2011年 · 第49卷第05期

Congenital papilla defects are often monocular. This disease is caused by incomplete ocular vesicle embryo closure at embryonic time, often accompanied by choroidal defect, but only optic disc defect is rare^[1]。 Patients are mostly found when symptoms such as poor vision, strabismus and nystagmus appear during visual development. The discovery of optic nerve defects in the neonatal period has not been reported. One case found by our neonatal eye disease screening is reported below.

Hereditary Protein C Deficiency (hereditary Protein C Deficiency) is a hereditary disease with recurrent venous thrombosis as the main clinical feature, which is more common in adults. The neonatal onset is rare and the prognosis is poor, and most of them die within 1 to 2 months after the onset. One case was admitted to our hospital, and the report is as follows.

A 10-year-old male was admitted to the hospital on October 7, 2008 due to "fever for 7 days". Physical examination at the hospital: clear, lethargic, slightly red throat, less smooth oral mucosa, no obvious ulcer, slightly congested conjunctiva in both eyes, coarse breathing sounds in both lungs, no rales, uniform heart rhythm, soft abdomen, no tenderness, no swelling of the liver and spleen under the costs, a 5 cm old surgical scar in the right lower abdomen, no obvious ulcer in the genitals, and negative neurological examination. Past history: The child underwent "appendicitis surgery" in a local hospital in 2007 due to "abdominal pain". Multiple colon ulcers and abdominal lymph node enlargement were found during the operation, which was considered to be Crohn's disease (details unknown) and was not treated. Blood routine after admission: WBC: 34.3×10⁹/L, N: 0.90, CR160 mg/L; ESR: 53 mm/1 h; Both chest X-ray and chest CT showed left lower pneumonia. Subsequently, active anti-infective treatment was given, and the child's body temperature dropped to normal from October 30th. From the 5th day after admission, the child developed multiple ulcers in the mouth (Figure 1), the child had transient facial skin erythema on day 7, and the skin prick test was negative. At that time, the child had no gastrointestinal symptoms such as abdominal pain and diarrhea. In order to understand the intestinal lesions of the child, a colonoscopy was performed on October 17th, which showed that hyperplastic mucosa was visible in the ileocecal region, partially red and brittle (Figure 2)。 Pathological biopsy of ileocecal mucosa showed that a large number of acute and chronic inflammatory cell infiltration in intestinal mucosa with surface ulcer formation, mucosal erosion and necrosis at the ulcer site with granulation tissue hyperplasia, fibrous necrosis of submucosal vascular wall, mainly arterioles, and a large number of neutrophil infiltration were seen. Multiple ulcers of intestinal mucosa with small vessel vasculitis (consistent with the changes of intestinal Behcet's disease) were considered (Figure 3)。 Capsule endoscopy on October 20th showed multiple ulcers of small intestine and colon. According to the clinical manifestations, endoscopic findings and pathological findings, the child was finally diagnosed as intestinal Behcet's disease, and was treated with mesalazine orally. After 4 months, the child's condition improved, and there was no abnormality in re-examination colonoscopy, so the drug was discontinued in April 2009. After 1 month of drug withdrawal, the child experienced diarrhea with fever again. Blood routine after admission: WBC: 17.9×10⁹/L, N: 0.85, CRP: 98 mg/L; ESR: 46 mm/1 h; Reexamination of colonoscopy showed multiple hyperplasia and swelling of the colon mucosa, and multiple huge puncture-like ulcers surrounding it, covered with white coating (Figure 4)。 Pathological biopsy revealed chronic active ileocecal mucosa with inflammatory necrosis. Considering the recurrence of Behcet's disease, continued treatment with mesalazine and additional anti-inflammatory treatment with methylprednisolone, and the child's condition improved and was discharged. Since then, he has been given oral treatment with prednisone and mesalazine, and his condition is well controlled, but he relapsed after hormone reduction.

arrhythmogenic right ventricular cardiomyopathy (ARVC), also known as arrhythmogenic right ventricular dysplasia (ARVD), is a genetically determined cardiomyopathy with multiple clinical phenotypes in which cardiomyocytes are gradually replaced by fat and fibrous tissue, and the lesions are mainly in the right ventricle. This disease occurs in young and middle-aged people. In the survey of sudden death of young people and athletes in Italy, it was found that 20% of the disease was caused by ARVD/C^[1]。 ARVD/C accounts for 17% of youth sudden deaths in the United States^[2]Therefore, ARVD/C has been recognized as one of the important causes of sudden death in young people. Pediatric case reports rare^[3,4]However, according to the available data, the clinical characteristics of pediatric patients are similar to those of adults, and they are prone to misdiagnosis and missed diagnosis due to hidden onset. There are still many aspects to be further studied about this disease, and the following is a summary of the current research progress.

Kawasaki disease (KD) is an acute febrile and eruptive disease mainly occurring in infants and young children under 5 years old. Coronary artery damage is the most serious complication. In severe cases, it can cause myocardial infarction and sudden death, which seriously threatens children's physical and mental health. In developed countries or regions, coronary complications caused by KD have become the leading cause of acquired heart disease in children, and may become one of the risk factors of ischemic heart disease in adulthood^[1]。 endothelial progenitor cells (EPC) are precursor cells that can circulate, proliferate and differentiate into vascular endothelial cells, but have not yet expressed the phenotypic characteristics of mature vascular endothelial cells. They are involved in the development of adult blood vessels and the repair of vascular endothelium after injury. At present, there are many studies on EPC in the treatment of adult coronary atherosclerotic heart disease, ischemic disease and malignant tumor, but the study of EPC in children with KD has not been reported in Chinese literature. In this paper, the biological characteristics and functions of EPC and the research progress of EPC in children with KD abroad are reviewed.

Changes in pediatric disease spectrum in recent 20 years^[1]Allergic diseases and autoimmune diseases continue to increase, with allergic diseases affecting about 25% of children^[2]。 The prevalence of food allergy among children in developed countries is about 3% ~6%; The prevalence of food allergies in children in the United States has risen by 18% in the past decade^[3]； The prevalence of peanut allergy among British children has doubled in recent 5 years^[4]。 At present, there is no national prevalence data of food allergy in infants and young children in China. In 1999, the detection rate of food allergy in infants from 0 to 24 months in Chongqing was 3.5% to 5.1%^[5]； After 10 years (2009), the results showed that the detection rate of food allergy in infants and young children was 7.0% ~9.2%^[6]It suggests that the prevalence of food allergy among children in China may also show an upward trend. Although most food allergies can heal themselves with age, studies have shown that food allergies in infant and early childhood may increase the incidence of other allergic diseases in later childhood^[7,8]。

The history of modern medical development shows that the breakthrough progress of future medicine depends on interdisciplinary research and cooperation, and the 21st century medicine pays more attention to the holistic medical view. Chen Zhu, Minister of Health, pointed out in the report entitled "Scientific Issues in Accelerating the Transformation of China's Medical Model and Promoting the Reform of China's Medical and Health System" at the S13th Academic Symposium of Xiangshan Science Conference in December 2010: Translational medicine is committed to bridging the gap between basic experimental research and development and clinical application, and opening up new ways for the research of revolutionary new treatment methods^[1]。 One of the principles of life science development in the 21st century is the unity of basic research and applied research. The so-called close combination and unity of basic and clinical research here is to emphasize multidisciplinary integration and cross-integration. Experts in different scientific fields communicate and cooperate with each other, and the research methods are complementary and borrowed, which generates new thinking at the intersection of different disciplines, which often stimulates innovation at the source^[2]。 In September 2009, Han Qide, vice chairman of the Standing Committee of the National People's Congress and chairman of China Association for Science and Technology, said at the 11th annual meeting of China Association for Science and Technology that the key point of today's scientific and technological development is cross-section and integration. In modern times, our country lags behind others in science and technology, largely because the subjects cannot cross or cross less^[3]。 In the "Eleventh Five-Year Plan" and "Twelfth Five-Year Plan" discipline development strategies and priority development areas of the National Natural Science Foundation of China both emphasize the emphasis on funding multi-disciplinary research^[4]。 It can be seen that the leadership of China's scientific community and health department have attached great importance to interdisciplinary research.

Children's health care is one of the most distinctive subspecialties of pediatrics. It is not only the foundation of pediatrics, but also has interdisciplinary intersection with many subspecialties of pediatrics. In recent years, the branch of developmental behavioral pediatrics, which has developed rapidly, has very distinct clinical specialty characteristics. So far, the English translation of Child Health Care is still not well unified, even in the textbooks of Child Health Care, there are inconsistencies, some translation is "Child Health Care", and some translation is "Primary Child Care"^[1,2]。 China's children's health care profession covers a wider range of professional contents, and it can't be directly connected with the corresponding professional classifications of major pediatric organizations in Europe and America. For example, a comparison of specialty classifications with the American Board of Pediatrics^[3]The assessment and monitoring of children's physical growth and development, children's nutrition assessment and consultation, disease prevention, early screening of diseases and children's health care in various stages involved in children's health care in China all belong to the category of general pediatrics, while the monitoring of neuropsychological behavioral development and disease diagnosis and treatment belong to the field of developmental-behavioral pediatrics, a subspecialty of pediatrics, while adolescent health care belongs to the subspecialty of adolescent medicine. Similarly, in the professional classification of the European Academy of Pediatrics, there is no suitable sub-specialty that can well cover the current work content of the child health profession in China^[4]。 In fact, the development of children's health care in China has strong historical characteristics. With the changes of domestic children's health care characteristics in different periods, its work tasks also change accordingly. In recent years, with the improvement and maturity of the development of children's health care, the sub-specialty branches within the profession are becoming more and more clear. However, how to subdivide subspecialties without dividing the overall observation of childhood growth and development and the understanding of the occurrence of related diseases is a major challenge.

Wang Cheng Yi Cheng Kai On(350001 Pediatrics, Fujian Maternal and Child Health Hospital) Q:

The 18th International Conference on the Prevention of Child Abuse and Neglect was held in Hawaii, USA, September 26-29, 2010. The theme of the conference was "One World, One Family, Multiculturalism". More than 800 pediatric, psycho-psychiatric, forensic experts, lawyers and social workers from five continents attended the conference. A total of 490 papers on 96 topics were exchanged in 12 sub-sessions of the conference. The main contents of the exchange were: medical identification of child abuse, medical treatment of abused children, treatment and rehabilitation of psychological trauma, neglect of children's dental health, and case analysis of child death.

A male child, 22 d, was admitted for "dyspnea with left pleural effusion for 22 d". The child was delivered spontaneously at 28 hours after premature rupture of membranes at 30 weeks of gestation in G4P2, with a birth weight of 1600 g. B-ultrasound at 24 weeks of pregnancy showed "left pleural effusion, heart compression and right shift". Apgar score was 2 at 1 min after birth. After tracheal intubation and cardiopulmonary resuscitation, left thoracocentesis was performed, and about 25 ml of pale yellow fluid was extracted. Apgar score was 8 at 5 min, and he was immediately transferred to the local hospital NICU. Multiple chest radiographs and B-ultrasound showed a large amount of pleural effusion on the left side, and repeated pleural puncture and fluid extraction (8 times in total, about 50-80 ml each time). Check the number of conventional cells in pleural effusion 40×10⁶/L ~180×10⁶/L, 80% ~85% of monocytes, negative for Li Fanta test, negative for chyle test, negative for pleural effusion culture, mycoplasma, mycobacterium tuberculosis and tumor cell examination. The child continued ventilator support due to dyspnea after birth, and failed to withdraw the machine many times; After fasting, intravenous nutrition and anti-inflammatory treatment with cefotaxime sodium and erythromycin for 22 days, the left pleural effusion did not improve, so he was transferred to our hospital for further treatment.