Humans ; Infant, Newborn ; Infant, Newborn, Diseases ; Lung Diseases ; Point-of-Care Systems ; Ultrasonography

A case of congenital nephrotic syndrome (CNS) combined with skull dysplasia was confirmed based on clinical findings, laboratory examination and skull CT scan. Hereditary features, clinical manifestations, treatment and prognosis of CNS were reported in this paper after literatm'e review.

Objective To evaluate the effects and mechanisms of bovine lactoferrin(bLF)on cell viability,proliferation,and the protective roles in intestinal epithelial cell-6(IEC-6)treated by lipopolysaccharide(LPS).Methods The rat jejunum epithelial cell lines IEC-6 were cultured in vitro.The effects of bLF on cell viability and proliferation in IEC-6 cells were detected by 3-(4,5-dimethyl-2-thiazolyl)-2,5-diphenyl-2-H-tetrazolium bromide(MTT)assay and 5-Bromo-deoxyuridine(Brdu)assay,respectively.Inflammatory cytokines and their mRNA of interleukin-1β(IL-1β),interleukin-6(IL-6),tumor necrosis factor-α(TNF-α)and interleukin-8(IL-8)were analyzed by real-time PCR and enzyme-linked immunosorbent assay(ELISA).Western blot was used to measure the levels of mitogen-activated protein kinase(MAPK)activation and nuclear factor kappa β(NF-κB)nuclear translocation.Results Dose dependent effects of bLF on cell viability and proliferation were observed in IEC-6 cells in vitro(F=3.825,5.861,all P<0.05),especially in a dose of 100 mg/L,and bLF significantly stimulated cell viability and proliferation compared with non-treatment group(q=5.240,3.765,all P<0.05).The mRNA levels of IL-1β,IL-6 and TNF-α were decreased by co-stimulation of bLF and LPS compared with the LPS treatments alone in IEC-6 cells in vitro(q=14.28,10.12,16.45,all P<0.001).The secretion of IL-6 and TNF-α was also decreased by co-stimulation of bLF and LPS(q=15.06,6.74,all P<0.01).In vitro,bLF treatment at dose of 100 mg/L could inhibit the activation of MAPK/NF-κB signal pathway induced by LPS(q=12.96,18.54,all P<0.001).Conclusion In vitro,bLF can promote IEC-6 viability and proliferation,and have anti-inflammatory effects via inhibited activation of MAPK/NF-κB nuclear translocation.

Objective To analyze the clinical characteristics and coagulation parameters of the infants with placental abruption . Methods Analysis was made on clinical and laboratory indexes of the hospitalized children of the NICU of Bayi Clinical Medical College of South Medical University ,enrolled from August 2012 to January 2013 ,including 60 infants with placental abruption as observation group and 60 infants without placental abruption as the control group .Results From clinical manifestations and lab date ,significant differences were found in gestational age ,polyembryony ,premature rupture of membrane ,birth weight ,intrauterine growth retardation ,motherhood gestational hypertension ,mother gestational diabetes mellitus ,asphyxia ,APTT ,D-dimer on admis-sion between the observation group and control group (P<0 .05) .Conclusion Placental abruption is the result of placental insuffi-ciency ,which may cause coagulation disorder and thus show the pathological state of high condensation in infants .

Objective To investigate the clinical outcome and treatment characteristics of viral encephalitis with bilateral thalamic damage so as to improve its prognosis.Methods Twelve cases of viral encephalitis with bilateral thalamic damage were collected during September 2012 to June 2013 by head MRI.These cases were retrospectively studied with the data of medical history,physical examination,laboratory and brain function monitoring and treatment.The relationship between treatment and prognosis was studied during 2 years of follow-up.Results All patients began with the rapid onset,accompanied by fever,coma,and convulsions.Delirium and involuntary movement occurred during the recovery period.Six cases(50.0％) received ventilator assisted ventilation.In the acute phase,electroencephalogram (EEG) showed diffuse slow wave and 4 cases(33.3％) had status epilepticus on EEG.Ten cases discharged from hospital had long-term oral anti epileptic drugs,which lasted 6 months in 3 cases,1 year in 4 cases,and 2 years in 3 cases for maintaining EEG stability.Head MRI indicated white matter demyelination besides the gray matter damage within the thalamus.All 12 patients underwent methylprednisolone impact treatment and 6 cases had effective reversal within 1 week of onset with better tolerance,and the other 6 cases received treatment in subacute stage and 5 of them accelerated recovery and 1 case had sense improvement,but died after giving up therapy.After 3 months courses,8 cases(66.7％) got gross motor and swallowing function recovered to normal,and 3 cases had left unilateral limb movement disorder.After 2 years of follow-up,11 cases had normal motor,intelligence returned to normal in 9 cases,and 2 cases had mild mental retardation.Conclusions Viral encephalitis complicated with bilateral thalamic damage is characterized by acute onset,serious manifestations,idiopathic progress;in the subacute stage it is most likely to develop white matter demyelination.The key to control the disease is to block the inflammatory immune response quickly,and give patients large dose methylprednisolone treatment can effectively curb the progress of the disease.In the sub acute phase,it can promote the recovery,safe and effective.The long-term prognosis will be good with the reasonable treatment at early stage of the disease.

Objective To evaluate the effectiveness of domestic exogenous pulmonary surfactant (EPS)-Calsurf in treating full-term neonatal respiratory distress syndrome (RDS).Methods (1) From January 2011 to June 2012,154 full-term neonates with RDS were divided into Calsurf group (74 cases) and Curosurf group (80 cases),comparing differences in changes of arterial blood gas,the length of hospital stay,hospitalization expenses,duration of mechanical ventilation,inspired oxygen time,the incidences of complications and repeated EPS use rates between two groups.(2) In order to compare the effectiveness of different doses of Calsurf in treating full-term neonatal RDS,from July 2012 to March 2013,80 RDS patients who received Calsurf were divided into mild-moderate group (grade Ⅱ ～ Ⅲ on chest X-ray,50 cases) and severe group (grade Ⅳ on chest X-ray,30 cases).Furthermore,each group was divided into two subgroups,which were low-dosage(per-time 30 ～40 mg/kg) and high-dosage group (per-time 30 ～40 mg/kg).Results (1) Before administrating Calsurf and after administrating it at 0.5 h and 6 h,the pH,PaCO2 and PaO2 of RDS patients were no significant difference in two groups (P ＞ 0.05).(2) There were no significant difference between the duration of mechanical ventilation,inspired oxygen time,length of hospital stay in two groups (P ＞ 0.05).But the hospitalization expenses in Calsurf and Curosurf groups were 28.778 versus 31.827 thousand yuan,respectively,with the average reduction of 9.6％ in Calsurf group (P ＜ 0.05).(3) There were no significant difference between the morbidities and mortalities of multiple organ failure,persistent pulmonary hypertension,acute renal failure and pneumothorax in two groups (P ＞ 0.05).(4) In mild-moderate grade RDS patients,the low-dosage or high-dosage of Calsurf had similar effectiveness in improving bloog gas (P ＞ 0.05),the repeated Calsurf use rates of both subgroups were 16.0％ (4/25) and 20.0％ (5/25) (P ＞ 0.05).While in severe RDS patients,high-dosage Calsurf got a better effectiveness in improving bloog gas than low-dosage Calsurf(P ＜ 0.05).Meanwhile,the repeated Calsurf use rates of the low-dosage and high-dosage subgroups were 20.0％ (3/15) and 33.3％ (5/15),with a 40％ reduction in high-dosage subgroup.Conclusion (1) Both Calsurf and Curosurf can significantly improve the conditions of hypoxemia and hypercapnia,moreover,the length of hospital stay and duration of mechanical ventilation of both groups are similar,with less hospitalization expense in Calsurf group.(2) Both low and high-dosage Calsurf have similar curative effect on mild-moderate RDS,but for severe RDS,high-dosage Calsurf works better,reducing the repeated EPS use rate to some extent.

Objective To construct a lentiviral vector carrying angiopoietin-1 (Ang-1) and DsRed gene, and to package a virus particles. Methods The Ang-1 lentiviral vector with DsRed (PLV.Ex3d.P/puro-CMV>Ang-1>IRES/DsRed-Express2) was constructed by Gateway technology, and identiifed by PCR and gene sequencing. The lentiviral vector was mixed with helper vector pLV/helper-SL3, pLV/helper-SL4 and pLV/helper-SL5 by Lipofectamine 2000 to prepare DNA-Lipofectamine?2000 complexes. The complexes were then added to transfect 293FT cells and package virus. The virus titers and infection ef-ifciency were determined by lfuorescence expression. Results Ang-1 lentiviral vector PLV.Ex3d.P/puro-CMV>Ang-1>IRES/DsRed-Express2 was constructed successfully as identified by PCR and gene sequencing. Lentivirus with high-efficiency infection was produced by transfection to 293FT cells and the virus titer was 5×108 TU/ml. Conclusions The recombinant len-tiviral vector for Ang-1 was successfully constructed by Gateway technology and the lentivirus with high-efifciency infection packaging can be used for further experiment of Ang-1 gene.

Objective Lung ultrasound has been used extensively to diagnose many types of lung disease.This study aimed to evaluate the pulmonary reasons for long-term oxygen dependence (LTOD) in premature infants using lung ultrasound.Methods Lung ultrasound was routinely performed in 50 premature infants clinically diagnosed with bronchopulmonary dysplasia(BPD).Results Among the 50 patients studied,there were 9 cases of atelectasis,4 cases of pneumonia,2 cases of severe pulmonary edema,and 3 cases of pulmonary edema and consolidation that coexisted with BPD.The babies' oxygen dependence either resolved completely or significantly decreased following appropriate treatments.Conclusion More than onethird of the cases of LTOD in premature babies were caused by either BPD alone or diseases other than BPD.Lung ultrasound plays an important role in differentiating pulmonary causes of LTOD in BPD patients,and the results of our study suggest that modifying the diagnostic criteria for BPD may be necessary.

Objective To survey the prevalence of undemutrition in elderly leprosy patients and explore the feasibility of nutritional assessment. Methods The nutrition status of 60 elderly patients with advanced leprosy was analyzed using Subjective Global Assessment (SGA) and objective methods. Results Undemutrition was determined in 22 patients (36. 6% ), including 5 patients ( 8. 3% ) were at a status of severe undernutrition. Kap-pa value of SGA and the objective items were 0. 619, 0. 571, 0. 476, 0. 454, and 0. 419 for triceps skin-fold thickness (TSF), pre-albumin (PALB), mid arm muscle circumference (MAMC), hemoglobin (Hb), and ser-um albumin, respectively. Gender, admission duration, type of leprosy, or disability grade was not correlated to all nutritional parameters except body height and cholesteroL Age and extent of ulceration were significantly correlated with some parameters such as BMI, TSF, lib, ALB, and PALB ( P < O. 01 ). Sensitivity and specificity of SGA on objective parameters screened were different. TSF and ulcer had the hrgest impacts on SGA. Conclusions The prevalence of undernutrition is not high in elderly leprosy patients. Any single objective parameter is not feasible for the assessment of nutrition status in these patients. A comprehensive method using SGA is preferred.

Objectives To detect genetic causes of dystrophic epidermolysis bullosa (DEB). Methods Next-generation sequencing was used to detect a neonate with DEB. Sanger sequencing was used to confirm the results and detect his parents and grandmother on his mother side from the family. Results The neonate was found to have heterozygous mutation c.6781C>T of exon 86 in COL7A1 gene.This mutation results in R2261X nonsense mutation in typeⅦcollagen. His mother and grand-mother on his mother side have the same mutation. Conclusion Next-generation sequencing technology is a useful tool for the detection of mutations of COL7A1 gene, which is valuable for clinical application.