2014年 · 第52卷第02期

aplastic anemia (AA) is a bone marrow failure disease characterized by decreased proliferation of bone marrow nucleated cells and peripheral pancytopenia. It is divided into two categories: hereditary and acquired. Hereditary bone marrow failure includes Fanconi anemia, congenital dyskeratosis, etc. There is no data on the incidence of this group of diseases in China. Acquired AA is divided into secondary acquired AA and idiopathic acquired AA according to the presence or absence of induced cause. Idiopathic acquired AA is the most common among acquired bone marrow failure diseases, and the incidence rate in China is 7.4/10⁶, higher than western countries^[1,2]It is one of the major blood diseases that seriously threaten children's health. Suggestions on Diagnosis and Treatment of Aplastic Anemia in Children jointly formulated by the Hematology Group of Pediatrics Branch of Chinese Medical Association and the Editorial Committee of Chinese Journal of Pediatrics^[3]It has been published for more than ten years. During this period, the international research on bone marrow failure diseases has been deepened, and many new discoveries have been made. Based on the research progress in this field^[3,4,5]We put forward the following new thoughts on the standardized diagnosis and treatment of AA in children.

aplastic anemia (AA) is a group of bone marrow failure diseases characterized by decreased proliferation of bone marrow nucleated cells and peripheral pancytopenia^[1,2,3]。 According to the international research progress in the field of bone marrow failure syndrome^[4]Combined with the actual situation of clinical diagnosis and treatment of AA in children in China^[5,6]The Hematology Group of the Pediatrics Branch of the Chinese Medical Association and the Editorial Committee of the Chinese Journal of Pediatrics organized relevant experts to formulate this edition of recommendations to standardize the diagnosis and treatment of acquired AA in children in China and improve the diagnosis and treatment level.

A 3-year-old male was admitted to the hospital for 5 days due to abnormal chest X-ray films found during physical examination. Two weeks before admission, a teacher in the kindergarten where the child was located was diagnosed with "pulmonary tuberculosis (cavitary type)", and the child had a history of close contact with him. Therefore, the local PPD examination 10 days before admission showed strong positive, and the chest X-ray examination in the local hospital 5 days before admission showed abnormality. The child occasionally has cough, not severe, no hemoptysis, no fever, no wheezing, no shortness of breath, no night sweats, no headache, no vomiting, diarrhea, no frequent urination, urgency, painful urination, acceptable stomach appetite, and no gradual weight loss. Then I went to our hospital, and the chest CT examination also showed abnormality. For further diagnosis and treatment, the outpatient clinic plans to admit "primary pulmonary tuberculosis to be discharged" to the pediatric respiratory department of our hospital.

Critically ill children often develop cardiovascular dysfunction in the early stage, but due to the lack of specific symptoms and objective indicators reflecting cardiovascular dysfunction, the early recognition of cardiovascular dysfunction is delayed, which leads to the occurrence of sequential multiple organ dysfunction. Serum N-terminal pro-brain natriuretic peptide (NT-pro BNP) has been widely used in the detection of heart function and the diagnosis of heart failure (HF)^[1]。 In recent years, more and more studies have confirmed that^[2,3]NT-pro BNP has exceeded the previous scope of being only a biomarker for the diagnosis of HF, and has wide application potential in the assessment, prognosis judgment and treatment guidance of early cardiovascular dysfunction in critically ill children.

Although modern medical technology has made great progress, malnutrition among hospitalized children, especially those admitted to pediatric intensive care unit (PICU), is widespread worldwide^[1]。 Studies show that 24% of children with acute or chronic malnutrition at the time of admission to hospital or PICU in developed countries^[2,3]While developing countries can reach 53 percent^[4]In addition, during hospitalization, the children's nutritional status is further deteriorated due to various factors, resulting in complications, infections, and increased mortality^[1,5]。 Therefore, nutritional risk screening and nutritional assessment of critically ill children during hospitalization are crucial. According to the results of nutritional risk screening and assessment, appropriate macro and micronutrient support treatment can improve the disease prognosis and avoid malnutrition or further nutritional deterioration. At present, the clinical application guidelines of pediatric enteral and parenteral nutrition support both at home and abroad propose that routine nutritional risk screening should be carried out for hospitalized children, so as to detect malnutrition and children at risk of malnutrition in time^[6,7,8]。 Further nutritional assessment is required when nutritional risk screening identifies possible nutritional problems. The methods and significance of nutritional risk screening and nutritional assessment in critically ill children are reviewed.

Gastro-esophageal reflux disease (GERD) refers to the reflux of stomach contents into the esophagus or even the oral cavity, causing a series of clinical syndromes with vomiting and acid reflux as typical esophageal symptoms and/or cough and asthma as main extraesophageal symptoms. Gastro-esophageal reflux cough (GERC) is a respiratory syndrome associated with GER with cough as the main manifestation. GERC is considered to be one of the three major causes of chronic cough, accounting for 10% to 41% of children with chronic cough^[1,2,3]。 Epidemiological investigation shows that the positive rate of GER is 30% ~80% in children with asthma, and 40% in children with chronic cough^[4]。 We performed 24-h esophageal pH monitoring in 4 048 children with reflux-related symptoms, and 2 171 cases were diagnosed with GER, with a positive rate of 53.6%; The detection rates of GER in children with recurrent bronchopneumonia, chronic cough, persistent pneumonia, bronchial asthma, non-cardiac chest pain and apnea were 58.4%, 53.8%, 44.8%, 34.7%, 56.4% and 48.0%, respectively^[5]。 The pathogenesis of GERC can be summarized as three main points: gastric acid micro-inhalation, esophageal-bronchial nerve reflex and airway hyperresponsiveness^[6,7,8]。 Micro-inhalation lacks evidence-based medicine in most children^[6]； Reflex theory believes that gastric acid reflux to the lower esophagus, causing neurogenic inflammation of the airway through vagus nerve or other nerve-mediated reflexes, inducing cough or aggravating asthma symptoms; Airway hyperresponsiveness is also believed to be related to airway neurogenic inflammation. The research progress of the pathogenesis of GERC is summarized as follows.