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Objective To analyze the clinical characteristics of thyroid dysfunction induced by camrelizumab in the treatment of classical Hodgkin lymphoma(cHL)and explore the influencing factors.Methods The medical records of cHL patients treated with camrelizumab from January 1st,2017 to December 31st,2020,were collected.The clinical characteristics of thyroid dysfunction induced by camrelizumab were analyzed,and the influencing factors of adverse drug reactions(ADRs)were discussed.Results A total of 47 patients were included,12 patients(25.53%)experiencing thyroid dysfunction after receiv-ing camrelizumab.Among them,3 patients had hypothyroidism,7 had subclinical hypothyroidism,and 2 had subclinical hyperthy-roidism.The severity of ADRs was between grade 1 and 2(mild to moderate).None of the patients with thyroid dysfunction dis-continued camrelizumab.Thyroid dysfunction occurred between 1 and 22 months after camrelizumab treatment,with a median time of 6 months.2 patients with hypothyroidism treated with levothyroxine,the thyroid function returned to normal in 1 patient and was improved in the other.1 patient with subclinical hypothyroidism was treated with levothyroxine with the thyroid function continued to be abnormal.The rest of the 9 patients with thyroid dysfunction received no intervention,and 4 of them returned to normal,and others remained with no obvious change or loss in follow-up.Thyroid dysfunction was associated with baseline thyroid stimulating hormone level(P=0.03).The level of thyroglobulin antibodies and thyroid peroxidase antibodies was increased in 2 patients a-mong 3 patients with moderate thyroid dysfunction.Conclusion The incidence of thyroid dysfunction induced by camrelizum-ab in cHL was high.Camrelizumab-induced thyroid dysfunction was related to TSH baseline level(P=0.03).The severity was between grade 1 and 2,which was related to the level of TgAb and TPOAb.The subtype of thyroid dysfunction was mainly sub-clinical hypothyroidism with a long period.Patients could continue camrelizumab treatment,and the thyroid function of patients with grade 2 hypothyroidism was improved after the treatment with levothyroxine.
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Objective:To summarize the clinical phenotype and genotypic characteristics of children with truncation variation in SMC1A gene. Methods:The clinical data of a child with late-onset cluster seizures caused by truncation variation in SMC1A gene diagnosed in February 2021 in Children′s Hospital Affiliated to Zhengzhou University were collected. The relevant literature was reviewed to summarize the clinical characteristics. Results:The proband was a 5-year-old girl, presenting with first seizure at the age of 5 and cluster seizures. She had poor response to multiple antiepileptic drugs, and had normal neurodevelopment before seizures. Whole exome sequencing results revealed a spontaneous heterozygous nonsense variation c.55C>T in SMC1A gene, causing a nonsense variant in the amino acid sequence p.Gln19Ter(p.Gln19 *), which has not been reported. There were a total of 14 relevant literatures, and there were in total 32 cases with truncation variation in SMC1A gene including this case. All children were female and 30 children had early-onset intractable epilepsy, and first seizure median age was 5 months (range: 4 weeks to 40 months); 78.1% (25/32) of them had cluster seizures; 93.8% (30/32) had mental retardation; Cornelia de Lange syndrome clinical score in 68.8% (22/32) of them was≥4. The truncation variations in SMC1A gene of 31 children were de novo, and there were 16 children with frameshift variation (16/32), 12 children with nonsense variation [12/32; 3 children (9.4%, 3/32) with c.2923C>T], 4 children with splice variation (4/32). Conclusions:This study further expands the clinical phenotype and genotype of cases with truncation variation in SMC1A gene. Case presenting with female late-onset cluster seizures has not been reported in China, and genetic testing can be beneficial for early diagnosis of hereditary epilepsy and precision treatment.
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Objective:To investigate the clinical characteristics of patients with combined oxidative phosphorylation deficiency type 4 (COXPD4) related to TUFM gene variation, in order to improve clinicians′ understanding of the disease. Methods:A case of COXPD4 with cystic leukodystrophy admitted to the Children′s Hospital of Zhengzhou University in June 2021 was taken as the study subject, and her clinical characteristics and genetic testing results were retrospectively analyzed. The "combined oxidative phosphorylation deficiency type 4" " TUFM gene" "cystic leukodystrophy" "combined oxidative phosphorylation deficiency 4" "COXPD 4" " TUFM" and "cystic leukodystrophy" were used as keywords, and the documents on COXPD4 related to TUFM gene mutations were reviewed from Wanfang Data Knowledge Service Platform, CNKI, PubMed Document Database, and National Center for Biotechnology Information (NCBI) until August 2021. The COXPD4 patients that have been reported internationally were analyzed for clinical features and variant types. Results:The patient was a 2-month-old girl with clinical manifestations of delayed development and progressive aggravation, elevated lactic acid in serum and cerebrospinal fluid, and diffuse white matter dysplasia with multiple cystic lesions in cerebral magnetic resonance imaging (MRI). Whole exome sequencing showed TUFM gene complex heterozygous variants c.684_684+4delGGTGA and c.1105C>T, which had not been reported in the past. A total of 5 cases of COXPD4 were reported in 4 English literatures. Together with 1 case in this study, there were 4 cases with detailed clinical history data, including 1 male and 3 females. The clinical manifestations were severe early-onset lactic acidosis and developmental lag, and 3 cases were accompanied by progressive infantile encephalopathy. Among them, 3 cases underwent head MRI examination, all of which showed diffuse white matter signal with multiple cystic lesions, 2 cases with basal ganglia involvement and multiple cerebellar gyri deformity. Genetic test indicated different types of TUFM gene variation. Conclusions:COXPD4 is a rare hereditary mitochondrial disease. For cases with COXPD4 clinical and imaging features, TUFM gene mutations can be screened first.
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Objective:To investigate the clinical phenotype and genotypic characteristics of Legius syndrome.Methods:The clinical data of a child with precocious puberty and scattered café-au-lait macules admitted to Department of Neurology of the Children′s Hospital Affiliated to Zhengzhou University in July 2021 were retrospectively analyzed. Trio-whole exome sequencing (trio-WES) was used for genetic analysis to confirm the molecular diagnosis of the family. The relevant literature was reviewed to summarize the clinical characteristics of the disease.Results:The proband was a 10-year and 9-month-old girl, presenting with more than 5 café-au-lait macules with diameter>5 mm on the face and trunk, freckles in the axillary, without Lisch tubercles of iris and tumor signs of neurofibromatosis type 1, diagnosed as central precocious puberty at the age of 8. trio-WES results of the family revealed a spontaneous heterozygous nonsense mutation c.751(exon7) C>T in SPRED1 gene, causing a nonsense mutation in the amino acid sequence p.Arg251Ter (p. Ter251 *). Literature review showed a total of 88 pathogenic mutations were reported in SPRED1 gene, including frameshift mutations (41/88), nonsense mutations (31/88), splice mutations (7/88), missense mutations (6/88), and others (3/88), and no mutational hotspots were found. Clinical phenotype was as follows:>5 café-au-lait macules accounted for 92.8% (168/181), armpit and inguinal freckles 43.5% (73/168), macrocephaly 21.4% (31/145), learning disability 18.0% (30/166), psychomotor retardation 13.8% (22/159), lipoma (adult) 13.7% (21/153), Noonan facial sign 12.1% (21/173), and tumor phenotype of neurofibromatosis type 1 was not reported. Conclusions:The central precocious puberty phenotype of Legius syndrome was not reported in China. The clinical phenotype of Legius syndrome was mild, with a large variation, but without neurofibromatosis type 1 tumor phenotype. Genetic testing can be beneficial for early diagnosis of Legius syndrome.
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Objective:To explore the relationship between body mass index (BMI) and clinical characteristics, imaging characteristics and prognosis of patients with primary brainstem hemorrhage (PBH).Methods:The clinical data of 114 PBH patients admitted in 909th Hospital of PLA (the Affiliated Dongnan Hospital of Xiamen University) from February 2016 to October 2019 were retrospectively analyzed. According to the BMI, the patients were divided into the normal group (BMI<24 kg/m 2, 71 cases) and the overweight/obese group (BMI≥24 kg/m 2, 43 cases). The clinical and imaging characteristics of the two groups were compared, and the factors affecting the prognosis of the PBH patients were analyzed by univariate analysis and Cox multivariate regression model. Results:More patients in the overweight/obese group had histories of hypertension (86.05% vs. 69.01%, P = 0.041), diabetes (37.21% vs. 9.86%, P<0.01), and heart disease (20.93% vs. 5.63%, P = 0.013) than the normal group. The systolic blood pressure, diastolic blood pressure, blood glucose, LDL, TG, WHR, and the incidences of coma, convulsions, central fever in overweight/obese group were significantly higher than those in normal group: (172.83±26.15) mmHg (1 mmHg = 0.133 kPa) vs. (159.28±31.67) mmHg, (98.36±15.57) mmHg vs. (89.52±13.26) mmHg, (8.64±1.53) mmol/L vs. (7.95±1.28) mmol/L, (3.51±1.29) mmol/L vs. (2.95±1.04) mmol/L, (5.94±2.27) mmol/L vs. (2.19±0.86) mmol/L, 1.29±0.21 vs. 2.19±0.86, 48.84% vs. 25.35%, 30.23% vs. 14.08%, and 44.19% vs. 23.94%, the GCS score was significantly lower than that in the normal group: (4.69±1.87) scores vs. (6.37±1.52) scores, and there was statistical difference ( P<0.05 or<0.01). Compared with the normal group, there were significant differences in the bleeding site ( χ2 = 5.64, P = 0.010), bleeding volume ( χ2 = 5.35, P = 0.013), combined ventricular hemorrhage ( χ2 = 9.21, P = 0.002) and hydrocephalus ( χ2 = 4.79, P = 0.029). Multivariate analysis showed that BMI ( OR = 4.11, 95% CI 3.84 to 4.64, P = 0.039), history of hypertension ( OR = 8.71, 95% CI 6.28 to 9.52, P = 0.021), GCS score ( OR = 0.05, 95% CI 0.03 to 0.12, P = 0.012), bleeding volume ( OR = 3.09, 95% CI 2.16 to 4.03, P = 0.041), combined ventricular hemorrhage ( OR = 4.34, 95% CI 2.96 to 5.12, P = 0.023) and hydrocephalus ( OR = 6.40, 95% CI 5.63 to 6.89, P = 0.027) were all independent risk factors affecting the prognosis of PBH patients. Conclusions:BMI has a greater impact on the prognosis of PBH. Overweight/obese PBH patients also have hypertension history, low GCS score, large amount of bleeding, ventricular hemorrhage, hydrocephalus, etc., which should be paid more attention to by clinicians, positively control the progress of the disease and improve the prognosis of patients.
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Objective:To report a rare case of early onset epileptic encephalopathy caused by YWHAG gene mutation, and discuss the clinical and genetic characteristics as well as the diagnosis, treatment and prognosis of the disease.Methods:Clinical data of the patient with YWHAG gene deficiency from Department of Neurology, Children′s Hospital Affiliated to Zhengzhou University were collected in January 2018. The whole exome sequencing was performed on the core members of the family, and the characteristics of gene mutations were analyzed.Results:The proband is a girl, three years and 10 months old, presented to the outpatient department of neurology with a history of six-month intermittent convulsions, manifested as epilepsy seizures, mental retardation, motor delay and gait instability, ataxia. The brain magnetic resonance imaging showed myelinated dysplasia, and long-term video electroencephalogram (EEG) showed extensive 1.5-3.0 Hz slow spikes, and multiple spikes during sleep. During the monitoring, the children had clinical seizures and abnormal EEG discharges, indicating that myoclonus was accompanied by atypical absence of consciousness. Whole exome sequencing on the proband detected a de novo mutation c.169C>T (p.Arg57Cys) in YWHAG gene. According to American College of Medical Genetics guidelines (2015), the mutation was considered potentially pathogenic.Conclusion:Early epileptic encephalopathy caused by YWHAG gene mutation is very rare, and the variation of YWHAG gene c.169C>T is the possible pathogenic variation of the genetic cause of early onset epileptic encephalopathy in the proband.
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Objective:To analyze the clinical and imaging characteristics of acute necrotic encephalopathy (ANE) in a child with human herpesvirus-6 (HHV-6) infection.Methods:Retrospective analysis was performed on the clinical data and imaging features of a case of HHV-6 related ANE from Children′s Hospital Affiliated to Zhengzhou University in March 2019.Results:The one year and seven month-old child had acute encephalopathy, recurrent convulsions, consciousness disorders, elevated serum transaminase. The number of cerebrospinal fluid (CSF) cells was normal and the protein increased. High throughput gene testing of CSF showed HHV-6. Cranial magnetic resonance imaging showed multiple symmetry damage in the bilateral thalamus, brainstem, and cerebellum. The symptoms improved after the treatment of glucocorticoids, intravenous immunoglobulin, and plasmapheresis.Conclusions:ANE is a rare severe encephalopathy, the characteristic imaging change of which is symmetry multifocal cerebral damage, especially in the bilateral thalamus. ANE should be considered for patients with frequent convulsions and disturbance of consciousness after virus infection.
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Objective:To investigate the clinical characteristics and gene mutation of seven cases of CDKL5 gene related early-onset epileptic encephalopathy diagnosed by next-generation sequencing.Methods:The clinical data of children with early-onset epileptic encephalopathy from February 2018 to December 2019 in the Department of Neurology, Children′s Hospital Affiliated to Zhengzhou University were retrospectively analyzed. The whole exome sequencing method was used to analyze the entire exome of the proband, and seven cases of CDKL5 gene mutation positive were screened out, and Sanger sequencing verification on family members was performed to identify the source and the characteristics of gene mutations were analyzed.Results:Among the seven children diagnosed with CDKL5 gene related early-onset epileptic encephalopathy, the ratio of male to female was 2∶5, and the age of onset was 15 days to five months of birth. The clinical phenotypes all included different degrees of developmental delay and repeated seizures, which were manifested as general seizures, myoclonic seizures, convulsive seizures or focal seizures; the outcome of use of antiepileptic drugs to control seizures was poor, and some applications of ketogenic diet had better effects. CDKL5 gene mutation sites were all denovo mutations, including NM_003159: c.772_776del (p.K258Efs *10) frameshift mutation, NM_003159.2 (exon: 9-15) heterozygous deletion, CDKL5 hemizygous deletion, NM_003159: c.268 (exon5) G>T (p.E90 *, 941) and NM_003159: c.2578C>T (p.Q860 *, 171) nonsense mutation, NM_003159: c.211A>G (p.Asn71Asp) and NM_001323289: c.545T>C (p.L182P) missense mutation. Among them, c.772_776del (p.K258Efs *10), c.268 (exon5)G>T and c.2578C>T (p.Q860 *, 171) have not been reported. Conclusions:CDKL5 gene related early-onset epileptic encephalopathy is an early onset epilepsy, which is more common in women, and has different forms of seizures. The early electroencephalogram is characterized as severe abnormal brain discharge, and the disease progresses in various forms. There are no specific changes in head magnetic resonance imaging. Different gene mutation sites may lead to different phenotypes and prognostic differences. Many anti-epileptic treatments are ineffective, and ketogenic diets are effective for some patients.
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Objective The experiment project was designed to explore the variation of NaI (Tl) gamma spectrometer channels with environmental temperature. 60Co and 152Eu were used to verify the reliability of the correction methods. Methods Two correction methods were applicated, which were curve fitting correction method and known measurement peak correction method. Results The experimental results showed that temperature changes had an effect on NaI (Tl) measured spectra peak. The relative peak will shift to the right at 5℃ by 9.6%, and to the left at 60℃ by 16%, with the reference temperature set at 25℃. The two methods are based on the channel change due to temperature changes, and they could effectively correct the temperature peak-drift. Conclusion In order to make the measured spectrum information accurate and reliable in field monitoring, it is suggested to monitor the environmental temperature so as to correct the measured data.
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Objective:To summarize the clinical features of a family of neurofibromatosis type I (NF1) and its NF1 gene mutation characteristics. Methods:The clinical data of a family of NF1 admitted to our hospital in May 2020 were collected. The proband was sequenced with NF1/ NF2 panel using second-generation sequencing. Sanger sequencing verification analysis was performed on the family members. The clinical characteristics of the proband and other family members were summarized and their gene mutations were analyzed. Results:The proband (V 2), a 1-year and 2-month old girl, had multiple Café au lait spots on the skin at birth, normal mental and motor development, and focal epileptic seizures in infancy. The father (IV 3), grandmother (III 2), and other 2 family members (II 2, I 2) in the family all had Café au lait spots or neurofibromatous changes without seizures. The mother's phenotype was normal. The proband had a heterozygous mutation in the NF1 gene, and the mutation site C.83-84delAG (P.N29Hfs*8) was a frameshift heterozygous mutation. After verification analysis by Sanger sequencing, the pathogenic genes of the father and grandmother were consistent with the proband, which was in line with the characteristics of heterozygous mutation in NF1 gene, dominant inheritance. Conclusion:NF1 is caused by NF1 gene mutation; the early clinical manifestations mostly include café-au-lait spots, and some have seizures; patients with multiple café-aulait spots with seizures should be diagnosed by genetic analysis as soon as possible.
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Objective:To investigate the clinical phenotype and summarize the genetic characteristics of children with neurofibromatosis type 1 (NF1) diagnosed by next-generation sequencing.Methods:The clinical data of 12 children with NF1 who were admitted to the Department of Neurology of the Children's Hospital Affiliated to Zhengzhou University from December 2017 to October 2019 were retrospectively analyzed. The next-generation sequencing method was used to sequence the NF1 gene of the probands and the mutations were verified by PCR-Sanger sequencing.Results:Among the 12 children diagnosed with NF1 (male: female=11: 1), who aged from seven months to 11 years old, the main complaints were seizures and skin with café-au-lait spots. Five children were found with freckles in axillae, and two with cutaneous neurofibroma. Six cases had seizures, two children suffered spastic seizures, two with generalized tonic-clonic seizures, one with typical absence seizure, one with focal seizure, one case had severe headache and vomiting. Fortunately for the children with seizures, anti-epileptic drugs had a good prognosis. There were five mutation types detected in 12 cases, including one case of loss of overall heterozygosity in NF1 gene; three missense mutations: c.7867C>A (p.L2623I), c.7855C>A (p.L2619I) and c.7792C>A(p.L2598I); three frameshift mutations: c.3162delC(p.N1054Nfs *8), c.540dupA (p.Q181Tfs *20) and c.2027dupA(p.V679Pfs *21); three nonsense mutations: c.1467T>A(p.Y489X, 2351), c.1318C>T(p.R440X, 2400) and c.1411C>T(p.K471X, 2369); two splicing mutations: c.2326-2(IVS10)G>C and c.1186-1(IVS10)G>C. Nine children were found with spontaneous mutations, one case was inherited from the father, and two cases were inherited from the mother. c.7867C>A(p.L2623I), c.7855C>A(p.L2619I), c.3162delC(p.N1054Nfs *8), c.1411C>T(p.K471X, 2369), c.2326-2(IVS10)G>C, c.1186-1(IVS10)G>C were unreported mutations in literature. Conclusions:NF1 is caused by NF1 gene mutation. The early clinical manifestations of children with NF1 defect presented with café-au-lait spots, and some suffered seizures. For patients with multiple café-au-lait spots and seizures in the clinic, genetic analysis should be performed as soon as possible to confirm the diagnosis.
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Objective@#To assess clinical and radiographic outcomes of short implants (length six mm) in the posterior region and early-loading with splinted fixed dental prostheses.@*Methods@#Forty-five patients were recruited at 3 centers and 2-3 implants (diameter 4 mm, length 6 mm) were implanted in each case. Totally, ninty-five implants were placed, using one-stage surgery protocol and loaded with a screw-retained splinted fixed prosthesis 6 weeks later. Clinical and radiographic examinations were performed preoperatively, post-surgery, at loading, and 6, 12 and 24 months after prosthesis placement.@*Results@#Four implants were removed before loading. A total of 36 patients with 76 implants completed the whole treatment and 2 year follow-up according to the research design. No implant was lost after loading, and the 2-year survival rate of the implant was 100%(76/76). In all cases, the response of swelling and pain after operation was mild, and the bone resorption around the implants was not obvious at 6 months [(0.04±0.29) mm], 1 year [(-0.11±0.44) mm], and 2 years [(0.00±0.53) mm] after loading. Bone loss less than 1.00 mm was found only in 26% (18/69) implants after 2 years of loading, and the height of the alveolar bone around 17% (12/69) of the implant increased. During 2-year follow-up, the probing depth of the implants increased by nearly 0.50 mm, but there was no clinical significance (P>0.05).@*Conclusions@#The 2-year results of this study showed that the 6-mm-long implants can be a predictable procedure, especially in situations with limited bone height in posterior edentulous regions.
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Objective To study the clinical characteristics and treatment of neurofibromatosis type Ⅱ (neuro-fibromatosis type 2 ,NF2 ,bilateral acoustic neuroma) ,and the effects of auditory brainstem implant for treating to-tal deafness after bilateral acoustic neuroma resection .Methods One case of bilateral acoustic neuroma and all clini-cal data in terms of diagnosis ,treatment and hearing -speech rehabilitation after surgery were retrospectively stud-ied .Results The patient was a thirteen years old boy .His clinical symptoms were hearing loss on the right ear ,tin-nitus ,hoarseness and gait instability three years .MRI showed space occupying lesion in the cerebellopontine angle . The postoperative pathological diagnosis was bilateral acoustic neuroma .The initial switch -on was peformed six weeks after the surgery ,and confirmed that all electrodes generated listening responses .As the extension of recov-ery time ,the correct recognition rate of patients on the natural environment sound ,vowel ,monosyllabic were on the rise and the pure tone hearing threshold gradually decreased .The vowel correct recognition rate of postoperative 6 , 9 ,12 ,24 ,and 36 months were 14% ,18% ,20% ,24% ,and 20% ,respectively .The recognition rate of monosyl-labic and open words at each postoperative rehabilitation stage were 0 .Conclusion The clinical characteristics and treatment of bilateral acoustic neuroma were different from the unilateral acoustic neuroma .The individualized treat-ment should be followed .Auditory brainstem implant could be performed in patients with post - bilateral acoustic neuroma resection .The accurate location of the cochlear complex during the surgery was the crucial point for the success of ABI .
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Objective To investigate the effect of autophagy response on neurological functions and the role of mitogen-activated protein kinases (MAPKs) signaling pathway in rats after traumatic brain injury (TBI).Methods Fifty-four healthy male SD rats were randomly divided into sham-operated group,TBI group and TBI+autophagy inhibitor 3-methyladenine (3-MA) group (n=18).TBI animal models of the later two groups were established using Feeney's method.Rats in the sham-operated group were only performed bone window opening without knock;rats in the TBI+3-MA group were given intraperitoneal injection of 3-MA(5 mg/kg) 30 min after modeling and rats in the other two groups were given the same volume of normal saline.Three and 7 d after modeling,the protein levels of S100B and neuron specific enolase (NSE) in serum were tested with enzyme linked immunosorbent assay (ELISA);modified neurologic severity scale (mNSS) was used to detect the movement,sense and reflex functions;brain water content was measured with wet-dry weight method.The autophagy related factors (LC3-Ⅱ and Beclin-1) and MAPKs signaling pathway related factors (c-Jun N-terminal kinase [JNK],phosphorylated [p]-JNK,extracellular signal-regulated kinase [ERK]1/2,p-ERK1/2,p38MAPK and p-p38MAPK) protein expressions in TBI cerebral cortex were determined by Western blotting.Results As compared with those in the sham-operated group,the brain edema level,mNSS scores,and S100B and NSE protein levels in the TBI group and TBI+3-MA group were significantly increased (P<0.05);TBI+3-MA group had significantly lower brain edema level,mNSS scores,and S100B and NSE protein levels than TBI group (P<0.05).The expression levels of autophagy and MAPKs signaling pathway related factors in the TBI group and TBI+3-MA group were significantly higher as compared with those in the sham-operated group (P<0.05).As compared with the TBI group,TBI+3-MA group had significantly decreased levels of LC3-Ⅱ,Beclin-1 and activation of JNK and p-p38MAPK signaling pathways (P<0.05).Conclusion Suppressing autophagy response markedly improves neurological outcomes after TBI,possibly mediated by inhibiting activation of JNK and p38MAPK signaling pathways.
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Objective To investigate the effects of omega-3 polyunsaturated fatty acids (ω-3 PUFA) supplementation on neuron apoptosis,brain edema,activation of microglia,inflammatory response and neural function after traumatic brain injury (TBI) in rats,so as to understand the protection of ω-3 PUFA in rats following TBI and its mechanism.Methods TBI model was established using Feeney's method.Ninety SD rats were randomly divided into 5 groups:sham operation group (sham group),TBI group,TBI + selective activator of c-Jun N-terminal kinase (JNK) anisomycin group (TBI + Aniso group),TBI + ω-3 PUFA supplementation group (TBI + ω-3 group),and TBI + ω-3 PUFA supplementation + JNK activation group (TBI + ω-3 + Aniso group).We measured rat behavioral outcomes by modified neurological severity score (mNSS) on day 1,3,and 7 after TBI.Brain water content was measured with wet-dry weight method.The neuron apoptosis and microglial activation (identified by specific marker IBA-1) in TBI cerebral cortex were determined by TUNEL staining and immunofluorescence.Inflammatory cytokines [tumor necrosis factor-α (TNF-α),interleukin (IL)-1α,IL-1β,and IL-6] and the JNK signaling pathway (JNK,pJNK) were tested with reverse transcription-polymerase chain reaction and Western blot,respectively.Results Compared with the sham group,the levels of brain cell apoptosis,brain edema,neuron apoptosis,and inflammatory-relatived factors (TNF-α,IL-1 α,IL-1β,and IL-6) were significantly increased in the other four groups (P < 0.05).Compared with the TBl group,ω-3 PUFA supplementation reduced brain water content following TBI,especially on day 3 after TBI [(78.14 ± 0.57) % vs.(82.31 ± 0.81) %,P < 0.01],and improved neurological function score (P < 0.05).Meanwhile,ω-3 PUFA supplementation suppressed neuron apoptosis,the activation of microglia,and the mRNA and protein expressions of inflammatory cytokines (TNF-α,IL-1α,IL-1 β,IL-6).The activation of JNK signaling pathway was also inhibited by ω-3 PUFA.Conclusion ω-3 PUFA supplementation may markedly reduce brain edema,suppress neuron apoptosis,and improve neurological outcomes after TBI in rats,possibly mediated by inhibiting JNK signaling pathway and microglial activation,reducing microglia-induced cerebral inflammatory responses,demonstrated as down-regulated expression of TNF-α,IL-1α,IL-1β,and IL-6.
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ObjectiveTo discuss the clinical features, diagnosis and treatment of mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome in children.Methods The clinical features and treatment process of two children with MELAS were retrospectively analyzed.ResultsThe main clinical features of MELAS were stroke-like epi-sodes, seizure, visual anomaly and lactic acidosis. Cephalic MRI ifndings performed during episode periods were in accord with the typical radiographic features of MELAS. Gene testing on the two children and their mothers showed the point mutation of A3243G in mitochondrial genome. The symptoms were improved signiifcantly after energy supply and corticosteroid treatment. Conclusions MELAS syndrome is easy to be misdiagnosed due to the varied clinical features. The diagnosis depends on the musclebiopsy and gene testing. Corticosteroid therapy is effective for MELAS syndrome.
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Objective:To prepare a bone substitute using microsphere scaffold containing adiponectin(APN)and to investigate the release behavior of the scaffold in vitro.Methods:Chitosan microsphere was developed by an emulsion-ionic cross-linking method. Poly (L-lactic-co-glycolic)acid (PLGA)and β-tricalcium phosphate (β-TCP)were used to prepare microsphere scaffold containing APN.The morphology,particle size,drug loading,incorporation efficiency and release behavior of the microsphere were examined. Results:The APN containing microsphere showed good spherical geometry,suitable size and microporosity under scanning electron microscope.The average diameter of the milipore was 20 -200 μm;the drug loading and incorporation efficiency were 1 .3% and 70.3% respectively.The controled-release process continued for 91 days.The extract solution from the APN microsphere-scaffold promoted MC3T3 cell proliferation without cytotoxicity.Conclusion:The APN microsphere-scaffold has sustained release function and may promote osteoblast proliferation.
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Objective:To understand the mode and effect of the consultations involved clinical pharmacists, and discuss the work-ing process and mode of the consultations. Methods:Based on the practical work and team discussion, the flow chart and consultation registration form for the clinical pharmacist consultations were established and applied. Totally 410 cases of clinica1 pharmacist consul-tations were retrospectively investigated from March 2009 to December 2012. Results:The consultation flow chart was strictly followed by the clinical pharmacists in our hospital, and the obvious effect was shown. The number of consultation was increased from 59 cases in 2009 to 122 cases in 2013. In all the consultation cases, 21 departments were involved, including the medical departments and the surgery departments. Among the cases,the adjustment in antibacterial drug treatment was the main requirements. Meanwhile, the a-doption rate of the clinical pharmacist suggestions was 95. 12%. Conclusion:Clinical pharmacists are playing an important role in clin-ical treatment. The consultation work can be regulated by the consultation flow chart and registration form with the improved adoption rate and consultation quality.
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Objective To compare the clinical performance of the self-etching and the phosphoric acid etching adhesive system for restoration of non-carious Class V restorations for a period of two years.Methods There were 37 patients with at least one pairs of similar-sized non-carious cervical lesions participated in the study.120 restorations were placed by one operator,of which 60 with phosphoric acid etching adhesive system(Spectrum TPH,Prime& Bond)and 60 with self-etch(Clearfil AP-X,SE Bond).The restorations were evaluated by ?2 statistics at the baseline,6th,12th and 24th month according to the modified Ryge"s USPHS criteria.Results At two years,106 teeth were reviewed in 33 patients.The retention rates for self-etching adhesive system were 89% and for phosphoric acid etching adhesive system 77%.The percentage of the retention rates of both adhesive systems was not found to be different when calculating the failure rates.No cases in both adhesive systems showed slight marginal discoloration problems.Some cases for each adhesive system had slight color change after the same period.Conclusions The performance of both adhesive systems is excellent during this two-year clinical trial.The self-etch adhesive system exhibites slightly better coloar match than the phosphoric acid etching system.
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Objective:To evaluate the treatment effects of the half-columnar shaped mandibular block bone onlay grafting technique for augmentation of the resorbed maxillary anterior alveolar ridge after single tooth missing.Methods: A total of 15 sites of 14 patients received ridge augmentation surgeries.The recipient sites were prepared with trephines,the half-columnar shaped bone blocks were harvested from the ramus and external oblique ridges with trephines according to diameters of the recipient sites.The bone blocks were placed as lateral onlay grafts on recipient beds and secured by means of titanium screws.Particulate bone was added and absorbable membranes were used to stabilize and protect the grafts.After a mean interval of 4.5 months of healing the flaps were re-opened,the screws were removed and non-submerged implants were placed.The width and height of the alveolar ridges were recorded.After 3 months,implant-supported crowns were provided to the patients.One year later,the peri-impant condition and the marginal bone resorption on the proximal sites were observed.Results: Mean lateral augmentation obtained at the time of bone grafting was(3.8?0.8) mm(x?s),5 out of 15 sites exhibited a mean of 3 mm of vertical augmentation.The mean healing time was 4.5 months,the mean percentage of horizontal and vertical bone resorption in the mean time were 8% and 7% respectively.No major complications were recorded at donor sites.No implant was lost during the study period.Clinical parameters and probing depth(≤4 mm) demonstrated the presence of a healthy peri-implant mucosa after 1 year of prosthetic reconstruction.The clinical and radiographic bone observations showed no more than 1.2 mm of resorption after bone graft and implant placement.Conclusion: The half-columnar shaped mandibular bone graft(from the ramus and external oblique ridge) is a promising technique for bone augmentation in localized alveolar ridge defects after single tooth missing.This procedure offers easy access,good bone quantity for localized repair,low morbidity,decreased complaints of postoperative sensory disturbances or discomfort,minimal graft resorption,and a shorter healing time as compared with other methods for bone repair.