Carbohydrates are the important energy source of body,including glucose,galactose,fructose and glycogen. Congenital enzymes defects will cause carbohydrates metabolic disorders. Most of the carbohydrate metabolic disorders could lead to hypoglycemia. Most patients presented with chronic disease course. But some patients with serious diseases,such as glycogen storage disease type I,fructose-1,6-bisphosphate deficiency,presented as acute onset with critical illness,resulting in hypoglycemia and multiple organ damage(encephalopathy,cardiomyopathy,hepatopathy and myopathy). Most of the carbohydrate metabolic disorders have good prognosis if the prompt diagnosis and proper intervention are available. Sudden death occurred in some severe cases. Post-morterm study by metabolic autopsy is important to conform the diagnosis and directive genetic counseling.

Cardiovascular diseases are important causes of sudden death in children. Cardiac and vascular structural,functional abnormalities and electrophysiological changes including congenital heart disease,cardiomyopathy,ion channel disease, myocarditis, hypertension, pulmonary hypertension and coronary artery diseases all perhaps lead to sudden death. Identifying these diseases in time will be helpful for recognizing children at risk of sudden death and differential diagnosis of successful resuscitated children,so as to ultimately reduce the risk of sudden death of the patients and the families.

Hypocalcemic cardiomyopathy could result in sudden congestive heart failure,cardiogenic shock,or sudden death. One of the causes of congestive heart failure is hypocalcemic cardiomyopathy. Beware of hypocalcemic cardiomyopathy at the diagnosis of dilated cardiomyopathy during infancy.Hypocalcemia is a biochemical characteristic,and cardiomyopathy is pathological feature in hypocalcemia cardiomyopathy.Vitamin D deficiency is the primary cause of cardiomyopathy.Hypocalcemia cardiomyopathy is based on vitamin D or calcium deficiency. Hypocalcemic cardiomyopathy responds well to vitamin D and calcium supplementation.

Electrolytes are essential substances that support life,which are important for maintain homeostasis. The extracellular and intracellular essential electrolytes,such as sodium,potassium,calcium and magnesium,are all important components in the process of nucleic acid and protein synthesis,plasma osmotic pressure and neuromuscular excitability. The patients present with irritability,tetany,tachycardia,arrhythmia and even sudden death. Inherited electrolyte imbalances are rare disorders. But the clinical diagnosis is easy by general biochemical examination. All of the diseases are treatable. If the patients were treated in time and correctly,the outcome of most patients should be favourable. Recent advances of genetic studies contribute to more understanding of the inherited electrolyte disturbances. This review describes the clinical characteristics,the diagnostic methods,treatment strategies and the genetic advances of the severe genetic electrolyte disturbances associated with sudden death.

Sudden unexpected death means a "healthy" person died suddenly of unknown diseases,usually within 6 hours after onset. It is reported that sudden unexpected death occurred from neonates(sudden infant death syndrome)to adults(sudden adult death syndrome). The patients suddenly died during daily activities,sleep or exercise. Underlying genetic diseases are main cause of sudden death. The etiological studies are performed in the patients of sudden death. Heart attack and encephalopathy due to varied genetic disorders are the two major causes. Sudden cardiac death accounts for more than half. It is known that some inherited metabolic diseases associated with sudden death sometimes. Mitochondrial diseases are a group of inherited metabolic diseases. Some patients of mitochondrial diseases suddenly died of acute heart failure,malignant arrhythmia or encephalopathy. With the advancement of genetic technology,post-mortem genetic diagnosis became available in some cases. The definite genetic diagnosis is the key for the genetic counseling of the families and prenatal diagnosis of their fetuses.

OBJECTIVE: To study the pathogenic spectrum and the clinical characteristics of viral diarrhea in children.METHODS: The study was conducted in 400 children with viral diarrhea hospitalized in Children's Hospital from January to December in 2016. The stool specimens(about 5 mL)were collected for detection from the children on the day of hospitalization. The ELISA method and PCR method were used to detect the genotypes of HUCV,HADV,HAsta V and HRV in stool specimens,and the clinical data of children were collected at the same time. RESULTS: In 2016,the total positive rate of HRV,HUCV,EAd V and HAst V in the stool specimens of children with viral diarrhea was 59%(236 cases). The positive detection rates of the four viruses were 178 cases(75.42%),68 cases(28.81%),12 cases(5.08%)and 5 cases(2.12%)respectively,of which 11.42% were double mixed infection and 41% were of unknown pathogens. The HRVG6(93.63%,147 cases)and P3(91.08%)as well as G6[P3](90.45%)combination were the popular superior type in 2016,and about 11.79%couldn't be typed. Norovirus accounted for 92.65% of the calicivirus viruses,and GⅡ accounted for 98.41%. About 81.79%of the children(193 cases)hospitalized for viral diarrhea were less than 24 months old. Pathogens were different at different peak age. The peak season of onset was fromJanuary to March and December. The main clinical manifestations were diarrhea,vomiting and fever. CONCLUSION: It is essential to determine the unknown pathogens and improve the pathogenic spectrum in children with viral diarrhea. Viral diarrhea is often accompanied by extraintestinal clinical manifestations.

Standardized residency training is the only way for medical students to grow into qualified doctors. Since the implementation of pediatric standardized residency training nationwide in 2014,the training has been progressing smoothly and has made remarkable achievements. In order to further adapt to the status of residential training,meet the needs of pediatric training nationwide and improve the quality of pediatric training,the Pediatric Professional Committee organized experts to brainstorm and revise the two criteria, "Pediatric Criteria for Standardized Residency Training Base" and "Pediatric Criteria for Standardized Residency Training Contents". The revision of these two criteria follows the principles of continuity,accessibility,advancement and consistency. Revision was focused on lowering the scale of the base,proposing the concept of professional group,making rotation of specialty flexible,and strengthening stratified training. We hope to improve the capability of base to improve the comprehensive ability of residential students without increasing the bed size. It is believed that the pediatric standardized residency training system will provide a solid foundation for the pediatric training through providing good policy guidance,support,and medical and educational cooperation.

Medical education after graduation is the necessary step for each medical graduate to become an independent practitioner. The present medical education system or standardized residency training system in China is similar to that of the United States and Canada. This system is designed to ensure that all trained physicians are competent in practicing medicine in their trained medical specialties. The article briefly summarizes the current status of the pediatric residency and neonatal-perinatal medicine fellowship training system in the United States. The critical roles of the American Board of Pediatrics and the Accreditation Council for Graduate Medical Education are also presented.

Autism spectrum disorder(ASD)is a developmental disorder disease,with social interaction and communication disorders,stereotyped behavior and narrow interest. As autism knowledge popularization,more and more autism was intervention,however,more concentrated the youth. Pediatricians should also pay attention to the intervention result and the other life stage.

The autism spectrum disorders(ASDs)are a complex group of neuropsychiatric conditions defined by impairment in three core behavioral domains:social interaction,verbal and non-verbal communication,and restricted interests/repetitive behaviors. Extensive genetic studies have led to the identification of many autism susceptibility genes,and increased understanding on the contribution of de novo and inherited copy number variation. Here,we seek to place recent genetic findings within a developmental and brain circuit context,and approach the basic understanding of autism neuropathology from multiple genetic,molecular,cellular and neural circuit domains. The authors reviewed literatures that interrogates brain mechanisms of ASDs utilizing animal models,primarily in mice. Understanding genetic data within a brain development context will shed light on how individual risk genes operate to determine patient symptomatology,which will inform circuit specific behavioral interventions leading to better intervention and disease outcomes.