2013年 · 第51卷第05期

Professor Wang Muti, a member of the 11th and 12th editorial boards of Chinese Journal of Pediatrics, a member of the Academic Steering Committee of the 13th and 14th editorial boards, a famous expert on pediatric genetic, metabolic and endocrine diseases in China, and a professor, chief physician and doctoral supervisor of Tongji Hospital affiliated to Tongji Medical College of Huazhong University of Science and Technology, died in Wuhan on February 25th, 2013 at the age of 80 due to ineffective treatment.

necrotizing enterocolitis (NEC) is the most common digestive emergency in newborns. Previously, the incidence of neonatal NEC was relatively low due to more early deaths in premature infants. In recent years, with the increase of the number of premature infants and the prolongation of survival time, the incidence of NEC in premature infants is increasing, especially in very low birth weight premature infants. The United States reported that the prevalence of NEC in premature infants with a birth weight of 500~1500 g was 7%^[1]The results of the 2011 National Neonatology Group Survey showed that the incidence of NEC in very low birth weight infants was 6.6% (170/2564). Once NEC occurs in premature infants, the mortality rate is as high as 20% ~30%^[1]It is significantly higher than that of full-term infants. At present, NEC is the main cause of death of premature infants in the late stage (2nd to 4th week), and has become one of the most difficult problems in neonatology. At the same time, the inflammatory response of NEC can affect the brain, causing long-term neurodevelopmental delay in children, and 25% of children with NEC will develop microcephaly or serious neurodevelopmental problems^[1,2]。 The prolonged hospital stay, surgical treatment and related complications caused by NEC significantly affect the children's quality of life and cost a lot of medical expenses. Therefore, strengthening the research on prevention and treatment of NEC is a very urgent issue in neonatology department^[3]。

Necrotizing enterocolitis (NEC) is an acquired inflammatory disease of the neonatal intestine that occurs mainly in premature infants, and the younger the gestational age and the lower the birth weight, the higher the incidence. So far, the prevalence of NEC, the related mortality and disability rate have not been improved, and even increased in some areas^[1]On the one hand, this is related to the increasing survival rate of premature infants with the continuous improvement of rescue technology, and on the other hand, it is also due to the lack of effective prevention and treatment measures. The mortality rate of NEC-related cases is about 20% ~30%, and 1/3 of the children need surgical treatment. Excessive inflammatory response can even affect distant organs such as the brain, causing long-term neurodevelopmental delays in children. In fact, 25% of children with NEC will develop microcephaly or severe neurodevelopmental problems^[1,2]。 The prolonged hospital stay, surgical treatment and related complications caused by NEC significantly affect the children's quality of life and cost a lot of medical expenses.

Allogeneic hematopoietic stem cell transplantation (allo-HSCT) and immunosuppressive therapy (IST) are the main methods for the treatment of aplastic anemia severe (SAA) and very severe (VSAA). Compared with IST, allo-HSCT has rapid hematopoietic reconstruction, low recurrence rate, high failure-free survival (FFS) (i.e., survival and treatment-effective, death, non-response to treatment at 6 months, disease progression or recurrence is defined as treatment failure), and fewer secondary malignancies. Therefore, HLA-matched sibling donor HSCT (MSD-HSCT) has been the preferred treatment option for SAA/VSAA in children. In recent years, with the development of matching technology, the improvement of pretreatment protocol, the use of new immunosuppressants and the advancement of supportive therapy such as anti-infection, the efficacy of allo-HSCT in the treatment of SAA/VSAA in children has been greatly improved, and the 10-year survival rate (OS) can reach 80% ~92%^[1,2,3]。 In terms of donor selection, alternative donor HSCT (AD-HSCT) including unrelated donor HSCT (URD-HSCT), cord blood transplantation (CBT) and haploidentical allogeneic hematopoietic stem cell transplantation (haplo-HSCT) It has also obtained exciting curative effect, and is an ideal treatment for refractory and relapsed SAA. The latest progress of allo-HSCT in the treatment of AA in recent years is reviewed.

Primary immune thrombocytopenia (ITP) is the most common hemorrhagic disorder in children, with an incidence rate of about (4~5) /100,000^[1]。 ITP in children is a benign self-limiting disease that often onsets within days or weeks of infection or vaccination, with platelet count (PLT) returning to normal within 12 months of diagnosis in 80% of cases. Given that ITP in children is mostly a self-limiting process, the aim of treatment is mainly to prevent severe bleeding, rather than to raise PLT to normal^[2,3]。 In order to further standardize the diagnosis and treatment of ITP in children, pediatric hematologists put forward the following suggestions on the diagnosis and treatment of ITP in children.

With the progress of clinical diagnosis technology, 25% ~43% of children with cardiomyopathy can be clearly identified^{[1,2,3,4,5,6,7,8]}Among them, inborn errors of metabolism (IEM) accounted for 11% to 34%^[4,5,6]。 Because some IEMs are targeted, it is possible to reverse, prevent or delay myocardial lesions and improve prognosis after treatment, so early recognition and definite diagnosis are very important. According to the relevant research data at home and abroad, after expert discussion and reach a consensus, this proposal is put forward, aiming at guiding clinicians to select appropriate examination methods for such children, clarify the cause of the disease early and give corresponding treatment.

primary immune thrombocytopenia (primary immune thrombocytopenia) is the most common hemorrhagic disorder in children, and used to be known as "idiopathic thrombocytopenic purpura (ITP)". At present, it is preferred to designate "immune thrombocytopenia", avoiding the use of "idiopathic" and choosing "immune" to emphasize its immune-related disease mechanism, retaining the abbreviation ITP^[1,2]。 Most scholars believe that this disease is caused by the body's production of platelet autoantibodies, resulting in excessive platelet destruction; and/or disorders of differentiation and maturation of bone marrow megakaryocytes, resulting in decreased platelet production, resulting in bleeding of skin, mucous membranes and organs^[3]。 Its pathogenesis involves a variety of immune cells and cytokines. The clinical manifestations of this disease are quite different. In mild cases, only peripheral blood platelets decrease without bleeding, while in severe cases, visceral hemorrhage or even intracranial hemorrhage can be life-threatening.

harlequin color change is a peculiar sudden skin color change that is most common in newborns, mainly in premature and low birth weight infants. It is manifested by extensive skin color changes throughout the body, or it can be a skin color change in specific parts of the body, which is manifested by the appearance of two distinct colors of the body with distinct boundaries, one side is red and the other side is white. Foreign literature reports that 10% of healthy newborns will have transient clown-like color changes^[1]However, this phenomenon is rare in actual clinical practice and has not been reported in China. On October 5, 2012, a case of clown-like skin color change in a newborn was treated in our hospital, which is reported as follows.

A 6-month-old male was admitted to the hospital with the main complaint of "intermittent strabismus in both eyes for more than 20 days". More than 20 days ago, parents found that the child had frequent upward strabismus in both eyes (Figure 1) (Parents consent to publish children's facial photos). Physical examination after admission: length 67 cm, weight 8.5 kg, unable to stare and follow objects in both eyes. Strong light stimulation without blinking reflex. Both pupils were dilated, 3 mm on the left side and 4 mm on the right side, and direct and indirect light reflections disappeared. Sound stimuli respond. Looking for sound sources is not sensitive. The children's great motor development is obviously backward, unable to vertical head, unable to turn over, sitting alone, and low muscle tone of the limbs. You can't actively grab objects with both hands. The vertical head is unstable. The external genitalia are short, about 1 cm, and the scrotum is full on both sides without cryptorchidism. Enjoy drinking water. The average daily water consumption is about 1200 ml. Excessive nocturia. The child was the first birth of the mother's first pregnancy, and the mother underwent cesarean section at 43 weeks +1 of pregnancy due to expired and no amniotic fluid. Birth weight 4.5 kg. Caregiver complained: Children usually cry less and sleep after breastfeeding. During the first 3 months of life, the average daily sleep duration was about 22 h. The mother was 19 years old at the time of delivery. The mother had a history of cold and fever at 5 months of pregnancy, and recovered after local drug treatment for 3 days. Parents married unrelated. Past history: Jaundice appeared the next day after birth and resolved quickly. After admission, the developmental quotient was 35, which belonged to moderate to severe low normal level. Fundus examination: bilateral optic atrophy. MRI of the head: septum pellucidum is absent, the lateral ventricle and the third ventricle are enlarged, and the frontal horns of both lateral ventricles are square (Figure 2)； The chiasmatic recess of the third ventricle was enlarged, and the optic nerve and chiasmatic recess were slender (Figure 3)。 Liver function is normal. The 24 h video EEG was normal. Visual evoked potentials: P wave latency was moderately prolonged and amplitude decreased at 70~120 ms in both eyes. Auditory evoked potentials: No abnormalities.

Sponsored by the Emergency Group of Pediatrics Branch of Chinese Medical Association, the Pediatrics Group of Emergency Branch of Chinese Medical Association and the journal Chinese Pediatric Emergency Medicine, and hosted by the Department of Critical Care Medicine of Children's Hospital affiliated to Chongqing Medical University, the 12th National Pediatric Critical Care Conference of Chinese Medical Association was held in Chongqing from November 2 to 5, 2012. This conference invited 22 well-known pediatric critical care experts from the mainland, Hong Kong and Taiwan to give special lectures, and 20 young doctors gave paper reports. The conference received 441 papers, and a total of 488 delegates from 30 provinces, municipalities, autonomous regions and municipalities directly under the Central Government, Hong Kong Special Administrative Region and Taiwan Province attended the conference, which was the largest scale and the largest number of delegates in all previous conferences. Part of the meeting is briefly described below.

The 17th National Academic Conference on Children's Cardiovascular Diseases, jointly organized by the Cardiovascular Group of Pediatrics Branch of Chinese Medical Association and the Editorial Committee of Chinese Journal of Pediatrics, was held in Guangzhou, Guangdong Province from June 13 to 16, 2012. 330 pediatric clinicians and researchers from all over the country attended the meeting, and had extensive exchanges on children's congenital heart disease (congenital heart disease), Kawasaki disease, arrhythmia, myocardial disease, pulmonary hypertension, childhood syncope and atherosclerosis. The conference received 298 academic papers, including 65 speeches and 233 exchanges. Three symposiums on congenital heart disease, Kawasaki disease and arrhythmia were held, and 22 experts were invited to give academic lectures on hot issues of everyone's concern.