Objective To investigate the clinical characteristics,treatment effect,long-term follow up results,guanosine triphosphate (GTP) cyrclohydrolase Ⅰ (GCH Ⅰ)gene and tyrosine hydroxylase(TH) gene mutations in patients with dopa-responsive dystonia (DRD).Methods The clinical features of 3 families with 4 affected members were analyzed and all of 4 patients were screened for mutations of the GCH Ⅰ gene and TH gene with DNA sequences.Results Four patients were females,average age at onset was (15.3 ± 5.6) years (range:from 9 to 20 years).The initial symptoms were a gait disorder,stiffness or tremor of the lower limbs in all patients presented with diurnal fluctuation.As the increase of disease duration,bilateral hand tremor was found in three patients,systemic torsion was found in one patient and torticollis was found in one patient.All patients' symptoms were in complete remission after administration of low dose of levodopa.Four patients were followed up for 0.5 to 10.0 years,and all were still responsive to the levodopa treatment and effective dosage was decreased as the increase of the disease duration.No longterm side effects of levodopa had occurred after long-term treatment.One patient was found to have c.607G ＞A(p,Gly203Arg) heterogenetic mutation in GCH I gene.Molecular analysis revealed a compound heterozygous mutation in the TH gene (p.Y447Ter and p.V468M) in one patient.No point mutations in both genes were found in other patients.Conclusions DRD patients have dramatic and sustained response to levodopa and no long-term side effects of levodopa after long-term treatment.The detection of GCH Ⅰ and TH gene mutations is helpful in early diagnosis but the negative results could not exclude the diagnosis of DRD.

Objective:To systematically assess the effectiveness and safety of acupuncture for spastic hemiplegia after ischemic stroke. Methods:Randomized controlled trials(RCTs)of acupuncture treatment for spastic hemiplegia after ischemic stroke meeting the inclusion criteria in Cochrane Library,Medline,Excerpta Medica Database(EMBASE),PubMed,China National Knowledge Infrastructure(CNKI),SinoMed,Chongqing VIP Database(VIP),and Wanfang Data Knowledge Service Platform(Wanfang)published from each database's inception to February 2023 were retrieved by computer.Two reviewers independently extracted data and evaluated the risk of bias using Cochrane's risk of bias tool.Review Manager 5.4 was used for data analysis.Continuous data were evaluated using mean difference(MD)with a 95%confidence interval(CI),and dichotomous data were analyzed using risk ratio(RR). Results:A total of 24 trials,including 1 970 participants,were included in the study.The meta-analysis of 7 trials showed that compared to the rehabilitation therapy,acupuncture therapy was more effective in improving the simplified Fugl-Meyer assessment score after 1-month treatments[MD=10.52,95%CI(7.81,13.23),P<0.001].The meta-analysis of 2 articles showed the same tendency after 6-month treatments[MD=19.18,95%CI(11.34,27.02),P<0.001],and the 6-month treatment course resulted in better outcomes than the 1-month course.The meta-analysis of 8 trials showed that acupuncture had a better improvement on the Barthel index score than rehabilitation therapy after 1-month treatments[MD=10.78,95%CI(8.91,12.64),P<0.001].The meta-analysis of 2 articles showed the same tendency after 6-month treatments[MD=19.94,95%CI(19.02,20.87),P<0.001],and the 6-month course was better than the 1-month course.The meta-analysis of 2 trials showed that the effective rate of the modified Ashworth scale score improvement was more notable in the acupuncture group after 1-month treatments[RR=1.20,95%CI(1.02,1.40),P=0.020].One trial reported no adverse event,and 1 trial reported 3 adverse events without severe influence. Conclusion:Acupuncture might be an effective and safe therapy for spastic hemiplegia after ischemic stroke,but more high-quality,large-sample objectively-evaluated RCTs are needed to validate the conclusion.

Objective To investigate the clinical effect of thin-layer free anterolateral thigh flap with iliotibial tract for reconstruction of heel soft tissue and Achilles tendon defect.Methods From January,2017 to December,2017,11 cases of heel soft tissue and Achilles tendon defect were repaired by thin-layer free anterolateral thigh flap with iliotibial tract.There were 5 cases with tibia/fibula fracture,3 cases with ankle fracture and 1 case with calcaneal fracture.The area of soft tissue defect was 6.5 cm×10.0 cm-8.0 cm×13.0 cm,and the length of Achilles tendon defect was 5.5-11.5 cm.All wounds were treated with debridement and negative pressure sealing drainage technique at first stage.After 6 days,the soft tissue defect around posterior calcaneal region combined with Achilles tendon defect were repaired by the thin-layer free anterolateral thigh flap with iliotibial tract.Reasonable rehabilitation training was established after operation.Results The flaps survived in 11 cases and the donor region healed in one stage.The patients were followed-up for 8 to 20 (mean,15.74) months by outpatient review,calling or WeChat.Slightly bloated in appearance in 2 cases,and were thinned by orthopedic operation 1 year later.The other flaps were well shaped and moderately thick.At the last follow-up,the flaps were soft in texture and elastic.The sensory recovery of the flap was good,and the two-point discriminant perception in the posterior region of the heel was 3.0-5.0(mean,4.11) mm.Thompson's sign was negative,double or one-foot heel lift test was negative,and there was no recurrence of Achilles tendon rupture or skin ulceration in the heel region.The range of motion (ROM) of affected side was (23.1 1±1.17)°of extension and (43.67±1.06)°of flexion,and the ROM of normal side was (23.79±1.03)°of extension and (44.03±0.94)°of flexion.There was no statistical difference between them (P＞0.05).Postoperative follow-up was conducted according to the Thermann's function evaluation system,the evaluated result was excellent in 8 cases,good in 2 cases,and receivability in 1 case.Conclusion It is a reliable and effective method to repair heel soft tissue and Achilles tendon defect by thin-layer free anterolateral thigh flap with iliotibial tract.And this method can better restore foot ankle shape and function.

OBJECTIVE To study the effect of Runzao zhiyang capsule （RZZY） on gut microbiota and its metabolites short- chain fatty acids （SCFA） in eczema rats， in order to reveal the improvement effect and possible mechanism of RZZY on eczema. METHODS The rats were divided into normal group， model group， desloratadine group （1.5 mg/kg）， and RZZY low-dose and high-dose groups （0.6， 1.2 g/kg）. Except for the normal group， the eczema model was established in other groups by applying the 2，4-dinitrochlorobenzene solution to the hair removal area to sensitize it； they were given relevant drugs intragastrically， once a day， for consecutive 2 weeks. After medication， the skin thickness of rats was measured， and the eczema area and severity index （EASI） scoring and pathology scoring were performed； the structure of gut microbiota was analyzed by 16S rDNA sequencing. The contents of SCFA （including acetic acid， propionic acid， and butyric acid） in feces were determined by gas phase-mass spectrometry. The expressions of signal transduction and activator of transcription 3 （STAT3） in the colon and skin were detected. RESULTS RZZY significantly alleviated skin swelling and ulceration and reduced EASI， skin thickness and pathological score in eczema rats （P＜0.05）. RZZY significantly increased ACE， Sobs and Shannon indexes （P＜0.05） and promoted the microbiota of eczema rats to return to normal structure. RZZY increased the abundance of Firmicutes and Lachnospira， Lactobacillus and Alistipes， while decreased the abundance of Bacteroidetes and Bacteroides. RZZY significantly increased the contents of propionic acid and butyric acid in the feces of eczema rats （P＜0.05）， while significantly down-regulated the expressions of STAT3 protein in the colon and skin （P＜0.05）. C ONCLUSIONS RZZY can improve SCFA levels by regulating gut microbiota， thereby inhibiting STAT3 expression， playing an anti- inflammatory role， and improving skin inflammation in eczema rats.