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.

Disease is closely related to nutrition,and disease state of hospitalized children with nutrient consumption and malnutrition is worrying. Malnutrition affects not only children′s growth,reduce the body func-tion,but also can increase the nutrition related complications and mortality. However currently the decision crite-ria of nutrition risk screening and evaluation method are not identical, there is no systemic, standard, unified plan,which cause a lot of malnutrition cases misdiagnosis and miss the timing of the early intervention. There-fore,it is necessary to do nutritional risk screening,nutrition assessment and nutritional surveillance for hospital-ized children,so that we can timely find the nutritional problems and take reasonable nutrition support and inter-vention measures. In this paper,the hospitalized children with nutritional risk screening and nutrition evaluation research progress are reviewed briefiy.

Objective To summarize the clinical characteristics and drug sensitivity of Pseudomonas aeruginosa in children. Methods The data of children with diagnosed Pseudomonas aeruginosa from 2006 to 2016 were retrospectively analysis. Results There were 14 patients who had diagnosis of Pseudomonas aeruginosa, most of whom were infants and toddlers ( 78 . 6%). The common complications were disseminated intravascular coagulation (DIC) and pulmonary hemorrhage. In 14 patients, 7 patients were cured, 4 were died, and 3 were given up treatment by their family because of serious illness. The antimicrobial susceptibility test showed that isolated pseudomonas aeruginosa had good sensitivity to aminoglycosides and quinolones. Conclusion Pseudomonas aeruginosa sepsis is more common in infants and toddlers, with high mortality. For suspected patients, the antibiotic that covers the Pseudomonas aeruginosa should be used in early antibiotic therapy.

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 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 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 evaluate the clinical efficacy of standardized house dust mite allergen specific immunotherapy (SIT) for rhinitis and asthma in children. Methods Forty-two children with allergic rhinitis and asthma who received a standardized house dust mite allergen SIT in our hospital were enrolled in our study. The result of allergen skin prick test, serum specific IgE levels (sIgE) of house dust mite and dust mite,pulmonary function and symptom scores were analysed before and at one year after treatment in all children. Results Skin indexes of house dust mite and dust mite, symptom score were significantly decreased at one year after treatment,but the levels of house dust mite and dust mite sIgE,lung function test (FVC,FEVt,MEF25-75) showed no significant differences. Conclusion Children with allergic rhinitis and asthma have significant improvements in their skin sensitivity and clinical symptoms by given SIT for one year,but the impact of SIT on airway inflammation needs further observation.

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 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.