2014年 · 第52卷第03期

In order to further improve and standardize the level of analgesia and sedation treatment in pediatric intensive care unit (PICU) in China and promote the development of comfort medical strategies in PICU in China, the Emergency Group of Pediatrics Branch of Chinese Medical Association, the Pediatrics Group of Emergency Branch of Chinese Medical Association and the pediatric Professional Committee of Critical care Physicians Branch of Chinese Medical Doctors Association jointly organized the writing of expert consensus on analgesia and sedation treatment of PICU. A kick-off meeting was held in Chongqing in November 2012 and a writing group was formed. After half a year's intense work such as literature retrieval, screening, reading and writing, the writing group submitted the first draft in April 2013 to solicit the opinions of the main members of the three groups. In April 2013 (Yangshuo), June 2013 (Ordos) and September 2013 (Changsha), some experts of the histology group conducted intensive discussions, and repeatedly revised it to form an expert consensus.

A 7-year-old child went to the outpatient clinic of Nanjing Maternal and Child Health Hospital in May 2009 due to "unexplained mental retardation". The child was delivered naturally at full term, with a birth weight of 3000 g and a length of 50 cm. The Apgar score: 1 and 5 min were 8 and 9, respectively. The anterior fontanel is closed for 6 months, it will lift its head for 6 months, it will turn over for 8 months, and it will sit alone for 9 months. Unable to walk and talk independently to see a doctor, unable to take care of yourself. Recurrent respiratory infections after birth. He had his first seizure at 6 years old and was treated with phenobarbital sodium for antiepileptic treatment. Parents deny consanguinity, history of toxic drug exposure during pregnancy, and family history of mental retardation.

The child, a female, began to develop a rash 1 month after birth. The rash was scattered throughout the body, focusing on the neck and knees, and gradually increased, with skin lesions and desquamation. The rapid plasma regain (RPR) and treponema pallidum particle assay (TPPA) were both positive in the local hospital 61 days after birth, and the diagnosis of "congenital syphilis (CS)" was given penicillin treatment for 3 days, that is, 64 days after birth, and was transferred to our department. Birth history: G3P1 (pregnant mother miscarried twice), full-term natural delivery, denied history of birth injury and asphyxia, birth weight about 3.35 kg, and both parents had a history of syphilis. Admission physical examination: body temperature 37.4 ℃, heart rate 152 beats/min, breathing 42 beats/min, weight 5.7 kg, blood pressure: 72/42 mmHg (1 mmHg =0.133 kPa), clear mind, general spirit, general development and nutrition, thin hair, eyelid edema, no yellowing staining of skin and sclera, no bleeding spots, pale complexion, no swelling of superficial lymph nodes throughout the body, skin peeling of some buttocks and neck, flat anterior fontanel, no congestion in the pharynx, uniform heart rhythm, medium heart sounds, coarse breathing sounds in both lungs, no dry and wet rales, flat and soft abdomen, 3.5 cm under liver and costs, soft texture and sharp edges, not under spleen and costs, normal intestinal sounds, no abnormalities in neurological examination, no edema in both lower limbs, and negative indentation of anterior tibial finger.

In the past 40 years, remarkable achievements have been made in the treatment of childhood tumors. According to the American Cancer Institute, the 5-year survival rate of malignant tumors in children aged 0 to 14 years has increased from 58% in 1975 to 1977 to 83% in 2003 to 2009, with the survival rate of acute lymphoid leukemia (ALL), the largest tumor in children, exceeding 90%^[1]。 However, there are still many deaths every year, and tumor recurrence and treatment-related complications are the main causes. Therefore, immunotherapy is a salvage strategy for refractory and recurrent malignant tumors. Moreover, in recent years, the field of tumor immunotherapy has made rapid progress, many new technologies and methods have emerged, and clinical trials are being carried out, making it a promising choice for the treatment of childhood tumors. The clinical research progress in this field in recent years is summarized as follows.

tuberous sclerosis complex (TSC) is an autosomal dominant disease with multi-system involvement. It is caused by chromosomes 9q34 and 16p13.3TSC1OrTSC2Mutations in two tumor suppressor genes cause the activation of mammalian rapamycin target protein (mTOR) signaling pathway, and the formation of multiple organ hamartomas, including cardiac rhabdomyoma (CR), is an important feature of this disease^[1,2]。 CR is one of the important clinical features of TSC in children, and its incidence is much higher than that in adults^[1,2,3]From the fetal period to adolescence, the occurrence and development of CR presents certain characteristics and regularities. Understanding these characteristics and regularities is helpful for the clinical diagnosis of TSC in children. At the same time, understanding the influence of tumor on cardiac function, cardiac hemodynamics and electrophysiology is of great significance for the disease judgment, clinical management and prognosis evaluation of this disease. Now, this paper summarizes the current situation of CR in children with TSC.

The 18th National Conference on Children's Cardiovascular Diseases was held in Chengdu, Sichuan Province from June 18 to 21, 2013. 295 pediatric clinicians and researchers from all over the country attended the meeting, and had extensive exchanges on children's congenital heart disease (CAD), myocardial disease, Kawasaki disease, arrhythmia, heart failure, pulmonary hypertension, gas signaling molecules and syncope in children. A total of 316 academic papers were received at the conference, of which 65 were read out at the conference, 2 symposiums on cardiovascular interventional therapy, myocarditis and cardiomyopathy were held, and 21 experts were invited to give wonderful academic lectures on hot issues of everyone's concern.