2012年 · 第50卷第01期

In the process of diagnosis and treatment of children's rheumatism, how should medical staff standardize the joint function examination of children, how to systematically evaluate the joint function of children with chronic arthritis, how to judge the improvement of children's general condition, and how to choose the evaluation criteria of validity, reliability and sensitivity according to the needs of many foreign joint function evaluation systems^[1]It has always been a difficult problem that has plagued domestic pediatric rheumatology colleagues. This paper briefly introduces the widely used joint function evaluation system of juvenile arthritis at present, hoping to help specialists to understand and apply it.

IgA nephropathy 1968 was first developed by Berger and Hinqlais^[1]It is reported that it refers to glomerular disease caused by diffuse deposition of IgA or IgA-based immune complex in the mesangial region of the glomerulus under immunofluorescence or immunohistochemistry. IgA nephropathy should be diagnosed by immunopathological examination of renal tissue, and secondary IgA nephropathy caused by systemic disease should be excluded.

Viral infection plays an important role in pediatric infectious diseases. Common viruses associated with pediatric infections include respiratory viruses, enteroviruses and others. The symptoms caused by different viruses of the same infection route are often similar, which is difficult to identify clinically. Antibiotic treatment is ineffective for viral infection, and abuse can induce bacterial resistance. Therefore, pathogen detection plays an increasingly important role in clinical diagnosis and treatment of viral infectious diseases. The detection of the virus itself generally includes virus isolation, electron microscopy observation, virus antigen detection and virus nucleic acid detection, and the detection results have more direct etiological diagnostic value; However, the detection of virus-specific antibodies is an indirect diagnostic index of virus infection. In application, it is necessary to comprehensively judge the detection results in combination with clinical practice, including the site of infection (systemic or local), the time of blood collection and the number of days of onset, etc.

hemophilia is an X-chromosome-linked recessive genetic disease that causes disorders in coagulation factor production due to abnormalities in the genes encoding coagulation factors. It usually occurs in men and is carried by women. The main manifestation of patients is repeated abnormal bleeding since childhood, and the common bleeding site is the joint, accounting for 70% to 80% of all bleeding manifestations. Repeated joint hemorrhage can cause degenerative changes, deformities, resulting in partial or complete loss of joint function^[1,2]。

leukemic stem cells (LSC) are also known as leukemia initiating cells^[1], refers to a class of cells with self-renewal ability in leukemia cells^[2]。 LSC accounts for a small proportion of leukemia cells and can reconstruct leukemia in immunodeficient mice. It is insensitive to chemotherapeutic drugs and is the root cause of refractory and recurrence of leukemia. Therefore, it is particularly important to distinguish between non-leukemic stem cells and LSCs and design targeted therapeutic drugs for LSCs. At present, the research on LSC involves many aspects: origin, phenotype, genotype, biological characteristics and relationship with microenvironment, etc.

The clinical diagnosis and treatment of children's rheumatic diseases has been carried out in China for nearly 30 years. At present, more than 30 hospitals or children's hospitals have opened children's rheumatology and immunology departments. However, as far as most children's rheumatic diseases are concerned, no matter in terms of diagnostic criteria, treatment plan, drug selection, efficacy evaluation and many other aspects, there are few high-grade evidence-based medicine research reports with wide recommendation value in China. Therefore, the Immunology Group of Pediatrics Branch of Chinese Medical Association and the Editorial Committee of Chinese Journal of Pediatrics decided to formulate the "Suggestions for Diagnosis and Treatment of Juvenile Idiopathic Arthritis (Multi/Oligoarticular Type)" (hereinafter referred to as the "Suggestions"). Confined to the current level of subject development, there must be many shortcomings in "Suggestions". The following expounds the relevant issues in the writing of "Suggestions" as follows.

juvenile idiopathic arthritis (JIA) is a group of chronic and systemic diseases of unknown cause, characterized by chronic joint synovitis, or accompanied by different degrees of damage to various tissues and organs^[1]。

leukocyte adhesion deficiency (LAD) is a kind of primary immunodeficiency disease, which is mainly divided into three types: LAD I, LAD II and LAD III. Here, we focus on the diagnosis, differential diagnosis and treatment of a clinically confirmed child with LAD Ⅰ, and the problems that should be paid attention to in this process, so as to improve the clinical understanding of this disease and achieve early diagnosis and treatment. LAD type I is due to the integrin β 2 (CD18) molecular subunit gene (ITGB2) Mutation^[1], resulting in defects in leukocyte adhesion function and chemotaxis function^[2,3]。 The disease is characterized by delayed umbilical cord shedding, repeated severe infections, periodontitis and delayed wound healing. Laboratory examination can find that: ① peripheral blood white blood cells are significantly increased; ②The expression of CD18 molecules on the surface of neutrophils decreased^[4]。

More than 80 kinds of human diseases are mainly caused by autoimmunity. The morbidity and mortality rate of autoimmune diseases in developed countries rank third after cardiovascular diseases and malignant tumors. In China, especially in the field of pediatrics, the epidemiological data of autoimmune diseases are still very lacking. However, this kind of disease has undoubtedly become one of the main sources of difficult and critical cases in pediatric clinic. The diagnosis and differential diagnosis are complicated, and the treatment is usually very difficult. It is a huge challenge for pediatricians in China, especially pediatric rheumatologists and immunologists. Complex diseases, shortage of specialists, inadequate training and other reasons lead to uneven diagnosis and treatment of children's autoimmune diseases in China and lack of standards. We should think about the clinical coping strategies in the next 5~10 years, and promote the leap-forward development of pediatric rheumatology and immunology while improving the clinical service level.

The "2011 National Symposium on Pediatric Rheumatic Diseases" jointly organized by the Immunology Group of Pediatrics Branch of Chinese Medical Association and the Editorial Committee of Chinese Journal of Pediatrics was held in Xi' an, Shaanxi Province on August 24-27, 2011. 171 representatives from 25 provinces, municipalities and autonomous regions across the country attended the meeting, and received a total of 101 manuscripts, covering common rheumatic diseases in children, as well as clinical and basic research on many rare diseases.