Objective:To explore the correlation between functional constipation （FC）and its related factors with acute appendicitis（AA）in children，so as to provide a reference for the prevention and clinical diagnosis and treatment of children with AA. Methods:A case-control study was conducted on 170 children diagnosed with AA in the Department of Pediatrics，the Second Affiliated Hospital of Air Force Military Medical University，from August 2022 to March 2023，and 170 non-AA children during the same period were selected as control objects.The clinical data，incidence of FC，symptoms related to FC，Bristol stool classification，past constipation history and other information were compared between two groups.Results:The incidence of FC in 170 children with AA was 22.9%（39/170），which was significantly higher than 10.6%（18/170）in the non-AA group（ P<0.01）；For children under four years old，the proportion of faecal retention in AA group was higher than that in non-AA group（25.6% vs. 9.3%, P<0.05）；For children ≥ 4 years old，the proportion of faecal retention and dyschezia in AA group were higher than those in non-AA group（28.2% vs. 6.9%，29.0% vs. 16.4%，respectively,all P<0.05）.The proportion of past constipation history in AA group was higher than that in non-AA group（29.4% vs.14.1%）.The duration of constipation in AA group was longer than that in non-AA group [0.00（0.00，1.25）month vs. 0.00（0.00，0.00）month,all P<0.01].The proportion of children with low Bristol stool classification in AA group was higher than that in non-AA group（ P<0.01）.Multivariate Logistic regression analysis showed that faecal retention[ OR=6.186（95% CI 2.336~16.380）] and long constipation time [ OR=1.310（95% CI 1.095~1.567）]were independent risk factors for AA in children（all P<0.05）. Conclusion:The incidence of faecal retention in children with AA is higher than that in children without AA，and the median duration of constipation is longer than that in children without AA.Fecal retention and long-term constipation are independent risk factors for AA in children.

Objective:To summarize the clinical, pathological and muscle magnetic resonance imaging (MRI) features of human immunodeficiency virus (HIV)-associated nemaline myopathy (NM; HIV-NM).Methods:The present patient was a 23-year-old man with HIV infection who developed progressive proximal weakness and atrophy for more than 10 months. He was admitted to the Department of Neurology of Beijing Ditan Hospital in early June 2021. Electromyography showed myogenic findings. The serum creatine kinase was 202.4 U/L. CD 4+ count was 585×10 6/L. Serum monoclonal immunoglobulin (M protein) was negative. The patient underwent MRI examination of bilateral thigh muscles, biopsy of left biceps brachii and gene detection. The clinical, pathological and muscle MRI changes of HIV-NM were summarized based on the literature review. Results:MRI examination of bilateral thigh muscles showed edema changes. Muscle biopsy showed nemaline structures in some muscle fibers, accompanied by fiber atrophy and regeneration. No gene mutation related to clinical phenotype was found by second-generation sequencing. After intravenous immunoglobulin combined with prednisone, the patient′s weakness symptoms were significantly improved. A total of 17 cases of HIV-NM (including the present case) were identified in the literature, who were aged (33.7±9.1) years. Fifteen were males and two were females. All patients developed proximal limb weakness. Creatine kinase was normal or slightly elevated. Serum monoclonal protein was positive in 3 cases (3/7). Immunosuppressive therapy was effective.Conclusions:The main clinical characteristics of HIV-NM are progressive proximal limb weakness and muscle atrophy. The muscle pathology shows a large number of nemaline structures in atrophic muscle fibers. Muscle edema can be seen on muscle MRI. This is the first report of HIV-NM in China, which may be a special subtype of immune myopathy.

To investigate the efficacy and safety of modified endoscopic retrograde appendicitis therapy (ERAT) in children with acute uncomplicated appendicitis complicated with intussusception. Data of 6 patients with acute uncomplicated appendicitis complicated with intussusception who received modified ERAT were collected from October 2018 to February 2020 in Pediatrics Department of Tangdu Hospital. The reduction rate of intussusception, the success rate of ERAT appendiceal intubation and endoscopic treatment, the remission time of clinical symptoms, the time of white blood cells return to normal, the length of hospital stay, complications and recurrence were summarized. All the 6 patients were confirmed to be acute uncomplicated appendicitis combined with ileocecal intussusceptions under colonoscopy. Endoscopic intussusception reduction and ERAT were performed successfully. The mean time of disappearance of abdominal tenderness was 1.3 days after the therapy, and the time of white blood cells return to normal was 1 day after surgery. The endoscopic treatment were all successful and the length of hospital stay was 3-6 days. No complications or recurrence of intussusception and appendicitis occurred during the follow-up period of 6-12 months. Modified ERAT can effectively, safely and minimally invitingly treat acute uncomplicated appendicitis with intussusception in children on the basis of definite diagnosis.

Objective:To summarize the clinical and gene mutation characteristics of a child with Cowden syndrome and review the literature.Methods:The clinical data of a child with Cowden syndrome treated in the First Affiliated Hospital of Xinxiang Medical University in June 2020 were analyzed retrospectively.Taking " Cowden syndrome" , " PTEN gene" , " hamartoma polyps" , "child" , " Cowden syndrome and child" and " PTEN and child" as key words, literature was retrieved from Chinese databases (China National Knowledge Internet database and Wanfang database) and the PubMed database from the establishment of the database to March 2021. Results:A 13-year-old male had intermittent abdominal pain and abdominal distension for 5 months.Electron microscopic gastroenteroscopy showed multiple polyps, and focal lymphocyte aggregation and infiltration were found in tissue biopsy.Whole exon sequencing revealed a hemizygous mutation of c. 475 (exon5) A>T in PTEN gene, which led to the transformation of the 159 th amino acid from arginine to tryptophan.The prediction results of the tertiary structure of the protein indicated that the variation might affect the spatial structure of the protein and damage the protein function.According to the clinical characteristics, Cowden syndrome was diagnosed.The pedigree confirmed that the variation was inherited from the mother, who had a similar phenotype.No qualified Chinese report was retrieved.Among 41 English studies on PTEN gene mutation in children, there were only two reports related to pediatric Cowden syndrome.The hemizygous mutation of PTEN gene was not reported. Conclusions:The missense mutation of c. 475 (exon5) A>T in PTEN gene in this study is a novel cause of Cowden syndrome, and the case is the first case report in China.

Objective:To study the value of ultrasound-guided endoscopic retrograde appendicitis therapy in children with uncomplicated appendicitis.Methods:This study was a single center, retrospective study, including all electronic cases of appendicitis diagnosed clinically in Department of Pediatrics, the Second Affiliated Hospital of Air Force Military Medical University from October 2018 to October 2020 and received ultrasound-guided endoscopic retrograde appendicitis therapy.The clinical features, treatment and prognosis of the children were retrospectively analyzed.Results:A total of 152 electronic cases were included, there were 77 males and 75 females, aged(6.84±3.09) years.All the 152 children were treated with ultrasound-guided endoscopic retrograde appendicitis therapy.Intubation success rate and clinical success rate was 98.03%(149/152 cases)and 97.99%(146/149 cases), respectively.The median time of endoscopic therapy was 42.50 (31.00, 56.00) minutes.Mean postoperative hospital stay was (2.81 ±1.41) days, and the mean total hospital stay was (4.19 ±1.71) days.A total of 139 patients were followed up with a median follow-up time of 5 (1, 26) months.During the follow-up, the recurrence rate was 7.19%(10/139 cases), and the median time of recurrence was 2 (1, 3) months.Conclusions:Ultrasound-guided endoscopic retrograde appendicitis therapy had high effective rate and low recurrence rate in children with uncomplicated appendicitis, preserved the physiological function of appendix and avoided radiation damage.It can be used as a safe and effective treatment for acute and chronic uncomplicated appendicitis in children.

Objective    To investigate the multidisciplinary management of patients with acute type A aortic dissection in late pregnancy. Methods    The clinical data of 3 patients admitted to the Second Affiliated Hospital of Army Medical University from 2018 to 2019 were analyzed. Their age ranged from 27 to 32 years, while gestational age was 34-37 weeks. Sudden chest pain was the main clinical symptom before operation. All 3 patients underwent Bentall surgery after cesarean section under general anesthesia, of whom 2 patients received total arch replacement (TAR) combined with frozen elephant trunk (FET) implantation and 1 received coronary artery bypass grafting (CABG) additionally. Results    No patient died during the perioperative period, and all the newborns were delivered successfully and survived healthily. The ICU stay was 3-5 d. The postoperative hospital stay was 15-18 d. The follow-up was 250-751 d. There was no recurrence or death. One patient who developed spontaneous pneumothorax and hydropneumothorax was cured in our center. Conclusion    The multidisciplinary diagnosis and treatment strategy plays a crucial role in saving the life of pregnant patients with acute type A aortic dissection.

Objective:To investigate the effect of periacetabular osteotomy (PAO) assisted by preoperative 3D printing planning on the treatment of early developmental dislocation of the hip (DDH).Methods:A retrospective study was conducted of the 39 patients (44 hips) with CROWE type Ⅰ DDH who had been treated at Department of Orthopaedics, Guizhou Provincial People＇s Hospital from May 2015 to April 2019. In the observation group of 19 patients (22 hips) who were treated by PAO assisted by preoperative 3D printing planning, there were 4 males (4 hips) and 15 females (18 hips) with a mean age of 23(19, 39) years; in the control group of 20 patients (22 hips) who were treated by mere PAO, there were 2 males (2 hips) and 18 females (20 hips) with a mean age of 23(20, 29) years. The 2 groups were compared in terms of operation time, intraoperative bleeding, hospital stay, fluoroscopic frequency, Wiberg lateral center-edge angle (LCE), acetabular index (AC), acetabulum coverage rate, Harris hip scores (HHS) and complications at the final follow-up.Results:There were no significant differences between the 2 groups in their preoperative general data, showing compatibility of the 2 groups ( P>0.05). Both groups were successfully followed up for 6 to 12 months (mean, 9.6 months). The operation time [100 (90, 120) min], intraoperative bleeding [160 (150, 180) mL], hospital stay [6 (5, 7) d], and fluoroscopic frequency [24 (22, 25) times] in the observation group were all significantly better than those in the control group [140 (100, 157) min, 200 (180, 250) mL,9(7, 11) d and 28 (24, 32) times] in the control group ( P<0.05). However, there were no significant differences between the 2 groups in LCE, AC or acetabulum coverage rate immediately after operation, or in HHS or complications at the final follow-up( P>0.05). Conclusions:Although PAO has a definite therapeutic effect on early DDH, PAO assisted by preoperative 3D printing planning can effectively reduce operation time, intraoperative bleeding, hospital stay and intraoperative fluoroscopy. Thus, combination of the two can result in a better therapeutic effect than PAO alone.

PURPOSE: Previous studies have confirmed that microRNAs play important roles in the pathogenesis of acute aortic dissection (AAD). Here, we aimed to explore the role of miR-145 and its regulatory mechanism in the pathogenesis of AAD. MATERIALS AND METHODS: AAD tissue samples were harvested from patients with aortic dissection and normal donors. Rat aortic vascular smooth muscle cells (VSMCs) were transfected with miR-145 mimic/inhibitor or negative control mimic/inhibitor. Gene and protein expression was measured in human aortic dissection tissue specimens and VSMCs by qRT-PCR and Western blot. Luciferase reporter assay was applied to verify whether connective tissue growth factor (CTGF) was a direct target of miR-145 in VSMCs. Methyl thiazolyl tetrazolium assay was used to detect VSMC viability. RESULTS: miR-145 expression was downregulated in aortic dissection tissues and was associated with the survival of patients with AAD. Overexpression of miR-145 promoted VSMC proliferation and inhibited cell apoptosis. Moreover, CTGF, which was increased in aortic dissection tissues, was decreased by miR-145 mimic and increased by miR-145 inhibitor. Furthermore, CTGF was confirmed as a target of miR-145 and could reverse the promotion effect of miR-145 on the progression of AAD. CONCLUSION: miR-145 suppressed the progression of AAD by targeting CTGF, suggesting that a miR-145/CTGF axis may provide a potential therapeutic target for AAD.
Animals ; Apoptosis ; Blotting, Western ; Connective Tissue Growth Factor ; Humans ; Luciferases ; MicroRNAs ; Muscle, Smooth, Vascular ; Rats ; Tissue Donors

Objective To investigate the efficacy of CT scanning for 3D reconstruction and 3D printing in the internal fixation for in situ femoral condyle fractures.Methods A retrospective study was conducted of the 111 patients with in situ femoral condyle fracture who had been treated by internal fixation at Department of Traumatic Orthopaedics,The First People's Hospital of Hefei from June 2013 to January 2018.They were 59 males and 52 females,aged from 20 to 59 years(average,47 years).In 74 of them (observation group),the internal fixation was assisted by 3D printing using 3D images.There were 38 males and 36 females with an age of 44.5 ±4.7 years;there were 20 cases of type C1,40 cases of type C2 and 14 cases of type C3 according to the AO classification.In the other 37 patients (control group),the operation was assisted only by 3 D scanning.There were 21 males and 16 females with an age of 43.1 ±4.6 year;there were 8 cases of type C1,19 cases of type C2 and 10 cases of type C3 according to the AO classification.The 2 groups were compared in terms of operation time,clinical fracture healing time,intraoperative blood loss,hospital stay,number of fluoroscopy,knee joint score of Hospital for Special Surgery (HSS),fixation modes and postoperative complications.Results There were no significant differences in the preoperative general data between the 2 groups,showing comparability (P ＞ 0.05).All the patients were followed up for 8 to 12 months (average,10.6 months).The observation group had significantly shorter operation time (62.3 ± 4.7 minutes),fracture healing time (21.2 ± 3.4 weeks) and hospital stay (14.9 ± 3.3 days) than the control group (89.5 ± 6.0 minutes,25.1 ± 3.5 weeks,23.5 ± 3.5 days) (P ＜ 0.05).In the observation group,the intraoperative blood loss (84.1 ± 11.1 mL),fluoroscopy number (2.1 ±0.3 times) and HSS score (94.1 ± 4.2 points) were significantly less than those in the control group (129.6 ± 14.7 mL,4.7 ± 0.4 times and 86.5 ± 4.6 points) (P ＜ 0.05).There were no significant differences between the 2 groups in the use of L type iliac plate,dynamic iliac screws,retrograde interlocking intramedullary nail or minimally invasive internal fixation system (P ＞ 0.05).There were no significant differences in abnormal healing,joint adhesion,fixation fracture or incision infection between the 2 groups either (P ＞ 0.05).Conclusions CT scanning for 3D reconstruction can be used for multi-angle observation of the bone fragments in patients with femoral condyle fracture and 3D printing based on 3D reconstruction for manufacture of physical model of individualized fracture to assist the internal fixation.Combination of the two can help choose an appropriate fixation mode to facilitate fracture reduction.

To investigate the clinical features, auxiliary examination and characteristics for anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis and its concomitant seizure.
 Methods: A total of 20 patients diagnosed as anti-NMDAR encephalitis were enrolled from January 2016 to September 2018 in Xiangya Hospital. The data including the clinical features, auxiliary examination, characteristics of seizure, treatment and prognosis were collected. The discharged patients were followed up for half a year.
 Results: The initial symptom in patients with anti-NMDAR encephalitis were mainly psychiatric symptom and seizure. Most of the EEG result were diffused slow waves. The mainly type of seizure in patients with anti-NMDAR encephalitis showed generalized tonic-clonic seizure. Patients occurred consciousness during the onset of the disease. MRI showed that patients with temporal lobe were more inclined to occur seizure than patients with anti-NMDAR encephalitis (P<0.05). After standardized treatment, 20 patients showed a significant improvement in modified Rankin Scale (mRS) scores and the seizure was under control within half a year. 
 Conclusion: Patients with temporal lobe affected in MRI should pay attention to the possibility of seizure occurrence. Anti-epileptic drugs and immunotherapy should be used promptly in patient with seizure. After standardized treatment, the prognosis of patients will be mostly good.
Anti-N-Methyl-D-Aspartate Receptor Encephalitis ; Humans ; Immunotherapy ; Magnetic Resonance Imaging ; Receptors, N-Methyl-D-Aspartate ; Seizures