BACKGROUND:Cilium is an antenna-like organele that plays a vital role in al organs generation and maintenance.Ciliopathiesrefer tomultisystem diseasescaused bydisordersofthecilia structure and function. OBJECTIVE:To introduce current understanding of the structure and function of the cilium,with afocus on the study progress ofciliopathies. METHODS:PubMed databasewas searched by computerusing the keywords of“primary cilium, motion cilium,ciliopathy”.Totally 371 relevantarticlespublished from 1959 to 2015 were retrieved. After excluding repetitive and irrelevant articles,we enrolled32 English literaturesregardingcilia structure, functionand ciliopathiesin accordance with the inclusion criteria. RESULTS AND CONCLUSION:Ciliopathiesalmostinvolve every system of the human body, such asthe brain, eyes, liver, kidney, bone and reproductive system.Most ciliopathies are related to human developmental or degenerative diseases, which characterized by wide-ranging clinical and genetic heterogeneity,such asprimary ciliary dyskinesia,ploycystic kidney disease, infertility, blindness and tumor. In view ofthemedical importanceof the cilium, scholars have devoted tostudyingthe underlying celular mechanisms.Thesein turnhave revealed thatciliopathiesare not only caused bydefectiveassemblyof the cilium,but also by impaired ciliarysignaling.

BACKGROUND: Steroid-induced avascular necrosis of the femoral head is a worldwide problem, and its pathogenesis still remains unclear. Its pathogenesis is still the focus of research in this field. OBJECTIVE: To discuss the role of autophagy gene Beclin1 in steroid-induced avascular necrosis of the femoral head and the effect of 3-methyladenine on Beclin1. METHODS: Thirty-six 5-month-old Japanese white rabbits (provided by Xi'an Dilepu Biological Resource Development Co., Ltd.) were included and randomly divided into control group (intramuscular injection of normal saline), avascular necrosis of the femoral head group (intramuscular injection of methylprednisolone) and 3-methyladenine group (intramuscular injection of methylprednisolone and 3-methyladenine), with 12 rabbits in each group. The injection was conducted four times, with 1 week in between. The animals from each group were sacrificed at 1, 2, 3 and 4 weeks for sample taken. Vacant bone lacunae were counted under optical microscope. mRNA and protein expression levels of Beclin1 were detected by RT-PCR and western blot assay. RESULTS AND CONCLUSION: (1) Histomorphological observation under optical microscope showed that the rate of vacant lacunae in the avascular necrosis of the femoral head and 3-methyladenine groups was significantly increased (P ＜ 0.05), and was significantly lower in the 3-methyladenine group than that in the avascular necrosis of the femoral head group (P ＜ 0.05). (2) Glucocorticoid could rapidly stimulate the expression of Beclin1 mRNA in the femoral head tissue, and the protein level was significantly increased at the first two weeks, suggesting that autophagy was activated. 3-Methyladenine could inhibit this process. (3) These findings indicate that enhanced autophagy gene Beclin1 expression is important for avascular necrosis of the femoral head. 3-Methyladenine can regulate Beclin1, thereby controlling autophagy, which alleviates the occurrence and development of avascular necrosis of the femoral head.

ObjectiveTo investigate the difference in protein expression between hepatocellular carcinoma (HCC) patients with recurrence and those with good prognosis, the differential expression and regulatory mechanism of miR-152-3p target proteins, and the role of miR-152-3p in the recurrence of HCC. MethodsTMT-labeled proteomic sequencing and RT-PCR were used to measure the expression of proteins and the expression of miR-152-3p in the HCC tissue of six patients with recurrence at 2 years after HCC resection and six patients with good prognosis at 5 years. Six databases were used to analyze the target genes of miR-152-3p, and Gene Ontology, DAVID, and REACTOME databases were used to perform target gene screening, enrichment annotation, and signal transduction pathway enrichment analysis. Gene mutation frequency and survival curve analysis were performed for the target genes of miR-152-3p to verify the role of miR-152-3p target genes in patients with HCC recurrence. The independent samples t-test was used for comparison of continuous data between two groups, and a Kaplan-Meier analysis was performed to investigate the survival rates of liver-related genes. ResultsCompared with the patients with HCC recurrence, the patients with good prognosis after HCC resection had a significantly higher transcriptional expression level of miR-152-3p in HCC tissue (P＜0.05). The results of protein sequencing showed that there were 365 differentially expressed proteins in HCC tissue between the patients with good prognosis and the patients with recurrence, and the analysis of HCC recurrence databases showed that 17 proteins were regulated by miR-152-3p. Further analysis of the signaling pathways showed that the function of the 17 target genes regulated by miR-152-3p was enriched in the translation and regulation of mitochondria and ribosome, and multiple enrichment revealed that six target genes were closely associated with mitochondrial respiratory chain complex, i.e., AKAP1, FOXRED1, MRPL28, MRPL50, SHC1, and STAU1. Gene mutation frequency and survival curve analysis showed that the loss or weakening of the function of mitochondrial respiratory chain-related target proteins seriously affected the prognosis and survival rate of patients. ConclusionThere is a significant difference in the expression of miR-152-3p in HCC tissue between patients with good prognosis and those with recurrence after HCC resection, and miR-152-3p may lead to the recurrence of HCC by regulating the target genes AKAP1, FOXRED1, MRPL28, MRPL50, SHC1, and STAU1, acting on the mitochondrial respiratory chain, and affecting the oxidative respiratory function of cells.

Objective:To investigate the efficacy of double S-shaped elastic stable intramedullary nailing in the treatment of paediatric fractures of the distal tibia diaphyseal metaphyseal junction.Methods:From January 2018 to January 2022, a total of 25 children with fracture of the distal tibia diaphyseal metaphyseal junction were treated at Department of Pediatric Orthopedics, The Second Affiliated Hospital of Inner Mongolia Medical University. All of them were treated with closed reduction and double S-shaped elastic stable intramedullary nailing. There were 16 males and 9 females with an average age of (10.4±3.3) years, and 14 left sides and 11 right sides. The operation time, imaging results and complications were recorded after operation. At the last follow-up, the American Orthopaedic Foot & Ankle Society (AOFAS) scoring was used to evaluate the efficacy.Results:Closed reduction succeeded in all patients. The operation time was (55.6±23.7) min. Follow-up lasted (20.5±4.7) months for this cohort. Bony union was achieved in all patients after (11.5±2.7) weeks. No postoperative complications occurred in the patients, like infection, loss of reduction, disparity in length of lower limbs, delayed union or non-union. The AOFAS scoring at the last follow-up yielded 23 excellent and 2 good cases, and an excellent and good rate of 100% (25/25).Conclusion:In the treatment of paediatric fractures of the distal tibia diaphyseal metaphyseal junction, double S-shaped elastic stable intramedullary nailing is a safe, effective and feasible option.

Objective:Compared with closed reduction and percutaneous pinning (CRPP) treatment, evaluating the clinical observation of utilizing a transolecranon pin joystick technique combined with CRPP in the teatment of multidirectionally unstable supracondylar humeral fractures in children.Methods:From thirty nine pediatric multidirectionally unstable supracondylar humeral fractures hospitalized between January 2012 and January 2019, twenty seven males (69.23%) and twelve females (30.77%) were included in the study, with a average age of 6.68±2.52 years (range, 2.17-13.75 y), twenty three fractures (65.7%) were treated with CRPP (CRPP group) and the remaining Sixteen fractures (41.03%) were treated utilizing a transolecranon pin joystick technique combined with CRPP (joystick group). Both groups were followed over 16 weeks. The paired sample t test or χ2 test and Fisher's exact test were used to compare the surgical time, times of fluoroscopy, quality of reduction and neurological or vascular complications, Baumann angle, carrying angle, lateralcapitellohumeral angle, postoperative range of motion as well as function-al outcomeduringthe Sixteen weeks and the last follow-up appointment. Results:All caseswere followed up for 1.98±1.43 years, and all fractures achieved clinical healing at 4 to 6 weeks postoperation. The surgical time and times of fluoroscopy were significantly shorter for patients in the joystick group (27.17±9.68 min, 24.25±5.92 times) when compared with CRPP group (48.59±15.75 min, 49.65±23.83 times, P<0.05). All cases showed restoration of the normal lateral capitellohumeral angle. Compared with Baumann angle of normal contralateral upper extremity during the sixteen weeks and the last follow-up appointment, the quality of reduction on the anteroposterior radiographic view was significantly better for patients in the joystick group than that of CRPP group ( P<0.05). The Baumann angle of the affected upper extremity was 77.70°±2.16°, and that of the normal contralateral upper extremity was 73.78°±4.04° in the CRPP group, joystick group was 73.06°±1.81° and 72.81°±3.45°, respectively at the sixteen weeks follow-up. The Baumann angle of the affected upper extremity was 77.13°±2.20°, and that of the normal contralateral upper extremity was 74.17°±4.17° in the CRPP group, joystick group was 72.69°±1.70° and 73.38°±3.48°, respectively at the last follow-up. The range of motion and clinical outcomes based on the criteria of Flynn were similar in both groups ( P<0.05). The elbow joint function of excellent and good rate of the criteria of Flynn was 82.61%, elbow flexion was 134.13°±8.61°, elbow extension was -3.48°±6.47° in the CRPP group, joystick group was 81.25%, 132.19°±9.48° and -3.44°± 4.37°, respectively at the sixteen weeks follow-up. The elbow joint function of excellent and good rate of the criteria of Flynn was 91.30%, elbow flexion was 140.14°±5.76°, elbow extension was -0.65±3.79° in the CRPP group, joystick group was 93.75%, 141.88°±5.12° and -0.31°±3.86°, respectivelyat the last follow-up. No immediate postoperative complications were observed. Conclusion:A transolecranon pin joystick technique combined with CRPP is a safe and effective method, can decrease surgical time and times of fluoroscopy and improve quality of reduction with no increasing risk of complications for closed reduction of multidirectionally unstable supracondylar humeral fractures in children.

Objective:To explore the clinical effect of observation and psychological intervention, splint or brace fixation as well as surgical treatment on habitual dislocation of the hip (HDH) and to combine the authors' data with a compilation of the cases from the literature, evaluingating the epidemiological characteristics of HDH and the treatment scheme to maintain the stability of hip joint by systematic literature review in children.Methods:A retrospective analysis of the relevant data of 11 patients (12 hips) with HDH were treated from March 2007 to March 2021, including 2 boys and 9 girls. The age of the first dislocation was 2.25 (1.66, 3.75) years old and 4.33 (3.33, 6.17) years old at the age of diagnosis. At the same time, the relevant literature reports were searched from 1932 to 2022, and 24 HDH patients reports and clinical studies were confirmed to be included in this study according to the inclusion and exclusion criteria. The data of 33 patients (38 hips) with HDH who were obtained in the literature, including 5 boys and 28 girls. The age of the first dislocation was 2.00 (1.50, 2.00) years old and 4.00 (2.55, 5.00) years old at the age of diagnosis. A total of 44 patients (50 hips) with HDH who were analyzed, including gender, age of first dislocation, age of diagnosis, mode of stimulating dislocation, side and direction of dislocation, accompanying symptoms, family history, trauma history and imaging examination. The data of 41 patients with HDH (3 patients were excluded due to lack of treatment description) were treated with observation and psychological intervention in 19 patients, splint or brace fixation in 13 patients, and surgical treatment in 9 patients. The femoral neck-stem angle, acetabular index, central edge angle (CE angle) and Reimers instability index were measured by AutoCAD software, and the hip function was evaluated by Harris standard. Meantime, the disappearance time of dislocation was recorded.Results:The average follow-up time of 44 patients (50 hips) with HDH were 4.05±2.93 years old, and the time of disappearance of dislocation after treatment were 1.28±1.19 years old. Patients were diagnosed as 7 boys and 37 girls, 30 right and 8 left as well as 6 bilateral, the age of the first dislocation was 2.64±1.54 years old and 4.52±2.64 years old at the age of diagnosis. The data of 39 patients took hip flexion, adduction, internal rotation or hip flexion and adduction as the mode of stimulating dislocation. There were 43 patients with posterior dislocation, 41 patients with an audible "click" sound during dislocation, 36 patients with painless dislocation, and 37 patients with "vacuum phenomenon" were captured at the moment of dislocation. All patients with HDH had no specific family history and obvious history of trauma. There was no significant difference in general data between observation and psychological intervention group, splint or brace fixation group as well as surgical treatment group ( P>0.05). Harris standard to evaluate hip function, CE angle and Reimers instability index of the affected side were significantly different from those before and after treatment at the moment of dislocation ( H=127.51, P<0.001; H=55.70, P<0.001; H=54.69, P<0.001). Compared with the immediate disappearance of dislocation in the surgical treatment group, the disappearance time of dislocation in the observation and psychological intervention group and the splint or brace fixation group were significantly longer, and the difference was statistically significant ( H=20.83, P<0.001). Conclusion:Without specific family history and obvious trauma at young girls, painless posterior dislocation of hip with an audible "click" sound and "vacuum phenomenon" at the moment of dislocation are the significant epidemiological characteristics of HDH. Observation and psychological intervention, splint or brace fixation are recommended as the initial treatment scheme. When conservative treatment is ineffective, surgical treatment is needed to stabilize the hip joint quickly.

Objective:To investigate the treatment strategy for pediatric humeral supracondylar fractures with callus formation and displacement neglected for over 1 week.Methods:A retrospective analysis was made of the clinical data of 36 children who had been treated at Department of Pediatric Orthopaedics, Medical Center, The Second Affiliated Hospital, Inner Mongolia Medical University from January 2011 to January 2021 for humeral supracondylar fractures with callus formation and displacement neglected for over 1 week. There were 22 boys and 14 girls, with an age of (6.7±2.7) years (from 2.3 to 12.8 years). All fractures were Gartland type Ⅲ. The patients were divided into 2 groups according to their treatment methods: a closed reduction and percutaneous pinning (CRPP) group of 15 patients subjected to the CRPP treatment only, and a leverage group of 21 patients subjected to CRPP assisted by the "lever technique" with posterior elbow Kirschner wire prying and pulling. The 2 groups were compared in terms of operation time, fluoroscopy frequency, quality of reduction, and recovery time for elbow range of motion; the elbow range of motion, visual analogue scale (VAS), Mayo elbow performance score (MEPS) and complications were assessed at the last follow-up.Results:The 2 groups were comparable because there was no significant difference between them in the general information before operation ( P>0.05). All patients were followed up for (26.2±16.3) months (from 6 to 96 months). All the fractures obtained acceptable reduction and clinical union 4 to 6 weeks after operation. The operation time [(28.2±6.8) min] and fluoroscopy frequency [(27.0±6.0) times] in the leverage group were significantly less than those in the CRPP group [(40.8±10.8) min and (43.3±11.4) times] ( P<0.05). The CRPP group was significantly better than the leverage group in the intraoperative Baumann angle (78.1°±1.6° versus 73.7°±4.1°), lateral capitellohumeral angle (58.3°±2.6° versus 49.6°±5.2°) and horizontal rotation rate (109.5%±3.0% versus 103.2%±4.9%) ( P<0.05). The intraoperative reduction in the CRPP group was significantly closer to the normal mean value than that in the leverage group ( P<0.05). There was no significant difference in the recovery time for elbow range of motion between the CRPP and the leverage groups ( P>0.05). At the last follow-up, the Baumann angle (75.4°±2.8°) and the lateral capitellohumeral angle (53.2°±3.6°) in the leverage group were still significantly better than those in the CRPP group (78.3°±1.5° and 57.5°±2.3°) ( P<0.05). However, there was no significant difference in the elbow range of motion, VAS, MEPS or incidence of complications between the 2 groups ( P>0.05). Conclusion:To treat humeral supracondylar fractures with callus formation and displacement neglected for over 1 week in children, CRPP assisted by the "lever technique" with posterior elbow Kirschner wire prying and pulling is an efficient and accurate method, because it can lead to more satisfactory reduction than CRPP only.

ObjectiveTo investigate the role and mechanism of DNA repair regulation in the process of hepatocellular carcinoma （HCC） recurrence. MethodsHCC tissue samples were collected from the patients with recurrence within two years or the patients with a good prognosis after 5 years， and the Tandem Mass Tag-labeled quantification proteomic study was used to analyze the differentially expressed proteins enriched in the four pathways of DNA replication， mismatch repair， base excision repair， and nucleotide excision repair， and the regulatory pathways and targets that play a key role in the process of HCC recurrence were analyzed to predict the possible regulatory mechanisms. The independent samples t-test was used for comparison of continuous data between two groups； a one-way analysis of variance was used for comparison between multiple groups， and the least significant difference t-test was used for further comparison between two groups. ResultsFor the eukaryotic replication complex pathway， there were significant reductions in the protein expression levels of MCM2 （P=0.018）， MCM3 （P=0.047）， MCM4 （P=0.014）， MCM5 （P=0.008）， MCM6 （P=0.006）， MCM7 （P=0.007）， PCNA （P=0.019）， RFC4 （P=0.002）， RFC5 （P<0.001）， and LIG1 （P=0.042）； for the nucleotide excision repair pathway， there were significant reductions in the protein expression levels of PCNA （P=0.019）， RFC4 （P=0.002）， RFC5 （P<0.001）， and LIG1 （P=0.042）； for the base excision repair pathway， there were significant reductions in the protein expression levels of PCNA （P=0.019） and LIG1 （P=0.042） in the HCC recurrence group； for the mismatch repair pathway， there were significant reductions in the protein expression levels of MSH2 （P=0.026）， MSH6 （P=0.006）， RFC4 （P=0.002）， RFC5 （P<0.001）， PCNA （P=0.019）， and LIG1 （P=0.042） in recurrent HCC tissue. The differentially expressed proteins were involved in the important components of MCM complex， DNA polymerase complex， ligase LIG1， long patch base shear repair complex （long patch BER）， and DNA mismatch repair protein complex. The clinical sample validation analysis of important differentially expressed proteins regulated by DNA repair showed that except for MCM6 with a trend of reduction， the recurrence group also had significant reductions in the relative protein expression levels of MCM5 （P=0.008）， MCM7 （P=0.007）， RCF4 （P=0.002）， RCF5 （P<0.001）， and MSH6 （P=0.006）. ConclusionThere are significant reductions or deletions of multiple complex protein components in the process of DNA repair during HCC recurrence.