2011年 · 第49卷第03期

Childhood non-Hodgkin lymphomas (NHL) are an umbrella term for a range of diseases originating from organs and cells of the lymphatic system, including all malignant lymphomas not classified as Hodgkin's disease. North America<The annual incidence of NHL in 15-year-old children is 8.3 per million. The statistical results of Shanghai Cancer Registry from 1992 to 1996 showed that the incidence of NHL in children aged 0-14 was 8.94/million, second only to leukemia and intracranial tumors.

Hemophilia is a group of hereditary hemorrhagic disorders, which are X-linked recessive inheritance. It is clinically divided into two types: hemophilia A (coagulation factor VIII deficiency) and hemophilia B (coagulation factor IX deficiency). It is clinically characterized by spontaneous or minor trauma of joints, muscles, internal organs and deep tissues, which is difficult to stop bleeding, often onset in childhood. It is very important for pediatrics to identify, diagnose and treat hemophilia actively and reasonably.

Hemophilia is a recessive hemorrhagic disease lacking coagulation factor VIII or factor IX linked to sex chromosomes. Large-scale epidemiological investigations have shown that the incidence of hemophilia is (15-20) per 100,000 population, without significant regional and ethnic differences^[1]China has a population of 1.3 billion, and it is estimated that the number of patients is huge. However, as of 2007, there were only more than 8,000 registered hemophilia patients in China, so the proportion of misdiagnosis and missed diagnosis of hemophilia in China may be high at present. According to reports, the first hemorrhage of children with hemophilia in China is mostly about 1 year old, but the initial diagnosis age is about 5 years old^[2,3], significantly later than the age at which symptoms first appeared. This delay in diagnosis may be associated with a low incidence of hemophilia, insufficient awareness of the disease by many physicians, and limited diagnostic conditions in primary hospitals. However, the delay of diagnosis inevitably leads to the delay of treatment, which is closely related to the occurrence of joint deformity. Therefore, clinicians' early diagnosis and treatment is of great significance to reduce the complications of hemophilia. For a long time, there has been a lack of guidelines for the diagnosis and treatment of children's hemophilia in China. Under this background, the Hematology Group of Pediatrics Branch of Chinese Medical Association and the Children's Group/Preventive Treatment Group of Hemostatic Thrombosis Group of Chinese Medical Association Hematology Branch jointly drafted the Suggestions for the Diagnosis and Treatment of Children's Hemophilia (hereinafter referred to as the Suggestions), in order to standardize the treatment and provide a reference for clinicians based on the experience of hemophilia diagnosis and treatment at home and abroad.

The 15th National Conference on Pediatric Hematology, co-sponsored by the Hematology Group of the Pediatrics Branch of the Chinese Medical Association and the Editorial Committee of the Chinese Journal of Pediatrics, was held in Guiyang, Guizhou Province from September 16 to 18, 2010. More than 200 delegates from all provinces, municipalities and autonomous regions attended the conference. The conference received 255 manuscripts, including 8 special lectures, 8 special speeches and 44 speeches at the conference. Different from previous sessions, this conference created three "firsts": the first time focused on non-neoplastic diseases; For the first time, foreign guests were invited to give a lecture; For the first time, an award session for outstanding papers was added. In addition, the conference also opened a special speech section, and made special reports on the research results that have been systematically researched for many years and published in authoritative journals at home and abroad, which achieved good results.

The 14th National Congress of Pediatric Nephrology was successfully held in Wuhan, Hubei Province on October 12-15, 2010. 316 delegates from all over the country attended the conference, the largest number of participants ever. A total of 273 submissions were received at the conference, with 8 themes, 8 expert lectures and 43 conference speeches exchanged at the conference. The venue was packed and the atmosphere of discussion was lively. The participants had full discussions and exchanges around primary glomerular disease, secondary kidney disease, hereditary disease and basic research.

The 26th International Congress of Pediatrics was successfully held in Johannesburg, South Africa, from August 5 to 9, 2010. More than 1,000 pediatricians and pediatric researchers from more than 50 countries attended the event. This is the first time that the World Congress of Pediatrics has been held in Africa, and it is also in the final stages of achieving the Millennium Development Goals (MDGs). Duration 5 d, schedule 1 d for workshop time. The opening ceremony was very grand, and the banquet for the 100th anniversary of the birth of the International Academy of Pediatrics (IPA) was held. A total of 1,377 papers were received at the conference, including 16 lectures, 257 symposiums, 36 oral speeches, 62 workshops, 194 Poster walk sessions and 812 posters. The lectures and oral speeches were clinically important issues, while basic research was discussed and exchanged in the form of posters. Nearly 100 representatives of pediatrics in China attended this conference, and 43 units (including Hong Kong and Macao Special Administrative Regions and Taiwan Province) submitted 373 articles, including 17 special lectures (accounting for 6.6%), 6 oral speeches (accounting for 16.67%), 2 special discussions (accounting for 3.23%), 43 poster discussions (accounting for 22.16%) and 305 posters (accounting for 22.14%).

The child, 11 years old and 5 months old, was admitted to the hospital with multiple pain throughout the body for 9 months and claudication for 4 months. The child first experienced knee pain, and then experienced multiple pain in the lower back, chest and ribs, and hip, accompanied by claudication. Weight loss, no polydipsia and polyuria. Physical examination: T 37.3 ℃, 20 breaths/min, 78 pulses/min, blood pressure 104/76 mm Hg (1 mm Hg =0.133 kPa). Clearness, no rash and jaundice, small superficial lymph nodes, no skull defect, no protrusion of both eyeballs, and no abnormalities in heart, lungs and abdomen. The upper body and pelvis are inclined to the left side, and they are lame. There is no redness, swelling, heat or damage on the skin of the whole body. There was no tenderness and percussion pain in the spinous processes of the cervical, thoracic and lumbar vertebrae, and tenderness in the sacrococcygeal region. The lumbar vertebrae was flexed forward by 40°, the left and right sides were bent by about 20°, and the left and right sides were rotated by 30°. The left hip was painful when squatting. The heel percussion test was negative, the left 4-character sign was positive, the bilateral straight leg elevation reinforcement test was negative, the Allis sign and Thomas sign were both negative, and the hip extension test was negative. The muscle strength and tone of the limbs were normal, the deep and superficial reflexes were normal, and the pathological signs were negative. The sensation and blood supply of the limbs are normal. Experimental examination: white blood cells 7.8×10⁹/L, neutrophils 0.56, lymphocytes 0.29, hemoglobin 108 g/L, platelets 324×10⁹/L. Erythrocyte sedimentation rate (ESR) was 38 mm/1 h, C-reactive protein (CRP) was 22.8 mg/L, lactate dehydrogenase (LDH) was 184.4 U/L, and there were no abnormalities in liver and kidney function, myocardial enzymes, electrolyte and coagulation. X-ray examination: There were no abnormalities in the lungs, flattening of the thoracic 2 and thoracic 10 vertebrae, reduced density of the lumbar 3, 5 and sacral 2 vertebrae, osteolytic bone destruction of the right iliac bone, and normal spaces of bilateral sacroiliac and hip joints. After admission, the right iliac bone lesion incision biopsy was performed under general anesthesia by C-arm machine positioning. During the operation, yellow granulomatous lesion tissue was seen. Under the pathological microscope, the bone marrow was obviously fibrotic with more foam cells (xanthoma cell-like), accompanied by a little lymphocyte infiltration, no eosinophilia, no obvious inflammation, and no malignant morphological cells (Figure 1，Figure 2，Figure 3)。 Immunohistochemistry: CD68 (+ +) (Figure 4), AACT (+ +), CD1a (-), S100 (-) (Figure 5)。 Diagnosis: Erdheim-Chester disease. Parent gives up treatment.

Dr. Zhou Juan(400014 Renal Immunity, Children's Hospital affiliated to Chongqing Medical University) Q:

The incidence of malignant tumors in children aged 0 to 15 years only accounts for a small fraction (about 2%) of all tumor patients. However, among the causes of childhood death, malignant tumors account for 10%, which is one of the main fatal diseases in children. With the continuous progress of medical science, more than 70% of children with malignant tumors have obtained long-term survival in developed countries. Now, the research progress of common malignant tumors in children is discussed with domestic colleagues in combination with several problems existing in clinical practice in China.

minimal residual disease (MRD) is an important prognostic index of acute lymphoblastic leukemia (ALL) in children. At present, it is generally believed that MRD detection has important guiding significance in evaluating treatment effect, judging prognosis and implementing individualized treatment^[1,2]。 If MRD is negative at the end of induction remission therapy, the prognosis of the child is excellent, and studies have explored the possibility of reducing the intensity of chemotherapy; Children with high MRD levels at this time, even at the time of consolidation therapy, have a poorer prognosis and need more intense chemotherapy or even hematopoietic stem cell transplantation.