Objective:To investigate the application of extracorporeal membrane oxygenation(ECMO)bridging heart transplantation in critically ill children.Methods:The clinical data of two cases of critical infants with venous-arterial ECMO(VA-ECMO)bridging heart transplantation and literature review were retrospectively analyzed.Results:Two cases received orthotopic heart allograft with VA-ECMO support, and were discharged uneventfully without significant postoperative complications.On the 13th day of ECMO assistance, the first child was treated with orthotopic heart transplantation in a hospital qualified for heart transplantation, and the ECMO was evacuated during the operation.After 21 days of the heart transplantation, the patient was discharged from the hospital.The patient was followed up to be healthy after heart transplantation, and had the same development as children of the same age, and had been taking anti-rejection drugs for a long time.On the 10th day of VA-ECMO treatment, the second case received awake ECMO after cardiac function improved.On the 12th day of VA-ECMO treatment, the patient was successfully evacuated from VA-ECMO and waited for heart transplantation.Cardiac orthotopic transplantation was performed after the 17 days after VA-ECMO evacuation.The patient was transferred to the general ward after 6 days of hospitalization in the intensive care unit, and was discharged 23 days after transplantation with conventional anti-rejection therapy.Discharge follow-up in good health, normal school life.Conclusion:When VA-ECMO cannot be withdrawn from the heart of the critically ill children and the end-stage heart, VA-ECMO bridging heart transplantation should be selected at the right time for the children who meet the indications for heart transplantation to create survival opportunity for the previously hopeless children, save the life of the end-stage children, and improve the quality of life.

Objective:To observe the efficacy of methimazole (MMI) combined with 1α-hydroxyvitamin D3 (alfacalcidol, ALF) in patients with Graves disease of high-titer thyrotropin receptor antibodies (TRAb) and to explore new clinical strategies to reduce serum TRAb in Graves disease.Methods:120 patients with Graves disease initially diagnosed in Quanzhou First Hospital Affiliated to Fujian Medical University and the People′s Hospital Affiliated to Quanzhou Medical College from June 2017 to June 2019 were prospectively selected as the research objects. All patients received conventional dose of MMI for anti hyperthyroidism treatment. The patients were randomly divided into three groups: group A [ n=40, treated with MMI combined with high-dose ALF (0.5 μg/d)], group B [ n=37, treated with MMI combined with low-dose ALF (0.25 μg/d)] and group C ( n=43, treated with MMI only). The treatment lasted for 24 weeks. The serum free triiodothyronine (FT 3), free thyroxine (FT 4), thyroid stimulating hormone (TSH) and TRAb in patients before and after above treatments were detected. The blood routine, liver function, alkaline phosphatase (ALP), 25(OH)D, serum calcium (CA) and serum phosphorus were detected regularly. Results:After drug treatment: ⑴ the thyroid function of the three groups returned to normal. The average daily dosage of MMI in group A was significantly lower than that in group B and C ( P<0.05), and that in group B was also lower than that in group C ( P<0.05), with significant difference. After 24 weeks of treatment, the daily dosage of MMI in group A and B was significantly lower than that in group C ( P<0.05). ⑵ There was no significant difference in thyroid function among the three groups. The concentration of serum TRAb in group A was significantly lower than that in group B and C ( P<0.05), and that in group B was also lower than that in group C ( P<0.05). ⑶ During the 24 week follow-up, there was no significant difference in serum 25(OH)D, ALP, Ca and P among the three groups ( P>0.05); no leukopenia in peripheral blood and no abnormal liver function were found in the three groups. Conclusions:MMI combined with ALF can effectively treat Graves′ disease, reduce the dosage of MMI drugs, decline the level of TRAb in the serum of Graves′ patients, and improve the prognosis of Graves′ disease.

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 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.

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

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.

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 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 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.