2016年 · 第54卷第09期

Since the effect of non-invasive positive pressure ventilation (NPPV) was clinically confirmed in the 1970s, its clinical use has gradually increased, especially in the past 10 years. The use of NPPV in pediatric intensive care units is increasingly prevalent in some developed countries^[1,2]It is also well used in children with chronic respiratory failure, and many are carried out in families^[3]。 In China, the use of NPPV in adults is more common, but the popularity of pediatric use varies greatly from place to place. Children's use of NPPV in families is still in its infancy.

Although the pathogenesis and clinical diagnosis and treatment of acute respiratory distress syndrome (PARDS) in children have been extensively studied in the past 40 years, it is still one of the diseases with high clinical mortality in pediatric intensive care unit (PICU). At the same time, there is a lack of effective treatment methods, and there are still too many clinical unknowns in the diagnosis and treatment of PARDS to be explored. Therefore, the research on the pathogenesis, early diagnosis and treatment of PARDS is still the main direction of pediatric critical care medicine.

Rare diseases, as the name implies, refer to uncommon diseases. What is rare is defined by different countries in the world, and different "definitions" of rare diseases may be given according to the population, medical insurance affordability, epidemiological characteristics of diseases, etc. of each country. For example, the United States defines as a rare disease any disease with fewer than 200,000 people affected nationally; The "threshold" for rare diseases in Japan is diseases with less than 50,000 patients or a prevalence of less than 1/2 500; The EU considers rare diseases to be chronic, fatal, requiring special efforts to develop treatments, and with a prevalence of 1 in 2 000^[1]。 Although there are different definitions of rare diseases from country to country, overall rare diseases include between 6,000 and 8,000 completely different diseases, of which about 80% have established genetic factors and about 50% of rare diseases occur in childhood. Rare diseases, also known as orphan diseases, aim to emphasize the severity of the disease, insufficient medical resources, lack of relevant knowledge, insufficient cognition, and the need for special conditions to develop new drugs.

noninvasive continuous positive airway pressure (NCPAP) is a ventilation mode that provides a certain pressure level through nasal congestion or mask under spontaneous breathing conditions, so that the airway maintains positive pressure throughout the whole respiratory cycle. It does not need to establish invasive artificial airway, so it can relieve children's pain and reduce complications of invasive ventilation. At present, it has become a commonly used auxiliary ventilation technology in clinic. In order to further promote and standardize the clinical application of NCPAP in children in China, improve the level of respiratory support, and reduce adverse reactions and complications, the Emergency Group of Pediatrics Branch of Chinese Medical Association, the Pediatrics Group of Emergency Medicine Branch of Chinese Medical Association and the Children's Critical Care Physician Branch of Chinese Medical Doctors Association referred to the existing guidelines and clinical research results at home and abroad and searched PubMed, Medline and related Chinese databases (Tsinghua Tongfang, Wanfang Database and VIP Chinese Science and Technology Journal Database), with the retrieval date as of May 2016, and formulated this expert consensus. Because newborns have their own characteristics, this expert consensus applies only to pediatric patients other than newborns.

The child was a male, 17 days after birth, with a gestational age of 40^＋Chow. In February 2016, I went to the local hospital due to "perioral cyanosis, dyspnea for 2 weeks, aggravated for 1 day". Physical examination at admission: blood pressure (50~60) / (25~35) mmHg (1 mmHg =0.133 kPa), percutaneous oxygen saturation 0.50~0.70, arterial blood gas analysis: pH 6.80, carbon dioxide partial pressure (PCO₂) 59 mmHg, oxygen partial pressure (PO₂) 37 mmHg, sodium (Na) 137 mmol/L, potassium (K) 5.9 mmol/L, calcium (Ca) 1.52 mmol/L, blood glucose 3.4 mmol/L, lactic acid>15 mmol/L, hematocrit (Hct) 0.45, bicarbonate (HCO₃^-) was not detected, and the residual alkali was not detected. Cardiac color ultrasound confirmed the diagnosis of "complete transposition of large arteries (intact ventricular septum)", and mechanical ventilation and anti-shock treatment were given at the same time to contact Bayi Children's Hospital. Consider the diagnosis of "complete transposition of large arteries (intact ventricular septum) and heart failure". The primary disease required emergency surgery. Since the local hospital could not perform the surgery, extracorporeal membrane oxygenation (ECMO) was decided to be transported. The head of the ECMO team convened a transport team (including 2 pediatric cardiovascular surgeons, 1 pediatric intensive care physician, 1 pediatric cardiopulmonary bypass physician, 1 nurse and 1 driver). Before departure, prepare and count items according to the checklist in the newborn ECMO transfer plan. ECMO equipment is the US Medtronic Bio-Console 560 centrifugal pump system, and consumables are Medtronic children's kits.

Child male, Iraqi nationality, first birth, first birth, 37 years pregnant^＋3Zhou gave birth naturally, with a birth weight of 2 850 g. He was transferred to the Children's Hospital affiliated to Zhejiang University School of Medicine in 2014 because of "abdominal mass found 5 h after birth". Abdominal CT revealed meconium peritonitis with encapsulated fluid pneumoperitoneum. After improving all preoperative preparations, exploratory laparotomy was performed on the second day after birth. During the operation, necrosis and perforation of the small intestine was found about 25 cm away from the Trigger's ligament, and the distal small intestine adhered into a mass. High proximal small intestinal fistula + abdominal drainage was performed. On 14 and 40 days after birth, he underwent two reoperations due to abdominal incision necrosis and infection. Finally, all small intestine and colon were found to be necrotic, only the horizontal part of the duodenum was left, where a duodenal drainage tube was placed. Final postoperative diagnosis: after meconium peritonitis, after necroctomy of small intestine and colon (without intestine), after duodenal drainage tube placement (Figure 1、Figure 2)。

Hyperuricemia is an ancient and common metabolic disease. Gout caused by Hyperuricemia mainly occurs in adults, but Hyperuricemia without gout is also very common in children. Bo Hui et al.^[1]A survey of blood uric acid levels of 1 515 school-age children in Tianjin showed that 12.94% of children had hyperuricemia, of which 19.57% were boys and 5.67% were girls. At the same time, more and more studies have shown that hyperuricemia is closely related to hypertension, cardiovascular diseases, metabolic syndrome, kidney diseases, diabetes, etc. However, people have relatively few studies on childhood hyperuricemia and its related kidney damage, and their understanding needs to be updated urgently.

The Bayley scales of infant and toddler development-third edition (Bayley-Ⅲ) is a diagnostic developmental scale for infants and young children between 1 and 42 months of age and takes 50 to 90 minutes. Specific contents include cognitive scale, language scale, action scale and caregiver questionnaire, among which caregiver questionnaire is used to evaluate social-emotional and adaptive behaviors of infants and young children. At present, Bayley-Ⅲ has been widely used in clinical evaluation and research internationally. Its evolution process and the application of Bayley-Ⅲ in infant development evaluation in clinical and scientific research in recent years are summarized as follows.

Congenital heart disease is a common congenital malformation with an incidence of about 8/1 000. About 20% of congenital heart diseases may have abnormalities of right ventricular outflow tract (RVOT) and/or pulmonary valve, such as pulmonary atresia, tetralogy of Fallot, common truncus arteriosus, misalignment of large arteries, etc. These children usually undergo surgical intervention at an early stage. Treatment of RVOT is typically reconstruction with valved tubes or patch enlargement^[1]。 RVOT obstruction and/or pulmonary regurgitation (PR) can occur as the child grows and develops and the tube and valve progressively degenerate. The former can cause an increase in right ventricular pressure load, and the latter can cause an increase in right ventricular volume load. Although the right ventricle can usually tolerate this change for a long time, with the progressive increase in pressure and volume load, the right ventricle can develop progressive enlargement, which affects right ventricular function and leads to decreased exercise capacity, atrial and/or ventricular arrhythmias, and even sudden death in children. Therefore, it is very necessary to treat RVOT and PR to restore right ventricular function. The management of RVOT obstruction and PR is mainly re-surgical placement of a valved tube. However, since the first transcatheter pulmonary valve replacement (TPVR) was reported in 2000, more than 6 000 cases of TPVR have been reported^[1]It has good short-to-medium-term results and is expected to replace resurgery. There are two main types of TPV in clinical use: Melody valve (Medtronic, USA) and Edwards valve (Edwards Lifescience, USA). Among them, Melody valve has been approved by the US Food and Drug Administration (FDA) for clinical use, and Edwards valve is in clinical trial stage. However, there are relatively few reports of TPVR in children. This paper introduces the timing and indications of surgery which are closely related to TPVR in children.

The pediatric clinical study took minors as the subjects. Minors do not have full capacity for civil conduct, cannot carry out civil activities independently, and must be under the supervision of a guardian or legal representative before they can participate in clinical research. Therefore, the informed consent form of minors to participate in clinical research must be signed by the guardian or legal representative. From what age is it appropriate for a minor to sign an informed consent form? This problem has strong practicality, but there is no uniform regulation at home and abroad. It is generally considered that minors under the age of 7 have poor cognitive level and have no informed consent ability; The cognitive level of 7-10 years old is still immature, but they already have basic informed consent ability, and they can probably understand questions such as "what to do with this research", "what I need to do", "will participation in the research hurt me", "will participation in the research be useful for me", "do I have to participate in this research", "can I withdraw at any time if I don't want to participate", etc.; 11~16 years old have strong cognitive ability, but are not fully mature, and can probably understand the research design scheme and research process of non-professional language description.