2013年 · 第51卷第03期

In recent years, the research on the pathogenesis of systemic lupus erythematosus (SLE) has been deepened, from the level of histopathology to the level of cellular and molecular biology, thus promoting the progress of pharmacotherapy of SLE. In March 2011, the U.S. Food and Drug Administration (FDA) approved the launch of a humanized monoclonal antibody, Belimumab, for the treatment of SLE, ending the embarrassing situation of no new drug for SLE for more than half a century. On the other hand, the continuous accumulation of evidence-based medical data has enabled rheumatologists to have a more comprehensive and in-depth understanding of the application of traditional treatment drugs for SLE, further improved the treatment strategy of SLE, and also put forward higher requirements for treatment goals^[1]。 At the same time, the progress of therapeutics has also greatly improved the survival rate and prolonged the survival time of patients, while the importance of long-term complications such as premature atherosclerosis and cardiovascular events has become increasingly prominent and a new challenge. In view of several hot issues in the clinical treatment of SLE, this paper introduces the research progress in recent years.

The adoption of evidence-based methods to formulate clinical practice guidelines has become the mainstream trend and consensus in the world. The widespread application of evidence-based clinical practice guidelines has also become the most substantial change in the clinical field in the past ten years^[1]。 The methodology used to develop evidence-based Clinical practice Guidelines has also matured, with the National Institute for Health and Clinical Excellence (NICE) and the Scottish Intercollegiate Guidelines Network (SIGN) And other authoritative institutions have formulated corresponding methodologies^[2,3]。 Since 2008, the number of evidence-based guidelines in clinical practice guidelines developed in China has increased year by year, and there is a trend of gradually replacing expert consensus guidelines^[4]。 However, in the field of pediatrics, the formulation of evidence-based guidelines is facing great challenges, including the lack of high-quality evidence, the lack of evidence-based concept and evidence-based methodology caused by the particularity of pediatric clinical research^[5]。 As of July 2010, only five evidence-based clinical practice guidelines have been formulated in the field of pediatrics in China^[6]。

In March, 2010, the Editorial Committee of Chinese Journal of Pediatrics of Chinese Medical Association organized the Immunology Group, Children's Health Group and Gastroenterology Group of Pediatrics Branch of Chinese Medical Association, as well as the Children's Dermatology Group, Respiratory Specialty and Evidence-Based Medicine Specialty of Dermatology and Venereology Branch of Chinese Medical Association to write the "Evidence-Based Suggestions for the Diagnosis and Treatment of Milk Protein Allergy in Chinese Infants" (referred to as evidence-based suggestions for short). After more than two years of literature retrieval, evidence grading, first draft writing, and many discussions and repeated revisions by expert groups, this evidence-based suggestion is formed on the basis of evaluating the evidence grades of nearly 1,000 documents retrieved item by item. Evidence-based suggestions mainly provide reference for clinicians to diagnose and treat children's milk protein allergy, and mainly involve the diagnosis, diet management, treatment, prevention and other clinical related contents of milk protein allergy.

The concept of "allergy", first proposed by Austrian pediatrician Clemens von Pirquet in 1906, has been more than 100 years old^[1]People are becoming more aware of food allergies. To help clinicians deal with food allergies in children, various countries have successively published consensus and guidelines on food allergies^{[2,3,4,5,6,7,8,9,10]}。

In July 2011, the Immunology Group of Pediatrics Branch of Chinese Medical Association and the Editorial Committee of Chinese Journal of Pediatrics jointly organized and formulated the "Recommendations for Diagnosis and Treatment of Systemic Lupus Erythematosus in Children" on the basis of sufficient evidence-based, which was published in Chinese Journal of Pediatrics, Volume 49, 2011, pages 506-514, and the interpretation of the diagnosis of this recommendation was published at the same time (Chinese Journal of Pediatrics, Volume 49, 2011, pages 515-518). Now, the treatment part of this suggestion is interpreted and supplemented, hoping to provide a reference for the treatment of systemic lupus erythematosus (SLE) in children.

Diffuse intravascular coagulation (DIC) is a clinical syndrome complicated by many diseases, and it is an important cause of death in newborns, especially premature infants. This disease occurs after endothelial cell injury, related cells and proteins are exposed to the surface of procoagulant substances, coagulation cascade is activated, fibrin is extensively deposited, micro-thrombosis in capillaries is formed, coagulation factors and platelets are continuously consumed, and then hyperfibrinolysis and bleeding occur, which is more common in the critical stage of the disease.

The incidence of acute myeloid leukemia (AML) accounts for about 20% of children with acute leukemia, and the 5-year event-free survival rate ranges from 50% to 60%^[1,2]。 In recent 30 years, with the combined application of intensive therapy, stem cell transplantation (SCT) and supportive therapy, the prognosis of children with AML has been greatly improved^[3]。 However, 30% ~40% of patients eventually relapse, suggesting that the current treatment plan cannot completely eliminate all malignant cells. The residual leukemia cells in the patient's body, that is, minimal residual disease (MRD), become the root cause of recurrence^[4]。 At present, minimal residual diseases are mainly detected by molecular biological techniques such as polymerase chain reaction (PCR) or molecular immunological methods such as flow cytometry (FCM). Despite the high sensitivity of the PCR technique, which can reach 10^－4to 10^－6However, only about 40% of AML patients have genetic markers suitable for minimal residual disease detection, such as AML1-ETO, CBFB-MYH11 or MLL recombinant gene products, etc^[5,6]Thus limiting its scope of application. The use of FCM technique to detect minimal residual disease in AML is suitable for almost all cases. On the other hand, there are many reports of using FCM to detect minimal residual disease of ALL in children^{[7,8,9,10,11]}, and has been generally accepted by hematological oncology workers, but its application in children with AML has been rarely reported, and the negative-positive judgment criteria are not uniform^{[12,13,14,15,16]}。 The research progress in this field in recent years is reviewed.

The ATP-binding cassette transporter A3 (ABCA3) gene was developed by Klugbauer and Hofmann^[1]Cloned from human medullary thyroid carcinoma cell line cDNA, mapped to 16p13.3, contains 33 exons, including 80 kb bases, directing protein synthesis containing 1704 amino acids. ABCA3 protein is the membrane protein of lamellar bodies (LB) in type Ⅱ alveolar epithelial cells (ATⅡ)^[2,3], driving substance transmembrane transport by consuming energy, affecting phospholipid transport and LB formation^[4,5]。 2004 Shulenin et al.^[6]It is reported that ABCA3 gene mutation is related to lethal pulmonary surfactant (PS) deficiency. Since then, a series of clinical and basic studies have been carried out abroad, but there are few related studies in China. This article mainly reviews the relationship between ABCA3 gene and neonatal respiratory distress syndrome (RDS) and pulmonary interstitial disease.

A 2-year-old and 5-month-old male was found to be a mass in the right upper abdomen for more than 1 month and went to the doctor 2 weeks after operation. The parents of the child inadvertently touched a mass in the right upper abdomen of the child while bathing the child in March 2011. On April 7, 2011, they went to the local hospital for MRI examination, and found a huge space-occupying lesion in the right kidney of the child, about 12.1 cm ×7.6 cm ×7.7cm in size, considered to be nephroblastoma (Figure 1), and performed "radical resection of right nephroblastoma + right renal hilar lymph node dissection + right adrenalectomy" in this hospital on April 23, 2011. The postoperative pathology was confirmed as right renal nephroblastoma by consultation in our hospital.

The child, 5 months old, was admitted to hospital for more than 1 month due to dyspnea. The child developed inspiratory dyspnea without obvious trigger more than 1 month ago, obvious after crying, intermittent facial cyanosis, cough, a small amount of white sticky sputum, and no fever. In the local hospital, considered "pneumonia" and "bronchial asthma", ipratropium bromide, budesonide aerosol antispasmodic and asthmatic treatment, dexamethasone anti-inflammatory (dose unknown), cefamandol anti-infective treatment, symptoms did not improve, dyspnea progressively aggravated, and then inspiratory wheezing sound appeared. He visited a hospital in Beijing 4 days ago, and the blood gas analysis showed pH 7.345, PO₂7.70 kPa, PCO₂6.58 kPa, SaO₂0.919. Chest CT showed that the middle trachea was not continuous, the soft tissue behind the vena cava was thickened, the pulmonary bronchial vascular bundle was increased and blurred, and the patchy high-density foci were seen in both upper lungs. Laryngoscopy was performed 3 days ago, and a spherical red mass was seen about 2 cm below the glottis, which originated from the posterior wall of the trachea. Transferred to our hospital for further diagnosis and treatment.