2017年 · 第55卷第10期

According to the surveillance, epidemiology, end results (SEER) database of the National Cancer Institute, which has a well-established childhood tumor registry, the annual incidence of cancer in children under 15 years of age from 1973 to 2010 was 155 per million, of which about 30% were leukemia, 20% were intracranial tumors, 10% were lymphomas, and the other 40% were mainly various malignant solid tumors. In the past half century, the prognosis of malignant solid tumors in children in developed countries has also made great progress, and the 5-year survival rate has reached about 80%. So far, China has not established a national-level childhood cancer registry system, so the exact national childhood cancer incidence and prognosis data are missing. In recent 20 years, the clinical diagnosis and treatment of childhood acute leukemia in China have made great progress, but the progress of childhood malignant solid tumor is relatively slow, especially in underdeveloped areas, and cases still often enter the misunderstanding of diagnosis and treatment. The diagnosis and treatment of malignant solid tumors is different from that of leukemia. Leukemia involves relatively few specialties, mainly blood specialties and laboratory specialties. Laboratory items can often be shared among hospitals or third parties. Adult leukemia also has a huge number. The development of adult leukemia testing technology also benefits childhood leukemia. The common tumor spectrum of malignant solid tumors is different from that of adults, and most of them are rare; Moreover, the diagnosis and treatment of malignant solid tumors involves many sub-specialties. The main core specialties include surgery, internal medicine, radiotherapy, imaging and pathology. Each specialty is only a part of the whole process of work, and the sub-specialties are strong and irreplaceable. Close communication and cooperation between sub-specialties are required. The absence or poor cooperation of one sub-specialty may affect the diagnosis and treatment of children, and ultimately affect the prognosis. However, the current hospital management system and busy medical tasks are often not conducive to the development of multi-disciplinary collaborative work mode. The current developments in the field of solid tumors in children are briefly reviewed.

rhabdomyosarcoma (RMS) is the most common soft tissue tumor in childhood, accounting for about 6.5% of childhood tumors^[1,2,3]。 The primary site is the head and neck, followed by the trunk, limbs and genitourinary system. RMS is sensitive to chemotherapy and radiotherapy, but the effect of single treatment is poor, so it needs comprehensive treatment of medical oncology, surgery, radiotherapy and other disciplines. After more than 20 years of clinical research, the Intergroup RMS Study Group (IRSG) and the European Pediatric soft tissue sarcomas Study Group (EpSSG) have divided RMS into three groups: low, medium and high risk according to age, tumor size, pathology and clinical stage, and carried out stratified and comprehensive treatment to continuously optimize chemotherapy regimen, further improve prognosis, and improve the efficacy of RMS year by year^[1,4,5,6]。 In September 2013, with the approval of the Chinese Children Cancer Group (CCCG), the RMS Collaborative Group for Chinese Children and Adolescents was established. The collaborative group firstly retrospectively analyzed the diagnosis and treatment effect of children with RMS systematically treated and followed up in 5 larger children's hospitals in China^[7]: The overall survival rate of children deviated, which may be related to the number of children with distant metastasis at the time of diagnosis, single treatment method and irregular management. Therefore, according to the above research results, combined with the research results of IRSG and EpSSG^{[4,5,6,8,9,10,11,12,13,14]}This diagnosis and treatment recommendation was formulated. All localities can take into account local reality and refer to it where feasible.

nephrotic syndrome (NS) is a clinical syndrome with a series of pathophysiological changes due to the increased permeability of glomerular filtration membrane to plasma protein and the loss of large amount of plasma protein from urine. The main clinical features are massive proteinuria, hypoalbuminemia, hyperlipidemia and edema. It can be divided into three types: primary, secondary and congenital NS. primary nephrotic syndrome (PNS) accounts for about 90% of the total number of NS in children^[1]It is one of the common glomerular diseases in children. The annual incidence of PNS in children (2~4) /100,000 was reported abroad, and the prevalence rate was 16/100,000^[2,3]Statistics from 37 cooperative hospitals in 19 provinces, 27 municipalities, 2 autonomous regions and 4 municipalities directly under the central government show that PNS accounted for about 20.0% of the total number of hospitalized children with urinary system diseases in the same period^[4]。 Oral glucocorticosteroids (hereinafter referred to as hormones) have been recognized as the first-line treatment for PNS since the 1950s.

In order to further standardize the diagnosis and treatment of rhabdomyosarcoma (RMS) in children and adolescents, in February and September 2016, the Pediatric Oncology Committee (CCCG) of Chinese Anti-Cancer Association, the Hematology Group of Pediatrics Branch of Chinese Medical Association and the Oncology Group of Pediatric Surgery Branch of Chinese Medical Association held two seminars. Based on the original draft and referring to the research progress of advanced treatment groups at home and abroad in recent years, a new version of "Recommendations for Diagnosis and Treatment of Rhabdomyosarcoma in Chinese Children and Adolescents (CCCG-RMS-2016)" was solemnly recommended.^[1]。

"Evidence-Based Guidelines for Diagnosis and Treatment of Common Kidney Diseases in Children (I): Trial Draft of Evidence-Based Guidelines for Diagnosis and Treatment of Hormone Sensitive, Relapsed/Dependent Nephrotic Syndrome" published in Chinese Journal of Pediatrics in March 2009^[1](hereinafter referred to as the "2009 Guidelines"), and referring to the latest research evidence at home and abroad, the Nephrology Group of Pediatrics Branch of Chinese Medical Association formulated the "Evidence-based Guidelines for the Diagnosis and Treatment of Hormone Sensitive, Relapsed/Dependent Nephrotic Syndrome (2016)" (hereinafter referred to as the "2016 Guidelines"). In view of the content of the updated part of the "2016 Guidelines", the opinions or suggestions put forward by the participating experts at the "2016 Seminar on Evidence-based Guidelines for the Diagnosis and Treatment of Common Kidney Diseases in Children" held in Beijing in April 2017, and the opinions or suggestions of the group members solicit feedback by email after the meeting are interpreted as follows.

Female, 6 years old and 4 months old, was admitted to Beijing Children's Hospital affiliated to Capital Medical University in December 2015 due to "inability to lie down at night, with mouth breathing for more than 4 months and aggravation for 2 months". Four months before admission, the child was unable to sleep at night, accompanied by mouth breathing, no snoring, shortness of breath, wheezing, cough, expectoration, dizziness and other symptoms, conscious weakness during the day, obvious after exercise, less physical strength and food intake than children of the same age, and liked to be quiet. Two months before admission, the above symptoms were aggravated, and echocardiography showed pulmonary hypertension and right ventricular hypertrophy. Severe obstructive sleep apnea syndrome (OSAS) was diagnosed by polysomnography (PSG) in other hospitals, but there was no improvement after continuous positive pressure ventilation (CPAP). The child is the first child, giving birth naturally at full term, with a vertical head at 5 months, sitting alone at 8 months, and walking alone at 15 months. He is poor in running and jumping since childhood, can go up and down stairs slowly, and has normal intelligence. No family history of hypertension, diabetes, neuromuscular disease. Pneumonia at 1 year old and severe pneumonia at 5 years old.

bronchopulmonary dysplasia (BPD) is a chronic lung disease that occurs in premature infants. With the development of neonatal intensive care technology, the survival rate of very low birth weight premature infants is obviously improved, and the incidence of BPD in premature infants is increased. A multicenter study of premature infants with a gestational age of 24 to 31 weeks in China from 2013 to 2014 showed that the gestational age<The incidence of BPD in premature infants at 32 weeks was 12.5%. The smaller the gestational age, the higher the incidence of BPD^[1]。 Inflammatory response plays an important role in the development of BPD, and glucocorticoids reduce the occurrence of BPD by inhibiting inflammatory response. Its possible mechanisms include promoting the production of pulmonary surfactants, stabilizing cells and lysosomal membranes, inhibiting inflammatory cell infiltration, and reducing bronchospasm, pulmonary edema and pulmonary fibrosis. However, since then, studies have found that systemic application of hormones can lead to a variety of adverse reactions, such as gastrointestinal bleeding, hypertension, hyperglycemia, restricted growth and development, and nervous system damage. How to use glucocorticoids rationally to prevent and treat BPD and reduce adverse reactions as much as possible has become the focus of current research. Neonatologists hope to achieve this goal by changing the route, dose, time and type of hormone administration, and some progress has been made in relevant clinical research. The development history and clinical progress of rational use of glucocorticoids in the prevention and treatment of BPD are reviewed.

With the rapid development of transcatheter interventional technology and instruments, more and more children with congenital heart disease (congenital heart disease) are treated by interventional surgery. In interventional surgery, X-ray fluoroscopy is routinely used to display the cardiac image and the position of the guide catheter. This process inevitably produces ionizing radiation, which poses a potential threat to the health of children. Therefore, the radiation protection (ALARA) guidelines recommended by the Pediatric Radiology Association should be followed during interventional surgery of children with congenital heart disease, that is, the exposure to ionizing radiation should be minimized as much as possible while ensuring the quality of images that meet the needs of the surgery^[1,2]。 The hazards of ionizing radiation, the determination of radiation dose, the reference value of ionizing radiation dose in interventional surgery for children with congenital heart disease and the effective means of reducing intraoperative radiation dose are summarized as follows, aiming at strengthening the awareness of intraoperative radiation protection and reducing radiation exposure as much as possible.

In clinical research, in order to establish a statistical association closer to causality, research design and multivariate analysis methods are often used to control confounding factors, so as to explore the relationship between target factors and a certain clinical outcome. For example, when exploring the relationship between treatment measures (X) and patient disease outcome (Y), other confounding factors (C) are controlled through study design or statistical analysis methods. These confounding factors have their own specific characteristics, the main characteristic of which is that C is both the "cause" of Y and the "cause" of X. That means that it is difficult to prove whether a factor is a confounding factor. We need to fully consider how this factor is related to X or Y in mechanism, and what are the research conclusions of others in the design and analysis stage.