2011年 · 第49卷第11期

Child, female, 3 h, G₂P₂, 41 weeks gestational age, fetal peritoneal effusion was found during abdominal ultrasound of pregnancy test for cesarean section, normal amniotic fluid, birth weight 3800 g, Apgar score 10 min 10 points. Non-coagulating fluid was extracted by abdominal puncture, and abdominal CT showed spleen rupture. Transferred to our hospital, physical examination: T 35.8 ℃, BP 80/50 mm Hg (1 mm Hg =0.133 kPa), mental reaction OK, pale complexion, stable breathing, no rash, bleeding spots and ecchymosis on the skin and mucous membranes of the whole body, flat and soft fontanel, normal heart and lung, abdominal swelling, good movement of limbs, and normal muscle tone. Blood routine: white blood cells 19.25×10⁹/L, hemoglobin 119 g/L, platelets 36×10⁹/L, coagulation routine: plasma prothrombin time 1.3 s, thrombin time 23 s, activated partial thromboplastin time 49.6 s, plasma fibrinogen 1.49 g/L, antithrombin III (activity) 74.2%, blood gas, electrolyte and blood culture were normal. Diagnosis: Spleen rupture (cause to be investigated). After fasting, sedation, transfusion of washed red blood cells, plasma and platelets, vitamin K1, etc. for two weeks, there was no obvious recovery of platelets. Bone marrow smear: about 100 megakaryocytes in whole tablet, rare platelets, active nucleated cell proliferation, granule: red ratio =5.6:1. Color ultrasound of the spleen showed that the peritoneal effusion disappeared, the size of the spleen was 73 mm ×24 mm, and the internal echo was not uniform, among which a medium echo area of about 36 mm ×22 mm was seen. Color Doppler ultrasound showed that there was blood flow inside. Abdominal CT + enhancement + delay: the spleen was significantly enlarged, and the enhancement was uneven, with multiple patchy low-enhancement foci. After 5 min delay, the original low-density foci were further slowly enhanced. Confirmed diagnosis: hemangioma of the spleen with thrombocytopenia syndrome. Hormone therapy was performed, dexamethasone 1 mg/ (kg·d) once a day for 3 days, and the platelet count gradually became normal. Prednisone was changed to oral administration of 2.5 mg twice a day, and the oral administration was reduced for 1 month. The outpatient follow-up was 1 year, and the growth, development and platelet count were normal.

As early as 1820, Bright had pointed out that proteinuria was related to kidney diseases. In the past 200 years, proteinuria has always been the focus of clinical and related basic research. At present, scholars at home and abroad have reached a consensus on the following views, that is, proteinuria is an important marker of kidney injury; Persistent proteinuria can lead to glomerular sclerosis and tubulointerstitial damage; Is an independent risk factor for sustained progression to end-stage kidney disease (ESRD); Early reduction of proteinuria can delay the progression of the disease^[1]。 The forefronts symposium held by the International Society of Nephrology (ISN) is titled proteinuria. It is worth noting that its slogan is "from glomerular filtration to tubular handling", which is undoubtedly a timely and useful enlightenment for our comprehensive understanding of proteinuria. For many years, glomerular proteinuria, which reflects the damage of renal parenchyma, has been mainly paid attention to proteinuria, while the role and significance of renal tubules and interstitium in the occurrence and development of proteinuria has been less paid attention to. Combined with the current situation of clinical diagnosis and treatment of pediatric nephropathy in China, this paper summarizes how to comprehensively understand proteinuria, in order to improve the diagnosis and treatment level.

acute leukemia (AL) is the most common malignant tumor in childhood, accounting for about 30% of all malignant tumors in childhood. AL is a hematopoietic malignant tumor. Coupled with various factors such as intense chemotherapy and radiotherapy, the nonspecific barrier function and specific immune function of children are impaired, and the risk of infection with various pathogenic microorganisms is significantly increased. Some children even die of severe infection.

As more and more children recover from the primary disease, the study of immune recovery and vaccination protocol for children with acute leukemia has become more and more important. Missing out on vaccination or needing a booster immunization is a matter of great concern to parents of children. We agree with this article that children with solid tumors have a more complete immune system recovery than children with hematological malignancies. Generally, immune labeling will recover 6 to 12 months after stopping chemotherapy. However, in our study, 18 months after stopping chemotherapy, CD4^＋and CD8^＋The number of cells remains below the normal range for a particular age^[1,2]And many subjects have lost a variety of protective antibodies that can prevent infectious diseases. Hepatitis B, measles, mumps and rubella affect more than whooping cough, diphtheria and tetanus.

The article "Clinical Characteristics and Long-term Follow-up Analysis of Three Cases of Neonatal Aristolochic Acid Nephropathy" is very valuable for reporting the results of clinical follow-up studies that lasted for 12 years. It seems that clinicians have accomplished their mission to actively diagnose and treat the disease in the acute stage or during hospitalization or consultation. However, long-term follow-up is often needed to understand and grasp the occurrence, development and prognosis of the disease. The follow-up of the disease not only helps to detect the changes of the disease in time, provide corresponding treatment and intervention, but also helps to manage the disease in the whole process; At the same time, it is also a necessary clinical research means to improve the understanding of the occurrence, development and even the nature of disease through the analysis of disease outcome.

primary nephrotic syndrome (PNS) accounts for about 90% of the total number of NS in childhood, and is a common glomerular disease in children. The annual incidence rate of PNS in children reported abroad is about 16/100,000^[1]Although there is still a lack of similar reports in China, the statistical data of the survey reports of hospitalized children in some provincial and municipal hospitals in 1982 and 1992 show that PNS accounts for about 21% and 31% of hospitalized children with urinary diseases respectively, and the incidence of PNS seems to be increasing^[2]。

primary nephrotic syndrome (PNS) is a common glomerular disease in children. 80% ~90% of children with PNS are sensitive to hormones and have good prognosis; 10% ~20% of children with PNS are hormone-resistant, that is, steroid-resistant nephrotic syndrome (SRNS), with poor prognosis^[1,2]。 30% to 40% of children with SRNS progress to end stage renal disease (ESRD) within 10 years^[3]。 According to the age of onset, SRNS are divided into three types: ① congenital nephrotic syndrome (CNS): onset within 3 months after birth and resistance to hormones; ②childhood SRNS (childhood-onset steroid-resistant nephrotic syndrome): onset from 3 months to 18 years old; ③ adult-onset steroid-resistant nephrotic syndrome (SRNS): onset after 18 years of age^[4,5]。 Early studies^[6]It was found that congenital nephrotic syndrome of Finland (CNF) was caused byNPHS1Caused by genetic mutations. Subsequent studies found detection in children with CNS in countries other than FinlandNPHS1Gene mutation^[7,8]。 Recent studies^[4,5]ConfirmNPHS1In addition to causing CNS, gene mutations can also cause SRNS in childhood and SRNS in adulthood.

Because of the heavy burden caused by respiratory syncytial virus (RSV) infection and the mortality rate of high-risk children, the prevention and treatment of RSV infection have been paid great attention. Despite this, no safe and effective vaccine has been available so far, and the anti-RSV monoclonal antibody Palivizumab is currently the only proven effective preventive drug, which can be used for the prevention of high-risk children. The treatment of RSV infection is still mainly based on supportive therapy, including keeping the respiratory tract open, inhaling oxygen, correcting dehydration and relieving dyspnea. Although bronchodilators, glucocorticoids and antiviral drugs are widely used in clinic, their efficacy has not been determined. The following introduces the research progress in the field of RSV infection prevention and treatment at home and abroad in recent years.

Syphilis is a sexually transmitted disease caused by treponema pallidum (TP). It can enter the fetal blood circulation through the placenta of pregnant women, causing miscarriage, stillbirth or systemic infection of newborns, that is, congenital syphilis, also known as fetal syphilis. At present, more than 1 million babies worldwide are born with fetal syphilis^[1]The number of fetuses and newborns killed by syphilis worldwide is conservatively estimated at more than 500,000^[2]。 In China, according to the statistics of China Syphilis Prevention and Control Plan (2010-2020), 109 cases of congenital syphilis were reported in 1997, with a reported incidence of 0.53/100,000 live births; In 2009, the number of reported cases was 10,757, with a reported incidence rate of 64.41 per 100,000 live births, representing an average annual increase of 49.2%^[3]。 It is harmful and has become a serious public health problem all over the world.

Worldwide, the incidence of type 1 diabetes among preschool children increases by 5 percent annually, compared with 3 percent annually among children and adolescents^[1]。 According to statistics, about 70,000 children under the age of 14 develop type 1 diabetes every year. With changes in eating habits and lifestyle, the number of children and adolescents with type 2 diabetes is also increasing significantly. Among children diagnosed with diabetes for the first time in the United States, 8% to 43% have type 2 diabetes^[2]。 If the blood sugar control of diabetic patients is not good, it can cause a variety of acute and chronic complications, which seriously threaten the health of patients. At present, some retrospective studies have shown that more diabetic children have glycosylated hemoglobin (HbA_1c) Level not up to standard^[3]Up to 50% of the children developed various complications and comorbidities in the course of the disease around 12 years^[1]。 A 30-year Diabetes Control and Complications Study (DCCT trial) and its subsequent Diabetes Intervention and Complications Epidemiology Study (EDIC trial) in adults demonstrated that intensive therapy including multiple daily injections (MDI) and continuous subcutaneous insulin infusion (CSII) can reduce or delay complications in diabetic patients^[4]。 This conclusion applies equally to children and adolescents with diabetes. The International Society for Pediatric and Adolescent Diabetes (ISPAD) has repeatedly pointed out the importance of intensive Diabetes treatment in its many published and continuously updated guidelines for the diagnosis and treatment of Diabetes in children and adolescents. At present, paediatric endocrinologists have generally accepted intensive insulin therapy as the first choice for type 1 diabetes.

The 15th IPNA Congress, sponsored by the International Society of Pediatric Nephrology (IPNA), was successfully held in New York, USA, from August 29 to September 2, 2010. More than 1,300 delegates from 89 countries and regions attended the event. There were 22 representatives from Mainland China, from Beijing, Shanghai, Guangdong and Fujian. Professor Ding Jie of Peking University First Hospital gave a keynote speech on "Epidemiological study of melamine-related urinary stones in children" in the symposium on "World Kidney Diseases", Professor Xu Hong of Shanghai Fudan University Pediatric Hospital presided over the latest progress of the plenary session on "Uric acid and hypertension", and Professor Zhao Mengzhun of Hong Kong Princess Margaret Hospital presided over the symposium on "Chronic dialysis".

In recent years, clinical multicenter study (multicenter study) has received more and more attention. Not only are researchers enthusiastic, planned and acting, but the project support of many funds also clearly (or tendently) supports multicenter research. It seems that clinical multicenter research in China is on the rise. International and domestic medical experts have noticed the advantage of the pathogen on the basis of China's large population, and it seems that our clinical multi-center research has come naturally. However, truly applying many central research methods and methods, obtaining objective, accurate and guiding research results for clinical practice, and ensuring the effective investment of manpower, material resources and financial resources, we still need to think calmly, and there is still a long way to go.