2017年 · 第55卷第04期

The incidence of congenital heart disease (CAD) is about 1% in live-born newborns, of which 1/4 to 1/3 are severe CAD, including critical and severe types, which refer to congenital heart diseases that require surgery or interventional treatment in neonatal and infancy, respectively^[1]。 If severe congenital heart disease is not treated in time, serious consequences such as heart failure, cardiogenic shock, acidosis and hypoxic brain injury can occur, which is one of the main causes of infant death and child disability^[2]。 Studies have shown that 12% ~50% of children with critical congenital heart disease in Western developed countries are not diagnosed after birth to obstetric hospital before discharge, but find the disease after severe symptoms. At this time, the vital signs of children are often in an unstable state, which increases the risk of surgery, seriously affects the prognosis, and also increases the socio-economic burden^[3,4]。 Based on this situation, the 2010 U.S. Advisory Committee on Hereditary Disorders of Neonates and Children (The Secretary's Advisory Committee on Hereditary Disorders in Newborns and Children, SACHDNC) It is suggested that general screening should be carried out for neonatal congenital heart disease, and a working group should be set up in the following year to promote related work^[5]。

In 1990, Dr. Van Hare of the University of California successfully treated a 10-month-old child with borderline tachycardia by catheter ablation for the first time^[1]In The same year, The Pediatric Electrophysiology Society established The Pediatric Radiofrequency Catheter Ablation Registry to evaluate The efficacy and safety of Radiofrequency Catheter Ablation in children^[2]； Based on the data from this registry, the North American Society for Pacing and Electrophysiology published an expert consensus on radiofrequency catheter ablation in children in 2002 (hereinafter referred to as the "2002 edition of the consensus")^[3]To standardize catheter radiofrequency ablation in children; The Guidelines for Radiofrequency Ablation in Children were also published by the Section of Cardiac Pacing and Electrophysiology of the Chinese Society of Biomedical Engineering in 2002^[4]。 These guidelines have played an important role in catheter radiofrequency ablation for the treatment of children's arrhythmias, and now catheter ablation has become the first-line method for the treatment of children's tachyarrhythmias. In 2016, the American Association for Pediatric and Congenital Electrophysiology (PACES) and the Heart Rhythm Association (HRS) released a new expert consensus on catheter radiofrequency ablation in children (hereinafter referred to as the "2016 edition consensus")^[5]This paper reflects the current status and progress of catheter ablation in the treatment of tachyarrhythmia in children. Since the first radiofrequency ablation of children was carried out in China in 1992^[6]The number of operations and the number of hospitals performing this operation are also increasing. Although domestic multicenter research data have been published^[6]However, the corresponding guidelines have not been formed to guide the domestic catheter ablation for the treatment of tachyarrhythmia in children. We should further strengthen the standardized operation of catheter ablation in pediatric clinical treatment of tachyarrhythmia in China.

primary immunodeficiency (PID) is a group of clinical syndromes in which gene mutations lead to defects in immune organs, immunoactive cells and immunoactive molecules (immunoglobulins, cytokines, complement and cell membrane surface molecules), which ultimately lead to abnormal immune function of the body. In 2015, the latest classification standard of the International Federation of Immunological Societies (IUIS) PID Expert Committee (PID EC) divided PID into 9 categories, involving more than 290 kinds of PID caused by more than 300 gene mutations: (1) T lymphocytes, B lymphocytes Combined immunodeficiencies affecting cellular and humoral immunity; (2) Immunodeficiency syndrome with other well-established phenotypes (combined immunodeficiencies with associated or syndromic features); (3) predominantly antibody deficiencies; (4) Immune Disorders (diseases of immune dysregulation); (5) Congenital defects in phagocyte number and function (congenital defects of phagocyte number, function, or both); (6) Innate Immunodeficiency (defects in Intrinsic and Innate Immunity); (7) autoinflammatory disorders; (8) complement deficiencies; (9) phenocopies of PID associated with autoantibodies^[1,2,3]。

Following the publication of the European Heart Rhythm Society (EHRA) and the European Association of Pediatric Cardiology (AEPC) "Expert Consensus on Pharmacotherapeutic and Non-Pharmacotherapeutic Treatment of Childhood Arrhythmias"^[1]In 2016, the American Association for Pediatric and Congenital Electrophysiology (PACES) and the American Heart Rhythm Association (HRS) released the "Expert Consensus on Catheter Ablation in Children and Patients with Congenital Heart Disease" (hereinafter referred to as the "2016 Edition Consensus")^[2]This consensus follows the last "Expert Consensus on Catheter Radiofrequency Ablation in Children with or without Congenital Heart Disease" published by the North American Pacing and Electrophysiology Society (NASPE) in 2002^[3](hereinafter referred to as the "2002 edition consensus") has been used for 14 years, which reflects the current status and progress of catheter ablation in the treatment of tachyarrhythmia in children, and will play a positive role in standardizing the rational development of this technology. This article now interprets the 2016 edition of the consensus published by PACES and HRS.

A 24-day-old male was admitted to the Children's Hospital affiliated to Zhejiang University School of Medicine in December 2015 due to "24 days of shortness of breath after premature delivery". The mother of the child was 36 weeks and 2 days pregnant. She was delivered by caesarean section due to the mother's low amniotic fluid, and her birth weight was 1 450 g. After birth, she was admitted to the local hospital due to shortness of breath and groaning. She was diagnosed with "pneumonia, sepsis, and baby smaller than gestational age". After treatment with respiratory support, anti-infection and gamma globulin immune support, she still had repeated cyanosis, and the total number of white blood cells and C-reactive protein could not be reduced to normal, so she was transferred to the Children's Hospital affiliated to Zhejiang University School of Medicine.

A male child, 9 months old and 27 days old, went to the neurology clinic of Beijing Children's Hospital in May 2016 because of "more than one month lagging in development". Because the child couldn't turn over or crawl at the age of 8 months, he didn't respond to his name, couldn't pronounce two syllables and came to the doctor without convulsions. The child was the first birth, the first birth, the full term natural birth, the birth weight was 3 500 g, and there were no abnormalities in the perinatal period. You can raise your head at 3 months and sit alone at 7 months. Healthy parents, denied inkin marriage and similar family medical history. Physical examination: clear consciousness, good mental response, no special face, can make laughter, both eyes have fixation, can chase light and objects, no response to names, limbs move autonomously, muscle strength and muscle tone are normal, tendon reflex is normal, pathological signs are negative, can raise your head, sit alone, can't turn over or crawl, can grab objects with both hands, and movements are inflexible. Auxiliary examination: Gesell Developmental Diagnostic Scale: total score 58, including 57 for large motor, 38 for fine motor, 63 for adaptive behavior, 67 for verbal behavior, and 70 for personal-social behavior. EEG normal. Cranial magnetic resonance imaging (MRI) showed: bilateral globus pallidus and dorsal pons showed symmetrical patchy slightly longer T1 and long T2 signals, and diffuse image (DWI) showed high signal (Figure 1)。 There were no abnormalities in blood biochemistry, lactic acid, blood ammonia, thyroid function tests and blood screening. Urine screening results showed an increase in glycerol 3 phosphate, which improved the detection of genes related to genetic metabolic diseases including fructose 1,6-diphosphatase deficiency.

pulmonary atresia with intact ventricular septum (PA-IVS) is an uncommon type of complex cyanotic congenital heart disease. It is characterized by fibrous atresia of the right ventricular outflow tract and intact ventricular septum. It can lead to right ventricular and tricuspid valve dysplasia and coronary artery abnormalities in the later stage. Although the right ventricular outflow tract of severe pulmonary stenosis with intact ventricular septum (CPS-IVS) is not completely atresed, the pathophysiology and clinical manifestations in the later stage are similar to those of PA-IVS, so it is reviewed here. Severe pulmonary stenosis (CPS) and PA-IVS account for approximately 5% of congenital heart diseases diagnosed prenatally^[1]The 5-year survival rate is only 59% ~67%, while the feasible biventricular circulation repair is only 29% ~33%^[2,3]。

Infant colic is a behavioral syndrome manifested by difficult to soothe irritable or crying behavior in healthy infants, occurring for more than 3 h daily, lasting ≥3 d weekly, and lasting for more than 3 weeks^[1]。 Infant colic usually attacks at 2 weeks of age, with the peak attack at 6 to 7 weeks of age, and disappears spontaneously at 3 to 4 months of age. About 20% of small babies suffer from it^[1]。 Although colic symptoms can resolve on their own with age, studies have shown that parents of infants with colic seek medical attention more frequently and extensively and are more likely to seek unproven, potentially risky ways to soothe crying infants than parents of infants without colic symptoms^[2]。 Moreover, infant colic can increase the occurrence of psychological distress and depression in caregivers, hinder the good development of mother-child relationship, and may lead to accidental injury and impaired family function^[3]。 A prospective study found that children with colic in infancy had an 11-fold increased risk of developing frequent abdominal pain after 10 years than those without colic in infancy, and had a higher risk of developing allergic diseases and psychological disorders^[4]。 Correct recognition of infantile colic can help to avoid improper diagnosis and treatment and the resulting adverse events. Recent research progress on the pathogenesis, diagnosis and intervention of infantile colic is reviewed.

Sponsored by the Chinese Medical Association and the Pediatrics Branch of the Chinese Medical Association and hosted by Guangdong Medical Association, the 21st National Pediatrics Academic Conference of the Chinese Medical Association was held in Zhuhai, Guangdong Province from October 20 to 23, 2016. At the request of the Pediatrics Branch of Chinese Medical Association, the 18th National Pediatric Nephrology Conference in 2016 was once again merged into the kidney special session of the 21st National Pediatric Conference of Chinese Medical Association and held at the same time.

The clinical guidelines put forward specific recommendations based on a systematic review of existing clinical evidence, an assessment of the risks and benefits of alternative treatments, and combined with local medical resources, patient needs and values. The evidence-based clinical guidelines are to link the above recommendations with relevant evidence, evaluate the existing evidence, and determine the recommendations and formulate guidelines based on the evaluation results. Evidence-based clinical guidelines increase the scientificity, pertinence and utility of the guidelines^[1]。