2012年 · 第50卷第04期

The child, female, 2 years old and 10 months old, was admitted to the hospital because "the heart shadow was found to be enlarged for half a month". Half a month ago, the child found that the heart shadow was enlarged due to cough and chest radiograph, so he was admitted to our hospital for treatment. He is usually healthy, denying shortness of breath and limited activity. Physical examination: The heart boundary enlarged to the lower left, the heart sound was strong, and a grade 1/6 diastolic blowing-like murmur could be heard between the costs at 2~4 of the right margin of the sternum. Electrocardiogram showed sinus rhythm, 98 beats/min, left ventricular hypertrophy. Orthographic radiograph of the chest: the shape of the heart shadow is enlarged, the left heart margin is round, the aortic node exists, and the blood of both lungs is normal. Echocardiography showed that the diameter of left ventricle and aorta was enlarged, with left ventricular end-diastolic diameter of 42 mm, aortic diameter of 16 mm, and aortopulmonary artery diameter of 13 mm; The right coronary artery was dilated and tortuously ran along the atrioventricular sulcus and opened in the posterior wall of the left ventricle. The diameter of the opening was 9 mm. The diameter and running of the left coronary artery were normal, and there was no stage dyskinesia in the ventricular wall. Heart function is normal. Orthogram of the root of the ascending aorta: the right coronary artery was obviously dilated, running tortuously and merging into the posterior wall of the left ventricle (the diameter of the opening was 9.8 mm). The fistula had multiple stenoses, the narrowest part was located in the lateral wall of the right ventricle, with an inner diameter of 4.1 mm, and no obvious branches were seen in the middle. The left coronary artery was normal in diameter and course (Figure 1)。 The child was successfully treated with interventional occlusion: the right femoral artery, right coronary artery and left ventricular track were established, and the domestic Lifetime 14 mm ventricular septal defect (VSD) occluder was placed in the narrowest part of the fistula. No residual shunt was found on angiography immediately after operation (Figure 2), there were no ST-T segment changes in ECG monitoring. After the operation, he was given oral low-dose aspirin 5 mg/ (kg·d) anticoagulation. There was no myocardial ischemia change in the reexamination of electrocardiogram, no residual shunt in echocardiogram, and the left ventricular diameter was smaller than that before the operation. He was discharged 3 days after the operation. The child has been followed up for 3 months at present, and the general condition is acceptable. There is no abnormality in electrocardiogram, echocardiogram indicates that the ventricular chamber diameter is within the normal range, and the occluder position is correct. He is still given low-dose aspirin oral maintenance.

A 7-year-old girl was admitted to hospital in January 2011 due to "edema, cola-colored urine for 30 days, anemia, thrombocytopenia, and increased serum creatinine for 25 days". The child had nausea and vomiting 30 days before admission and 1 week later developed eyelid edema, cola-colored urine, decreased urine output, 150~500 ml per day, poor spirit, less speech, less movement, and no diarrhea. 25 days ago, the edema was aggravated, accompanied by pale complexion. He went to a local hospital and checked for an increase in blood pressure of 157/130 mm Hg (1 mm Hg =0.133 kPa), anemia, and no yellowing stain on the skin. Assay: Hemoglobin minimum 58 g/L, platelet minimum 33×10⁹/L; Red blood cell fragments were visible in peripheral blood smears; Urinary routine: protein (+ + +), red blood cells (+ + +), 24 h urine protein quantification 1.442 g. Serum creatinine increased progressively up to 507 μ mol/L, blood urea nitrogen (BUN) up to 28.6 mmol/L, lactate dehydrogenase (LDH) 1369 U/L, total bilirubin 24.5 μ mol/L and indirect bilirubin 17.8 μ mol/L. Blood complement C3 0.42 g/L (normal value 0.5-1.5 g/L), C4 0.22 g/L (normal value 0.2-0.7 g/L), Coombs test negative; Anti-nuclear antibody (ANA), anti-neutrophil cytoplasmic antibody (ANCA) and extractable nuclear antigen (ENA) antibody profiles were all negative. The proposed diagnosis of "hemolytic uremic syndrome (HUS)" was given symptomatic treatment such as diuresis, antihypertensive and anti-inflammatory, and intermittent transfusion of washed red blood cells and plasma, plasma exchange once and renal replacement therapy. The child's edema resolved, and the monitored hematological parameters recovered slightly, but urine protein (+ + +), red blood cells (+ + +), oliguria (50-150 ml per day) and serum creatinine 287-383 μ mol/L were still present. Despite oral antihypertensive therapy, blood pressure control was not satisfactory. During the course of the disease, nausea, vomiting and abdominal distension were repeated, and slightly improved after gastrointestinal decompression. Transferred to our hospital for further diagnosis and treatment.

Hand-foot-mouth disease (HFMD) was first reported in Shanghai in 1981. Since then, it has been reported in more than a dozen provinces such as Beijing, Hebei, Tianjin, Jilin, Shandong, Hubei, Qinghai and Guangdong. An outbreak of HFMD caused by coxsackievirus A16 (Cox A16) occurred in Tianjin in 1983, with more than 7,000 cases occurring between May and October alone. After two years of low-level sporadic epidemics, another outbreak occurred in 1986. Enterovirus 71 (EV71) was isolated from patients with HFMD in Wuhan Institute of Virology in 1995, and EV71 was also isolated from children with HFMD in Shenzhen Health and Epidemic Prevention Station in 1998. It has been established that the pathogens causing HFMD are a variety of enteroviruses, including Coxsackievirus group A 16, 4, 5, 7, 9, 10, group B 2, 5, 13, ECHO virus and EV71, among which Cox A16 and EV71 are the most common^[1]。

hand foot mouth disease (HFMD) and herpangina (herpangina) in children caused by enterovirus 71 (EV71) infection have become important issues for disease prevention and control systems and pediatricians, especially pediatric critical care specialists, to face. The disease occurs all over the country between April and July every year and causes many infant and child deaths. The proportion of neurological complications after EV71 infection is particularly high. Brainstem encephalitis and cerebral hernia cause relative sympathetic nerves, resulting in neurogenic pulmonary edema (NPE) and circulatory failure. China's Ministry of Health has issued relevant guidelines or consensus, but there are still many controversies regardless of the mechanism and treatment plan.

human cytomegalovirus (HCMV), officially named as human herpes virus 5 (HHV-5), is extremely widespread in China. The positive rate of HCMV antibodies in general population is 86% ~96%, about 95% in pregnant women, and 60% ~80% in infants. Primary infections mostly occur in infants and infants. HCMV has a latent-activated biology that, once infected, lasts for life. Although HCMV is a weak pathogenic factor, it is not obviously pathogenic to individuals with normal immune function, and most of them are asymptomatic infections; However, HCMV is a common pathogen that causes diseases in pathologically and physiologically immunocompromised people, including developmental immunodeficiency in fetuses and newborns. It is also one of the important causes of AIDS, severe diseases and increased mortality in organ and bone marrow transplant patients.

Hand, foot and mouth disease is an acute infectious disease mainly manifested as maculopapular rash and herpes in hands, feet, mouth and other parts. Most of them are benign clinical processes, but a few cases may appear severe and critical manifestations such as meningitis, encephalitis, encephalomyelitis, pulmonary edema, circulatory disturbance, etc. Enterovirus 71 (EV71) is the main pathogen of these cases. Acute pulmonary edema secondary to brainstem encephalitis is an important cause of death in critically ill children^[1]However, factors such as acute left heart failure, cytokine release and increased vascular permeability also play a role in the formation and progression of pulmonary edema. At present, the mechanism of acute pulmonary edema caused by EV71 is still unclear, and its research progress is summarized as follows.

neuroblastoma (NB) is the most common extracranial tumor in children. More than 50% of children have extensive metastasis in bone, bone marrow, liver and other organs at the time of diagnosis, but the incidence of metastasis in central nervous system is still very low^[1]。

human respiratory syncytial virus (HRSV) is the most common pathogen of acute lower respiratory tract infection in infants and young children, which seriously endangers their health^[1,2]。 Children aged 2 have had at least one infection, 50% of whom have had two infections^[3]。 HRSV infection is one of the main causes of hospitalization in infants and young children in winter and spring. According to the WHO, about 64 million people worldwide are infected with HRSV every year, including 160,000 deaths^[4]。 According to a new study, HRSV infection is associated with 20% of children under 5 years of age who are hospitalized with acute respiratory infections between November and April of each year^[5]。 The incidence of HRSV infection is global, and local outbreaks can prevail, which has become a global public health problem.

Hand, foot and mouth disease is a common acute infectious disease in children caused by enterovirus and transmitted mainly through fecal-oral and respiratory routes. Most children have mild clinical symptoms, with fever and rash or herpes in hands, feet, mouth, buttocks and other parts as the main clinical manifestations, while a few severe children can involve the nervous system and develop cardiopulmonary symptoms, even leading to death^[1]。

hemolytic uremic syndrome (HUS) is the most common cause of acute renal failure in children. It belongs to systemic thrombotic microvascular disease, and is clinically characterized by microvascular hemolytic anemia, thrombocytopenia and acute renal failure. Previously, HUS was classified as post-diarrheal HUS according to the presence or absence of diarrheal symptoms (D^＋HUS, i.e., typical HUS) and non-diarrheal HUS (D^－HUS, i.e. atypical HUS). Among them, atypical HUS accounts for 5% ~10% of children's HUS. In the past, this type lacked specific treatment methods and had poor prognosis. More than 50% of children needed long-term dialysis treatment, which is one of the difficult and severe diseases in pediatrics. In recent years, there have been some new understanding of the pathogenesis of atypical HUS, and new classification methods have emerged internationally^[1]HUS was classified into infection-induced (including post-diarrheal HUS and Streptococcus pneumoniae-associated HUS), complement regulation abnormality-associated, Thrombomodulin genetic defect-associated, vitamin B12 metabolic defect-associated HUS, and others. In the new classification, post-diarrheal HUS is the typical HUS defined in the past. Whether Streptococcus pneumoniae-related HUS is classified as typical or atypical HUS is still controversial, and the rest types belong to atypical HUS in the previous classification. Among atypical HUS, complement-dysregulation-related HUS account for more than half^[2]And new understanding of its mechanism of occurrence and treatment is also gained, which is helpful to improve the treatment and prognosis of children with atypical HUS.

A 6-year-old child was admitted to hospital on July 22, 2011 due to rash for more than 1 month and intermittent fever for more than 10 days. More than one month before admission, the child had scattered red herpes on his limbs, the blister fluid was clear, the surrounding skin was red and swollen, accompanied by pain and itching, the herpes crashed after ulceration, and the scab was not easy to fall off for a long time. After falling off, there was vaccinia-like scar without obvious pigmentation. Fever appeared more than 10 days before admission, with the highest body temperature of 39.5℃, and the fever type was not obvious. During the period, the body temperature was normal at the longest interval of 3 days, and the treatment of antipyretic drugs could reduce it to normal. Occasionally, there was headache, nausea, no chills, convulsions, no abdominal distension, diarrhea, no fatigue, night sweats, etc. during fever. Since the onset of the disease, the child has good mental reaction, good appetite, and normal urine and stools.

The 9th National Conference on Pediatric Infectious Diseases and the 10th National Conference on Children's Liver Diseases was held in Yangzhou, Jiangsu Province from April 10 to 13, 2011. The conference was jointly sponsored by the Infectious Diseases Group of the Pediatrics Branch of the Chinese Medical Association, the Children's Liver Diseases and Infectious Diseases Group of the Infectious Diseases Branch of the Chinese Medical Association and the Editorial Committee of the Chinese Journal of Pediatrics. More than 150 representatives from more than 20 provinces, municipalities and autonomous regions across the country attended the meeting, received a total of 122 manuscripts, 10 experts gave special lectures, 31 representatives spoke at the congress, and had a lively discussion on the Diagnostic Guidelines and Treatment Principles of EBV-related Diseases in Children. The meeting also invited Canadian experts to host the symposium on "Challenges of Pediatric Infectious Diseases in the Next 10 Years". The main contents of the meeting are introduced as follows.

The 11th National Academic Conference on Pediatric Endocrine Genetic Metabolic Diseases jointly organized by the Endocrine Genetic Metabolology Group of Pediatrics Branch of Chinese Medical Association and the Editorial Committee of Chinese Journal of Pediatrics was held in Dongshan Hotel, Yantai, Shandong Province on October 13-16, 2011. 450 official representatives of the pediatric genetic metabolic endocrinology specialty from all over the country attended the conference. A total of 168 papers were received at the conference, including 117 papers on endocrine diseases and 51 papers on genetic metabolic diseases, of which 17 papers were selected as speakers at the conference. The conference set up 10 special lectures, and invited Professor Jan Lebl, President of the European Society of Pediatric Endocrinology, and experts in pediatric endocrine genetic and metabolic diseases from Europe, America, Asia-Pacific and China to give special reports on diabetes, sexual development disorders, metabolic bone diseases, lysosomal storage diseases and other related diseases. Seven satellite meetings (including 10 lectures) were arranged; For the first time, two special clinical cases of sexual development abnormalities and genetic metabolic diseases were discussed at the group meeting. The participants had extensive exchanges and lively discussions with domestic and foreign experts on hot issues of children's endocrine genetics and metabolism.