2017年 · 第55卷第02期

2017 is the first year after the establishment of the 15th editorial board of the Chinese Journal of Pediatrics. As the new editor-in-chief of Chinese Journal of Pediatrics, I look back on the development process of the journal and look forward to the future of the journal's development, with a surging heart and thinking. In the past years, under the correct leadership of the Journal of Chinese Medical Association, and with the support and dedication of readers, authors and editors, Chinese Journal of Pediatrics has successfully completed the editing and publishing tasks and achieved remarkable achievements. The magazine adheres to the purpose of running a journal, and practically combines theory with practice, emphasizing practice; Adhere to the combination of basic and clinical, focusing on clinical; Striving to combine popularization and improvement, emphasizing improvement, has made important contributions to promoting academic exchange and development in the field of pediatric medicine in China, improving the quality of medical services and cultivating pediatric medical talents in China. With few staff and heavy tasks, the editorial department of this journal thinks hard and innovates constantly. Using the main columns set up, it publishes a large amount of academic information, including clinical guidelines, consensus and norms of disease diagnosis and treatment experts, high-quality multi-center clinical research, clinical case discussion and experience sharing, and original scientific research results, etc., and gives full play to the important role of leading academic orientation, serving society and gradually expanding international influence.

The first working meeting of the 15th editorial board (editorial board) of Chinese Journal of Pediatrics was held in Beijing on November 19th, 2016. 145 editorial board consultants, editorial board members, special editorial board members and corresponding editorial board members from all over the country attended the meeting. The morning meeting was chaired by Professor Gui Yonghao, Editor-in-Chief of the 14th Editorial Board, and Professor Du Lizhong, Deputy Editor-in-Chief. At the beginning of the meeting, all participants watched the video promotion video carefully prepared by the editorial department, reviewed the history and achievements of Chinese Journal of Pediatrics, and listened to the messages of four old editors-in-chief. President Jiang Yongmao of Chinese Medical Association Magazine read out the list of editorial committee of Chinese Medical Association and delivered a speech; Professor Zhao Zhengyan, Chairman of Pediatrics Branch of Chinese Medical Association and Deputy Editor-in-Chief of the 15th Editorial Board, delivered a speech; Deputy Editor-in-Chief Professor Li Tingyu and Professor Feng Zhichun read out the list of special editorial board members and corresponding editorial board members respectively; Professor Gui Yonghao and Professor Du Junbao, editor-in-chief of the 15th editorial board, delivered keynote speeches respectively; Professor Lu Quan, Professor Li Haiqi and Professor Chen Zhimin introduced the work done for the magazine on behalf of the outstanding deputy editor-in-chief, outstanding editorial board and outstanding correspondence editorial board of the 14th editorial board respectively; Li Wei, deputy director of the editorial department, was entrusted to give a work report on behalf of the 14th editorial board. The afternoon meeting was chaired by Professor Du Junbao, Editor-in-Chief of the 15th Editorial Board, and Professor Wang Tianyou, Deputy Editor-in-Chief. Professor Huang Guoying, Professor Mao Meng and Professor Wang Tianyou, Deputy Editor-in-Chief, read out the list of outstanding editorial members, corresponding editorial members and reviewers of the 14th Editorial Board respectively. The editorial board of the meeting made suggestions on the future planning of the magazine. The main contents of this meeting are briefly introduced below.

Thank you to the editorial department for giving me this opportunity to speak. I would like to share with you my experience and thoughts and wishes for the future development of Chinese Journal of Pediatrics during my five years as the editor-in-chief of Chinese Journal of Pediatrics.

Today, the 15th editorial board (editorial board) of Chinese Journal of Pediatrics was formally established here, which is an important moment of historical significance in the development history of Chinese Journal of Pediatrics. This means that the new editorial board has taken over the baton of running the magazine from the previous editorial board, and continues to work hard to push the magazine to a new height. At this solemn moment of mission handover, on behalf of the Journal of Chinese Medical Association, I would like to express my deep gratitude to all the experts of the 14th editorial board with Professor Gui Yonghao as the editor-in-chief, and to express my heartfelt congratulations on the establishment of the 15th editorial board and to all the experts for being hired as members of the 15th editorial board.

I am very pleased to attend the 15th editorial board (editorial board) meeting of Chinese Journal of Pediatrics. First of all, on behalf of the Pediatrics Branch of Chinese Medical Association, I would like to express my warm congratulations on the establishment of the 15th editorial board of Chinese Journal of Pediatrics. At the same time, I would also like to express my warm congratulations to my colleagues who were elected to the editorial board, corresponding editorial board and special editorial board, and to Professor Du Junbao who was elected editor-in-chief. Under the leadership of editor-in-chief Gui Yonghao, the last editorial board made great progress in the development of the magazine. Here, I would like to express my gratitude to Professor Gui. The teachers in the editorial department are serious and responsible, and they have worked hard to run the magazine well. I also express my sincere gratitude to them.

In October 2015, the Chinese Pediatric Oncology Professional Committee conducted another retrospective summary of the WT-2009 protocol based on the American Nephroblastoma Collaborative Group (NWTS) -5 protocol. 361 kidney tumors entered the analysis, with a median follow-up of 57 months and a 5-year disease-free survival rate (EFS) of 80%. Among them, the pathological type was good prognosis (FH) in 300 cases (83.1%), and the 5-year EFS was 81.2%; 32 cases (8.9%) with poor prognosis (UFH) and 71.7% with 5-year EFS; 17 cases (4.7%) of renal clear cell sarcoma, 5-year EFS 68.8%; Renal rhabdoid tumor was found in 5 cases (1.4%) and 5-year EFS was 60.0%. In the whole group, 104 cases (28.8%) were in stage Ⅰ, 99 cases (27.4%) in stage Ⅱ, 102 cases (28.3%) in stage Ⅲ, 47 cases (13.0%) in stage Ⅳ, and 9 cases (2.5%) in stage Ⅴ. Preoperative chemotherapy was performed in 60 cases, accounting for 37.9% of the patients in stage Ⅲ ~ Ⅴ. Among the 300 cases of pathological FH, 93, 87, 81, 31 and 8 cases were in stage Ⅰ ~ Ⅴ, respectively, and the 5-year EFS were 91.7%, 78.0%, 78.3%, 77.2% and 50.4%, respectively (P＝0.019)。 177 patients should receive radiotherapy according to the protocol, but only 97 of them received radiotherapy according to the protocol, and the other 80 patients did not receive radiotherapy according to the protocol. The 5-year EFS of the two groups was 83.1% vs 64.5% (P＝0.002)。 There were no reports of intraoperative ulceration in preoperative chemotherapy patients, and there was no intraoperative ulceration rate of 5% in stage III preoperative chemotherapy patients^[1]。

A 4-year-old child was admitted to hospital for "intermittent abdominal pain with diarrhea for nearly 1 month". One month ago, the child intermittently developed abdominal pain, diarrhea and fever. Abdominal pain is mostly located around the umbilicus, with excessive sweating during pain, lasting for several minutes, 3 to 4 times/d, accompanied by watery diarrhea 2 to 3 times/d, no mucus and bloody stool, no jam-like stool. The fever peak is about 39 ℃, 1 to 2 times/d, showing relaxed fever type, without chills and chills. No vomiting, nausea, etc. After short-term anti-infective treatment in the outside hospital, the symptoms can be temporarily improved. Symptoms were repeated after drug withdrawal. One month after the onset of the disease, the weight loss was 4 kg.

Example 1Male, 12 years old. The main cause was "edema and proteinuria for 3 months". Sufficient amount of prednisone was given orally for 4 weeks outside the hospital. The urine protein continued to not release, the edema gradually aggravated, and the blood pressure increased more than 10 days before admission (140/90 mmHg, 1 mmHg =0.133 kPa); During the course of the disease, there were no discomfort such as photosensitivity, rash, oral ulcer, joint swelling and pain.

The male child, 1 h after birth, was admitted to the Department of Cardiology and Pediatrics of the First Affiliated Hospital of Tsinghua University in April 2016 mainly because of the discovery of congenital complete atrioventricular block. The mother found a slow fetal heart rate at 25 weeks of pregnancy, ultrasound showed fetal atrioventricular block, and ultrasound at 35 weeks of pregnancy showed complete atrioventricular block (atrial rate 135 beats/min, ventricular rate 44 beats/min), whole heart enlargement, cardiothoracic ratio 0.7, left ventricular end diastolic diameter 20 mm, ejection fraction 59%, and no abnormalities in cardiac structure. The fetal growth and development were still normal, the gestational age was 36 weeks, the birth weight was 2.5 kg, and the Apgar score was 9, 9 and 10 at 1, 5 and 10 min, respectively. Admitted to our department for "pacing therapy". The child is the second child and the second birth. His sister (12 years old) is in good health, with no special birth history and past history. The mother (43 years old) and father (44 years old) are in good health in the past, and they deny the family history of syncope and sudden death. The mother's autoantibody spectrum [antinuclear antibody, Sjogren's syndrome (SS) -A, SS-B, double-stranded DNA (dsDNA) antibody, anticardiolipin antibody, etc.] is negative.

The child, 1 year old and 4 months old, was admitted to the Department of Neurology of Wuhan Children's Hospital in February 2016 due to "right upper limb and perioral jitter for 6 days". The child was the second child and the second birth, with a birth weight of 2.8 kg. He was delivered by cesarean section at full term. He was breastfed all the time after birth, and complementary food was only added at the age of 1 year and 3 months. Motor development is backward, 8 months old can sit, still can't walk alone, can only call "Dad" and "Mom". Half a month ago, the child was admitted to a hospital due to lung infection and pale complexion. The auxiliary examination in the hospital showed that hemoglobin was 56 g/L, mean red blood cell volume (MCV) was 100.7 fl, and white blood cells were 3.54×10⁹/L, platelets 137×10⁹/L, and then multiple dynamic reexaminations of blood routine showed abnormalities; Bone marrow puncture showed that the nucleated cells of bone marrow proliferated obviously and actively. The granulocyte system was 0.415, mainly middle and late granulocytes, and some cells showed megaloblastic metamorphosis, especially late granulocytes and rod-shaped nuclear granulocytes, and the erythrocyte system was 0.405, mainly middle and late granulocytes, and some cells showed megaloblastic metamorphosis. Metabolic examination found that folic acid 15.7 nmol/L (reference: 4.8~31.6 nmol/L), vitamin B₁₂Cannot be detected (<12.8 pmol/L, reference: 320~1 483 pmol/L), so it was diagnosed as "vitamin B₁₂Deficiency: megaloblastic anemia ". Alternate-day vitamin B during hospitalization in an outside hospital₁₂(Cyanocobalt vitamin, 1 000 μ g/d) intramuscular injection, sudden limb tremor and myoclonus occurred on the 4th day of injection, mainly in the right upper limb and perioral, tongue muscle twitching when the tongue was extended, and sleep also persisted. Suspected "state of convulsion" was brought to hospital.

2009 Glocker et al.^[1]A case of very early onset inflammatory bowel disease (VEO-IBD) caused by mutations in the interleukin (IL) -10 receptor (IL-10R) gene is reported for the first time. Domestic Xu Yongbin et al^[2]Liu Lili, et al^[3]And Peng Kaiyue, etc.^[4]A total of 4 cases of VEO-IBD with neonatal onset were reported, and IL-10RA gene mutation was confirmed by gene sequencing. In order to improve the understanding of this disease and avoid missed diagnosis and delayed diagnosis, the clinical characteristics, diagnosis, treatment and related progress of VEO-IBD caused by mutation of IL-10 gene and IL-10R gene are reviewed.

Congenital megakaryocytic dysplastic thrombocytopenia (CAMT) is a rare hereditary bone marrow failure syndrome presenting with severe thrombocytopenia, bone marrow megakaryocytopenia or absence without specific somatic malformations, and ultimately progressing to aplastic anemia or secondary bone marrow malignancy^[1]。 In early reports, most children eventually developed bone marrow failure and died due to the inability to perform timely treatment such as platelet transfusion and hematopoietic stem cell transplantation (HSCT). In recent years, with the in-depth research on the pathogenesis of this disease and the extensive development of HSCT, many children have been successfully treated. Domestic cognition of this disease is not very perfect^[2,3]。 The etiology, pathogenesis, clinical manifestations, diagnosis and treatment of CAMT are summarized as follows.

The 21st National Pediatric Academic Conference of Chinese Medical Association was held in Zhuhai, Guangdong Province from October 20 to 23, 2016. 7,452 delegates from all provinces, municipalities and autonomous regions attended the conference, and 3,182 manuscripts were received to the conference. Among them, there were 173 participants in the hematology and tumor special conference, 15 special reports and 37 speeches. At the three-day meeting, the reports of experts were brilliant, and they interacted closely with other experts and doctors present; The speeches at the conference were rich in content, covering many sub-specialties of hematology and oncology, which benefited every participating delegate a lot. It is briefly introduced as follows.

Randomized controlled trials (RCTs) are a type of study design with a very high level of evidence in evidence-based medicine, but their feasibility is greatly challenged. In addition, RCT provides average efficacy and does not guide individuals well in clinical decision-making. N of 1 trial, as a complementary design of RCT, well compensates for the shortcomings of RCT. N of 1 trial was earlier used in psychological research, and was introduced into clinical research by the Evidence-Based Medicine Working Group of McMaster University in 1986.