Objective To discuss the perioperative nursing measures and their effectiveness for patients with malignant airway stenosis who are receiving airway stent implantation once more due to airway re-stenosis.Methods The clinical data of 7 patients,who received airway stent implantation once more due to airway re-stenosis during the period from May 2014 to July 2016,were retrospectively analyzed.The effectiveness of nursing intervention for stent implantation was assessed.Results The symptoms of dyspnea were relieved immediately after the stent re-placement procedure in all the 7 patients,and no severe complications,such as massive hemorrhage,respiratory failure or asphyxia occurred.Conclusion The key points to ensure a successful second airway stent implantation include following measures and preparations:cooperation mode of medical care integration,preoperative individualized psychological intervention,guiding the patients to cooperate with the treatment,ready for in-operation emergency rescue,rapid and effective assistant cooperation for stent placement,and postoperative close observation for the occurrence of respiratory tract infection,bleeding and other complications.

Objective To review the ophthalmic features of granulomatosis with polyangiitis (GPA).Methods Clinical data of 164 GPA patients diagnosed in Peking Union Medical College Hospital during the past 10 years were retrospectively reviewed.Results Among 164 patients,48 (29.3％) had ocular involvement with a male to female ratio of 1.4:1 and an average age of (45 ± 15) years.Nine patients (5.5％) were first diagnosed at the ophthalmology department.Scleritis (15.9％,26/164),orbit lesions (13.4％,22/164) and peripheral corneal ulcer (9.1％,15/164) were the three most common ophthalmic complications.Twenty-two patients (13.4％) had positive findings in the orbit imaging.Six patients (3.7％,6/164) lost their vision due to GPA.Conclusion The ocular manifestations of GPA are diverse and complicated.GPA may affect every component of the eye,sclera,orbit and cornea are the most frequently affected sites of the eye.

Objective:To explore the value and risk of electronic bronchoscope applied in perioperative management of children with congential tracheoesophageal fistula.Methods:Sixty-five children with congential tracheoesophageal fistula performed electronic bronchoscope examination from September 2014 to November 2020 were enrolled in this study.The results of examination and complications were analyzed.Results:Sixty-three children with congenital tracheoesophageal fistula were diagnosed by electronic bronchoscopy.The diagnosis rate was 96.92%.Fifty-four children with congenital tracheoesophageal fistula were diagnosed by esophagography.The diagnosis rate was 91.53%.Sixty-one children with congenital tracheoesophageal fistula were diagnosed by multislice spiral computed tomography.The diagnosis rate was 93.85%.Airway anatomic abnormity was found in 27 children, including three cases of nasopharyngeal soft tissue collapse, 14 cases of laryngomalacia, five cases of tracheal stenosis, nine cases of tracheobronchomalacia, and nine cases of tracheobronchial and abnormal opening of the bronchus.The incidence was 41.54%.Three children with difficult ventilator weaning were related to tracheobronchial stenosis or tracheobronchomalacia.They were gradually weaning from ventilator after a long period of mechanical ventilation and treatment.Two children with transient decrease in oxygen saturation were noticed as complication.Conclusion:Electronic bronchoscopy is a safe and effective method for the diagnosis of congenital tracheoesophageal fistula and recurrence after operation.It is of great value to the airway management after operation by early detection of respiratory anatomic abnormity.

Covalent organic nanospheres(CONs)were explored as a fiber coating for solid-phase microextraction of genotoxic impurities(GTIs)from active ingredients(AIs).CONs were synthesized by an easy solution-phase procedure at 25℃.The obtained nanospheres exhibited a high specific surface area,good ther-mostability,high acid and alkali resistance,and favorable crystallinity and porosity.Two types of GTIs,alkyl halides(1-iodooctane,1-chlorobenzene,1-bromododecane,1,2-dichlorobenzene,1-bromooctane,1-chlorohexane,and 1,8-dibromooctane)and sulfonate esters(methyl p-toluenesulfonate and ethyl p-toluenesulfonate),were chosen as target molecules for assessing the performance of the coating.The prepared coating achieved high enhancement factors(5097-9799)for the selected GTIs.The strong affinity between CONs and GTIs was tentatively attributed to T-T and hydrophobicity interactions,large surface area of the CONs,and size-matching of the materials.Combined with gas chromatography-mass spectrometry(GC-MS),the established analytical method detected the GTIs in capecitabine and imatinib mesylate samples over a wide linear range(0.2-200 ng/g)with a low detection limit(0.04-2.0 ng/g),satisfactory recovery(80.03％-109.5％),and high repeatability(6.20％-14.8％)and reproducibility(6.20％-14.1％).Therefore,the CON-coated fibers are promising alternatives for the sensitive detection of GTIs in AI samples.

Objective To assess the feasibility of the log file-based three-dimensional independent dose verification system for the quality assurance of clinical radiotherapy.Methods The statistical values of the percentage depth dose,off-axis curves and output factor calculated by the Mobius system were statistically compared with the measured data by three-dimensional water tank.The three-dimensional independent dose verification in clinical radiotherapy plan and the acceleratr log file-based three-dimensional dose verification during the treatment were performed in 17 patients with nasopharyngeal cancer.The accuracy of dose calculation and reconstruction of Mobius system was assessed.A statistical analysis was performed on the intra-fractionalγpass rate (3％/3 mm) for each patient to evaluate the stability of intra-fractional radiotherapy.Results The percentage depth dose,off-axis curve and output factor statistically calculated by the Mobius system matched well with the data measured by three-dimensional water tank.The dose-volume histogram (DVH) parameters between the target area and organ at risk during clinical radiotherapy plan were statistically compared in 17 patients with nasopharyngeal cancer.The maximum deviation was-2.16％ for the three-dimensional independent dose verification in the clinical radiotherapy plan,and 0.18 Gy for the accelerator log file-based three-dimensional dose verification.The averageγpass rate for 17 nasopharyngeal cancer patients was 99.26％,and the maximum deviation of intra-fractional radiotherapy was below 0.5％.Conclusions The function of dose reconstruction and independent calculation of the Mobius system yeilds the same accuracy with the treatment planning system,which can quickly perform three-dimensional independent dose verification in the clinical radiotherapy plan and accelerator log file-based three-dimensional dose verification throughout the treatment,thereby guarantting and providing the safe and reliable technical support for clinical treatment.

OBJECTIVE: To investigate the clinical manifestations, biochemical abnormalities and pathogenic variants among children with Short/branched-chain acyl-CoA dehydrogenase (SBCAD) deficiency detected by neonatal screening. METHODS: A total of 2 730 852 newborns were screened from January 2016 to December 2021 with liquid chromatography tandem mass spectrometry. Suspected SBCAD deficiency patients were diagnosed by urine organic acid analysis and high-throughput gene sequencing analysis. The clinical, biochemical and genetic changes of the confirmed cases were analyzed, in addition with guidance for diet and life management, L-carnitine supplement, and survey of growth and intellectual development. RESULTS: Twelve cases of SBCAD deficiency were diagnosed, which yielded a prevalence of 1/227 571. The lsovaleryl carnitine (C5) of primary screening blood samples was between 0.6 and 2.1 µmol/L, all exceeded the normal range. C5/acety1 carnitine (C2) was between 0.02 and 0.12, with 6 cases exceeding the normal range. C5/propionyl carnitine (C3) was between 0.1 and 1.16, with 5 cases exceeding the normal range. Free carnitine (C0) was between 18.89 and 58.12 µmol, with 1 case exceeding the normal range. Three neonates with abnormal screening results were recommended to have appropriate restriction for protein intake and two were given L-carnitine. During follow-up, their C5 has ranged from 0.22 to 2.32 µmol/L, C5/C2 has ranged from 0.01 to 0.31, C5/C3 has ranged from 0.14 to 1.7. C5 or C5/C2 and C5/C3 were transiently normal in all patients except for case 8 during the neonatal screening and follow-up. C0 was 17.42 ∼ 76.83 µmol/L Urine organic acid analysis was carried out in 9 of the 12 cases, and 2-methylbutyroglycine was elevated in 8 cases. Urine organic acid analysis was carried out in 9 cases, and 2-methylbutyrylglycine was increased in 8 cases. Genetic analysis was carried out for 11 children, and in total 6 ACADSB gene variants were identified, which included 4 missense variants (c.655G>A, c.923G>A, c.461G>A, c.1165A>G), 1 frameshift variant (c.746del) and 1 nonsense variant (c.275C>G). Among these, the C.461G>A variant was unreported previously. The most common variants were c.1165A>G (40.9%) and C.275C>G (22.7%). The patients were followed up for 18 days to 55 months. Only one patient had mental retardation, with the remainders having normal physical and mental development. CONCLUSION SBCAD deficiency is a rare disease. The detection rate of newborn screening in this study was 1/227 571. Early intervention can be attained in most asymptomatic patients through neonatal screening. In this study, the common gene variants are c.1165A>G and c.275C>G.
Humans ; Infant, Newborn ; Amino Acid Metabolism, Inborn Errors/genetics* ; Carnitine ; Neonatal Screening/methods*