Objective To study the clinical and laboratory characteristics and treatments in children with chronic myelogenous leukemia (CML). Methods Respective analyze of the clinical and laboratory data of twelve pediatric patients with CML from December 1994 to October 2009 was performed. Results The disease predominately affected children from 5 to 9 years old (50 % of the patients), with a higher prevalence in boys than girls (gender ratio: 5:1). The duration before the diagnosis was different from three days to one year. The main presenting symptoms were fever, bleeding,asthenia, and complaints related to splenomegaly.Some cases were diagnosed incidentally. Markedly raised leukocyte and platelet counts were common.Conclusion CML in children is rare, and average level of WBC count seems to be much higher than adult.

Objective To investigate the clinical and laboratory characters of infantile organic acidemia(OA) accompanied with acute metabolic crisis.Methods We analyzed retrospectively datum of infants with OA diagnosed in our unit from April 2006 to October 2014.Results Fity-three cases(37 male and 16 female,aged under 1 year old) were enrolled in this study,in which,28 cases were methylmalonic acidemia,11 cases were propionic acidemia,3 cases were biotinidase deficiency,3 cases were glutaric acidemia type Ⅱ,2 cases were glutaric acidemia type Ⅰ,2 cases were isovaleric acidemia,1 case was variety of coenzyme A carboxylase deficiency,1 case was glycerol kinase deficiency,1 case was 3-methylcrotonyl-CoA carboxylase deficiency and 1 case was holocarboxylase synthetase defect.Tweny-five of the 53 cases(47.2％)developed metabolic crisis within 7 days after onset,main manifestations included feeding difficulties,frequent seizures dyspnea,et al.The most common abnormal laboratory findings manifested severe hypoglycemia,intractable metabolic acidosis,hyperammonemia,et al.Twenty cases had family history.All patients were given symptomatic relief and supportive treatment,including colleting hypoglycemia,reducing hyperammonemia,keeping water,electrolyte and acid base balance,maintaining function of vital organs,suppling metabolic cofactor and special fomula,et al.After treatment,32 cases (60.4％) improved markedly while death occurred in 15 cases(28.3％).Conclusion OA confirmed under 1 year old is especially vulnerable to acute metabolic crisis,which characterized by sudden onset,rapid progress and is difficult to treat.Feeding difficulties,frequent seizures and dyspnea were the very common presentations.Early diagnosis and timely treatment are critical for improving the prognosis.Clinicians should be aware of it,and an early metabolic disorders screening should be intervened in patients with hypoglycemia of unknown etiology or refractory metabolic acidosis.

Objective To explore the risk factors of extremely low birth weight infants(ELBWI) with bronchopulmonary dysplasia(BPD).Methods The clinical data in 151 ELBWI in NICU of the Provincial Hospital Affiliated to Shandong University and Sichuan Provincial People's Hospital from 2010 to 2015,admitted at 24 after birth and survived until to 28 h after birth,were performed the survey analysis.The newborns of oxygen dependency(FiO2 ＞ 21％) more than 28 d were included into the BPD group,on the contrary,the newborns were included into the non-BPD group.The maternal pregnant factors,neonatal factors and disease treatment factors were compared between the two groups.Results (1) Among 151 ELBWI,57 cases developed BPD with incidence rate of 37.7 ％.(2) The univariate analysis results showed that maternal chorioamnionitis,premature rupture of membrane more than 18 h,birth weight,gestational age,sepsis,patent ductus arteriosus (PDA),neonatal respiratory distress syndrome (NRDS),mechanical ventilation(MV),MV time,parenteral nutrition time,frequency of blood transfusion were associated with the BPD occurrence in ELBWI.Caffeine use was a protective factor.(3)The multivariate Logistic regression analysis displayed that MV (OR=6.975,95％CI:1.464-33.219) and maternal chorioamnionitis(OR=3.890,95％CI:1.656-9.140) were the independent factors of BPD occurrence in ELBWI.Conclusion Reducing intrauterine infection and preterm birth as far as possible and reasonable respiratory support after birth can effectively reduce the incidence rate of BPD in ELBWI.

Emergency Medical Services ; Health Promotion ; Humans ; Life Style ; Nurses ; psychology

Objective To study the early survival rate and its influencing factors of extremely preterm infants and extremely low birth weight ( ELBW ) infants.Method All extremely preterm infants and/or ELBW infants in Shandong Provincial Hospital from January , 2010 to December 2015 were studied retrospectively.The factors affecting their survival rate and their complications were analyzed retrospectively . All cases were assigned into the survival group and the death group .On the other hand , they were also assigned into two groups according to their birth , pre-2014 and post-2014.Result A total of 142 extremely preterm infants and/or ELBW infants were enrolled, their gestational age was 28 (27, 29) weeks, birth weight was 925 (830, 965) g.76 cases (53.5%) survived, 66 cases (46.5%) died.Factors associated with the survival rate were early onset sepsis , placental abruption , perinatal asphyxia , birth weight and pulmonary hemorrhage (P<0.05).There were 67 cases pre-2014 in which 30 cases survived (44.8%), while 75 cases post-2014 in which 46 cases survived ( 61.3%) .Comparative analysis between the two groups pre-2014 and post-2014 revealed that the survival rate was significantly different (χ2 =3.900, P=0.048).The top 2 underlying causes of death before 2014 were perinatal asphyxia and early onset sepsis . Furthermore, early onset infection became the first cause of death after 2014.Conclusion Along with the prevalence of neonatal resuscitation program and the optimization of respiratory support strategies in premature infants , the early survival rate of extremely preterm infants and /or ELBW infants has improved significantly.However, early onset sepsis may have been the crucial cause for their perinatal mortality .