Adenoid cystic carcinoma is a malignant tumor of the head and neck, this article reports a case of a large adenoid cystic carcinoma of the skull base, with the lesion involving the sphenoid sinus, sphenoid bone wings, pterygopalatine fossa, nfratemporal fossa, hard palate, and other structures. The treatment plan consisted of surgical excision, primary reconstrction of the surgical defect,and postoperative radiotherapy, resulting in a favorable prognosis for the patient.
Humans ; Carcinoma, Adenoid Cystic/surgery* ; Maxillary Sinus/surgery* ; Maxillary Sinus Neoplasms/surgery* ; Palate, Hard/surgery* ; Skull Base Neoplasms/surgery* ; Surgical Flaps

Objective:To retrospectively analyze the clinical features and surgical efficacy of congenital cholesteatoma （CC） and acquired cholesteatoma （AC） in children. Methods:Clinical data of 169 children with middle ear cholesteatoma were reviewed in the Department of Otorhinolaryngology Head and Neck Surgery, Beijing Children's Hospital, Capital Medical University from January 2010 to July 2020. The clinical characteristics, stages, surgical methods, and postoperative recurrence rates were analyzed and summarized. Results:The age distribution of enrolled children ranged from 2 to 14 years. The mean age of the CC group was （5.60±2.48） years compared with （6.45±2.48） years in the AC group, and the difference was statistically significant （P<0.05）. Preoperative hearing in the CC group was （40.06±13.52） dB HL, which was better than in the AC group at （48.40±13.84） dB HL （P<0.05）. The proportion of stage Ⅰ in the CC group was lower than that in the AC group according to EAONO/JOS staging （P<0.05）. The recurrence rate after primary surgery was 19.23% （10/52） in the CC group compared with 36.29% （45/124） in the AC group （P<0.05）. The mastoid retention rates after all operations were 28.85% （15/52） in the CC group and 5.65% （7/124） in the AC group （P<0.05）. Conclusion:Compared with congenital cholesteatoma, acquired cholesteatoma in children is more aggressive and has more complications, higher postoperative recurrence rate, and less possibility of mastoid retention. Early clinical detection and treatment are required, and canal wall-down tympanoplasty should be considered in surgery.
Humans ; Cholesteatoma, Middle Ear/congenital* ; Child ; Retrospective Studies ; Child, Preschool ; Adolescent ; Male ; Female ; Recurrence ; Cholesteatoma/congenital* ; Tympanoplasty ; Treatment Outcome

Objective:To investigate long-term auditory changes and characteristics of Alport syndrome（AS） patients with different degrees of renal injury. Methods:Retrospectively analyzing clinical data of patients diagnosed AS from January 2007 to September 2022, including renal pathology, genetic detection and hearing examination. A long-term follow-up focusing on hearing and renal function was conducted. Results:This study included 70 AS patients, of which 33（25 males, 8 females, aged 3.4-27.8 years） were followed up, resulting in a loss rate of 52.9%.The follow-up period ranged from 1.1to 15.8 years, with 16 patients followed-up for over 10 years. During the follow-up, 10 patients presenting with hearing abnormalities at the time of diagnosis of AS had progressive hearing loss, and 3 patients with new hearing abnormalities were followed up, which appeared at 5-6 years of disease course. All of which were sensorineural deafness. While only 3 patients with hearing abnormalities among 13 patients received hearing aid intervention. Of these patients,7 developed end-stage renal disease（ESRD）, predominantly males （6/7）. The rate of long-term hearing loss was significantly different between ESRD group and non-ESRD group（P=0.013）. There was no correlation between the progression of renal disease and long-term hearing level（P>0.05）. kidney biopsies from 28 patients revealed varying degrees of podocyte lesion and uneven thickness of basement membrane. The severity of podocyte lesion was correlated with the rate of long-term hearing loss（P=0.048）, and there was no correlation with the severity of hearing loss（P>0.05）. Among 11 cases, theCOL4A5mutationwas most common （8 out of 11）, but there was no significant correlation between the mutation type and hearing phenotype（P>0.05）. Conclusion:AS patients exhibit progressive hearing loss with significant heterogeneity over the long-term.. THearing loss is more likely to occur 5-6 years into the disease course. Hearing abnormalities are closely related to renal disease status, kidney tissue pathology, and gene mutations, emphasizing the need for vigilant long-term hearing follow-up and early intervention.
Male ; Child ; Female ; Humans ; Nephritis, Hereditary/pathology* ; Retrospective Studies ; Kidney ; Deafness ; Hearing Loss/genetics* ; Kidney Failure, Chronic/pathology* ; Mutation

Objective To investigate the effect of Tongsheng Prescription on trimethylamine N-oxide(TMAO)and related metabolites substrate in patients with acute cerebral infarction after intervention and to evaluate its clinical efficacy.Methods A prospective randomized controlled study was conducted in 48 patients with acute ischemic stroke with large vessel occlusion(AIS-LVO)who had received endovascular treatment(EVT).The patients were divided into an observation group(25 cases)and a control group(23 cases).After emergency EVT,the control group was treated with routine western medicine,and the observation group was treated with nasogastric feeding or oral use of Tongsheng Prescription on the basis of treatment for the control group.The course of treatment lasted for seven days,and then the patients were followed up for three months.Before operation,7 days and 90 days after operation,National Institutes of Health Stroke Scale(NIHSS)scores in the two groups were observed.The functional prognosis evaluated with Modified Rankin Scale(MRS)scores was compared between the two groups,and the effects of Tongsheng Prescription on serum TM AO and related metabolites choline,betaine and L-carnitine were investigated.Results(1)On the postoperative day 7 and 90,the NIHSS scores of the two groups were decreased compared with those before operation(P＜0.01),and the decrease of NIHSS scores on the postoperative day 7 and 90 in the observation group was significantly superior to that in the control group(P＜0.01).(2)On the postoperative day 90,the rate of good functional outcome in the observation group was 96.00％(24/25),which was significantly higher than 69.57％(16/23)in the control group,and the difference was statistically significant(P＜0.05).(3)On the postoperative day 7 and 90,the levels of TMAO,L-carnitine and betaine in the observation group tended to decrease compared with those before operation,but the difference was not statistically significant(P＞0.05).However,the levels of TMAO,L-carnitine and betaine in the control group were significantly increased compared with those before operation(P＜0.01).On the postoperative day 7,the levels of TMAO,L-carnitine and betaine in the observation group were significantly lower than those in the control group,and the differences were statistically significant(P＜0.05).(4)Spearman correlation analysis showed that TMAO was positively correlated with NIHSS score,L-carnitine and choline(P＜0.05 or P＜0.01).Conclusion Tongsheng Prescription has a significant effect on AIS-LVO patients seven days after EVT.The Prescription is beneficial to obtain a good functional outcome,and its mechanism may be related to the decrease of TMAO level through L-carnitine and betaine pathways,so as to improve the neurological deficit symptoms of stroke patients.

Objective To investigate the effects of Tianma Jiannao Granules(derived from Banxia Baizhu Tianma Decoction)combined with hyperbaric oxygen on cognitive function,activities of daily living(ADL),and the expression levels of serum inflammatory factors,matrix metalloproteinase 9(MMP-9),homocysteine(Hcy)and superoxide dismutase(SOD)in patients with vascular dementia(VD).Methods A total of 100 VD patients with wind-phlegm and stasis obstruction type were randomly divided into a trial group and a control group,50 cases in each group.The patients in the two groups were given symptomatic treatment such as controlling blood pressure,lowering blood glucose and regulating blood lipid.Additionally,the control group was given Oxiracetam Capsules orally,and the trial group was given Tianma Jiannao Granules orally combined with hyperbaric oxygen therapy on the basis of treatment for the control group.The course of treatment covered 12 weeks.The changes of traditional Chinese medicine(TCM)syndrome scores,Mini-Mental State Examination(MMSE)scores,ADL scores,and the expression levels of serum inflammatory factors,neurotransmitters,apoptosis-related factors,MMP-9,Hcy and SOD in the two groups were observed before and after treatment.Results(1)The scale scoring showed that after treatment,the TCM syndrome scores and ADL scores of the two groups were significantly decreased compared with those before treatment(P＜0.05),and the MMSE scores were significantly increased compared with those before treatment(P＜0.05).The decrease of TCM syndrome scores and ADL scores and the increase of MMSE scores in the trial group were significantly superior to those in the control group(P＜0.01).(2)After treatment,the serum levels of inflammatory factors such as tumor necrosis factor α(TNF-α),C-reactive protein(CRP)and interleukin 6(IL-6)in the two groups were significantly decreased compared with those before treatment(P＜0.05),and the decrease of serum TNF-α,CRP and IL-6 levels in the trial group was significantly superior to that in the control group(P＜0.01).(3)After treatment,the serum levels of neurotransmitters such as norepinephrine(NE),dopamine(DA)and 5-hydroxytryptamine(5-HT)in the two groups were significantly increased compared with those before treatment(P＜0.05),and the increase of serum NE,DA and 5-HT levels in the trial group was significantly superior to that in the control group(P＜0.01).(4)After treatment,the serum level of apoptosis-related factor of heme oxygenase-1(HO-1)in the two groups was significantly increased compared with that before treatment(P＜0.05),and the levels of serum soluble apoptosis-related factors(sFAS)and their ligands(sFASL)were significantly decreased compared with those before treatment(P＜0.05).The increase of serum HO-1 level and the decrease of serum sFAS and sFASL levels in the trial group were significantly superior to those in the control group(P＜0.01).(5)After treatment,the serum SOD level in the two groups was significantly increased compared with that before treatment(P＜0.05),and the levels of serum MMP-9 and Hcy were significantly decreased compared with those before treatment(P＜0.05).The increase of serum SOD level and the decrease of serum MMP-9 and Hcy levels in the trial group were significantly superior to those in the control group(P＜0.01).Conclusion Tianma Jiannao Granules combined with hyperbaric oxygen is effective on improving the cognitive function and ADL of VD patients,and has certain effects on counteracting inflammatory response and oxidation and on regulating the expression of neurotransmitters.

This article summarized Professor Ning Wei-Min's experience in treating chronic migraine with the method of soothing liver,relieving depression and expelling pathogens.Chronic migraine is a common neurological disease with relatively high incidence.Currently,its treatment is mainly through oral administration of drugs,but there exist the disadvantages of unstable therapeutic effect,obvious adverse reactions after long-term medication,low treatment compliance,drug dependence,and easily ignorance of emotional co-morbidities such as anxiety and depression.Professor NING Wei-Min,a famous traditional Chinese medicine(TCM)physician of Guangdong Province,believes that chronic migraine affects the region along the meridians of the liver and gallbladder,and is closely related with the liver's function of ensuring the free movement of qi.Once the liver-qi stagnates,internal retention of phlegm and blood stasis easily induced,and persisting illness causes stagnant liver qi turning into heat,and then the headache will attack by the trigger of a variety of factors of external pathogens such as wind,cold,summer-heat,dampness and heat,as well as the emotional disorders,which leads to the stirring of liver-wind and the stagnation of phlegm-fire insidious pathogens in the meridian.For the treatment of chronic migraine,therapies of soothing liver,relieving depression and expelling pathogens can be utilized,and oral administration of Kaiyu Touxie Prescription combined with acupuncture is recommended.Kaiyu Touxie Prescription is composed of Chuanxiong Rhizoma,Angelicae Dahuricae Radix,Asari Radix et Rhizoma,Bupleuri Radix,Gastrodiae Rhizoma,Curcumae Radix,Paeoniae Radix Alba,Bombyx Batryticatus,Scorpio,Malloti Apeltae Radix Et Rhizoma,and Glycyrrhizae Radix et Rhizoma.Acupuncture is performed mainly on the acupoints along the meridians of the liver and gallbladder,such as Fengchi(GB20),Shuaigu(GB8),Toulinqi(GB15),Wangu(GB12),Xuanli(GB6),Yangbai(GB14),Yanglingquan(GB34),Taichong(LR3)and Xingjian(LR2),alternatively on acupoints of Waiguan(TE5),Hegu(LI4),Baihui(GV20),Touwei(ST8),etc.Professor NING Wei-Min's experience in treating chronic migraine with oral administration of Chinese medicine combined with acupuncture based on the method of soothing liver,relieving depression and expelling pathogens will provide a reference for clinical treatment of chronic migraine.

Objective:To establish a cell line stably expressing the transient receptor potential melastatin 2(TRPM2)channel for screening TRPM2 inhibitors based on PiggyBac transposition system.Methods:A plasmid PiggyBac-human TRPM2(pPB-hTRPM2)eukaryotic expression vector was constructed using PiggyBac transposition system.The plasmid and a helper plasmid were co-transfected into HEK293T cells to express TRPM2,which was identified by fluorescence and patch-clamp assays.The high throughput screening performance was assessed with the Z'factor.Calcium imaging and patch clamp techniques were employed to assess the initial activity of eleven compound molecules,confirming the inhibitory effects of the primary molecules on TRPM2.The protective effect of the screened compounds on damaged cells was validated using the oxygen-glucose deprivation/reperfusion(OGD/R)injury model and CCK-8 kit.The level of cellular reactive oxygen species(ROS)was detected by flow cytometry.The neuroprotective effects of the compounds were evaluated using a transient middle cerebral artery occlusion(tMCAO)mouse model.Results:The HEK293T cells transfected with pPB-hTRPM2-EGFP showed high TRPM2 expression.Puromycin-resistant cells,selected through screening,exhibited robust fluorescence.Whole-cell patch results revealed that induced cells displayed classical TRPM2 current characteristics comparable to the control group,showing no significant differences(P＞0.05).With a Z'factor of 0.5416 in calcium imaging,the model demonstrated suitability for high-throughput screening of TRPM2 inhibitors.Calcium imaging and electrophysiological experiments indicated that compound 6 significantly inhibited the TRPM2 channel.Further experiments showed that 1.0 μmol/L of compound 6 enhanced cell viability(P＜0.05)and reduced the level of ROS(P＜0.05)of SH-SY5Y under OGD/R injury.0.3 and 1.0 mg/kg of compound 6 reduced the cerebral infarction volume in tMCAO mice(both P＜0.05).Conclusions:A stable TRPM2 gene expressing cell line has been successfully established using PiggyBac gene editing in this study.TRPM2 channel inhibitors were screened through calcium imaging and patch clamp techniques,and an inhibitor compound 6 was identified.This compound can alleviate cell damage after OGD/R by reducing cellular ROS levels and has a protective effect against cerebral ischemia-reperfusion injury in mice.

Patients with severe trauma require an extremely timely treatment and transfusion plays an irreplaceable role in the emergency treatment of such patients. An increasing number of evidence-based medicinal evidences and clinical practices suggest that patients with severe traumatic bleeding benefit from early transfusion of low-titer group O whole blood or hemostatic resuscitation with red blood cells, plasma and platelet of a balanced ratio. However, the current domestic mode of blood supply cannot fully meet the requirements of timely and effective blood transfusion for emergency treatment of patients with severe trauma in clinical practice. In order to solve the key problems in blood supply and blood transfusion strategies for emergency treatment of severe trauma, Branch of Clinical Transfusion Medicine of Chinese Medical Association, Group for Trauma Emergency Care and Multiple Injuries of Trauma Branch of Chinese Medical Association, Young Scholar Group of Disaster Medicine Branch of Chinese Medical Association organized domestic experts of blood transfusion medicine and trauma treatment to jointly formulate Chinese expert consensus on blood support mode and blood transfusion strategies for emergency treatment of severe trauma patients ( version 2024). Based on the evidence-based medical evidence and Delphi method of expert consultation and voting, 10 recommendations were put forward from two aspects of blood support mode and transfusion strategies, aiming to provide a reference for transfusion resuscitation in the emergency treatment of severe trauma and further improve the success rate of treatment of patients with severe trauma.

Objective To observe the effects of different doses of Kiwi fruit essence on fibroblast growth factor 2(FGF2)and extracellular regulatory protein kinase 1/2(ERK1/2)in lung tissue of rats with pulmonary fibrosis.Methods SD rats were divided into blank control group(0.9％NaCl),model group(0.9％NaCl),positive control group(5 mg·kg-1 prednesone acetate),experimental group-L,-M,-H(60,120,180 mg·kg-1 Kiwi fruit essence),except the blank control group was injected with 0.9％NaCl at one time.The other groups were injected 5 mg·kg-1 bleomycin A5 into the trachea to construct the rat pulmonary fibrosis model.The expressions of FGF2 and ERK1/2 proteins and mRNA were detected by Western blot and real-time fluorescence quantitative polymerase chain reaction.Results The relative expression levels of FGF2 protein in blank control group,model group,positive control group and experimental group-L,-M,-H groups on the day 28 were 0.07±0.01,0.63±0.15,0.14±0.02,0.16±0.01,0.26±0.02 and 0.44±0.03,respectively;the relative expression levels of ERK1/2 protein on day 28 were 0.06±0.00,0.41±0.05,0.09±0.00,0.10±0.00,0.14±0.02,0.21±0.02,respectively;the relative expression levels of FGF2 mRNA on day 28 were 0.98±0.05,9.32±1.03,2.21±0.32,2.87±0.44,5.32±0.21,6.37±0.66,respectively;the relative expression levels of ERK1/2 mRNA on day 28 were 1.00±0.01,9.22±0.85,1.93±0.63,2.36±0.54,5.34±0.44 and 6.85±1.09,respectively.The above indexes in the model group were significantly different from those in the blank control group(all P＜0.05).The above indexes in the positive control group and the experimental-L,-M,-H groups were significantly different from those in the model group(all P＜0.05).Conclusion Kiwi essence may inhibit pulmonary fibrosis by regulating the expression of FGF2 and ERK1/2.