大骨节病诊断学
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Preface

Kashin-Beck Disease(KBD)is an endemic and deformative osteoarthropathy.The primary lesions of KBD in growing children are the multiple and symmetrical degeneration and necrosis of epiphysis cartilage,epiphyseal plate cartilage and articular cartilage,and the secondary lesion is degenerative osteoarthritis.Brachydactylia,nanomelia,gnomes and permanent disability could be seen in severe KBD patients.It had been more than 160 years since the first report of KBD in 1849.KBD mainly distributed in Heilongjiang,Jilin,Liaoning,Hebei,Beijing,Shandong,Henan,Inner Mongolia,Shanxi,Shaanxi,Gansu,Qinghai,Sichuan provinces and Tibet Autonomous Region.Disease areas are located in a panhandle region from the northeast and southwest part of China and extended to Eastern Siberia and a few regions in the northern part of North Korea.In addition to those areas,there were no other KBD disease areas in the world.In 2011,the former Ministry of Health entrusted the Center for Endemic Disease Control to make an examination and evaluation on the epidemic status of KBD in Beijing,all experts came to a conclusion that KBD in Beijing had achieved the elimination.

Diagnostics research on KBD is very limited outside of China and related report is only embodied in clinical diagnosis.Clinical diagnosis and classification standard of KBD was derived from the report by a Russian doctor named Beck in 1906.The basic characteristics include that finger joints enlargement is degreeⅠ,brachydactylia is degreeⅡ,brachydactylia and gnomes is degreeⅢ.This standard is still in use now and has been accepted by most occupational workers for KBD prevention and control.Chinese researchers have several main contributions to the diagnostics research of KBD.Firstly,they confirmed the children hands x-ray was very applicable for KBD diagnosis.Distal end of phalanx image change was a specific hallmark and metaphyseal image change was a sensitive hallmark for KBD diagnosis.These confirmations reflected the characteristics of children KBD patients,showed the pathological mechanism from osteochon drosis in early stage to osteoarthritis in late stage and could be used to make a scientific description for the gradual deterioration process from non-cases,early cases,typical cases to severe disability cases.Secondly,Chinese researchers confirmed KBD mainly affected endochondral bone,which showed atrophy,degeneration and necrosis of the cartilage,accompanied by the hyperplasia,repair and adaptation.With the progression of KBD,the obstacle of endochondral ossification appeared eventually.The clinical characteristics were joint deformity and gnomes.These confirmations revealed the pathologic basis and development law of the KBD x-ray basic image changes.

According to specific clinical signs and exposure history,it is not difficult for the correct diagnosis of typical KBD patients,while it is difficult for the diagnosis of atypical and earlystage KBD patients.KBD had been listed into the category of International Classification of Diseases.Professor Tokio Takamori in Japan had compiled a book named “Kashin-Beck's disease”.Professor Qian Zhizhong in China had compiled another book named “Identification of Kashin-Beck disease and bone disease”.However,a systematic and scientific book for KBD diagnostics hasn’t been published yet.This book covers the research achievements of KBD in epidemiology,clinic,X-ray imaging and pathological changes,and fills up a vacancy of KBD diagnostics and has great application value for the workers engaging in teaching,scientific research and disease prevention and control.

With the support of Chinese Center For Disease Control And Prevention,Center for Endemic Disease Control collaborated with Institute of Endemic Diseases of Xi􀆳an Jiaotong University Health Science Center,Institute of Endemic Diseases of Sichuan Center for Disease Control and Prevention,Jilin Provincial Second Institute for Endemic Disease Control,Heilongjiang Provincial Center for Disease Control and Prevention and West China Hospital of Sichuan University,and compiled precious photos of clinic,x-ray images and pathology accumulated over the years.With pictures and detailed explanations,this book respectively interpreted the KBD epidemiology,the diagnosis and differential diagnosis for KBD child patients and adult patients,KBD pathological changes and so on.

Owing to certain restriction in overall recognition and limited capacities on the part of the authors,some shortcomings are surely inevitable.Criticism and advices,therefore,are more than welcome.

Dianjun Sun

November,2016,Harbin