2015年 · 第53卷第12期

The Chinese government issued the Outline of Child Development in China three times in 1992, 2001 and 2011, respectively, which played a key leading role in promoting the development of children's health care in China. According to the data released by the United Nations, China has achieved ahead of schedule the goal of reducing the infant and under-5 mortality rate to two-thirds of 1990 by 2015: the under-5 mortality rate was 1.4% in 2012 and 5.4% in 1990; The infant mortality rate was 1.03% in 2012 and 5.01% in 1990. In 2011, the United Nations Children's Fund ranked the mortality rate of children under the age of 5 in 195 countries and regions around the world, and China ranked 115th from the highest mortality rate. The improvement of children's living conditions and the level of growth and development in China has been ahead of the developing countries and close to the level of developed countries^[1]。 This is an amazing achievement in the development of children's health in China, which has a profound impact on the international community^[2,3]It is also the success of a children's health care system with Chinese characteristics, including the hard work of generations of children's health care practitioners.

Neonatal screening refers to the screening of genetic metabolic diseases, congenital endocrine abnormalities and some serious genetic diseases in the newborn population by rapid and sensitive laboratory methods. The purpose is to make the sick newborn pass the screening before the clinical symptoms appear or when the manifestations are mild, so as to be diagnosed and treated early, so as to prevent irreversible damage to body tissues and organs, and avoid mental retardation, serious disease or death. Newborn screening is an effective way to prevent and treat certain genetic diseases after birth. In 1963, Guthrie bacterial inhibition was used for the first time to detect phenylketonuria, which opened the prelude to neonatal screening^[1]。 In 1970 – 1980, hypothyroidism was also included in the screening program, which was widely carried out in European and American countries^[2]。 At present, the newborn screening rate in developed countries such as Europe and the United States has reached more than 95%, and dozens of diseases are included in the scope of newborn screening. In recent years, primary immunodeficiency disease (PID), as a large group of genetic diseases that seriously endanger children's life and health, has been highly valued by governments and their disease control departments. Developed countries have introduced a series of public health intervention policies mainly focusing on newborn screening, in an attempt to greatly reduce the negative impact of PID on economic and social development^[3]。

Growth is an important indicator of children's health, and evaluating children's physical growth is the daily work of pediatricians, especially children's health care physicians. Measurement and evaluation of children's physical growth is the simplest, economical and non-invasive method to monitor and intervene on individual and group children's health and nutrition status. It is of great significance for early diagnosis of nutritional, chronic systemic and endocrine diseases. Intervention has potential significance for reducing childhood morbidity and mortality. The World Health Organization (WHO) and Chongqing have conducted investigations on the use of growth curve in children. The results show that the use rate of growth curve is low and decreases with age. At the same time, some health workers still have many problems in tracing and interpreting growth curve, which shows that the development and effectiveness of growth monitoring and evaluation in children are not satisfactory all over the world^[1,2]。 In order to give full play to the role of growth monitoring and evaluation in the field of children's health services, and to facilitate children's health care doctors to understand children's growth regularities in their work, correctly use growth curves, detect children's growth deviations early and intervene in time, the editorial committee of Chinese Journal of Pediatrics and the children's health care group of Pediatrics Branch of Chinese Medical Association formulated this recommendation with reference to the internationally commonly adopted children's physical growth evaluation methods. This recommendation is also applicable to clinical pediatricians and physician assistants, general practitioners, child nutritionists, senior nurses, health workers and public health departments to help them use standard tools and methods to measure and evaluate children's physical growth indicators and carry out growth monitoring.

Primary immunodeficiency disease (PID) is a group of rare diseases dominated by single gene genetic variation. Due to gene mutation, immune cells and immune molecules are functionally defective, resulting in immune insufficiency or loss of the body. At present, there are more than 200 PIDs with clear gene mutation. Foreign data show that the overall incidence of PID with clinical manifestations is about 1/2 000. According to this calculation, the number of PID patients in China should reach more than 150,000, but the number of confirmed cases at present is less than 1/10. The main clinical characteristics of PID are repeated infection since childhood, predisposition to autoimmune diseases and malignant tumors. With the continuous progress of immunology and molecular biology technology, especially the widespread application of deep sequencing technology in clinical work, it has been gradually discovered that new PID diseases can be mainly manifested as uncontrolled inflammation and severe allergy^[1]。 Therefore, the clinical manifestations of PID are becoming increasingly complex. Even for immunologists, it is not easy to accurately identify the clinical phenotype of PID and confirm PID. Although the incidence rate of PID is low, it is usually extremely harmful, which seriously threatens the life and quality of life of children. At the same time, the pathogen may replicate, re-toxic and drug resistance in children for a long time, which brings a great threat to public safety^[2]。 Therefore, early diagnosis and rational treatment are the keys to control prevalence, reduce mortality and improve quality of life.

Since 1978, China has implemented a planned vaccination system throughout the country, that is, according to epidemic surveillance and population immunity analysis, according to the prescribed immunization procedures, the planned use of vaccines for vaccination, so as to improve the immunity level of the population and achieve the purpose of controlling and even eliminating corresponding infectious diseases. In 1982, the national immunization program for children was unified. In 2008, 15 infectious diseases, such as hepatitis A and meningococcal encephalitis, which can be effectively prevented through vaccination, were included in the national immunization program. Therefore, at present, for the vaccination of healthy children, the National Center for Disease Control and Prevention (CDC) of China has a relatively complete vaccination plan and practice guide (the Regulations on the Administration of Vaccine Circulation and Vaccination promulgated by the State Council on April 19, 2005). However, there is no unified opinion or norm on whether, when and how to vaccinate children with abnormal immune function. However, these children are susceptible to vaccine-preventable diseases due to abnormal immunity, so they need to be vaccinated more. At the same time, because the immune response after vaccination is closely related to the immune function state of the body, the immune response of individuals with abnormal immune function to vaccination will be significantly affected, for example, the vaccination cannot produce sufficient protective antibodies, and the vaccination may affect the diseases existing in children. The purpose of this consensus trial draft is to provide Chinese pediatric clinicians with practical basis for immunization of children with immune dysfunction, which is in line with China's national conditions and has operability. However, due to space limitations, it will be published in several parts. This part mainly involves the vaccination of children with primary immunodeficiency disease (PID).

Logistic regression is a kind of probabilistic nonlinear regression, which is a multiple regression analysis method to study the relationship between the outcome of classification and the influencing factors. Logistic regression analysis is widely used in clinical research. The odds ratio in Logistic regression analysis (OR) can visually reflect the strength of the association between influencing factors and outcome. But researchers oftenORThe role of the understanding is insufficient, this paperORThe meaning of is simply sorted out.

Vaccination is the most effective means of preventing and controlling infectious diseases. At present, countries all over the world have very perfect immunization procedures for healthy children and adults, thus preventing the occurrence of many infectious diseases. However, for children with abnormal immune function, whether the routine planned immunization procedure can be followed, who is responsible for judging whether these children can be vaccinated, whether there are special indications and contraindications, and whether these children should be given Class II vaccine vaccination are clinical decision-making difficulties that often trouble children's health care doctors and immunologists. Children with immune dysfunction are susceptible to various pathogenic bacterial infections, and they need vaccination to prevent them. At the same time, the risk of inappropriate vaccination is higher, which are urgent to be solved in the vaccination procedure. In recent years, scholars at home and abroad have attached great importance to the vaccination of these children, and immunologists have been paying attention to the vaccination of these groups^{[1,2,3,4,5,6]}However, a national unified norm or practice guide has not yet been formed. Therefore, the Immunology Group of Pediatrics Branch of Chinese Medical Association, in conjunction with the Editorial Committee of Chinese Journal of Pediatrics, collected and analyzed the domestic and foreign literature on immunization, referred to the suggestions or guidelines of other international professional committees, and reached the consensus of this trial draft after many discussions by relevant experts, striving to provide pediatric clinicians in China with a practical basis for immunization of children with immunocompromised function that is in line with China's national conditions. However, it should be emphasized that this consensus is not a legal or regulatory document, and it is not binding by laws and regulations. It is only used as a reference for clinicians in dealing with vaccination of children with immunocompromises.

Professor Guo Di is the pioneer of children's health care and the founder of developmental and behavioral pediatrics in China. Professor Guo graduated from St. John's University School of Medicine in 1935 with a Doctor of Medicine degree, and then went to the Institute of Pediatrics of the Soviet Academy of Medical Sciences and the School of Continuing Medical Education of the University of Pennsylvania to study pediatrics. When he returned to China in 1937, he actively participated in the rescue of the wounded in Shanghai Red Cross Ambulance Hospital, and also engaged in health care in Shousen Children's Shelter in Wanshou. In the early days of liberation, Professor Guo first responded to the call and invested in the construction of Shanghai Second Medical College. Later, he served as the director of Shanghai Institute of Pediatric Medicine and the director of the World Health Organization Children's Growth and Development Cooperation Center.

A 4-year-old girl was admitted to hospital with "pharyngeal mass for 16 d and neck mass for 14 d". The child accidentally found a pharyngeal mass 16 days ago and a neck mass 14 days ago. He had no fever, headache, sore throat, difficulty swallowing, or choking and coughing after drinking water. The local hospital diagnosed him as "acute tonsillitis". After anti-inflammatory treatment for 2 days, the tonsils did not shrink, so he went to our hospital.

A 9-year-old child was admitted to hospital for "multiple joint swelling and pain for more than 3 years, muscle soreness for 2 days, and fever for half a day". After catching cold more than 3 years ago, the child developed swelling and pain of the right elbow joint, the 2nd to 5th metacarpophalangeal joints of the right hand, and both ankle joints, and limited movement, accompanied by fever, no rash, erythema, alopecia and oral ulcer, no foamy urine and edema, and rheumatoid factor (+) was examined. He was diagnosed as "juvenile idiopathic arthritis (JIA)" outside the hospital. He was given prednisone 10 mg twice/d, methotrexate and naproxen orally (details unknown), and was followed up regularly and the hormone dose was reduced. During this period, joint swelling and pain were repeated. Three months before this admission, the child's swelling and pain in both knees and ankle joints were aggravated by cold, accompanied by fever, and the highest body temperature was 39.4℃. He went to the outpatient clinic of West China Second Hospital of Sichuan University, and the blood white blood cells were 24.8×10⁹/L, neutrophils 0.818, C-reactive protein (CRP) 171.7 mg/L, erythrocyte sedimentation rate 74 mm/1 h, serum ferritin (SF) 1.59 mg/L; Rheumatoid factor 101 kU/L, ANA (–), anti-ENA antibody (–), peripheral blood CD3, CD4 T cells, IgA, IgM and IgG were not abnormal; Blood culture (-), seroblade, chlamydia antibody (-); Plain X-rays of both hands: narrowing of the joint space between both wrists; Lumbar spine digital X-ray and chest CT showed no abnormalities; No abnormalities were observed in bone marrow smears; Polyarticular JIA (rheumatoid factor positive) was considered, and prednisone 15 mg, 3 times/d orally, etanercept 12.5 mg, 1 time/5 d subcutaneous injection, leflunomide 10 mg, 1 time/d orally, and naproxen 200 mg, 2 times/d orally were given. The joint swelling and pain of the children were significantly improved after about 2 weeks of treatment. Reexamination of white blood cells 5.4×10⁹/L, neutrophils 0.616, CRP 15 mg/L. Two months before this admission, the child was admitted to the hospital due to "cough and sputum with fever". He was diagnosed with "severe pneumonia and sepsis" and anti-infection for 3 weeks. During this period, the above-mentioned drugs related to JIA were stopped. After anti-infective treatment, he recovered and was discharged. After discharge, the aforementioned JIA-related treatment continued: prednisone 15 mg, 3 times/d orally, naproxen 300 mg, 2 times/d orally. Five days before admission, the child did not complain of discomfort at the outpatient follow-up visit, and the white blood cells were 11.9×10⁹/L, neutrophils 0.751, hemoglobin 139 g/L, platelets 132×10⁹/L, CRP<1 mg/L. Two days before admission, the child developed muscle soreness, no tenderness and decreased muscle strength, and was admitted to the hospital again due to fever for half a day.

Adult SLE (aSLE) and rheumatoid arthritis are risk factors for premature atherosclerosis compared with population prevalence. Whether in children or adult patients, pericarditis is known to be the most common clinical phenomenon of cardiac involvement in SLE, accounting for about 20% to 30%, and even pericardial tamponade. Followed by enlarged heart, vasculitis. Electrocardiogram abnormalities, mitral and tricuspid insufficiency, and left ventricular systolic and diastolic dysfunction can occur even in the absence of clinical manifestations. Myocardial infarction or stroke caused by atherosclerosis and abnormal coronary artery injury rarely occur in children with SLE (pSLE). After adulthood, its incidence is 6-9 times that of the general population, and it is also significantly higher and earlier than that of aSLE cases. The prevalence age is between 24 and 43 years old, and it becomes more obvious with the increase of age. Therefore, the importance of coronary vascular involvement in pediatric-onset SLE for early detection in childhood and prognosis in early adulthood is gradually being recognized^[1,2,3]。

Over the past 40 years, the incidence of childhood obesity has increased rapidly worldwide and has become a serious health problem for children^[1]。 It is well known that the potential risk factors of childhood obesity mainly focus on health risk behaviors, such as sedentary lifestyle, eating habits and physical activity^[2,3,4]。 Not only that, but obesity may also be related to children's neuropsychological function, especially executive function. The research progress of the relationship between different components of executive function and childhood obesity is reviewed.

nonhomologous DNA end joining (NHEJ) is the main pathway for the repair of DNA double strand breaks (DSBs) in human and mammalian cells. If the defects of protein factors in this pathway prevent DNA damage from being repaired in time, it will lead to VDJ recombination disorder and/or immunoglobulin class switching recombination defect, resulting in combined immune deficiency, chromosome instability, cell death and tumor^[1]。 Currently, the core components found to be involved in NHEJ include DNA-dependent protein kinase (DNA-PKS), X-ray repair cross-complementary protein 4 (XRCC4), DNA ligase IV (LIGⅣ), Cernunnos, Artemis, and other DNA damage repair factors^[2]。 At present, more and more NHEJ pathway deficiency diseases such as Cernunnos, LIGⅣ and Artemis have been reported abroad, but no NHEJ pathway deficiency cases have been reported in China. The clinical molecular characteristics of 40 NHEJ pathway deficiency cases included from Mutation@a Glance (http: / /rapid.rcai.riken.jp/mutation) are reviewed (Table 1) and immune mechanism, among which LIGⅣ deficiency cases are numbered P1~ P13, Cernunnos deficiency cases are numbered P14~ P24, and Artemis deficiency cases are numbered P25~ P40, aiming to promote pediatricians' understanding of this rare disease and improve the diagnosis and treatment level.

The incidence rate of patent ductus arteriosus (PDA) in premature infants varies from 10% to 70%. The smaller the gestational age and the lower the birth weight, the higher the incidence rate and the greater the harm to premature infants^[1]。 PDA is one of the main reasons affecting the survival rate and sequela incidence of premature infants^[2,3,4]。 At present, the treatment of PDA in premature infants mainly includes drug therapy and surgical ligation. Among them, drug therapy is the first choice for clinical treatment of PDA in premature infants. Traditional drug treatments are non-steroidal anti-inflammatory drugs (NSAIDs), including indomethacin and ibuprofen, which are non-selective cyclooxygenase (COX) inhibitors and can reduce prostaglandin E by inhibiting COX activity₂(prostaglandin E₂, PGE₂), resulting in the closure of the ductus arteriosus. Indomethacin and ibuprofen are divided into two dosage forms: oral and intravenous drip, and the effective rate for PDA is 70% ~85%. However, these two drugs have strong adverse reactions to inhibit COX-1, which can cause peripheral vasoconstriction, gastrointestinal bleeding or perforation, oliguria and thrombocytopenia. Oral administration is more likely to cause gastrointestinal complications. At present, there are no intravenous indomethacin or ibuprofen preparations in China, only oral ibuprofen preparations. Because PDA itself can cause clinical symptoms such as feeding intolerance and gastrointestinal bleeding in children, oral ibuprofen can aggravate gastrointestinal bleeding, even necrotizing enterocolitis and gastrointestinal perforation. In very low birth weight infants, transient acute renal failure has been reported, thus limiting its clinical application. Acetaminophen is a commonly used over-the-counter antipyretic and analgesic drug in pediatrics. It has been widely used in newborns and even premature infants, with few adverse reactions, few contraindications and good safety^[5]。 Hammerman et al.^[6]In 2011 atPediatricsFirst publication of a case report on the successful treatment of PDA in premature infants with acetaminophen. A growing body of research evidence suggests that acetaminophen may replace indomethacin and ibuprofen as the first-line treatment for PDA in premature infants. The research progress of acetaminophen in the treatment of PDA in premature infants is reviewed.

The 5th National Academic Conference on Children's Syncope, jointly organized by Kaifeng Children's Hospital of Henan Province, Peking University First Hospital, Xiangya Second Hospital of Central South University, Children's Hospital Affiliated to Shanghai Jiaotong University, Lanzhou University Second Hospital and Cardiovascular Group of Pediatrics Branch of Chinese Medical Association, was held in Kaifeng, an ancient city, on July 25th, 2015. More than 300 experts and representatives from all over the country attended the meeting.