OBJECTIVE TTo evaluate the clinical manifestations of the laryngeal amyloidosis and its laryngoscopic characteristics.METHODS The clinical data of 11 cases with laryngeal amyloidosis confirmed pathologically was studied retrospectively. RESULTS There were 4 males and 7 females in this study.The age of the patients ranged from 16 to 64 years old (average 43.36?4.16).The duration of symptoms was 4 months to 10 years (average 2.85?0.87 years).Hoarseness presented in 10 patients (90.90%) and dyspnea in 4 patients.The false vocal cord was involved in 8 patients,laryngeal ventricle in 6 patients,true vocal cord in 5 patients and subglottic area in 3 patients observed with fibrolaryngoscope. The appearance of the lesions was mainly described as waxy yellow or yellow-gray submucosal plaques and nodules without ulceration of the overlying mucosa.The laryngostroboscopy demonstrated that the wave of the mucosa and vibration of the involved vocal cords were adynamic or disappeared.CONCLUSION The chief complaint of the laryngeal amyloidosis was hoarseness. Laryngeal amyloidosis is a slowly progressive disease. The lesions commonly involve the false vocal cord, laryngeal ventricle,true vocal cord and subglottic area.

ObjectiveTo investigate the correlation of MRI features and phenotypes and genetic mutations in Pelizaeus-Merzbacher disease.Methods Sixteen boys with clinical diagnosis of PelizaeusMerzbacher disease (PMD) were included in this study.Their ages ranged from 22 months to 9 years.They were examined by pediatric neurologists,and clinical classification was made according to the symptoms and physical signs.An experienced radiologist reviewed the cranial MRI images and analyzed the brain involvement,including pallidus globus,pyramidal tract,corpus callosum,cerebellar white matter,semiovale centrum,brain atrophy and ‘ tigroid sign’.ResultsThere were 8 patients with classic form,7 patients with transitional form and one patient with connatal form.They all showed diffuse delayed myelination in the white matter,with involvement of pallidus globus in 13 cases,pyramidal tract in 7 cases,corpus callosum in 11 cases,cerebellar white matter in 7 cases,semiovale centrum in 12 cases.Cerebral atrophy was found in 5 patients and eerebellar atrophy was found in one patient.Five cases depicted ' tigroid sign'.In patients with PLP1 gene point mutation,pyramidal tract and cerebellar white matter involvement showed a high incidence.Cerebellar white matter lesions were relatively frequent in children with transitional form and connatal form.In contrast,‘ tigroid sign' was often related to classic form,which indicated a better myelination and outcome.ConclusionPMD patients show distinct imaging features in their brains,which may be correlated with the phenotype and genetic mutation.

Objective To detect the changes of cerebral perfusion in patients with MELAs syndrome by using MR perfusion technique.Methods Thirteen patients with MELAS syndrome and 13 controls with normal neurological conditions were scanned with the sequence of flow-sensitive alternating inversion recovery exempting separate T1 measurement(FAIREST).Their rCBF values were obtained in regions of bilateral basilar nuclei and thalami,as well as bilateral temporal lobes and occipital lobes.Regression analysis was carried out to determine the effect of location and side on the measurement of rCBF in controls.One-way ANOVA was conducted to compare rCBF values among the control group.the lesion ROIs and normal ROIs of the MELAS syndrome group.Results The values of rCBF were 0.83±0.23,1.17±0.30.0.93±0.28,and 1.11±0.25 for the left basilar ganglia,thalamus,temporal lobe,and occipital lobe respectively,while they were 0.77±0.15,1.03±0.34,1.06±0.23,and 1.09±0.23 for the right basilar ganglia,thalamus,temporal lobe.and occipital lobe respectively.Regression analysis revealed no effect of location and side on the rCBF (P＞0.05).The rCBF value for control group was 1.00±0.28,while it was 1.01±0.31 for the normal ROIs and 1.95±0.43 for the lesion ROIs in the MELAS syndrome group(F=54.99.P＜0.01).The rCBF of the lesion ROIs in the MELAS syndrome group was significantly higher than the normal ROIs and the control group.Conclusion CBF maps can reveal changes of cerebral blood flow in patients with ietal MELAS,which suggests increased perfusion in the stroke-like lesions.

Objective To observe the effect of Ro 20-1724 on social interaction ability of developing rats after repeated ketamine anesthesia.Methods 32 rats with 21 days old were randomly divided into four groups,control group (C group),ketamine group (K group),ketamine + Ro 20-1724 group (K + R group),ketamine + ethanol group (K + E group).Ethanol was used as a solvent of Ro 20-1724.Ketamine 70 mg· kg-1 was intraperitoneal injected,30 min later,to give or not give Ro 20-1724 0.5 mg · kg-1 or equivalent ethanol solvent for once each day for seven consecutive days.Then the rats were fed for three days.On the fourth day after the last administration,the social interaction ability were assessed in all rats.The expression of brain-derived neurotropic factor (BDNF) in hippocampal CA1 region was detected using conventional ELISA.Results Comparing with rats in C group,the time spent on the cage of lifeless body ((60 ± 29) min vs (109 ± 33) min,P ＜ 0.01),unfamiliar rats (103 ±35)min vs (151 ±42)min,P＜0.01;((123 ±34)min vs (184 ±46) min,P＜0.05) and familiar rats (89 ± 25) min vs (140 ± 38) min,P ＜ 0.01) in the social interaction test was significantly less in K group.The time spent significantly prolonged in group K + R,comparing with K group (lifeless body:(94 ± 34) min vs (60 ±29) min,P＜0.01) ;unfamiliar rat 1:(140 ±41) min vs (103 ±35)min,P＜0.05) ;unfamiliar rat 2:(171 ±45)min vs (123 ±34)min,P＜0.01) ; familiar rat:(133 ±35)min vs (89 ±25) min,P＜0.01).And there was no difference between K group and K + N group (P ＞ 0.05).The expression of BDNF in hippocampal CA1 region was significantly lower in K group,comparing with C group ((8.6 ± 2.7) ng/ml vs (11.8 ± 2.4) ng/ml,P ＜0.01) ; and there was a significant increase in K + R group,comparing with K group ((10.1 ± 3.6) ng/ml vs (8.6 ± 2.7) ng/ml,P ＜ 0.05).Conclusion Ro 20-1724 significant rescued social interaction impairment induced by ketamine anesthesia in developing rats.And BDNF in hippocampal CA1 region contribute to the reversal process.

Objective:To analyze the clinical characteristics of primary acute myeloid leukemia (AML) (non-M3 type) in children suffering from different levels of platelet count(PLT).Methods:In the Tumor Hospital of Zhengzhou University from January 2014 to December 2018, laboratory and clinical data of 247 de novo primary AML pediatric patients were retrospectively reviewed.According to the PLT before treatment, patients were divided into very low platelet group (VLG), low platelet group (LG) and non-lowing platelet group (NLG), with<50×10 9/L, ≥50×10 9/L but <125×10 9/L and ≥125×10 9/L as the boundaries.All patients were followed up until June 30, 2019.Meanwhile, the follow-up data was obtained by consulting medical records or by telephone.SPSS 17.0 software was applied for data analysis. Results:In general clinical features, a different group of hemoglobin (Hb) content, fusion gene AML- ETO and clinical risk stratification were statistically significant in different PLT groups ( χ2=11.270, 12.115 and 12.848, respectively, all P<0.05). However, the differences of other indicators in different groups of PLT were not statistically significant (all P>0.05). There were no statistically significant differences in terms of 3-year disease-free survival(DFS) rate (59.3%, 36.3%, 50.4%) among the 3 groups (all P>0.05). The median total survival(OS)time(40.5 months)and 3-year OS rate(41.0%) of NLG patients were significantly higher than those of VLG(23.1 months, 30.1%)and LG(14.1 months, 18.2%)patients, with statistically significant differences( χ2=7.798 and 6.553, respectively, all P<0.05). The univariate analysis of gender, white blood cell(WBC), Hb, PLT, lactic dehydrogenase(LDH), FLT3-ITD, NPM1, DNMT3A, CEPBA, C-KIT, AML-ETO, molecular genetic prognosis, complete remission(CR), and hemopoietic stem cell transplantation(HSCT) displayed that DNMT3A mutation was an adverse factor that affects patients′ OS ( χ2 =5.834, P<0.05), and the positive factors that influences OS were non-reducing PLT before treatment, and obtaining CR and subsequent HSCT ( χ2=7.798, 79.168, and 31.337, respectively, all P<0.05). Multi-factor analysis revealed that the independent protective factors that affect patients′ OS were the non-reducing PLT before treatment, and obtaining CR and subsequent HSCT( Wald=42.760, 15.918, and 10.183, respectively, all P<0.05). Conclusions:Before treatment, non-reducing PLT is a protective factor for primary childhood AML patients, and the prognosis is satisfying.

OBJECTIVE: To study the anatomic characteristics of recurrent laryngeal nerve during thyroid surgery. METHOD: A retrospective review of surgical data of 307 patients undertook thyroid surgery was conducted. RESULT: Total 342 recurrent laryngeal nerves were identified during the surgery(184 on the right side, left 158). 215 (62.9%) nerves were deep to the inferior thyroid artery, 106(31.0%)were superficial to the artery, 21(7.5%) were between the arterial branches. A nerve bifurcation was found in 203(59.4%). None of nerve bifurcation was found in 136(39.8%). 3(0.9%)were confirmed to hold non-recurrent laryngeal nerves during operations. No patient showed permanent laryngeal recurrent nerve paralysis postoperatively. CONCLUSION The careful dissection and protection of the recurrent laryngeal nerve was an effective method to prevent its injury during thyroid surgery.
Arteries ; Cranial Nerve Diseases ; etiology ; prevention & control ; Dissection ; Humans ; Postoperative Period ; Recurrent Laryngeal Nerve ; Retrospective Studies ; Thyroid Gland ; surgery ; Thyroidectomy ; Vocal Cord Paralysis

Purpose:To investigate the effectiveness of intralymphatic infusion of anti- cancer agents and cytokines in the treatmrnt of malignancy.Materials anti methods:23 patients suffering from advanced metastatic cancers anti 2 primary lymphomas,unresponsble to the standard therapies or intra-arterial chemotherapy,were treated with lymphatic injections of an- ticancer ddrugs or combiation with biochemotherapy.Results:Follow-up study about one month after the therapy,comparing with findings on lymthatic radiographies anti computed tomographic scans,revealed decrease of lymphnodes in size in 23 cases.Conclusion:This therapeutic ap- proach proved to be an effective and safe method for the palliative treatment of advanced lym- phatic metastases and lymphomas.The procedure was feasible without serious compllications.

Objective To study the clinical efficacy of intra-operative implantation of I125 seeds to treat advanced pancreatic cancer.Methods We retrospectively analyzed the clinical data of 28 patients who underwent intra-operative implantation of I125 between January 2009 and June 2010 to treat advanced pancreatic cancer.Patients who received conservative treatment (n =136) at the same period were used as the control group.Results The mean tumor diameter was (5.7 ± 1.7) cm.The average number of implanted seeds were 50.There were no patients who suffered from pancreatic fistula or post-operative bleeding,and no patient died from the treatment.Twenty-five patients were regularly followed up,and the follow-up rate was 85.7％.The survival rates at one month,half a year and one year were 100％,70.8％ and 26.9％,respectively.The average survival time was (9.9 ± 1.4) months.The survival data were significantly higher than that in the control group.Conclusion Intra-operative implantation of I125 seeds was an efficacious treatment for patients with advanced pancreatic cancer.

Objective p38 Mitogen-activated protein kinase (MAPK) is a crossing center of various pathways. In this study, protein transduction system based on human immunodeficiency virus (HIV)-1 transactivator of transcription (TAT), which is an efficient delivery peptide of the foreign proteins into cells, was employed to study p38 MAPK functions in eukaryotic cells. Methods p38 And its dominant negative form, p38AF, were constructed into pET-His-TAT vector correctly to verify that the recombinant plasmids were well-founded through restriction enzyme digestion and DNA sequencing. The two proteins, His-TAT-p38 and His-TAT-p38AF, were expressed and purified in Escherichia coli by SDS-PAGE. Then they were incubated with ECV304 cells respectively and readily transduced into cells in a time-dependent and dose-dependent manner. The cells were stimulated by sorbitol. Activating transcription factor (ATF) 2 phosphorylation level was checked using Western blot to assess the activity of endogenous p38. Results Compared with controls, it was found that His-TAT-p38 increased the level ofATF2 phosphorylation in sorbitol-stimulated ECV304 cells, while His-TAT-p38AF inhibited it, indicating p38 MAPK protein delivery system based on TAT was constructed successfully. TAT-p38 and its dominant negative form possessed high biological activity after transduction into ECV304 cells by TAT protein delivery system. The results showed that p38AF fused with TAT could inhibit the transduction of endogenous p38 signal pathway in part, and other pathway might regulate p38 phosphorylation. Conclusions Our study provides a novel pathway to inhibit p38 signal pathway and establish a new method to study p38 function.