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 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 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 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.

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.

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.

Objective To evaluate the diagnostic value of lung ultrasonography for transient tachypnea of new-born (TTN).Methods From January to December 201 4,a total of 1 358 children were hospitalized in the Neonatal Intensive Care Center and underwent lung ultrasonography at the Bayi Children′s Hospital,Beijing Military General Hospital.According to the patients′medical histories,clinical presentations,arterial blood gas analysis,chest X -ray examinations,and lung ultrasound examinations,there were 41 2 cases of patients without pulmonary lesions,228 cases of TTN,358 cases of respiratory distress syndrome (RDS),85 cases of meconium aspiration syndrome (MAS),21 5 ca-ses of infectious pneumonia,and 60 other cases at the time of hospital admission.In a resting state,the patients were placed in a supine,lateral recumbent or prone position.By using the anterior and posterior axillary lines as boundaries, the lung was divided into 3 regions:anterior,lateral,and posterior.The probe was perpendicular or parallel to the ribs, and each region of both sides of the lung was scanned.The scan results were compared to the conventional chest X -ray results.Results The main ultrasonic characteristics of TTN was pulmonary edema.In severe cases,the ultrasound showed a white lung or compact B -line.Compact B -line had 1 00.0% sensitivity and 95.3% specificity for diagno-sing severe TTN.Mild TTN presented as pulmonary interstitial syndrome or double lung point.Double lung point might appear during the recovery period of mild TTN or other diseases,such as RDS,MAS,and pneumonia,among others. Lung consolidation and air bronchogram were not observed in patients with TTN.Pleural effusion might occur in a varie-ty of lung diseases,and pleural line abnormality,A -line disappearance,and B -line or pulmonary interstitial syndrome were common ultrasonic manifestations of a variety of lung diseases.Conclusions Ultrasonic diagnosis of TTN,mainly based on pulmonary edema without lung consolidation and air bronchogram,is accuracy and reliable;however,double lung point is not a specific sign of TTN,whereas the identification of a white lung and compact B -line is a sensitive and specific sign of severe TTN.

Objective To evaluate the value of lung ultrasound in diagnosing infectious pneumonia (IPN) of newborns. Methods Seventy-two infants hospitalized from September 1, 2012 to May 30, 2013, in Bayi Children's Hospital, General Hospital of Beijing Military Command were divided into two groups. The study group consisted of 32 newborn infants diagnosed as IPN by medical history, clinical manifestations and chest X-ray, while the control group consisted of 40 neonates without any lung diseases. In a quiet state, the infants were placed in supine, side or prone position for lung ultrasound examination. The lung field was divided into three areas by the anterior and posterior axillary line. The regions of the bilateral lung were scanned by the probe which was vertical with the ribs. Fisher's exact was performed for stastical analysis. Results The most important ultrasound imaging findings of IPN included lung consolidation of varying size and shape with irregular and serrated margins (100%, 32/32), dynamic air bronchograms (100%, 32/32), A-line disappearance (100%, 32/32) and interstitial syndrome (100%, 32/32). Other common ultrasound signs included pleural line abnormalities (91%, 29/32), lung pulse (38%, 12/32) and dynamic air bronchograms (66%, 21/32) under real-time ultrasound. Pleural effusion was an infrequent sign that existed in 9%(3/32)of IPN patients. None of these abnormalities was observed in the control group. Conclusion Lung ultrasound is reliable for the diagnosis of IPN and can be routinely performed in neonatal intensive care units, and a large area of lung consolidation with irregular margins A-line disappearance, interstitial syndrome and pleural line abnormalities are the main image findings.