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Uncovering Cassin-Baker disease: Why are children the main victims of this rare disease?
Kashin–Beck disease (KBD) is a chronic and endemic osteochondrosis, mainly distributed in 15 provinces from northeast to southwest China, with the highest incidence rate in Tibet. The disease, which typically affects children between the ages of 5 and 15, has affected more than a million people.
Symptoms of Cassin-Baker disease include joint pain, morning stiffness, difficulty in flexing and extending the elbow, enlargement of the interphalangeal joints, and limited movement of many joints.
The basic pathological feature of KBD is the death of chondrocytes on the growth plate and articular surface, which may lead to growth retardation and secondary osteoarthritis. Although the disease has been known for more than 150 years, its cause is still not fully understood. Studies indicate that potential causes of Cassin-Baker disease include mycotoxins in grains, trace mineral deficiencies in nutrients, and high levels of fulvic acid in drinking water.
Among these factors, deficiencies of selenium and iodine are considered to be important factors leading to KBD. Contamination of grains with toxins produced by fungi may trigger KBD, especially T-2 toxin, which causes lesions in hematopoietic, lymphatic, intestinal and cartilaginous tissues, especially in chondrocytes. High levels of fulvic acid directly damage cartilage cells. In addition, selenium supplementation measures have shown the potential to prevent KBD in some areas but failed to show significant effects in other areas, which means that selenium deficiency may not be the main factor causing KBD.
Genetic studies have recently shown that a single nucleotide polymorphism (SNP rs6910140) in the COL9A1 gene is significantly associated with the development of Cassin-Beck disease, indicating the role of COL9A1 in KBD.
Prevention of Cassin-Beck's disease
Prevention of KBD has a long history, and intervention strategies are usually based on one of three major theories of its causes. Selenium supplementation, along with the use of antioxidants (such as vitamins E and vitamin C), has been reported as successful preventive measures, but other studies have shown no significant reduction in incidence compared with control groups.
In some areas, this may be due to logistical difficulties (e.g. daily or weekly supplementation and medication supply), potential toxicity (with poorly controlled supplementation strategies), associated iodine deficiencies, and low compliance. Making selenium supplementation a thorny issue.
In response to the mycotoxin theory, Guangxi Province proposed to treat grains before storage, but the results have not been reported in international literature. Changing the source of grains has shown efficacy in Heilongjiang Province and North Korea. Regarding the impact of drinking water, changing the water source to deep well water has been reported to reduce the X-ray display rate in different environments. Overall, the effectiveness of preventive measures remains controversial, mainly due to methodological issues (lack of randomized controlled trials) and inconsistencies in results.
Treatment of Cassin-Beck's disease
Treatment for KBD is primarily palliative. Orthopedic surgeons in China and Russia have successfully performed some surgical corrections. Until 1992, Médecins Sans Frontières launched a physiotherapy program in Nyemo County, Lhasa, aimed at reducing the symptoms of KBD patients (mainly adults) suffering from severe joint injuries and pain. Physical therapy has a significant effect on joint mobility and pain in patients with KBD. Over time, the program was expanded to other counties and regions in Tibet from 1994 to 1996.
Epidemiology
The incidence of Cassinian-Baker disease is limited to 13 provinces and two autonomous regions in China. In addition, the disease has also been reported in Siberia and North Korea, but with socioeconomic development, the incidence rate in these areas has declined. It is estimated that approximately 2 to 3 million people in China are affected by KBD, with 30 million people living in endemic areas. Life span in the KBD area is significantly reduced by selenium deficiency and Keshan's disease (endemic cardiomyopathy).
In some villages, clinical symptoms of KBD among children aged 5 to 15 show that the incidence of suspected KBD is as high as 100% as the epidemic changes.
As the number of cases of KBD continues to increase and its root cause remains unsolved, it begs the question: In the journey to combat this rare disease, can we find truly effective prevention and treatment methods to create a better world for the next generation? A healthy future?
