No full-text available
To read the full-text of this research,
you can request a copy directly from the author.
The clinical course of Kashin-Beck Disease
Abstract
Kashin-Beck or Urov disease is described with authority by an astute scholar and clinician, Prof. Nesterov, Director of the Institute for Rheumatism and member of the Academy of Medical Sciences of the USSR in Moscow. The disease is a chronic, disabling, degenerative, generalized osteoarthrosis involving peripheral (symmetrical) joints and spine un-associated with systemic or visceral manifestations. It occurs principally in childhood and results in growth disturbances. It is endemic in Eastern Siberia, Northern China and Northern Korea and is believed to be due to ingestion of cereal grain grown in these areas and infected with the fungus, Fusaria sporotrichiella. The incidence has been considerably reduced and hopefully will soon be eradicated by importing cereals from unaffected areas. This is a classic example of a generalized degenerative joint disease due to environmental causes, unrelated to genetic factors or inborn errors of metabolism.Le morbo Kashin-Beck o Urov es describite con autoritate per le astute scholar e clinico, Professor Nesterov, Director del Instituto pro Rheumatismo e membro del Academia de Scientias Medical del URSS in Moscova. Le morbo es un osteoarthrosis chronic, incapacitante, degenerative, generalisate que implica le articulationes peripheric (symmetric) e le rhachis sin manifestationes systemic o visceral. Illo occurre principalmente in le infantia e resulta in disordines de crescentia. Illo es endemic in Est-Siberia, Nord-China, e Nord-Corea. On opina que illo es debite al ingestion de granos cereal crescente in iste regiones, e inficite con le fungo Fusaria sporotrichiella. Le incidentia del morbo ha essite reducite considerabilemente. On spera que illo tosto va esser eradicate per le importation de cereales ab areas non inficite. Illo es un exemplo classic de un generalisate morbo degenerative articulatori que es debite a causas in le ambiente, sin relation a factores genetic o a innate errores de metabolismo.
... The authorities decided to move the populations of some villages towards the Argun River. Unfortunately, there was no medical follow-up of these villagers and no conclusions can be drawn from this experiment (Nesterov, 1964). ...
... These signs are, most of the time, bilateral and become progressively installed during childhood and adolescence. According to most of the studies, it is the interphalangeal and metacarpophalangeal joints and the joints of the wrists that are the first to be affected (Nesterov, 1964;Sokoloff, 1988;Yin, 1990). Later on, the elbows, the knees and the ankles are also affected. ...
... Beck and most of the Russian doctors distinguished three stages in the evolution of the disease (Nesterov, 1964;Voshchenko et al., 1990;Kolsteren, 1992). These are defined by clinical criteria with which radiological signs are associated. ...
... The authorities decided to move the populations of some villages towards the Argun River. Unfortunately, there was no medical follow-up of these villagers and no conclusions can be drawn from this experiment (Nesterov, 1964). ...
... These signs are, most of the time, bilateral and become progressively installed during childhood and adolescence. According to most of the studies, it is the interphalangeal and metacarpophalangeal joints and the joints of the wrists that are the first to be affected (Nesterov, 1964;Sokoloff, 1988;Yin, 1990). Later on, the elbows, the knees and the ankles are also affected. ...
... Beck and most of the Russian doctors distinguished three stages in the evolution of the disease (Nesterov, 1964;Voshchenko et al., 1990;Kolsteren, 1992). These are defined by clinical criteria with which radiological signs are associated. ...
Abstract This chapter describes the objectives and methodology of a proposed randomized
clinical trial that will evaluate the effect of a combined micronutrient supplementation on the
incidence and evolution of Kashin-Beck disease among residents in Tibet Autonomous
Region, China. This supplementation trial will include iodine, selenium, vitamin A, vitamin C,
vitamin E, manganese, zinc and copper supplements.
... The authorities decided to move the populations of some villages towards the Argun River. Unfortunately, there was no medical follow-up of these villagers and no conclusions can be drawn from this experiment (Nesterov, 1964). ...
... These signs are, most of the time, bilateral and become progressively installed during childhood and adolescence. According to most of the studies, it is the interphalangeal and metacarpophalangeal joints and the joints of the wrists that are the first to be affected (Nesterov, 1964;Sokoloff, 1988;Yin, 1990). Later on, the elbows, the knees and the ankles are also affected. ...
... Beck and most of the Russian doctors distinguished three stages in the evolution of the disease (Nesterov, 1964;Voshchenko et al., 1990;Kolsteren, 1992). These are defined by clinical criteria with which radiological signs are associated. ...
Kashin-Beck disease is probably one of the most neglected diseases. It occurs in only a very limited part of the world, in a crescent shape area situated from South East Siberia to North West China. It affects mainly the rural population of these remote regions and more specifically the children. They are suffering from a lot of joint pain and lose their mobility. They stop to grow properly and they became severely handicapped. Since more than 15 years, an international team together with his partner, the Centre for Disease Control and Prevention of Tibet Autonomous Region, are studying this disease with, sometimes, very limited means but always in a marvellous environment. Their daily workmates are the lovely children, the yaks and other animals of the Himalaya region as well as the unique variety of flowers and plants, under the beautiful shadow of some of the highest peaks of the world. Their unique multidisciplinary approach of the complex issue of the Kashin-Beck disease is done with a constant worry of a better knowledge of the environment and its population. Through a lot of beautiful pictures and some more scientific data, the authors, with an immoderate enthusiasm, will drive you along the valleys of the high Tibetan plateau and will accompany you for a visit from one village to another to meet these patients and understand the difficulties of their daily life.
... The important function of bone can be easily recognized in day-to-day life, where millions of people suffer from bone disease such as osteoporosis, which is, in part, caused by an imbalance between bone formation and bone resorption [1,2]. The better understanding of the regulation of bone formation will provide new insights into the molecular mechanisms of Kashin-Beck disease (KBD), which usually afflicts children between the ages of 5 and 13 years with epiphyseal plates showing focal or irregular premature closure, wherein in the normal bone formation, endochondral ossification ends up about 20 years after birth in humans [3][4][5]. Its initial pathologic changes are multiple degenerative and necrotic lesions within the growth plate cartilage. ...
... The serum levels of OPG were highest in the KBD-affected group rats. Based on these previous studies [3][4][5][6][7][8][9], the aetiology of KBD not only causes epiphyseal plate chondrocyte necrosis but also decreases epiphyseal plate bone formation both by decreasing the rate of bone formation and increasing the bone resorption. Thus, the mycotoxin, which are seen as exogenous free-radical carriers, are important environmental factors in the pathogenesis of Kashin-Beck disease [41,42], and selenium, vitamin C and vitamin E, which inhibit free-radical formation, are considered to be protective [12,33]. ...
Objective
There were no studies on the macrophage colony-stimulating factor (M-CSF), receptor activator of NF-kappaB ligand (RANKL) and osteoprotegerin (OPG) in the pathogenesis of Kashin-Beck disease (KBD). The objective of the present study was to investigate the serum M-CSF, RANKL and OPG in rats fed with KBD-affected diet.Methods
Ninety Wistar rats were divided into five groups. The rats received standard commercial feed with or without T-2 toxin additive, low protein feed with or without or T-2 toxin additive and the KBD-affected feed. The serum bioactivity of M-CSF, RANKL and OPG was tested by enzyme-linked immunosorbent assay.ResultsThe serum levels of M-CSF in E group rats were higher than those in the other groups in the five groups (P¿<¿0.01). The serum levels of RANKL and OPG in E group rats were highest in the five groups and have significant difference compared to the other groups (P¿<¿0.05).Conclusions
The molecule of M-CSF, RANKL and OPG may be involved in the regulation of epiphyseal plate injury and repair in KBD, and its participation in the pathogenesis of KBD should be studied in the future.
... Kashin-Beck Disease (KBD) is an endemic, chronic and degenerative osteoarthropathy affecting approximately 3 million people in China [1, 2]. It principally occurs in childhood [3], with the breakdown of cartilage starting in children as early as 2 or 3-years-old [1], and results in growth disturbances [3]. Its main clinical symptoms include diarthrodal joint deformation and limited joint mobility. ...
... Kashin-Beck Disease (KBD) is an endemic, chronic and degenerative osteoarthropathy affecting approximately 3 million people in China [1, 2]. It principally occurs in childhood [3], with the breakdown of cartilage starting in children as early as 2 or 3-years-old [1], and results in growth disturbances [3]. Its main clinical symptoms include diarthrodal joint deformation and limited joint mobility. ...
Kashin-Beck Disease (KBD) is an endemic, chronic and degenerative osteoarthropathy principally occurring in children. The characteristic pathological change of KBD is chondrocyte necrosis in hyaline articular cartilage. Proteoglycans are one of the major components in the extracellular matrix of articular cartilage, and disrupted proteoglycan metabolism and loss of proteoglycans in articular cartilage from KBD patients has been observed. In this mini-review, we discuss the close relationship between chondrocyte death including necrosis and loss of proteoglycan, and its potential mechanism during KBD onset and development, which may provide new clues for KBD research.
... Kashin-Beck disease (Sokoloff 1985) has been variously attributed to a toxin from the fungus Fusarium Sporatrichiella contaminating wheat (Nesterov 1964), inorganic iron in drinking water (Hiyeda 1939) and selenium Content courtesy of Springer Nature, terms of use apply. Rights reserved. ...
The clinical appearance and radiological pattern of osteoarthritis have been identified in the skeletons of dinosaurs some 50–70 million years old, and in Egyptian mummies, and in ancient skeletons in England. Osteoarthritis patterns of joint involvement, often referred to as primary osteoarthritis, can be seen in the hands, spinal facet joints, hips, knees and feet, but can also be termed secondary osteoarthritis when seen in any joint that has had trauma, sepsis, surgery or metabolic insult. The prevalence of osteoarthritis increases with age. The histology and pathophysiology both demonstrate an inflammatory process. While there have been studies of genetic predisposition, the basic cause of primary osteoarthritis has not been determined.
... Soil fertilization is one of the major agronomic practices for Se biofortification and plays an essential role in agriculture (Wu et al., 2020). Se deficiency has been considered a global health issue that causes endemic diseases, e.g., Kashin-Beck disease in Eastern Siberia, Northern China and North Korea (Fordyce, 2007;Nesterov, 1964). A survey showed that half to one billion people are Se-deficient around the world (Combs, 2001). ...
Selenium (Se) is an essential element with application in manufacturing from food to medical industries. Water contamination by Se is of concern due to anthropogenic activities. Recently, Se remediation has received increasing attention. Hence, different types of remediation techniques are listed in this work, and their potential for Se recovery is evaluated. Sorption, co-precipitation, coagulation and precipitation are effective for low-cost Se removal. In photocatalytic, zero-valent iron and electrochemical systems, the above mechanisms occur with reduction as an immobilization and detoxification process. In combination with magnetic separation, the above techniques are promising for Se recovery. Biological Se oxyanions reduction has been widely recognized as a cost-effective method for Se remediation, simultaneously generating biosynthetic Se nanoparticles (BioSeNPs). Increasing the extracellular production of BioSeNPs and controlling their morphology will benefit its recovery. However, the mechanism of the microbial production of BioSeNPs is not well understood. Se containing products from both microbial reduction and abiotic methods need to be refined to obtain pure Se. Eco-friendly and cost-effective Se refinery methods need to be developed. Overall, this review offers insight into the necessity of shifting attention from Se remediation to Se recovery.
... Korea and China [1][2]. Clinical manifestations include joint pain, enlarged deformed joints, mobility disorders, and limb muscle atrophy [3][4][5]. KBD is prevalent in 14 provinces in China, with an incidence of 640,000 cases. Patients are mainly concentrated in the remote areas of western China, including Sichuan, Gansu, and Qinghai provinces, as well as Tibet [6]. ...
Kashin-Beck disease (KBD) in western China is not well controlled. The objective of this study is to evaluate prevention and control children with KBD through a meta-analysis of a community-based trial. Web of knowledge, PubMed, Elsevier, the Chinese National Knowledge Infrastructure (CNKI), VIP and Wanfang data had been electronically searched up to February 2015. Search terms included the trial terms "Salt rich selenium" and "Kashin-Beck disease." Eligible studies were prospective trials of salt-rich selenium in endemic villages. Data extraction was performed by two authors using predefined data fields that also included quality evaluation. Of 292 potentially relevant articles initially screened, reporting 11 community-based trials with a total enrollment of 2652 participants were included, from five provinces in China. The pooled odds ratios (OR) and 95 % confidence intervals (CI) of primary prevention in healthy children were 0.16 and 0.08∼0.33, respectively. The OR and 95 % CI of clinical improvement in KBD children were 6.57 and 3.33∼12.93, respectively. The OR of repairing rate of metaphysis lesions was 5.53 (95 % CI 2.92∼10.47) based on X-ray film, which was statistically significantly different in favor of salt-rich selenium. The combined standard mean difference (SMD) of selenium content in hair was 2.54 (95 % CI 1.21∼3.87) which was significantly higher in selenium group. Current evidence showed that supplement salt-rich selenium was effective in reducing new incidence in healthy children and clinical improvement including repairing metaphysis lesions instead of repairing distal end of phalanx lesions in KBD children.
... Prolonged oral iron therapy has been reported in a few cases to give rise to secondary haemochromatosis (Turnberg, 1965;Johnson, 1968), and in Manchuria a generalized siderosis with a crippling polyarthritis, Kashin-Beck disease, is localized to areas where the water and food have a very high iron content (Hiyeda, 1939). Recently, however, the arthritis has been attributed to the ingestion of a fungus (Nesterov, 1964). ...
... However, mice fed diets high in saturated fats show evidence of damage to chondrocytes () whereas ascorbic acid fed to guinea pigs resulted in less cartilage damage in experimental osteoarthritis (Schwartz et al., 1981). Kashin – Beck or Urov disease in Eastern Siberia, Manchuria and North Korea (Sokoloff, 1985) has been attributed by Russian workers to an arthrotoxic toxin from the fungus, Fusaria sporotrischiella, contaminating wheat (Nesterov, 1964), while Chinese scientists believe it is due to selenium dee ciency (Westermark, 1984). Mseleni disease is an arthropathy indigenous to Northern Zululand (DuToit, 1979), which has been attributed to manganese dee ciency (Sokoloff, 1985). ...
... Taken together, we believe that it is better to understand the pathogenesis of KBD and OA by determining the location of protein expression of CSGalNAcT-1 and Hapln-1 in morphological zones. KBD, an endemic osteoarthropathy, principally occurs in childhood [24]. The many severe KBD patients also exhibited decreased limb length and short stature due to the focal and irregular closure of the growth plates [25]. ...
Purpose
Kashin-Beck disease (KBD) is an endemic degenerative osteoarthritis associated with extracellular matrix degradation. The aim of this investigation was to evaluate the role of targeting genes in the pathogenesis of KBD and primary osteoarthritis (OA) involved in extracellular matrix degradation.
Methods
Agilent 44 K human whole-genome oligonucleotide microarrays were used to detect the gene expression in KBD and OA cartilage. The mRNA and protein expressions of CSGalNAcT-1 and Hapln-1 in chondrocytes were verified by reverse transcription polymerase chain reaction (RT-PCR) and western blot, and their expression in cartilage were verified with immunocytochemical analysis. Meanwhile, CSGalNAcT-1 and Hapln-1 protein levels in the selenium intervention group of KBD with different concentrations (0.25, 0.1and 0.05 μg/ml) were detected by western blot.
Results
CSGalNAcT-1 and Hapln-1 were down-regulated in KBD and OA at both mRNA and protein levels, and were increased in Se(Selenium) groups compared to KBD free-Se group. However, Wnt 3a, β-catenin and Runx-2 were up-regulated in OA and KBD at protein levels. Additionally, immunohistochemical staining showed that CSGalNAcT-1 and Hapln-1 were reduced in all zones of KBD and OA articular cartilage, but not significantly reduced in the up zone of OA articular cartilage.
Conclusions
The CSGalNAcT-1 and Hapln-1 were down-regulated in both KBD and OA cartilage. CSGalNAcT-1 may be involved in the damage of articular cartilage of KBD and OA by regulating Hapln-1 in the Wnt/β-catenin signalling pathway. It was indicated that CSGalNAcT-1 and Hapln-1 may play important roles in the pathogenesis of KBD and OA.
Background
To study the epidemiological characteristics of atypical Kashin–Beck disease cases without characteristic hand lesions such as interphalangeal joint enlargement and brachydactyly and the characteristics of ankle joint lesions.
Methods
We investigated Kashin–Beck in the endemic villages in Heilongjiang Province. The patients were judged according to the “Diagnosis of Kashin–Beck Disease” (WS/T 207–2010). The severity of foot lesions was judged based on the changes of X-ray images. Residents of non-Kashin–Beck disease area were selected as normal controls in Jilin Province.
Results
A total of 119 residents over 40 years old were surveyed in a natural village in the non-endemic area. A total of 1190 residents over 40 years old were surveyed in 38 endemic areas of Kashin–Beck disease. A total of 710 patients with Kashin–Beck disease were detected, including 245 patients with grade I, 175 patients with grade II, 25 patients with grade III, and 265 atypical patients. Among all investigated patients, 92.0% (653/710) had ankle joint changes, and it was 80.0% (196/245) in grade I patients and 95.4% (167/175) in grade II. Varying degrees of ankle joint changes were found in both grade III and atypical patients. The grade of Kashin–Beck disease was correlated with the degree of ankle joint change ( P < 0.001), and the correlation coefficient r s = 0.376. Atypical Kashin–Beck disease patients in mild and severe endemic area of Kashin–Beck disease were younger than those with typical Kashin–Beck disease.
Conclusions
We found a correlation between the degree of ankle joint change and the grade of Kashin–Beck disease. The higher the grade of Kashin–Beck disease, the more serious the change of the ankle joint.
Objective:
Kashin-Beck disease (KBD) is an endemic osteoarthropathy, which causes disability and heavy socioeconomic burdens. The preventive measures have been taken in the past few decades. However, recent KBD-epidemiological trend and comprehensive effect of its preventive measures need to be evaluated.
Methods:
By employing typical survey, cross-sectional survey, case-control study, intervention trial, and national surveillance, the present study summarizes comprehensive role of KBD-preventive measures.
Results:
The endemic KBD is distributed in a long and narrow area of the world. The latest epidemic began in the late 1950s and lasted until the end of 1980s. Epidemiology of the KBD was characterized by early-onset, gender equality, agricultural area, regional discrepancy, family aggregation, annual fluctuation, etc. Multivariate regression analysis suggested that etiology of the KBD was food-related factors such as fungal contamination of grains, selenium deficiency, imbalance of protein intake, etc. A series of intervention measures for KBD control had been implemented since 1990s, and involved more than 300 million residents. National incidences were 22.1% in 1990, 16.0% in 1995, 12.3% in 2000, 5.5% in 2005, 0.38% in 2010, and 0.18 in 2015, respectively. Although new patients were annually decreased, it still affected 22,567,600 inhabitants and there were 574,925 patients in 2016.
Conclusions:
Etiology of the KBD is food-sourced. Its decreased incidence may attribute to an effective implementation of preventive measures. It is possible to eradicate KBD from the earth in the near future.
A systematic analysis of the published results of studies on the effect of trace elements such as barium (Ba) and strontium (Sr) on functional systems of the human body. With sufficient intake of stable Sr isotopes, a decrease in pulse arterial pressure is observed in the morning, associated with a decrease in mortality among patients with ischemic heart disease and arterial hypertension. There is a statistically significant linear relationship between the ratio of the Ba and Sr content in the hair and the circadian heart rate index, that is associated with an increase in the incidence of thrombosis, particularly during treatment with the Strontium ranelate at therapeutic doses. A statistically significant negative correlation was found between the Ba tissue concentrations and the increase in the patient’s age that is also associated with an increase in the risk of progressive degenerative brain disorders, as well as chronic obstructive pulmonary disease. The possible association of Sr and Ba tissue concentrations in the body with the risk of Kashin-Beck disease is discussed. For early detection of this disease in the population residing endemic areas and the areas of its sporadic cases, it is advisable to evaluate the function of active flexion of the distal joints of II - IV fingers of the hands in people aged 4 to 26 years with imaging the bone structure of these fingers in the people at risk within acceptable doses of radiation. © 2019, Northern State Medical University. All rights reserved.
Der Krankheitsbegriff der Osteogenesis imperfecta wäre mit der wörtlichen Übersetzung „unvollständige Knochenbildung“ nur unvollkommen wiedergegeben. Auch die 1940 von Bauer und Bode angewandte Bezeichnung „erbliche unvollkommene Knochenbildung“ ist nach unserer heutigen Auffassung unzureichend, da der Osteogenesis imperfecta vermutlich ein enzymatischer Defekt in der Ausreifung des Kollagens zugrunde liegt, wodurch es im Reticulinstadium stecken bleibt. Die Auswirkungen am Skeletsystem sind hierbei am bedeutungsvollsten. Daneben sind vielfach charakteristische Veränderungen an den Augen, Ohren, Zähnen sowie der Haut vorhanden.
The objective of this study was to evaluate the reliability and validity of the joint dysfunction index (JDI) for assessment of therapeutic efficacy for Kashin-Beck disease (KBD). In an initial survey, completed questionnaires were obtained from 276 of 281 patients (98.2 %). A follow-up survey was completed with 64 KBD patients among 276 cases. A third survey selected 60 KBD patients who underwent intra-articular injection of sodium hyaluronate in the knees ascertained from the findings of the second questionnaire. Reliability was assessed using test-retest, "split-half" reliability and Cronbach's alpha coefficient. Factor analysis and item-to-domain correlation were used to analyze validity. The coefficient of variation (CV) was used to measure the sensitivity of scale. Feasibility assessment included consideration of completion time, rate of recovery, and time of completion. Reliability analysis comprised a test-retest correlation coefficient of 0.404-0.546 and a kappa test of 0.404-0.546. Internal consistency analysis comprised a Cronbach alpha coefficient of 0.689 and a split-half coefficient of 0.677. Principal component factor analysis for validity testing extracted a common factor with a cumulative variance contribution of 45.44 %. The JDI score from 276 KBD cases revealed no significant difference associated with age, gender, education, or the body mass index. Sensitivity analysis showed that there was no significant difference between pre-treatment and post-treatment values, with a CV of 96.55-172.06 %. In conclusion, the JDI can be used to evaluate the efficacy of agents used to treat KBD.
Die nachfolgende alphabetische Übersicht berücksichtigt Syndrome, die klinisch und radiologisch von Bedeutung sind; sie dient einer leichteren Orientierung auf diesem durch Eigennamen und viele Synonyma unübersichtlich gewordenen Gebiet. Für die Nomenklatur und Schreibweise wurde das „Würterbuch der klinischen Syndrome“ von Leiber/Olbrich als maßgeblich angesehen. Auf Abbildungen im Textteil ist an entsprechender Stelle verwiesen.
This chapter provides an overview of the general pathology of the peripheral joints. Synovial joints are functional interfaces; they lie at the line of demarcation between hydrostat and rigid framework and are so formed that their physiological and pathological behavior is dominated by either characteristic or by a combination of the two. Disorders of synovial joints are disturbances of the cells of non-mineralized connective tissue, of mineralized tissue, or of both. In osteoarthrosis (OA), the marginal osteophytes may be predominantly hyaline cartilage, fibrocartilage, compact or cancellous bone, and bone marrow. The bone of OA is often hyperemic. Raised bone metabolism prevails; excess reactive bone deposition and excess bone resorption are prominent, and trabecular microfracture is associated with these changes. Subchondral bone cysts develop inconstantly. They may reach a diameter of 2.0 cm; there is a dense, bony wall, a fibrous lining, and a content of loose myxoid material which may be fluid. Osteophytic outgrowths of bone develop both at the chondro–synovio–osseous margin of an affected joint and near attachments of the capsule and of the articular ligaments to bone. Many systemic and local conditions may lead to secondary OA. Among these are mucopolysaccharidoses; metabolic bone disease; some forms of aseptic bone necrosis; rheumatoid, infective, and traumatic arthritis; and hemophilia.
Synonyma: Chondrocalcinosis (inter-)vertebralis, Pyrophosphat-Spondylopathie; englisch: Spondylopathy of Calcium pyrophosphate dihydrate (CPPD) crystal deposition disease (Pseudogout-Syndrome), Pyrophosphate Spondylopathy; französisch: spondylopathie de la chondrocalcinose articulaire, spondylopathie chondrocalcinosique
The differential diagnosis of arthritis is exhaustive (see table of contents; Chapter 2). The 1988 edition of the Primer on the Rheumatic Diseases,1 prepared by a committee of the American College of Rheumatology (ACR), lists over 100 disorders in which arthritis may be a significant manifestation. A few of these conditions have not yet been reported in children. We have also found it necessary to add to this exhaustive list. Several additional disorders were recently first seen in children in our own clinic, and there continue to be new additions to this list each year.
This is perhaps the commonest form of joint disease. Its prevalence is difficult to estimate accurately, but it is generally believed that about five million people in the United Kingdom are affected, causing a loss of 4.7 million days from work per year (Wood, 1976). Even so, it was not recognized as a separate entity until the last century. The archaic name arthritis deformans gave way to osteoarthritis; but, as it is widely regarded as a degenerative disease, the term osteoarthrosis has been in vogue during the last two decades. However, the simplistic concept of ‘wear and tear’ occurring in those of advancing years is no longer tenable as new evidence sheds light on the likely series of pathogenetic changes that take place in the joint.
Pour les rhumatismes dégénératifs de la colonne vertébrale, comme pour les rhumatismes dégénératifs des articulations, les seuls facteurs étiopathogéniques dont le rôle soit bien établi, sont: le facteur héréditaire (surtout net pour les arthroses digitales), les facteurs mécaniques (surtout nets pour les arthroses de la hanche), et l’avancée en âge (dont le rôle est évident pour tous les rhumatismes dégénératifs). En ce qui concerne le rôle des troubles métaboliques, ce que l’on sait est actuellement peu de choses auprès de ce que l’on ignore.
Zwei Tatsachen allgemeiner Bedeutung sind es, die einleitend zur Röntgenologie des Handskeletes erwähnt werden müssen: „Die Hand hat zusammen mit dem Arm durch den aufrechten Gang eine Entwicklung erfahren, die sie zu einem für den Menschen charakteristischen und nur ihm eigentümlichen Organ gestaltet hat. Sie ist daher seit frühesten Zeiten zu einem Symbol des ganzen Menschen geworden, unlösbar verbunden mit der Entwicklung der Menschheit“(Lanz und Wachsmuth). M. Bürger schreibt: „Die Hand, dieses vielgestaltige und wohl wirksamste Ausdrucksmittel menschlicher Tätigkeit und menschlichen Geistes, ist neben dem Gehirn dasjenige Organ, durch welches wir uns wesentlich vom Tier unterscheiden. Der aufrechte Gang hat dem Menschen ermöglicht, seine Hände zum Wirken auszubilden und als Werkzeug zu benutzen. Alles, was wir mit dem Geist in die Tat umsetzen wollen, alle unsere „Hand“lungen, bedürfen letztlich der Vermittlung unserer Hände. Ist der Mensch in seinem Wirken durch Krankheit gehemmt oder beeinträchtigt, so wird dies auch Spuren an der Hand hinterlassen.“
Synonyma: Chondrocalcinosis articularis; Gelenkchondrokalzinose, Pseudogicht; Pyrophosphat-Arthropathie; englisch: calcium pyrophosphate dihydrate (CPPD) crystal deposition disease, pseudogout syndrome, pyrophosphate arthropathy, pyrophosphate synovitis; französisch: chondrocalcinose articulaire, arthropathie lors de chondrocalcinose.
A great number of reviews have evidenced the importance of selenium for our organism since it was proved to be an integral part of the enzyme glutathione peroxidase, which protect tissues from oxidative break down. Diet is the only source of selenium. Therefore, an adequate nourishment from chilhood is important in order to cover our need of this mineral.
Background To investigate serum and epiphyseal plate expression of cartilage oligomeric matrix protein (COMP) in rats fed with Kashin-Beck disease (KBD)-affected diet. Methods Two hundred Wistar rats were divided into five groups. Group A was fed with a normal diet as control; group B was fed with a normal diet and trichothecene (T-2) toxin; group C was fed with a low nutrition diet and T-2 toxin; group D was fed with a low nutrition diet; and group E was fed with a KBD-affected diet. The serum bioactivity of COMP was tested by enzyme linked immunosorbent assay. The epiphyseal plate COMP messenger RNA (mRNA) was detected by reverse transcription-polymerase chain reaction (RT-PCR). Histological sections were stained with hematoxylin and eosin (HE). Results After 4 weeks, the epiphyseal plate showed more intense necrosis of chondrocytes in groups B, C, and E. The serum levels of COMP and epiphyseal plate COMP mRNA levels in group E at 12 weeks were the highest of all groups and had significant difference when compared with other groups (P<0.05). Compared with other groups, the variation trend of the serum levels of COMP and epiphyseal plate COMP mRNA levels in group C were similar to group E from week 1 week to week 12. Conclusions These findings demonstrate COMP metabolism in serum and epiphyseal plate expression were altered in KBD and may play a role as a potential biomarker for monitoring pathogenic progression of cartilage destruction of KBD.
Arthritic diseases are a world-wide problem affecting every race, but there are large differences in the prevalence and manifestations even of the more common conditions in different populations and countries. The author reviews some of the more recent epidemiological studies on disorders such as osteoarthrosis, rheumatoid arthritis and gout, and discusses factors which appear to be involved in the prevalence of other rarer arthropathies. It is considered that a greater study of these differences might ultimately prove helpful in the understanding of the aetiology of arthritic disorders.
Es werden unsere heutigen Kenntnisse über akute und chronische Erkrankungen durch den Verzehr Mykotoxin-haltiger Nahrungsmittel beschrieben. Ausführlich wird auf epidemiologische Untersuchungen im Zusammenhang mit dem Auftreten von primärem Leberkrebs in Thailand und verschiedenen Regionen Mittel- und Südafrikas eingegangen.
Cette étude est une synthèse de travaux portant sur l’histoire naturelle de la maladie de Mseleni, une arthrose endémique invalidante, ses conséquences socioéconomiques, ses paramètres démographiques, alimentaires, les données géologiques et la génétique de cette maladie. Quelques nouvelles pistes de recherche sont de plus proposées pour l’étude de l’étiologie de cette maladie, telles que l’analyse des isotopes stables et les mécanismes épigénétiques. La maladie de Mseleni est une chondrodysplasie décrite pour la première fois en 1970. Elle est géographiquement limitée à une zone éloignée de la région Maputaland au nord du KwaZulu-Natal, en Afrique du Sud. Cette maladie touche la plupart des articulations et principalement la hanche. Les premiers symptômes sont une douleur et une raideur articulaires et l’évolution progressive peut compromettre la marche. La maladie de Mseleni se caractérise par deux anomalies distinctes, une protrusion acétabulaire (qui touche principalement les femmes et dont la fréquence augmente avec l’âge), et une dysplasie de la hanche, également plus fréquente avec l’âge. Beaucoup de travaux ont été réalisés sur des sujets atteints de cette maladie et sur leur environnement. La maladie de Mseleni est encore mal comprise malgré de nombreux travaux portant sur son étiologie. L’étude des mécanismes épigénétiques et l’analyse des isotopes stables de dents donnent des pistes pour l’étiologie de cette maladie. Ces méthodes peuvent également s’appliquer à d’autres chondroplasies d’étiologie inconnue.
Mycotoxins are naturally occurring toxic chemical compounds produced by fungi infesting agricultural crops both during crop growth and storage. Such secondary metabolites, when ingested, can produce toxic syndromes in humans. This study is the first survey that documents the occurrence of mycotoxins in stored barley in Tibet Autonomous Region [P.R. China]. Twenty-five samples of barley collected from Tibet were analyzed for the presence of aflatoxins, fumonisins, ochratoxins, zearalenone, deoxynivalenol, and T-2 toxin using an easy, sensitive, competitive direct enzyme-linked immunosorbent assay. Ninety-six percent of the samples were contaminated with zearalenone at concentrations ranging from 25 to 270 µg/kg. Seventy-six percent of the samples were contaminated with T-2 toxin at concentrations ranging from 1 to 163 µg/kg. In contrast, deoxynivalenol was observed in only 12% of the samples, with toxin concentrations ranging from 25 to 270 µg/kg. Aflatoxin was observed in only 4% of the contaminated samples.
Selenoproteins are a protein family in which the amino acid, selenocysteine (Sec) is incorporated cotranslationally at UGA
“stop” codons. Selenoprotein synthesis requires unique RNA and protein factors in order to recode UGA. In eukaryotes, both
the biosynthesis and incorporation of Sec employ nucleo-cytoplasmic shuttling of multiple protein factors, and this shuttling
appears to play crucial roles in the functions of these factors. A dynamic series of supramolecular complexes may increase
the efficiency of Sec incorporation, as well as the recycling of the Sec biosynthesis factors. This chapter will review the
putative functions of these factors and explore the protein-nucleic acid and protein-protein interactions that define their
biological roles. In addition, preliminary attempts to isolate supramolecular translation complexes from in vivo and in vitro systems will be discussed. Recent evidence suggests that both the biosynthesis of Sec-tRNA and the incorporation of Sec are
physically coupled within supramolecular complexes that retain and coordinate the many factors required, tRNA channeling systems
for the standard 20 amino acids rely on cotranslational complexes for spatial and temporal regulation of the multiple enzymatic
factors responsible for aminoacyl-tRNA recycling. This chapter draws parallels between these functionally equivalent systems
in eukaryotes.
To investigate the association between variants in the HLA-DRB1 gene and Kashin-Beck disease (KBD), as well as associations of selenium and iodine deficiencies with KBD in a Tibetan population.
Fourteen single-nucleotide polymorphisms (SNPs) were genotyped around the HLA-DRB1 gene, and HLA-DRB1 allele genotyping was performed in a discovery cohort, composed of 605 patients with KBD and 393 control subjects, and/or a replication cohort, composed of 290 patients with KBD and 295 controls. Plasma concentrations of selenium and iodine were measured and compared by t-test in 299 patients with KBD and 280 controls from the same villages.
Four SNPs (rs6457617, rs6457620, rs9275295, and rs7745040) in the HLA-DRB1 gene locus were significantly associated with KBD in both the discovery cohort and replication cohort (combined cohort odds ratios [ORs] 1.307-1.402, P = 0.0039-0.0006). The protective haplotype GTCC and the risk haplotype ACGT, each generated by the 4 SNPs, showed a significant association with KBD (for GTCC, OR 0.77, P = 0.0031; for ACGT, OR 1.40, P = 0.0014). HLA-DRB1 allele genotyping revealed that the frequencies of HLA-DRB1*08 and *11 were significantly different between patients with KBD and controls (for HLA-DRB1*08, OR 0.731, P = 0.00564; for HLA-DRB1*11, OR 0.489, P = 0.000395). Moreover, plasma concentrations of selenium and iodine were significantly different between patients with KBD and controls from the same villages (P = 0.0013 and P = 1.84 × 10(-12) , respectively).
These findings, obtained in plasma samples from Tibetan patients with KBD and healthy control subjects from the same regions, confirm the role of selenium and iodine deficiencies in the development of KBD. Moreover, genetic variants in the HLA-DRB1 gene significantly increase the susceptibility to KBD in this population.
In this paper past research on the natural history of Mseleni joint disease, a crippling endemic osteoarthritis, its socio-economic impacts, the demographics, diet, geology and the genetic background of affected people are reviewed. In addition, some new research ideas are suggested to continue the search for etiological avenues for this disease such as stable isotope analysis and epigenetic mechanisms.
Mseleni joint disease is a chondrodysplasia first described in 1970. It is geographically confined to a remote area in the Maputaland region in northern Kwazulu Natal, South Africa. This disease affects most joints but primarily those of the hip; it is a progressive condition beginning with pain and stiffness until the patient's ability to walk becomes compromised. Mseleni joint disease is characterized by two distinct abnormalities, protrusio acetabuli that mainly affects females and increases in frequency with age, and hip dysplasia that is more frequent with age. Much research has been conducted on the people with the disease and their surrounding environment.
Despite intensive investigations into the etiology of Mseleni joint disease, it remains unknown. As a result the examination of epigenetic mechanisms and stable isotope analysis of teeth are suggested as a means of providing information on the etiology of the disease. These methods can also be applied to other chondroplasias of unknown etiology.
This article presents a model where firms may endogenously externalize part of their production process. We start from the premise that adaptation to uncertainty cannot be contracted upon in the worker/employer relationship. Vertical separation then balances flexibility gains against hold-up costs of opportunistic behavior by outside contractors. In equilibrium, the degree of separation is shown to depend on the degree of product market competition, contractor's bargaining power, and the volatility of demand shocks. Our main result is that an increase in the degree of vertical separation amplifies the elasticity to demand shocks of firms' sales and employment. It does not, however, amplify aggregate uncertainty. Evidence from firm-level data is shown to be largely consistent with the main implications of our theory. (JEL: L16, L23, L24) (c) 2007 by the European Economic Association.
The Urov disease (endemic osteohondritis)
- Dobrovolsky L. O.
To the problem of disforming endemic osteoarthritis in the Baikal area
- Beck E. B.
The toxity of some forms Fusarium sporotrichiella, picked from grains of Eastern Siberia
- Perckel N. B.
The information of spreading of goitre and cretinism within the limits of the Russian empire
- Kashin N. I.
About the reasons of the Urov disease's origin (geo-chemical study)
- A P Vinogradov
The Urov (Kaskin-Beck) disease
- N. I. Damperov
To the problem of histology of the Urov disease
- V A Donskov
The escription of endemic and other disease, prevailing in the Urov-river area
- N. I. Kashin
To the problem of Kashin-Beck disease
- O B Nickolaev
The Urov disease its prevention and treatment
- L F Kravchenko
So called “Urov disease” or endemic deforming osteo-arthritis in Zabaikalye
- M I Schwartzman
Geological chemistry of the Urov endemic's area
- Vinogradov
The main principles and tasks of conservative treatment of the Urov disease patients
- L F Kravchenko
Clinical picture of the Urov disease at children. The All-Union Conference of Endocrinologists M
- A M Popov
Experimental food micotoxical enchondral dystrophy (to etiology and pathogenesis of Kashin-Beck disease)
- Y I Rubinstein
Deforming arthritis. The News of Modern Med
- N A Schenk
To the problem of osteoarthritis deformans endemica in the Baikal area. St-Petersberg Medical Military Academy Theses
- E B Beck
The goitre and cretinism in the limits of Russia and outside.The records of the Scientific Medical Society of the Eastern Siberia Irkutsk
- N I Kashin
Röntgenodiagnostics of articular involvement
- Rokchlin
Experimental food micotoxical enchondral dystrophy (to etiology of Kashin-Beck disease). Hygiene and sanitation N 12 1949 p
- Y I Rubinstein
Pathomorphological changes in joints of the Urov disease patients according to the operation material
- A A Florensov
The teaching hypotesis) of articular diseases from the clinical point of view
- N A Velyaminov
Röntgenopaleanthropological investigation of bone materials of the Zabaikalye
- D G Rokchlin
To the problem of geographical prevalence of the Beck disease and rudimentary Beck's changes. News of Rontgenology and Radiology ” v. 21 part 2 M-L
- D G Rokchlin
Clinical classification of the Urov disease
- F P Sergievsky
To the problem of osteoarthritis deformans endemica in the Baikal area. St-Petersberg
- Beck
Urov disease” or endemic deforming osteo-arthritis in Zabaikalye
- Schwartzman
About the Kashin-Beck disease
- Turner
Röntgenodiagnostics of articular involvement. L. v.I. 1935 v.2
- D G Rokchlin
Geological chemistry of the Urov endemic's area. Archives of Biological Sciences v. 53 part 2
- A P Vinogradov
The escription of endemic and other disease prevailing in the Urov-river area. The records of physicomedical scientific society attached to the McScow University
- N I Kashin
About the Kashin-Beck disease. Orthopedia and Traumatologia. N 4 Kcharkov
- G I Turner