الملخص
Objective @#To compare the ultrasound signs of symptomatic joint lesions in rheumatoid arthritis (RA) and gouty arthritis ( GA) , musculoskeletal ultrasound ( MSUS) was utilized. @*Methods @#A retrospective analysis was performed for 85 hospitalized patients with RA and 55 hospitalized patients with GA in the same period , and the differences in general data , diseased joints and ultrasound signs between the two groups were compared. @*Results@#The gender, age and diseased joints of the two groups were statistically significant (all P < 0. 05) . The detection rate of knee joint lesions was the highest; the RA group had high sensitivity , high specificity of meniscal injury , and high diagnostic efficiency of bone erosion , while the diagnostic performance of the three combined ultrasound signs of punctate strong echo , double track sign and tophi in the GA group was higher than that of any individual diagnosis , and the sensitivity and specificity were also higher. The course of disease in the RA group was positively correlated with bone erosion (P < 0. 05) , and the course in the GA group was positively correlated with tophi (P < 0. 05) .@*Conclusion @#The ultrasound signs of RA and GA are different , and MSUS has good value in the diagnosis and differential diagnosis of the two.
الملخص
Objective:To compare the effects of deep anesthesia extubation with sugammadex and neostigmine on perioperative respiratory adverse events (PRAE) and the duration of anesthesia recovery room (PACU) in children undergoing adenoidectomy.Methods:The clinical data of 470 children with adenoidectomy who underwent deep anesthesia extubation from February 2021 to April 2022 in Shenzhen Hospital of Southern Medical University were retrospectively analyzed. According to the use of muscle relaxant, they were divided into sugammadex group (group S) and neostigmine group (group N). Group S was intravenously injected with sugammadex 2 mg/kg after operation, and group N was intravenously injected with neostigemine 0.05 mg/kg combined with atropine 0.02 mg/kg when the train-of-four (TOF) value was >25%. The extubation time and PACU residence time of the two groups were recorded. Changes of heart rate (HR) and mean arterial pressure (MAP) immediately after surgery (T 0), within 1min after extubation (T 1), 5 min after extubation (T 2), and 20 min after extubation (T 3). PRAE was record. Results:There were 138 cases of propensity score matching in group S and group N respectively. The extubation time and PACU residence time in group S were shorter than those in group N (all P<0.05). At T 1, T 2 and T 3, HR and MAP in group S were significantly lower than those in group N (all P<0.05). The incidence of persistent cough and decreased blood oxygen in group S was lower than that in group N (all P<0.05). Conclusions:Compared with neostigmine, postoperative deep anesthesia extubation with sugammadex antagonistic residual muscle relaxant can shorten the duration of extubation and PACU retention after adenoidectomy, accelerate the recovery of children, make the peri-extubation cycle more stable, and reduce the occurrence of PRAE.
الملخص
Objective@#To explore the relationship between different mutation characteristics and clinical phenotype of children with Dravet syndrome (DS) with SCN1A gene mutation, and to summarize the drug efficacy.@*Methods@#The clinical data of children diagnosed as DS from the Department of Pediatrics Neurology, the Third Affi-liated Hospital of Zhengzhou University from January 2014 to May 2018 were collected.The peripheral blood DNA of the children was detected by adopting next generation sequencing for epilepsy-related gene-panel, while the parents′ were screened by using Sanger sequencing for family verification.Multiple ligation-dependent probe amplification technology was used to detect large fragment variation of SCN1A gene if the mutations of the children were negative.The Gesell scale and cavity Wechsler intelligence scale for children(C-WISC) were used to evaluate the intelligence of children.@*Results@#A total of 50 cases of DS were collected, 38 cases of them were positive for SCN1A mutation, and the mutation rate was 76.0%(38/50 cases), of which, the missense mutation[50.0%(19/38 cases)] and frameshift mutation[28.9%(11/38 cases)] were dominant.The average onset age of 50 patients was 6 months, of which onset of seizures was triggered by fever(temperature>37.5 ℃) in 68.0%(34/50 cases)of children, the history of seizures in hot baths was found in 60.0%(30/50 cases) of children, status epilepticus was found in 74.0%(37/50 cases), cluster-like episodes was found in 80.0%(40/50 cases), ≥2 seizure types was found in 92.0%(46/50 cases). Mental retarda-tion was found in most of the children, of which 30.0% (15/50 cases) were mild mental retardation, 38.0% (19/50 cases) were moderate mental retardation, 14.0% (7/50 cases)were severe intelligence retardation.Interictal abnormalities of electroencephalogram(EEG) before 1 year old was found in 24.0%(12/50 cases), and the average age of EEG abnormalities was 30.12 months old; the top three drug efficacy rates were 70.0% (28/40 cases) of Topiramate, 48.0% (24/50 cases) of Sodium Valproate, 45.7% (16/35 cases) Clonazepam or Clobazam.The time of onset of myoclonus and atypical absence of the truncation mutation group was earlier than that of the missense mutation group(14.75 months vs.21.20 months; 16.82 months vs.26.00 months), and the difference was statistically significant(P<0.05). The proportion of clustered episodes in the truncation mutation group was higher than that in the missense mutation group [94.7%(18/19 cases)vs.63.2%(12/19 cases)], and the difference was statistically significant (P<0.05). There was no significant correlation between the SCN1A gene mutation (type and position) and age of onset, type of seizure, proportion of convulsion persistence, the mental development or abnormal proportion of EEG and seizure frequency before 1 year old(all P>0.05).@*Conclusions@#The SCN1A gene mutation rate is high in children with DS, and the SCN1A gene mutation characteristics has a certain correlation with DS clinical phenotype.Topiramate is the most effective drug among children with DS.
الملخص
Objective To explore the relationship between different mutation characteristics and clinical phenotype of children with Dravet syndrome (DS) with SCN1A gene mutation,and to summarize the drug efficacy.Methods The clinical data of children diagnosed as DS from the Department of Pediatrics Neurology,the Third Affiliated Hospital of Zhengzhou University from January 2014 to May 2018 were collected.The peripheral blood DNA of the children was detected by adopting next generation sequencing for epilepsy-related gene-panel,while the parents' were screened by using Sanger sequencing for family verification.Multiple ligation-dependent probe amplification technology was used to detect large fragment variation of SCN1A gene if the mutations of the children were negative.The Gesell scale and cavity Wechsler intelligence scale for children(C-WISC) were used to evaluate the intelligence of children.Results A total of 50 cases of DS were collected,38 cases of them were positive for SCN1A mutation,and the mutation rate was 76.0％ (38/50 cases),of which,the missense mutation[50.0％ (19/38 cases)] and frameshift mutation[28.9％ (11/38 cases)] were dominant.The average onset age of 50 patients was 6 months,of which onset of seizures was triggered by fever(temperature ＞ 37.5 ℃) in 68.0％ (34/50 cases)of children,the history of seizures in hot baths was found in 60.0％ (30/50 cases) of children,status epilepticus was found in 74.0％ (37/50 cases),cluster-like episodes was found in 80.0％ (40/50 cases),≥2 seizure types was found in 92.0％ (46/50 cases).Mental retardation was found in most of the children,of which 30.0％ (15/50 cases) were mild mental retardation,38.0％ (19/50 cases) were moderate mental retardation,14.0％ (7/50 cases)were severe intelligence retardation.Interictal abnormalities of electroencephalogram(EEG) before 1 year old was found in 24.0％ (12/50 cases),and the average age of EEG abnormalities was 30.12 months old;the top three drug efficacy rates were 70.0％ (28/40 cases) of Topiramate,48.0％ (24/50 cases) of Sodium Valproate,45.7％ (16/35 cases) Clonazepam or Clobazam.The time of onset of myoclonus and atypical absence of the truncation mutation group was earlier than that of the missense mutation group(14.75 months vs.21.20 months;16.82 months vs.26.00 months),and the difference was statistically significant (P ＜ 0.05).The proportion of clustered episodes in the truncation mutation group was higher than that in the missense mutation group [94.7％ (18/19 cases)vs.63.2％ (12/19 cases)],and the difference was statistically significant (P ＜0.05).There was no significant correlation between the SCN1A gene mutation (type and position) and age of onset,type of seizure,proportion of convulsion persistence,the mental development or abnormal proportion of EEG and seizure frequency before 1 year old(all P ＞ 0.05).Conclusions The SCN1A gene mutation rate is high in children with DS,and the SCN1A gene mutation characteristics has a certain correlation with DS clinical phenotype.Topiramate is the most effective drug among children with DS.