)D by liquid chromatography mass spectrometry in dried blood spot samples and utilized a questionnaire to assess lifestyle factors.
In summer, we sampled 1206 participants from 40 communities in Armenia. Mean 25-OH D level among women aged 18-24 was 20 ± 8 ng/mL; aged 25-64 was 21 ± 7 ng/mL; and >65 was 18 ± 8 ng/mL. The country-wide mean of the entire female population was 20 ± 8 ng/mL. A majority (>54%) had 25-OH D levels <20 ng/mL with nearly 13% having 25-OH D levels <12 ng/mL. Participants who reported calcium, vitamin D, or multivitamin supplementation had higher levels of 25-OH D (p values 0.004, 0.0002, and 0.03 respectively) as did pre- vs. postmenopausal women (p = 0.01), pregnant vs. nonpregnant women (p = <0.0001), and women who had experienced a sunburn in the past year (p = 0.004).
In Armenia, there is a high prevalence of vitamin D insufficiency. This information provides data that can be used to inform public health directives to address this pervasive threat to optimal health.
We report a case of a patient with a de novo germline heterozygous truncating variant of CTNNB1 gene (c.2172del, p.Tyr724Ter) causing neurodevelopmental disorder with spastic diplegia and visual ...defects syndrome (NEDSDV) associated with a new clinical feature — severe pediatric-onset osteoporosis and multiple fractures. A functional effect of the identified variant was demonstrated using adipose-tissue derived primary mesenchymal stem cells, where we detected the alteration of CTNNB1mRNA and β-catenin protein levels using real-time PCR and Western blot analysis.
•Patients with NEDSDV syndrome caused by CTNNB1 variants might develop severe fracture osteoporosis•Attention to bone health in patients with NEDSDV caused by CTNNB1 variants can reveal osteoporosis at young age•Оsteoporosis-associated CTNNB1 variant leads to alteration of protein expression in progenitor mesenchymal stem cells
Summary
Age-specific intervention and assessment thresholds based on FRAX® were developed for eight Eurasian countries participating in the EVA study (Armenia, Belarus, Georgia, Moldova, Kazakhstan, ...the Kyrgyz Republic, the Russian Federation, and Uzbekistan). The intervention thresholds (major osteoporotic fracture) ranged from 3.6 (Armenia and Georgia) to 12.3% (Uzbekistan) for people at age 50 years, and from 16 (Armenia) to 27% (Belarus) at the age of 90 years. These thresholds enable a substantial advance in the ease of detection of individuals at high fracture risk.
Introduction
The purpose of this study was to derive and compare FRAX-based intervention and BMD assessment thresholds for 8 Eurasian countries in the EVA study.
Methods
The intervention threshold (IT) was set at a 10-year probability of a major osteoporotic fracture (MOF), calculated without BMD, equivalent to a woman with a prior fragility fracture but no other clinical risk factors, and a body mass index (BMI) of 25.0 kg/m
2
. The lower assessment threshold was set at a 10-year probability of a MOF in women with BMI of 25.0 kg/m
2
, without previous fracture or other clinical risk factors. The upper assessment threshold was set at 1.2 times the IT.
Results
The age-specific intervention thresholds ranged from 3.6 (Armenia and Georgia) to 12.3% (Uzbekistan) for men and women at the age of 50 years and from 16 (Armenia) to 27% (Belarus) at the age of 90 years. The difference between countries was most evident at younger ages and become progressively less with advancing age.
Conclusions
For the 8 Eurasian countries, the newly established FRAX-based intervention thresholds provide an opportunity to improve the clinical detection of both men and women with a high risk of fracture and improve treatment rates.
To extend elderly patients’ active longevity, practitioners all over the world need a precise clinical instrument to assess the 10-year osteoporotic fractures probability and choose the optimal ...preventive intervention. The purpose of this research is to assess major osteoporotic (OP) fractures incidence in CIS countries and to create country-specific computing FRAX clinical prediction tools. Method. We carried out a cohort population-based study (EVA) in Russia, Belarus, Armenia, Moldova, Kazakhstan, and Uzbekistan in 2011-2018. The age- and sexspecific OP fracture incidence values were incorporated into the computing FRAX models. Results. We revealed that the highest standardized HP incidence was obtained in Kazakhstan (338 and 255 per 100 000 per year for women and men older than 50 years resp.) and Moldova (331, 0 and 155, 0 per year for women and men resp.), the lowest in Armenia (201 and 136 per 100 000 per year for women and men resp.). The FRAX models showed that in Kazakhstan, Uzbekistan, and Moldova, elderly people had the highest hip fracture probabilities. Estimates reveal that the OP fractures numbers in the EVA-project countries will grow by 1.5-3 times by 2050. Conclusion. The created national FRAX instruments should enhance the accuracy of determining fracture probability among older people, help clinicians to make decisions concerning OP prophylactic, and to step forward to reducing the burden of age-related diseases.
Bisphosphonates is a first-line therapy for treatment of osteoporosis. In the last decade, the number of atypical femur fracture (AFF) cases during long-term treatment with bisphosphonates has ...increased. The aim of this article was to analyze the literature data on this problem, to define the diagnostic criteria of AFF and to present the case of AFF in the patient who received treatment with alendronate for 3.5 years.
A 78-year-old woman, receiving oral bisphosphonate for severe postmenopausal osteoporosis for 3.5 years, suddenly started feeling pain in her right thigh while walking. Three months later, she had got a fracture in middle third of the right femur after falling from her standing height. According to instrumental diagnostics, this fracture had all criteria of AFF. Blocking intramedullary osteosynthesis with shafts was performed. A retrospective analysis of soft tissue magnetic resonance imaging in the area of right thigh, done before the fracture, showed the presence of undiagnosed incomplete right femur fracture in the middle third, which subsequently led to a complete fracture.
Presented clinical case demonstrates the complexity of AFF diagnostics. The purpose of the publication is to draw attention of medical specialists to the issue of this rare side effect of bisphosphonate treatment.
The meeting of the Russian Council of Experts for the use of the novel anabolic agent Forsteo®(teriparatide) to treat osteoporosis (OP) was held in Moscow on June 29, 2013. The meeting was attended ...by leading Russian OP specialists, including the members of the Russian Osteoporosis Association and its Presidium. The participants of the Council of Experts noted the great social importance of severe OP, which was associated primarily with its consequences, such as fractures causing an increase in disability and mortality rates in elderly people, and discussed the possibilities and benefits of the new approach to treating OP with teriparatide, the first drug permitting the formation of new bone tissue. The experts have recommended teriparatide for use within the registered indications in the following groups of patients: those who have severe OP (≥1 vertebral fractures, hip fracture of vertebral bodies or a fracture of the proximal femur, multiple recurrent fractures of skeletal bones) as first-line therapy; those who had ineffective previous antiosteoporotic therapy (new fractures occurring during the treatment and/or a continuing decline in bone mineral density), those who are intolerant to other medications for OP, or who have contraindications to their use.
Bisphosphonates are one of the most effective medications widely used in the treatment of osteoporosis. In the last 15 years, descriptions, and observations of unusual low-energy femoral fractures ...with atypical localization for osteoporosis after prolonged use of these drugs have been published.
A 78-year-old patient applied to the osteoporosis center. She received parenteral bisphosphonate therapy for severe osteoporosis for 4 years. In 2017, a spontaneous fracture of the middle third of the femoral shaft was registered (the fracture occurred while walking). Regarding the fracture, metal osteosynthesis with a plate was performed. Subsequently, there was a delayed consolidation of the fracture with the formation of a false joint and the need for repeated surgical intervention. Examination of the patient revealed vitamin D deficiency, and significant negative trend in densitometry.
A feature of this case is poor fracture consolidation and long-term uncompensated vitamin D deficiency. Physicians should be aware of this rare but serious complication of bisphosphonate therapy, as it requires discontinuation of antiresorptive drugs and the appointment of bone anabolic therapy for osteoporosis, which, in addition to treating the underlying disease, also promotes consolidation fracture.
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