ObjectiveTo explore the clinical features,etiological factors,diagnosis and treatments in children with hemophagocytic syndromes (HPS).MethodsA retrospective study was carried out to analyze etiological factors,clinical symptoms and sighs,laboratory findings and outcomes of 32 children with HPS in our hospital.Results( 1 ) Etiological factors:virus infection cases accounted for 75.0％ (24/32),in which Epstein-Barr virus infection cases were predominant,making up to 68.8％ (22/32) ; (2) Main clinical features:included persistent fever,hepatosplenomegaly,lymphadenopathy; ( 3 ) Characteristic laboratory findings:included decrease of three lines or two lines of peripheral blood cells,hyperferrttinemia,abnormal liver function and coagulation function,hypertriglycerdema and decreased fibrinogen,hemophagocytosis in bone marrow,high soluble interleukin-2 receptor level,decreased natural killer cell activity.(4) Treatments and outcomes:twenty-four(24/32) virus infection cases received antivirus and high dose of immunoglobulin treatment.Twenty-two cases were treated with glucocorticoid and cyclophosphamide,resulting in 11 cases improvement,5 cases with complete remission,2 cases giving up and 4 death.The other 10 cases were treated under the guide of HLH-2004 protocol treatmemt with 4 cases improved and 6 cases with complete remission.Conclusion HPS is mainly triggered by infections,especially Epstein-Barr virus infection.Serum ferrifin,interleukin-2 receptor level and natural killer cell activity are biomarkers of the disease,so that they should be monitored during the progress of chemotherapy.HLH-2004 protocol is a good guide for the treatment of this disease.

BACKGROUND: Aerobic exercise is a popular and effective way of fitness. All kinds of changes occur in the human system, especially the changes in the cardiovascular system. Enhancing the cardiovascular system functions is the most important role in the aerobic exercise. OBJECTIVE: To sum up effects of aerobic exercise on blood indicators, cardio-vascular function. RETRIEVAL STRATEGY: A computer-based search was conducted in Pubmed for literatures related to aerobic exercise and cardiovascular system published between January 1998 and December 2006 with the key words of "aerobic exercise, cardiovascular" in English. Simultaneously, China Journal Full-text Database was looked for relevant articles published from January 2001 to December 2006 with the same key words in Chinese. The data were selected firstly. Inclusive criteria: ①the influence of aerobic exercise on cardiovascular function, ②influence of aerobic exercise on blood indexes. The exclusive criterion for deletion was the repetition of the same research. There were 81 literatures in accordance with the above-mentioned criteria. LITERATURE EVALUATION: 51 papers of repetitive research were excluded, 30 of them were accorded with the inclusive criteria and were included. DATA SYNTHESIS: Aerobic exercise plays the most important role in fitness. Enhancing the cardiovascular system functions is the most important role in the aerobic exercise. Epidemiological studies and experimental studies have indicated that aerobic exercise not only can increase energy consumption, improve vitality lipoprotein and lower blood lipid content, but also can reduce blood pressure in patients with hypertension and decrease the incidence of arteriosclerosis and other cardiovascular diseases, and can effectively enhance cardiac function. CONCLUSION: Appropriate aerobic exercise can lead to the enhancements of cardiac function and vascular function, and plays an important role in the prevention and treatment of cardiovascular diseases.

Objective To explore the clinical characteristics, treatment and prognosis of pediatric patients with bronchiec-tasis caused by Mycoplasma pneumonia (MP) and Chlamydia pneumonia (CP). Methods The clinical data from 27 MP and CP pneumonia pediatric patients with bronchiectasis suggested by the high resolution CT were retrospectively analyzed. Results The morbility rate of bronchiectasis caused by MP and CP pneumonia is 0.56%. The mean age of these patients was 75.4 ± 52.7 months. Among them, 27 cases (100%) had cough, 19 cases (70.4%) had fever, 10 cases (37%) had respiratory distress and 20 cases had lung auscultation. Sixteen cases were MP-IgM positive, 5 cases (18.5%) were CP-IgM positive and 6 cases (22.2%) were positive of both. Eight cases were combined with other pathogens infections, in which 6 cases were bacterial infections. The imaging ifndings showed diffuse bronchiectasis in 13 cases (48.1%) and local bronchiectasis in14 cases (51.9%). The bronchoscopy found endothelium in-lfammation, mucosal swelling, partial erosion and follicular hyperplasia in 16 cases (66.7%), the formation of short column sputum bolt in 5 cases (20.8%), in which 1 case had plastic bronchitis. All patients were treated with macrolides antibiotics, 10 cases (37%) combined with methylprednisolone, 3 cases (11.1%) combined with immunoglobulin and 20 cases (74%) combined with other anti-biotics. The average length of hospitalization was 12±4.3 days. The bronchiectasis sign disappeared within 4 months in 23 cases (92%). Two cases (8%) still had bronchiectasis after 9 to 15 months, with the recurrent pneumonia. Conclusions MP and CP pneumonia can lead to acute bronchiectasis. Most of patients are recoverable with effective treatment.

Objective To analyze the etiology and clinical characteristics of infectious mononucleosis-like syndrome (IMLS).Methods Eighty-seven children with IMLS in a hospital from January 2006 to September 2013 were per-formed pathogenic detection,then clinical features were summarized and analyzed.Results The top 5 pathogens in IMLS were Mycoplasma pneumoniae (MP,n=20 ),cytomegalovirus (CMV,n=13),simplex herpes virus(HSV, n=11),Chlamydia (CP,n=11),and parvovirus B19 (Hpv B19,n=9 ),clinical manifestations of all kinds of dis-eases were similar,but the occurrence rates were slightly different.MP infection were mainly fever,hepatospleno-megaly,and lymphadenopathy,and most were associated with respiratory complications(including laryngitis,bron-chitis,and pneumonia),CMV infection were also showed fever and hepatosplenomegaly,but the latter was more ob-vious;rubella patients didn’t appear skin rash,but the other symptoms were obvious.Conclusion Etiologies of in-fectious mononucleosis-like syndrome are varied,MP infection is most common,the next is CMV.Pathogens should be ascertained to provide theoretical basis of treatment.

Objective To study the epidemic characteristics and disease spectrum of Epstein-Barr virus (EBV) infected children in Zhongshan region, Guangdong province. Methods Clinical data from the children with positive EBV-DNA detected by real-time lfuorescent quantitative PCR between 2011 and 2013 was retrospectively analyzed. Results A total of 409 cases were detected with EBV-DNA positive from 3402 cases, with a total positive rate 12%, and the positive rate is 8.1%in 2011, 10.4% in 2012, 19.5% in 2013, there were significant differences among positive rate (χ2=6804.00, P<0.05). There was no statistically signiifcant difference in the positive rate of EBV-DNA between different gender (χ2=0.239, P>0.05) and different age groups (χ2=136.96, P<0.05). The positive rate of pre-school group is the highest. EBV infection can cause multiple system diseases. The most common disease caused by EBV infection was infectious mononucleosis (61.6%), followed by respiratory tract infection (26.7%), neck lymphadenitis (3.4%), idiopathic thrombocytopenic purpura (2.4%), etc. Among the 409 cases of EBV infection, the concurrent other pathogen speciifc IgM positive cases as MP-IgM positive (n=79), CP-IgM positive (n=47), Parvovirus B19-IgM positive (n=20), HSV-IgM positive (n=11), CMV-IgM positive (n=10), and RV-IgM positive (n=4) were found. Conclusions Infectious mononucleosis is the leading disease in children infected by EBV in Zhongshan region, the annual positive rate is increasing. Multiple pathogen speciifc IgM may be detected positive in children with EBV infection, which should be interpreted in combination with clinical status.

Objective To investigate the diagnostic value and safety of bronchovideoscope in the pediatric upper airway obstruction.Methods Bronchovideoscope was performed in 91 pediatric patients with upper airway obstruction.The etiology was analyzed and summarized.Results (1) Our study showed that the etiology of pediatric upper airway obstruction were as follow in turn:congenital laryngo-trachemalacia (45 cases),subglottic foreign body (13 cases),laryngotracheal occupying lesion (9 cases),vocal area edema (6 cases),tracheal stenosis (5 cases),laryngopharyngeal lesion (3 cases of retropharyngeal abscess,2 cases of tumor),vocal area hyperplasia (3 cases),subglottic stenosis (2 cases),glottic paralysis (2 cases),laryngeal web (1 case).(2) The common etiology of pediatric upper airway obstruction was different with age.Neonatal period:glottic paralysis,laryngomalacia in turn;1~6 month:laryngo-trachemalacia,tracheal stenosis,subglottic foreign body in turn;1~3 year:subglottic foreign body,vocal area edema,laryngo-trachemalacia in turn,>3 year:laryngeal papilloma.(3) Specific disease had predominant age:laryngo-trachemalacia predominated in infants less than 6 month;tracheal foreign body was most common in child aged 1~3 years old;epiglottic cyst was most common in infant less than 3 month.(4) Complication:during procedure,16 patients had transient decrease of saturation of blood oxygen and 11 patients had tracheobronchial spasm.After procedure,8 patients had transient fever and 6 patients had transient aggravation of laryngeal stridor.Conclusion Bronchovideoscope plays an important role in the diagnosis of pediatric upper airway obstruction.It can directly identify position and nature of disease,and then guide treatment.

Objective To investigate the clinical significance of testing serum kisspeptin in central precocious puberty (CPP) girls. Methods Sixty eight CPP girls and 68 healthy girls was studied from December 2012 to December 2014. HEK293 cells were cultured. Luciferase reporter assay was performed to verify the binding of miR-137 to the 3′UTR of KISS1. Serum miR-137 level was levaluated by qRT-PCR. Level of serum luteinizing hormone , prolactin , follicle stimulating hormone , thyrotropin , free thyroxine and estradiol was evaluated by chemi-luminescence immunoassay. The level of serum kisspeptin was detected by ELISA. Results MiR-137 was confirmed to bind to the 3′UTR of KISS1. The level of serum miR-137 was downregulated and kisspeptin was enhanced in CPP girls. The expression of miR-137 and kisspeptin was negatively correlated. Serum miR-137 level was negatively related to bone age and bone age advancement. According to the results of GnRH stimulating test, serum miR-137 was related to peak LH and peak/basal LH ratio. Conclusions MiR-137 could bind to the 3′UTR of KISS1. MiR-137 may be a potential biomarker for CPP assisted diagnosis.

Objective To analyze the clinical feature of pediatric severe influenza A(H1N1)cases.Methods To summarize the clinical manifestation,diagnostic and therapeutic process of eight pediatric severe influenza A(H1N1)cases.Results All eight cases couldn't provide contact history.Four cases had fundamental diseases,which were nephrotic syndrome,congenital hypothyroidism,bronchial asthma and moderate anemia.All cases had cough and fever,which was productive cough and hyperpyrexia(5 cases).All cases had tachypnea,which presented at the course of 0.5～6 days and progressively aggravated to respiratory failure 3～24 hours later.Chest x-ray showed localized exudation,which was similar to mycoplasma pneumonia.Seven cases had increased percentages of neutrophil.Six cases had increased CRP.All cases had respiratory failure;two cases were complicated with toxic encephacopathy.Treatment included anti-virus and support therapy.All cases received immunoglobulin and some cases received glucocorticoid.Six patients received mechanicai ventilation.Time of mechanical ventilation was 3～6 days.No patients died.Conclusion Pediatric severe influenza A(H1N1)case is severe pneumonia with characteristic of severe hypoxemia.Acute respiratory distress syndrome and death can be prevented through effective and in-time therapy.

Objective To investigate hospitalized children's nutritional risk and malnutrition occurrence,and to provide clinical basis for nutrition support.Methods Nutritional risk screening tool STRONGkids was applied to 651 cases of hospitalized children with nutritional risk screening,and through physical measurement to assess children' s nutritional status,at the same time during the patient's diagnosis,the length of time data for analysis was recorded.Results Of 651 cases of hospitalized children,7.07％ (46 cases) were of highly nutritional risk,80.95％ (527 cases) with moderate nutrition risk,and 11.98％ (78 cases) were of low nutrition risk.Malnutrition rate was 22.58％,moderate malnutrition in 111 cases (17.05％),severe malnutrition in 36 cases (5.53％).The first three high risk diseases were congenital heart disease,chronic liver disease and chronic kidney disease(x2 =21.43,P ＜0.01).According to the result of nutrition evaluation concluded with congenital heart disease,chronic kidney disease occurred with severe malnutrition was far higher than other diseases(x2 =16.53,P ＜ 0.05).Children with highly nutritional risk were more likely to have weight loss than the children with relatively low nutritional risk (P ＜ 0.05),and their length of hospital stay were obviously longer than the children with low or moderate nutritional risk (P ＜ 0.05).Conclusions The hospitalized children have high incidence of malnutrition,and different nutritional risks lead to different clinical outcomes.STRONGkids score method helps to evaluate nutritional risk in hospitalized children and detect malnutrition and potential deterioration,which is conducive to early comprehensive nutritional assessment and proper nutritional treatment,thus to improve their clinical outcomes.

Objective To analyze the clinical features, diagnosis and treatment of X-Linked Agarnmaglobulinemia (XLA). Methods Clinical features, cellular and humoral immune functions, treatment and prognosis from 3 patients with XLA were retrospectively reviewed. Results The age of onset were from 11 months to 6 years in these 3 cases, however, the median age of diagnosis was 12 years. All patients showed multiple recurrent bacterial infections, arthritis involved large joints such as knee, ankle, elbow and hip. Laboratory examination revealed the decrease of serum gammmaglohulin and absence of B lymphocytes in the peripheral blood. All 3 patients were identiifed BTK mutations, which were frameshift mutation and nonsense mutation in exon 3, frameshift mutation in exon 10, missense mutation in exon 18. After XLA was diagnosed, the patients were managed by intravenous gammagloulin (IVIG) replacement. The non-steroidal anti-inflammatory drugs (NSAIDs) were administrated in patients combined arthritis. The small dose of hormones had been applied. All patients had a significantly improvement. Conclusions The clinical features of XLA have greater variability, with recurrent bacterial infections. Markedly decreased and absent tosils and lymph nodes, serum immunoglobulin may be one of the warning signs for early diagnosis of XLA. IVIG and NSAIDs can be jointly treatment of XLA with arthritis. The steroid and immunosuppressant agents should be used with caution.