Objective:To summarize the clinical manifestations, diagnosis, treatment and prognosis of acute flaccid myelitis (AFM) in children.Methods:Clinical characteristics of 4 AFM cases from Department of Neurology, Children′s Hospital Affiliated to Capital Institute of Pediatrics, from September 2018 to November 2022, were analyzed retrospectively.Results:The age of 4 children with AFM was 7 years, 4 years and 3 months, 7 years and 1 month, 6 years and 5 months, respectively. There were 2 boys and 2 girls. Prodromal infection status showed 3 children of respiratory tract infection and 1 child of digestive tract infection. The main manifestation was asymmetrical limb weakness after infection, and the affected limb range was from monoplegia to quadriplegia. Cranial nerve injury was involved in 1 child, no encephalopathy. Magnetic resonance imaging in the spinal cord of all 4 children showed long T1 and T2 signals, mainly involving gray matter. Cerebrospinal fluid cell-protein separation was observed in 2 children. Pathogen detected in 1 child pharyngeal swab was enterovirus D68. Antibody IgM to adenovirus was positive in the blood of 1 child. Antibody IgG against Echo and Coxsackie B virus were positive in the blood of another child. After glucocorticoid, human immunoglobulin or simple symptomatic treatment and at the same time under later rehabilitation training, muscle strength recovered to different degrees, but there were disabilities left in 3 children.Conclusions:AFM should be considered in children with acute and asymmetrical flaccid paralysis accompanied by abnormal magnetic resonance imaging signal in the central region of spinal cord, especially post-infection. The effective treatment is limited and the prognosis is poor.

Objective:To investigate the impact of serum vitamin A levels on all-cause mortality risk in diabetes patients.Methods:Diabetes patients aged 20 years and above who participated in the US National Health and Nutrition Examination Survey during 2003-2004 and 2005-2006 were enrolled as the study population,with death data up to 2019 as the endpoint.Cox proportional hazards regression models were employed to calculate the hazard ratios of all-cause mortality in diabetes patients with different serum vitamin A levels,both unadjusted and adjusted for confounders.Restricted cubic spline methods were used to analyze the dose-response relationship between serum vitamin A levels and all-cause mortality risk in diabetes patients.Results:A total of 484 diabetes patients were included,with a median follow-up period of 13.7 years,during which 211 deaths occurred.Cox proportional hazards analysis showed that compared to the lowest quartile of serum vitamin A,higher quartiles of serum vitamin A were not associated with all-cause mortality risk in diabetes patients without adjusting for confounders.However,after adjusting for confounders,higher quartiles of serum vitamin A significantly reduced the all-cause mortality risk.The dose-response analysis indicated a significantly increased risk of all-cause mortality in diabetes patients with lower serum vitamin A levels.As the vitamin A levels increased,the mortality risk gradually decreased.A significant reduction in all-cause mortality risk was observed when serum vitamin A levels were between 2.17 and 2.50 μmol/L.Beyond this range,there was a tendency for increased all-cause mortality risk with further increases in vitamin A levels.Conclusion:Lower serum vitamin A levels increase the all-cause mortality risk in diabetes patients,while moderate serum vitamin A levels help reduce the all-cause mortality risk in diabetes patients.

More than 85% of patients with uveal melanoma (UM) carry a GNAQ or GNA11 mutation at a hotspot codon (Q209) that encodes G protein α subunit q/11 polypeptides (Gα_q/11). GNAQ/11 relies on palmitoylation for membrane association and signal transduction. Despite the palmitoylation of GNAQ/11 was discovered long before, its implication in UM remains unclear. Here, results of palmitoylation-targeted mutagenesis and chemical interference approaches revealed that the loss of GNAQ/11 palmitoylation substantially affected tumor cell proliferation and survival in UM cells. Palmitoylation inhibition through the mutation of palmitoylation sites suppressed GNAQ/11^Q209L-induced malignant transformation in NIH3T3 cells. Importantly, the palmitoylation-deficient oncogenic GNAQ/11 failed to rescue the cell death initiated by the knock down of endogenous GNAQ/11 oncogenes in UM cells, which are much more dependent on Gα_q/11 signaling for cell survival and proliferation than other melanoma cells without GNAQ/11 mutations. Furthermore, the palmitoylation inhibitor, 2-bromopalmitate, also specifically disrupted Gα_q/11 downstream signaling by interfering with the MAPK pathway and BCL2 survival pathway in GNAQ/11-mutant UM cells and showed a notable synergistic effect when applied in combination with the BCL2 inhibitor, ABT-199, in vitro. The findings validate that GNAQ/11 palmitoylation plays a critical role in UM and may serve as a promising therapeutic target for GNAQ/11-driven UM.
Humans ; Mice ; Animals ; Lipoylation ; NIH 3T3 Cells ; Uveal Neoplasms/genetics* ; Melanoma/genetics* ; Cell Proliferation ; Proto-Oncogene Proteins c-bcl-2 ; GTP-Binding Protein alpha Subunits, Gq-G11/genetics*

Objective Toevaluatetheclinicalvalueofmagneticresonanceenterography (MRE)indiagnosingCrohn’sdisease (CD).Methods ThearticlesconcerningthediagnosisofCD byusing MRE weresystematicallysearchedindatabasesincluding PubMed,EMbase,CochraneLibrary,WebofScience,CNKI,CBM,WanFangandVIPdata.Tworeviewersindependentlyscreenedliterature, extracteddata,andassessedbiasriskofincludedstudiesbyusingtheQUADAS-2.Then,thisMeta-analysiswasperformedbyusing Stata12.0software.Thepooledweightedsensitivity,specificity,positivelikelihoodratio(PLR),negativelikelihoodratio (NLR)and diagnosticoddsratio(DOR)werecalculated,thesummaryreceiveroperatingcharacteristiccurve(sROC)wasdrawnandtheAUC wascalculated.Results Atotalof16studieswereincluded,involving1276patientsand919bowelsegments.TheresultsofMeta-analysisshowed that,thepooledsensitivity,specificity,PLR,NLR,DORandAUCofMREdiagnosingCDwere0.87(95%CI:0.79,0.92),0.92(95%CI:0.89, 0.94),10.6(95%CI:7.4,15.2),0.15(95%CI:0.09,0.24),72.69(95%CI:32.7,161.51),0.95(95%CI:0.93,0.97),respectively.Theresultsof subgroupanalysissuggestedthat,thestudytype,MRT-field,pathogenicsiteanddiagnosticcriteriaplayedlittleeffectonthevalueof MREdiagnosingCD (P>0.05).Conclusion MREhadhigheraccuracyfordiagnosingCDand mayservedasanefficientimaging methodfordiagnosingCD.

Objective To explore the clinical significance of monitoring drainage fluid parathyroid hormone (dPTH) for estimating the in situ reserves and function of the parathyroid by analyzing the change of serum calcium,serum parathyroid hormone(sPTH) and dPTH after thyroid surgery.Methods According to the operative method,the total of 144 patients with thyroid disease were divided into five groups:unilateral lobectomy,unilateral lobectomy plus isthmectomy with unilateral lymph node dissection,total thyroidectomy,total thyroidectomy with unilateral lymph node dissection,and total thyroidectomy with bilateral lymph node dissection group.The blood calcium,sPTH and dPTH level of patients were tested before operation and on the 1st,2nd,3rd and 4th day after operation.The depression of serum calcium,hypocalcemia and hypoparathyroidism were observed after operation.The serum calcium,serum PTH and dPTH level were summarized and analyzed statistically in order to evaluate the in situ reserves and postoperative function of the parathyroid.Results Among the 114 cases,the decline of serum calcium level mostly happened on the 2nd day after operation(70 cases,61.4％).There were 36 patients with hypocalcemia (31.58％) and 34 patients with hypoparathyroidism (29.82％).Serum calcium level increased gradually in all of the patients.Although sPTH level swung,it had a rising trend on the whole.The level of serum calcium and sPTH was positively correlated.The level of dPTH was discrete and decreased along with time.The decline level of dPTH among different groups had statistical difference.Conclusions It is a promising method to evaluate the in situ reserves and function of the parathyroid by monitoring the level and changes of dPTH after thyroid surgery,and it is of value for preventive calcium supplementation after thyroid surgery.

Objective To apply intervention of integrated pharmaceutical care (IPC) for asthma-COPD overlap syn-drome patients,so as to reduce the side effects of drugs,enhance medication compliance,promote reasonable drug application,cut down the medical expenses in ACOS patients. Methods A total of 60 ACOS patients were randomly divided into IPC group (group A) (n=34) and contrast group(group B) (n=26).The patients in group A were given IPC measurements such as noso-comial guidance,classroom teaching,regular follow-up,life coaching and psychological advice.While the patients in group B were not given any intervention measures. Results In group A,patients' awareness rate of action and side-effects of drugs were ob-viously increased;Knowledges of inhalation preparation were greatly improved;the ratio of ADRs was significantly reduced;The FEV1 and the value added of FEV1 was dramatically improved.Furthermore,the differences showed statistical significance as com-pared with group B(P<0.05).Total medical costs and anti-bacterial drug costs per year were significantly lower in group A than group B. Conclusion IPC is beneficial to enhance drug compliance,promote reasonable drug application and bring down the medical expenses in ACOS patients.

Objective To investigate the prevalence of pertussis in infants and children with persistent cough in Beijing during 2011-2016.Methods The eligible infants and children from over ten hospitals who were suspected to have pertussis from 2011 to 2016 were enrolled for detection.Nasopharyngeal secretions and blood samples were collected.Multiplex-PCR was performed for Bordetella pertussis and real-time PCR was performed for nucleic acid of Bordetella pertussis.Results A total of 1 318 eligible cases were enrolled,including 820 males and 498 females.Pertussis was detected positive in 534 cases,including 81.3％ (434/534) of B.pertussis positive cases and 31.8％ (170/534) of IgG positive cases.There were 13.1 ％ (70/534) had double positive for bacteria and antibodies.From 2011 to 2016,the enrolled patients were increased from 103 cases per year to 460 cases per year,and the test positive patients were increased from 29 cases to 194 cases.Among the pertussis patients,466 (87.3 ％) cases were younger than one year old.From the first quarter to the fourth quarter of the year,There were 65 cases,151 cases,205 cases,and 113 cases,respectively.In further analysis of the 268 cases from Children's Hospital affiliated to Capital Institute of Pediatrics,90.7％ of the patients who had whooping cough were scattered children;185 cases (69.0％) of the patients had not begun programmed immunization,71 cases (26.5％) did not complete programmed immunization and 12 cases (4.5％)completed the programmed immunization.Of all the inpatients,21.6％ were critical ill,0.8％ (2 cases) dead,and the remaining patients were recovered and discharged.Conclusions The prevalence of pertussis is increasing,especially in summer.Infants are the most susceptible population.Bordetella pertussis is one of the most important pathogen that can induce persistent and chronic cough.

Objective To explore the effects of different degrees of intermittent hypoxia (IH) on the activation and the secretion of extracellular matrix in MLg lung fibroblast cell line. Methods MLg lung fibroblast cells in logarithmic growth phase were exposed for 5%O2 for 100 seconds and 21%O2 for 120 seconds in 1 h, 4 h and 8 h groups (IH1, IH4 and IH8) and normoxia group (21%O2 for 8 h, N group). The cells in each group were collected at the end of experiment. Real-time PCR was used to measure the mRNA expression levels ofα-SMA and typeⅠcollagen (COL1) A1, and Western blot assay was used to detect the protein expression levels ofα-SMA and COL1. Results The mRNA and protein expression levels ofα-SMA and COL1 were significantly increased in IH1, IH4 and IH8 groups than those in N group (all P < 0.05). Furthermore, expression levels of α-SMA and COL1 showed a time-dependent increase with IH exposure time. Conclusion The intermittent hypoxia can promote the cell activation and the extracellular matrix secretion of mouse lung fibroblast cells, which may be related with the oxidative stress.

Objective To study the epidemiological and clinical characteristics ofpertussis in infants younger than three months.Methods Infants younger than three months were enrolled from January 1,2011 to December 31,2015 with one or more of the following symptoms:persistent cough,spasmic cough,cyanosis of unknown causes,asphyxia and apnea.Multiplex polymerase chain reaction(PCR) assay was performed to identify Bordetella pertussis and enzyme-linked immunosorbent assay was used to detect antibody to pertussis toxin.Clinical features,complications,treatments and prognosis of the infants confirmed with pertussis were analyzed.Results Altogether 202 cases were enrolled in the five years,and 59 (29.2％) of which were positive for pertussis confirmed by multiplex PCR.Among the 59 cases,37 were boys and 22 were girls.The youngest baby was 13 days and the oldest one was 85 days.Length of stay ranged from 7 to 21 days.Twelve cases had a contact history with family members having chronic cough.Symptoms occurred in spring or summer in 46 cases (78.0％),and in autumn or winter in 13 (22.0％) cases.Symptoms of spasmic cough,cyanosis after coughing,vomiting after coughing and conjunctival hemorrhage were respectively found in 41 (69.5％),36 (61.0％),39 (66.1％)and 33 (55.9％) cases,while only six (10.2％) presented with inspiratory whooping sound on coughing.Fortynine cases (83.1％) showed increased lymphocyte count (≥ 10 × 109/L).Twenty-eight cases (47.5％) developed severe pertussis.Complications including apnea and bradycardia after coughing,respiratory failure and heart failure,pertussis encephalopathy as well as highly increased leucocyte count (≥ 60× 109/L) occurred in 23 (39.0％),18 (30.5％),five (8.5％) and four (6.8％) cases,respectively.Twenty-four cases with severe pertussis required respiratory support,of which six received invasive ventilation and 18 received non-invasive ventilation.Fifty-eight infants were recovered and discharged,while one baby died.Conclusions Bordetella pertussis infection is an important cause of persistent cough in unimmunized infants under three months of age.The symptoms of pertussis in infants are untypical,but the incidence of severe pertussis is high.Thus early diagnosis and timely treatment are necessary.