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1.
??Abstract?? Systemic vasculitis is a group of vasculitis with common inflammation of the blood vessel and involves multiple organs. It can involve all kinds of vessels of the organs??showing different clinical manifestations and lacking specificity. Through summarizing the clinical manifestation and diagnosis of some common systemic vasculitis in children??we aim to improve pediatricians’s awareness of these diseases??in order to early discover??diagnose and improve the prognosis.  相似文献   
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??Objective??To analyze the clinical and gene mutation characteristics of Duchenne progressive muscular dystrophy ??DMD????summarize the gene mutation hotspots in 97 cases and to explore the correlation between clinical manifestations and genotype. Methods??Totally 97 patients with DMD diagnosed by genetic examination from January 2014 to 2018 were collected and analyzed. The clinical manifestations??serum analyses and gene mutation results were analyzed. Results??The main clinical manifestations of 97 patients??96 boys?? were feeding difficulties?? increased muscle enzyme and limb weakness. Creatine kinase??CK???? lactate dehydrogenase??LDH?? and aspartate aminotransferase??AST?? muscle enzymes were significantly increased. By combining deep-sequencing technologies??the large deletions of DMD gene mutation was in 62 cases??63.92%????there were 11 cases??11.34%?? of large duplication mutation??and 24 cases??24.74%?? of point mutation. All of the mutations could occur in any position in the DMD gene??but there were two hot spots??45 cases were located in the central region gene exon 45??55??72.58% ????12 cases of deletion mutation were located in 5’exon end exon 2??19 area??19.35%??. Conclusion??The main clinical manifestations of the DMD children are feeding difficulty??increased muscle enzyme and limb weakness. The patients with significantly increased muscle enzyme should receive a timely defection of DMD gene.  相似文献   
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??Objective??To investigate the efficacy and safety of propofol used as anesthesia and deep sedation during flexible bronchoscopy in children. Methods??The clinical data of 206 children with atelectasis who underwent flexible bronchoscopic alveolar lavage in the Endoscopy Room of the Respiratory Department of Hebei Children’s Hospital from January 2016 to January 2017 were retrospectively analyzed. Children for ASA??/?? level were divided into two groups according to the sedation method :there were 106 cases in the propofol group??2 mg/kg?? and 100 cases in the midazolam group??0.1 mg/kg??. To compare the onset time of anesthetict??heart rate??HR????respiratory rate??RR????mean arterial pressure??MAP????percutaneous oxygen saturation??SPO2?? before and after anesthesia induction??T0??T1????during endoscopy placement??T2??and after awakening??T3?? at 4 time points??operative duration??the waked duration of postoperation??the rate of adverse reactions??hiccups??respiratory depression??in the operation and Ramsay score between two groups. All data were analyzed by SPSS 20.0 statistical software. Results????1??There were statistically significant differences at the onset time of anestheticts??operative duration and the rate of side-effects between the two groups??P??0.05??. There was no statistical differences in awake time??P??0.05??.??2??There was no significant difference in MAP or SPO2 at time points of T0??T1??T2??T3??P??0.05????whereas the difference in HR and RR at time points of T1??T2??T3 between the two groups was statistically significant??P??0.05??. ??3??The sedative effect of propofol group was significantly better than that of midazolam group in Ramsay sedative scoring. Conclusion??Propofol used for anesthesia and deep sedation works fast??safely and effectively in flexible bronchoscopy for children??the time to gain consciousness is short??the operation time is short and there is fewer side effects??which is worth promoting.  相似文献   
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??Pulmonary surfactant??PS?? is one of the treatments for acute respiratory distress syndrome??ARDS????but the curative effect is still unsure. The results of current studies indicate that different kinds??doses and delivery methods of PS may affect the outcomes. This paper makes a review of the current status of PS in the treatment of ARDS.  相似文献   
6.
??Airway mucus hypersecretion is an important feature of chronic respiratory diseases. Viral infection is a common factor inducing airway mucus hypersecretion. Understanding the relationship between the viral infection and airway mucus hypersecretion and mechanism is very important to preventing and reducing airway mucus hypersecretion??decreasing the mortality of chronic respiratory diseases. The increase of MUC5AC expression is the major pathological basis of airway mucus hypersecretion??so it is necessary to discuss the signal transduction pathway of MUC5AC secretion caused by common respiratory virus infection.  相似文献   
7.
??Objective??To study the clinical features of childhood acute lymphoblastic leukemia??ALL?? with secondary myelofibrosis. Methods??The clinical data of 208 cases of newly diagnosed childhood ALL were analyzed retrospectively??who had difficulty in sucking the bone marrow fluid and underwent bone marrow biopsy from November 2001 to September 2012 in Hematology Hospital of Chinese Academy of Medical Sciences. The clinical characteristics and prognosis of childhood ALL patients with and without myelofibrosis were compared. Results??All the cases were separated into two groups??group A included 33 cases??who were diagnosed with ALL combined with bone marrow fibrosis confirmed by pathology??175 patients diagnosed with ALL without bone marrow fibrosis were enrolled into group B. From November 2001 to December 2007??we treated patients using CAMSBDH-ALL regimen??and from January 2008 to September 2012 we used CCLG-ALL 2008 regimen. In group A??42.4% of patients showed pancytopenia of peripheral blood. The 5-year overall survival rate??OS?? was 64.3%. The 5-year OS was 58.3% in the patients treated with CAMSBDH-ALL regimen. In group B??15.6% of patients showed pancytopenia of peripheral blood??lower than that of group A. The 5-year OS was 85.0%??which was significantly higher than that of group A ??P??0.010??. The 5-year OS was 68.8% in the patients treated with CAMSBDH-ALL regimen??which was significantly lower than that of patients treated with CCLG-ALL2008 regimen ??5-year OS being 87.2%?? in group B ??P??0.044??. Conclusion??Pancytopenia in childhood ALL with myelofibrosis are more common and with poorer prognosis than those without myelofibrosis. The overall survival of ALL patients without bone marrow fibrosis can be improved by modifying the treatment strategies??but the prognosis can’t be improved for those ALL patients with myelofibrosis.  相似文献   
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??Early blood transfusion??high serum ferritin level??poor compliance with chelation therapy and splenectomy are the main risk factors for endocrine complications in patients with severe thalassemia. Endocrine system complications are manifested in the following aspects??firstly??growth retardation and hypothalamus-pituitary-gonad axis disorder??manifested as puberty stagnation??delay and hypogonadism??secondly??hypoparathyroidism??HPT?? and subclinical hypothyroidism. finally??impaired pancreas function??characterized by abnormal glucose tolerance test and symptomatic diabetes mellitus.  相似文献   
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