2015年 · 第53卷第01期

Opening this volume brings us to another new year. Farewell to the old and welcome the new. In 2015, the 65th anniversary of the founding of the Chinese Journal of Pediatrics will usher in its centenary. We look forward to the continuous innovation and development of the medical cause of the motherland, and leap to new heights.

Functional cardiovascular disease in children refers to a group of diseases caused by abnormal function of the autonomic nervous system without organic lesions of the cardiovascular system, including orthostatic intolerance (OI), cardiac beta receptor hyperfunction and inappropriate sinus tachycardia (IST), among which OI includes vasovagal syncope (VVS), orthostatic tachycardia syndrome (POTS), orthostatic hypotension (OH) and orthostatic hypertension (OHT). Functional cardiovascular diseases in children are more common in OI, among which VVS accounts for a large proportion^[1,2]。 At present, there is a lack of detailed epidemiological data on functional cardiovascular diseases in children at home and abroad. It is reported that 15% to 20% of children and adolescents have experienced at least one syncope^[2]。 The study of VVS was carried out earlier, and an epidemiological study in the United States showed an increasing incidence, from 71.9 per 100,000 to 125.8 per 10,000 in the 1950s and 1990s^[3]。 With the support of China's "Tenth Five-Year Plan" and "Twelfth Five-Year Plan", a number of hospitals in Beijing, Hunan, Shanghai, Hubei, Jilin and Chongqing jointly carried out joint research, and found that the most common underlying diseases of syncope in children are VVS and POTS, accounting for about 73% of syncope in children^[1]。 Therefore, continuously improving the diagnosis and treatment level of VVS and POTS in children is a major clinical issue in this field today.

Congenital heart disease (congenital heart disease) is the most common heart disease in childhood, accounting for the first place in the incidence and mortality of major birth defects in China^[1]。 Since the 1980s, interventional cardiac catheterization for congenital heart disease has been developed and become an important means of treatment for congenital heart disease. In 1998, the American Heart Association (AHA) issued the "Scientific Statement on Interventional Cardiac Catheterization in Pediatrics"^[2]。 In the past 20 years, with the emergence of new interventional materials, technologies and treatment concepts, the number of interventional therapies for congenital heart disease has increased rapidly, and the types, scope and operation techniques of interventional therapy have also made breakthroughs. Therefore, AHA published an updated "Scientific Statement on Indications for Cardiac Catheterization and Interventional Therapy of Pediatric Heart Disease" in 2011.^[3]。

pulmonary hypertension (PH) is a group of clinical pathophysiological syndromes characterized by increased pulmonary blood pressure caused by different etiologies. It can occur at all ages in childhood, and in severe cases it can cause right heart failure or even death^[1,2]。 In recent 20 years, the definition and classification of PH have been updated with the deepening of research on its pathogenesis^[3,4,5]。 Children's PH has many similarities with adults in etiology and pathophysiological mechanism, but children have their own characteristics, so we should be cautious when learning or applying the experience of adult PH diagnosis and treatment. In recent years, studies on the pathogenesis of PH in children have shown that the formation of PH is influenced by environmental and genetic factors. Various vasoactive molecules (endothelin, angiotensin Ⅱ, prostacyclin, nitric oxide, carbon monoxide, hydrogen sulfide and sulfur dioxide), ion channels and signal pathways are involved in the formation of PH^{[6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21]}。 Much has also been achieved in the diagnosis and treatment of PH in children, and the overall prognosis of children has been significantly improved. In order to let domestic pediatricians better understand the latest progress in the field of children's PH and further standardize the diagnosis and treatment of children's PH in China, the Cardiovascular Group of Pediatrics Branch of Chinese Medical Association and the Editorial Committee of Chinese Journal of Pediatrics organized relevant domestic experts to write the consensus.

A 6-year-old male was admitted to hospital for "repeated tachycardia for 6 years". The child developed tachycardia immediately after birth, with a ventricular rate of 200-250 beats/min, and the electrocardiogram showed "pre-excitation syndrome" during sinus rhythm. 2 to 3 episodes of supraventricular tachycardia a year, each lasting several minutes to 1 h. One year ago, he underwent radiofrequency ablation (two-dimensional and three-dimensional mapping, respectively) twice in a foreign hospital, but was unsuccessfully diagnosed as "pre-excitation syndrome, parabundular bypass" by intracardiac electrophysiological examination. After operation, the child suffered from continuous episodes of supraventricular tachycardia with ventricular rate of 170-220 beats/min. After taking amiodarone, sotalol and propafenone successively, the ventricular rate decreased, but it could not effectively reverse and maintain sinus rhythm. When the sinus rhythm was briefly reverted, the electrocardiogram showed I ° atrioventricular block (PR interval 247 ms). He was admitted to our hospital for "cryoablation".

Kawasaki disease is an acute eruptive disease in children with systemic non-specific vasculitis as the main pathological change, which has replaced rheumatic fever as the first cause of acquired heart disease^[1,2,3]。 intravenous immunoglobulin (IVIG) combined with aspirin significantly reduced the risk of coronary artery lesion (CAL), but 9% to 25% of children still had their body temperature not dropped to normal 36 to 48 hours after IVIG treatment, and other clinical manifestations were not alleviated, which was called IVIG unresponsive or refractory Kawasaki disease, and the incidence of CAL in these children was higher^[4,5]。 Studies have shown that significant increases in inflammatory factors play an important role in the progression of Kawasaki disease. As the most active proinflammatory factor and inflammatory factor, tumor necrosis factor α (TNF-α) has become a new therapeutic target, which has attracted more and more researchers' attention. Anti-TNF-α therapy is used in the treatment of Kawasaki disease. The research in the treatment is continuously in depth. The research progress of anti-TNF-α treatment of Kawasaki disease is reviewed.

Since Alport syndrome was first reported and named in 1927, human beings have been continuously researching and understanding it for nearly 90 years. About 85% of patients with Alport syndrome are X-linked dominant hereditary Alport syndrome caused by COL4A5 gene mutation. Among them, male patients are seriously ill, with 90% of renal failure before the age of 40, and female patients are relatively mild. About 15% of patients with Alport syndrome are autosomal Alport syndrome caused by COL4A3 or COL4A4 gene mutations. Among them, patients with autosomal recessive Alport syndrome are the main ones, and almost all of them develop renal failure before the age of 30^[1,2]。 It can be seen that Alport syndrome is a disease that seriously endangers the health of young adults.

Located at No.399 Wanyuan Road, Shanghai, in the cardiology ward of Pediatric Hospital affiliated to Fudan University, no matter in hot summer or cold winter, every Friday morning, a professor over 80 years old will conduct English teaching rounds here. He is Ning Shoubao, a tenured professor of Pediatrics Department of Shanghai Medical College who has been working in this hospital since 1956. Listen to Professor Ning's ward rounds include intern medical students, doctors from other places and doctors at all levels who are already in our hospital. In order to make every participant gain something, Professor Ning started by reviewing the basic knowledge, discussed the diagnosis, treatment and treatment plan with everyone from the symptoms, signs and examination results of selected children, and strived to make every participant gain something. In order to do the ward rounds in advance, Professor Ning will never "cram his feet". Every Thursday, we go to the ward to know the medical history, do a comprehensive examination of the children, consult the information, and provide relevant English reference materials for the participants in the ward rounds to read. He also regularly asks the responsible doctor to write an English medical history and give guidance, which strengthens the training of professional English for young doctors. An undergraduate student from Xi'an Jiaotong University School of Medicine wrote in his feelings: "When I handed him the medical record folder, he actually took it with both hands and said 'thank you' with a smile. This is a 'thank you' I received for doing such small things, and it came from an 80-year-old professor. His speech and behavior, that kind gesture, that kind of instruction, that bit by bit of movement, made me call him 'grandpa' in my heart; He liked to ask us questions but never made people nervous. What do you think after his interlocking questions? ', and the look of expectation and encouragement that naturally accompanied it, is still fresh in my memory. "

Randomized controlled trials (RCTs) are a type of study design with a very high level of evidence in evidence-based medicine. In the GRADE evidence pyramid, RCT is second only to systematic review and meta-analysis, and belongs to the secondary level of evidence, with a higher level of evidence than case-control studies, cohort studies, and non-randomized controlled trials. However, RCT also has flaws, and the results of RCT research need to be viewed and interpreted correctly. The main limitations of the RCT study are explained below.