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AIM: To investigate the clinical features and genetic background of autosomal recessive Wolfram syndrome caused by WFS1 gene mutation.METHODS: A pedigree with autosomal recessive Wolfram syndrome was studied by clinical examination, gene analysis and bioinformatics.RESULT: It was found that the proband and his brother had diabetes, color weakness and optic neuropathy. In addition, his brother had diabetes insipidus. Whole-exome sequencing(WES)analysis showed that there were two heterozygous variations in the WFS1 gene exon 8 of the two brothers: c.941G>A(p.W314X)and c.2309T>G(p.F770C), and were co-separated from the clinical phenotype in this family.CONCLUSION: The compound heterozygous mutation of WFS1 gene is associated with Wolfram syndrome in this pedigree. Among them, c.941G>A(p.W314X)has not been reported yet.
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Objective: To evaluate the associations of body mass index (BMI) and waist circumference with hospitalization risk of kidney stones in Chinese adults. Methods: This study used long-term follow-up data from China Kadoorie Biobank. After excluding participants with chronic kidney disease, cancer, and extreme BMI or waist circumference values at baseline, 502 096 participants were included in the present analysis. Cox proportional hazards regression models were used to estimate the associations of BMI and waist circumference with hospitalization risk of kidney stones. Results: During an average follow-up period of (10.7±2.2) years, 12 396 participants were hospitalized for the first time with kidney stones. Using the participants with BMI (kg/m2) of 20.5-22.4 as reference, the multivariable-adjusted HR (95%CI) for those with BMI of <18.5, 18.5-20.4, 22.5-23.9, 24.0-25.9, 26.0-27.9, 28.0-29.9, and ≥30.0 were 0.96 (0.87-1.05), 0.94 (0.88-1.00), 1.11 (1.05-1.17), 1.25 (1.18-1.32), 1.29 (1.21-1.37), 1.39 (1.28-1.50), and 1.54 (1.40-1.71), respectively. For every 1 kg/m2 increase in BMI, the HR of kidney stones hospitalization increased by 4% (HR=1.04, 95%CI: 1.04-1.05). Using the participants with waist circumference (cm) of 75.0-79.9 as reference, the multivariable-adjusted HR (95%CI) for those with waist circumference of <65.0, 65.0-69.9, 70.0-74.9, 80.0-84.9, 85.0-89.9, 90.0-94.9, and ≥95.0 were 0.82 (0.74-0.91), 0.85 (0.80-0.92), 0.95 (0.89-1.00), 1.16 (1.09-1.22), 1.22 (1.15-1.30), 1.28 (1.19-1.38), and 1.46 (1.35-1.58), respectively. For every 5 cm increase in waist circumference, the HR of kidney stones hospitalization increased by 9% (HR=1.09, 95%CI: 1.08-1.10). After mutually adjusting for BMI and waist circumference in the above models, the association between BMI and hospitalization risk of kidney stones disappeared. In contrast, the association between waist circumference and hospitalization risk of kidney stones remained unchanged. Conclusions: BMI and waist circumference were positively associated with hospitalization risk of kidney stones. The increased waist circumference was an independent risk factor for kidney stones among Chinese adults.
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Adult , Humans , Body Mass Index , Hospitalization , Kidney Calculi/epidemiology , Risk Factors , Waist CircumferenceABSTRACT
Objective:To analyze the clinical and molecular genetic characteristics of a Chinese family with congenital cataract-microcornea syndrome.Methods:The method of pedigree investigation was adopted.A Chinese Han family with congenital cataract-microcornea syndrome was recruited in Xiamen Eye Center of Xiamen University.All the family members received detailed ophthalmologic examination including the best corrected visual acuity, intraocular pressure measurement by handheld applanation tonometry, slit lamp biomicroscopy, color fundus photography, B-scan ultrasonography, corneal diameter, anterior segment optical coherence tomography, ultrasound biomicroscopy, corneal endoscopy, and corneal topography.Genomic DNA was extracted from peripheral venous blood from some patients and unaffected family members.Targeted high-throughput DNA sequencing was performed on the proband.The sequencing chip contained 188 known pathogenic genes related to lens abnormalities.Suspected pathogenic genes were verified by Sanger sequencing in phenotypically normal family members to identify the co-segregation and the disease-causing gene.Bioinformatics analysis was performed to analyze the pathogenicity of variants by REVEL.Conserved protein domains were analyzed by InterPro.Physicochemical property of the mutant protein was analyzed by ProtParam.The deleteriousness of the protein was predicted by PolyPhen-2.Homology of the variants in pathogenic gene was analyzed by NCBI website to compare the conservation among various species.This study followed the Declaration of Helsinki.The study protocol was approved by the Ethics Committee of Xiamen Eye Center of Xiamen University (No.XMYKZX-LW-2009-003).Written informed consent was obtained from each subject prior to entering the study cohort.Results:There were 39 members of 4 generations in this family including 11 patients with an autosomal dominant inheritance pattern.Clinical features of the patients included congenital cataract and microcornea.No obvious abnormality was found in ophthalmic and general examination.A heterozygous mutation c. 61C>T in the CRYAA gene was found, resulting in the mutation of the amino acid from arginine to tryptophan (p.Arg21Trp) at position 21, consistent with co-segregation.The number of cationic cluster in the mutant protein decreased, and the hydrophilicity and stability were reduced.The variant was predicted to be deleterious and was highly conserved in multiple species. Conclusions:A novel heterozygous mutation c.61C>T p. Arg21Trp in CRYAA gene is considered as the causal gene of this family.It is the first time this variant has been reported in China.
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Normal tension glaucoma (NTG) is almost the most difficult type of glaucoma to diagnose.The difficulty lies in the lack of specificity of the symptoms and signs of glaucomatous optic neuropathy (GON), so the reevaluation of NTG is a new understanding of GON.As a subtype of primary open-angle glaucoma (POAG), NTG is difficult to accurately conceptualize.One of the reasons is that the intraocular pressure (IOP) is closely linked to the occurrence of GON in POAG but not in NTG.GON seems to be secondary to a number of local or systemic disorders, including vascular dysfunction in the optic nerve head induced by compression (elevated IOP) or ischemia, hypoxia, migraine, Flammer syndrome, intracranial hypotension, low body mass index, low estrogen levels, nocturnal hypotension, obstructive sleep apnea-hypopnea syndrome, Alzheimer disease, Parkinson disease, and genetic background, which may influence the flow in the radial peripapillary capillaries (RPCs) and the underfilling of RPCs causing retinal ganglion cell damage.In some cases, GON does not progress with systemic diseases under control, and these systemic diseases are not risk factors for NTG but may be the cause of GON (or GON is one of the manifestations of these diseases). If these causes are not excluded and NTG is diagnosed only on the basis of GON, chamber angle opening, and normal IOP, it is easy to cause misdiagnosis, and the complete exclusion of these diseases is short of clinical significance in practice.In conclusion, the diagnosis of NTG may not be made before the other ocular or systemic disorders capable of presenting with GON are ruled out, and the concept and diagnostic criteria of NTG should be reconsidered.
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Objective@#To examine the effects of obesity and central obesity on hypertension, so as to provide insights into the prevention and control measures of hypertension.@*Methods@#From September to December 2018, residents at ages of 35 to 75 years were sampled using the multi-stage random sampling method in Baiyin District, Baiyin City, Gansu Province, and subjected to questionnaire surveys and physical examinations. The interaction between obesity/central obesity and hypertension was evaluated using logistic regression analysis. The synergy index ( SI ), relative excess risk due to interaction ( RERI ) and attributable proportion due to interaction ( AP ) were calculated using Excel compiled by Andersson et al.@*Results@#A total of 6 246 questionnaires were allocated and 6 169 valid questionnaires were recovered, with an effective recovery rate of 98.77%. The respondents included 3 038 men ( 49.25% ) and 3 131 women (50.75%), with a mean age of ( 52.05±8.78 ) years. There were 832 respondents with obesity ( 13.49% ) and 2 278 with central obesity ( 36.93% ). The crude and standardized prevalence rates of hypertension were 35.89% and 33.05%, respectively. Multivariable logistic regression analysis showed that obesity ( OR=2.020, 95%CI: 1.705-2.393 ) and central obesity ( OR=1.622, 95%CI: 1.433-1.836 ) were statistically associated with hypertension. There was no multiplicative interaction between obesity or central obesity and hypertension ( OR=1.011, 95%CI: 0.655-1.560 ), and no additive interaction was detected between obesity or central obesityand hypertension ( SI=1.405, 95%CI: 0.815-2.424; RERI=0.658, 95%CI: -0.298 to 1.614; AP=0.201, 95%CI: -0.075 to 0.476 ).@*Conclusions@#Obesity and central obesity increase the risk of hypertension; however, no interaction is detected between obesity or central obesity and hypertension.
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Hepatic cystic echinococcosis is a chronic parasitic disease caused by the infection with the larvae of Echinococcus granulosus in human or animal liver tissues. As a chronic active infectious disease, tuberculous empyema mainly invades the pleural space and then causes visceral and parietal pleura thickening. It is rare to present comorbidity for hepatic cystic echinococcosis and tuberculous empyema. This case report presents a case of hepatic cystic echinococcosis complicated with tuberculous empyema misdiagnosed as hepatic and pulmonary cystic echinococcosis, aiming to improve clinicians’ ability to distinguish this disorder.
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Objective:To evaluate arthroscopy-assisted tibial bone tunnel fixation combined with double-row anchors in the treatment of tibial eminence fracture.Methods:The 23 patients were retrospectively analyzed who had been treated at Department of Orthopedics, Beiing Shunyi District Hospital for tibial eminence fractures by arthroscopy-assisted tibial bone tunnel fixation combined with double-row anchors from October 2015 to December 2019. They were 15 males and 8 females, aged from 12 to 55 years (average, 35.5 years). All the injuries were unilateral (14 right and 9 left sides). According to the modified Meyers-McKeever classification, 2 cases belonged to type Ⅱ, 18 cases to type Ⅲ and 3 cases to type Ⅳ. Range of motion of the knee, Lysholm and International Knee Documentation Committee (IKDC) scores were observed before surgery, 1 month and 12 months after surgery. The anterior tibial slope angle (ATSA) on CT was measured preoperatively and 1 month after surgery for evaluation of fracture reduction.Results:All the 23 patients were followed up for an average of 23 months (from 12 to 52 months). Postoperatively, limited knee movement was observed in 2 patients and non-anatomic reduction in one patient. At 1 month and 12 months after operation, the Lysholm scores (61.4 ± 3.5 and 90.4 ± 4.3) and IKDC scores (69.6 ± 4.2 and 88.5 ± 3.0) were significantly improved compared with the preoperative values (45.4 ± 6.8 and 49.6 ± 3.9, respectively) ( P<0.05). ATSA was significantly restored from preoperative 4.2° ± 5.7° to -11.7° ± 2.9° at 1 month after operation ( P<0.05). Conclusion:In the treatment of tibial eminence fracture, arthroscopy-assisted tibial bone tunnel fixation combined with double-row anchors can achieve anatomical reduction and firm fixation, leading to satisfactory surgical outcomes.
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Imaging and serological approaches play an important role in the diagnosis and treatment of alveolar echinococcosis; however, they also suffer from some problems during their applications in clinical practices, which urges the identification of potential diagnostic markers. Novel serological, genomics and proteomics diagnostic markers alone or in combination may increase the sensitivity and specificity in early diagnosis of alveolar echinococcosis, which play vital roles in monitoring of disease courses and prognostic evaluation. This review mainly presents the advances in the studies on novel diagnostic markers for alveolar echinococcosis.
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Rotator cuff tears are one of the three most prevalent musculoskeletal disorders, with the supraspinatus tendon as the most common lesion site. It may lead to chronic shoulder pain, weakness and limited joint motion, and eventually to secondary degeneration of the shoulder. As traditional biomechanical experiments are limited by measurement techniques and ethical issues, it is almost impossible to clarify the stress distributions at the rotator cuff under physiological and pathological conditions. Recently, advances in computer science, software development and image processing have rapidly improved finite element models of shoulder joint which promote the researches into pathogenesis of rotator cuff tears and their surgical techniques, making finite element analysis an indispensable means in the biomechanic research of rotator cuff. This paper reviews the recent literature available in China and abroad to expound on the element models of shoulder joint applied in the researches into pathogenesis and surgery of rotator cuff tear.
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Objective:To investigate the application value of triangular modal construed for planning approach of laparoscopic local resection of liver tumors located in superior part of central liver lobe.Methods:The retrospective and descriptive study was conducted. The clinicopathological data of 10 patients who underwent local laparoscopic resection of liver tumors located in superior part of central liver lobe at the Affiliated Hospital of Qingdao University from January to June 2020 were collected. There were 6 males and 4 females, aged from 41 to 63 years, with a median age of 54 years. Preoperative triangular model was constructed for all patients to plan approach of laparoscopic local resection of liver tumors located in superior part of central liver lobe. Observation indicators: (1) preoperative general situations of patients; (2) surgical situations; (3) follow-up. Follow-up was conducted by outpatient examination or telephone interview to detect tumor recurrence and survival of patients up to February 2021. Measurement data with normal distribution were expressed as Mean± SD. Count data were expressed was absolute numbers. Results:(1) Preoperative general situations of patients: hepatocellular carcinoma was found in 7 cases, inthahepatic cholangiocarcinoma was found in 2 cases and ovarian cancer with liver metastasis was found in 1 case. Three cases had tumors located in S4a segment, 2 cases had tumors located in ventral subsegment of S8 segment, 2 cases had tumors located in dorsal subsegment of S8 segment, and 3 cases had tumors across the ventral segment of S4a+S8. The diameter of tumors was (3.4±1.0)cm. (2) Surgical situation: all the 10 patients underwent R 0 resection successfully, with no intraoperative blood transfusion or conversion to open surgery. The operation time of 10 patients was (149±59)minutes, the volume of intraoperative blood loss was (294±163)mL, the minimum surgical margin of specimen was (1.1±0.2)cm. The alanine aminotransferase was (324±151)U/L on the postoperative first day, the aspartic aminotransferase was (401±113)U/L on the postoperative first day, and the duration of postoperative hospital stay was (9±4)days. No bile leakage, hemorr-hage, reoperation or other complications occurred in all patients. (3) Follow-up: 10 patients were followed up for 7?13 months, with a median follow-up time of 11 months. All patients had no margin recurrence or distant metastasis. Conclusion:It is safe and feasible to construct triangular modal for planning approach of local laparoscopic resection of liver tumors located in superior part of central liver lobe.
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Objective To investigate the changes of mitochondrial metabolic functions of macrophages following Echinococcus multilocularis infections, so as to provide insights into the pathogenesis of alveolar echinococcosis. Methods Two groups were assigned according to different treatment methods. In the culture group, mouse leukemic monocyte macrophage RAW264.7 cells were cultured with 2 000 E. multilocularis at a ratio of 500∶1, while RAW264.7 cells in the control group were given no treatment. Then, both the culture and control groups were further divided into the 24 h and 72 h subgroups. Mitochondria were stained with MitoTracker® Deep Red FM and the mean fluorescence intensity of macrophage mitochondria was measured with the Cytation 5 Cell Imaging Multi-Mode Reader. The mitochondrial DNA copy number was quantified using the quantitative real-time PCR (qPCR) assay, and the mitochondrial energy metabolism was monitored using the Seahorse XF assay. In addition, the mitochondrial reactive oxygen species and mitochondrial membrane potential were detected using flow cytometry. Results The mean fluorescence intensities of macrophage mitochondria were significantly lower in the 24 h (15 341 ± 2 532 vs. 17 823 ± 3 429; t = 6.379, P < 0.01) and 72 h (18 102 ± 3 505 vs. 21 511 ± 5 144; t = 17.680, P < 0.01) culture subgroups than in the corresponding control subgroups, and lower mitochondrial DNA copy numbers were measured in the 72 h culture subgroup than in the 72 h control group [(3.23 × 109 ± 1.78 × 107) vs. (4.39 × 109 ± 3.70 × 107); t = 8.85, P < 0.001]. The oxygen consumption rates were significantly greater in the 24 h [(241.70 ± 73.13) pmol/min vs. (69.05 ± 52.30) pmol/min; t = 7.89, P < 0.01] and 48 h culture groups [(249.50 ± 42.06) pmol/min vs. (60.28 ± 40.66) pmol/min; t = 8.64, P < 0.01] than in the corresponding control groups, and a higher extracellular acidification rate was seen in the 48 h culture group than in the 48 h control group ([ 111.6 ± 17.49) mpH/min vs. (35.05 ± 7.57) mpH/min; t = 16.90, P < 0.01]. In addition, flow cytometry detected higher mean fluorescence intensity of mitochondrial reactive oxygen species (58 264 ± 10 087 vs. 4 307 ± 97; t = 12.930, P < 0.01) and lower mitochondrial membrane potential (9.833% ± 2.285% vs. 2.667% ± 0.208%; t = 6.645, P < 0.01) in the 72 h culture group than in the control group. Conclusions E. multilocularis infection may impair mitochondrial functions and inhibit oxidative phosphorylation of macrophages, resulting in increased macrophage glycolysis. It is speculated that the alteration of macrophage metabolic states may contribute to the mechanisms underlying the development and progression of alveolar echinococcosis.
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Dendritic cells (DCs), a type of antigen-presenting cells (APC), are recognized as an important regulator of immune response and immune tolerance, and play a critical role in the host innate immunity and adaptive immunity. Previous studies have shown that the long-term parasization of Echinococcus in the host is strongly associated with the host immune tolerance induced by DCs. This review summarizes the research progress of the role of DCs in host immune tolerance caused Echinococcus infection, aiming to provide the theoretical basis and insights into the management and immunotherapy of Echinococcus infections.
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A patient with cystic echinococcosis was presented with primary lesions in the waist and hip. The case was misdiagnosed as subcutaneous abscess at initial diagnosis, and then definitively diagnosed as echinococcosis by means of imaging examinations and anti-Echinococcus antibody test. This case was reported with aims to improve the awareness of cystic echinococcosis among clinical physicians to avoid and reduce the misdiagnosis and missing diagnosis.
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The patients with hepatic alveolar echinococcosis have unapparent early symptoms due to invasive and slow growth, and the disease is usually at an advanced stage upon diagnosis, which has a poor prognosis. Early diagnosis of hepatic alveolar echinococcosis is therefore of great importance. The advances in the ultrasound techniques continues to improve the accuracy for the diagnosis of hepatic alveolar echinococcosis, and ultrasound plays a critical role in the treatment and assessment of therapeutic efficacy for hepatic alveolar echinococcosis. This paper mainly reviews the application of ultrasound in the diagnosis and treatment of hepatic alveolar echinococcosis.
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ObjectiveThere are many kinds of intestinal cleansing drugs in clinical practice at present, but there is no universal and effective intestinal cleansing program. In this study, sodium polyacrylate was used as a candidate drug for intestinal preparation to explore its feasibility, efficacy and safety for intestinal preparation in mice.Methods24 mice fasted for 12 hours were divided, with random number table method, into 4 groups (6 mice in each): blank group, sodium phosphate group, polyethylene glycol group and sodium polyacrylate solution group. Except that the blank group was given isotonic saline (0.6mL/20g) to fill the stomach, the other groups were given sodium phosphate (0.5mL/20g), polyethylene glycol(0.6mL/20g) and sodium polyacrylate solution (0.6mL/20g) to fill the stomach, and the small intestinal propulsion (carbon powder propulsion), the defecation and intestine volume in mice were observed to explore the effect of sodium polyacrylate on the mice colon cleansing.ResultsAfter administration by gavage for 15min, compared with the blank group [(62.72±6.58) %] and the sodium phosphate group [(66.40±9.53) %], the carbon powder propulsion rate of the sodium polyacrylate solution group [(81.17±4.75) %] significantly increased (P<0.05). The number of fecal excretion [(11.5±2.4) granules] in the sodium polyacrylate solution group after 2 hours of gavage was significantly higher than that in the blank group [(4.5±1.0) granules], the sodium phosphate group [(6.2±2.0) granules] and the polyethylene glycol group [(8.5±1.0) granules] (P<0.05). Compared with the blank group [(39.7±11.60) mg] and the sodium phosphate group [(77.2±15.91) mg], the defecation quality of sodium polyacrylate solution [(162.4±16.69) mg] significantly increased within 2h after gavage (P<0.05). Compared with the blank group [(2.25±0.29)g], the sodium phosphate group [(2.72±0.24)g] and the polyethylene glycol group [(2.95±0.19)g], the intestinal mass of the sodium polyacrylate solution group [(3.30±0.16)g] significantly increased (P<0.05).ConclusionOral administration of sodium polyacrylate solution can accelerate intestinal peristalsis in normal mice, promote defecation in mice, and significantly reduce intestinal absorption of water. As a potential intestinal preparation drug, it has the advantages of small dose, high efficiency, safety and reliability.
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With the development of instruments and the innovation of techniques, gastrointestinal endoscopy is expanding the scope and scale in its application. As an important component of endoscopic therapeutic techniques, the development of endoscopic resection techniques is undoubtedly remarkable. The representative techniques including endoscopic submucosal dissection, submucosal tunneling endoscopic resection and natural orifice transluminal endoscopic surgery have made endoscopic resectable scope gradually extend from the initial intramucosal to the submucosal, and even extraserosal lesions. This article reviews the state of the art and advances of main endoscopic resection techniques.
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With the development of instruments and the innovation of techniques, gastrointestinal endoscopy is expanding the scope and scale in its application. As an important component of endoscopic therapeutic techniques, the development of endoscopic resection techniques is undoubtedly remarkable. The representative techniques including endoscopic submucosal dissection, submucosal tunneling endoscopic resection and natural orifice transluminal endoscopic surgery have made endoscopic resectable scope gradually extend from the initial intramucosal to the submucosal, and even extraserosal lesions. This article reviews the state of the art and advances of main endoscopic resection techniques.
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Objective:To evaluate whether posterior cruciate ligament (PCL) avulsion fracture was correlated with intercondylar notch and tibial slope.Methods:A total of 48 patients with PCL avulsion fracture were compared with 48 age- and sex-matched healthy controls without PCL avulsion fracture from January 2012 to October 2018 in our hospital. There were 25 males and 23 females with an average age of 54.35±14.06 years (range 27-82 years) in PCL avulsion fracture group. MRI of the knee joint were acquired in the sagittal, coronal, and axial sequences. The measurements included intercondylar notch's angle, width, and height, medial/lateral condylar widths, condylar width, medial/lateral posterior tibial slopes and coronal tibial slope. Notch shape index and notch width index were also calculated to adjust for size variations. The predictive accuracy of Risk factors were analyzed using the area under the receiver operating characteristic curve (AUC). The differences of morphological measurements between the two groups were compared. The morphological measurements with statistical significance were included for further multivariate analysis. Risk factors of PCL avulsion fracture were determined by binary logistic regression analysis. The inter- and intra-observer reproducibility of the measurements was determined by the intraclass correlation coefficients (ICC).Results:Except for coronal intercondylar notch height (0.709), medial posterior tibial slope (0.699) and lateral posterior tibial slope (0.705), the other intra-observer ICC ranged from 0.802 to 0.980, which indicated that the parameters had desirable reproducibility. Similarly, except for coronal intercondylar notch height (0.700), the other intra-observer ICC ranged from 0.778 to 0.971. Parameters of patients with PCL avulsion fracture, including the axial notch angle (52.56°±6.56°), coronal notch angle (54.81°±7.29°), axial intercondylar notch width (19.62±2.32 mm), axial notch shape index (0.68±0.08) and medial posterior tibial slope (8.27°±3.53°), were all significantly larger than those of the control group. The binary logistic regression analysis showed that the coronal notch angle ( β=0.102, OR=1.108, P=0.030) and medial posterior tibial slope ( β=0.151, OR=1.163, P=0.046) were the risk factors or PCL avulsion fracture. The AUC for coronal notch angle (0.678) and medial posterior tibial slope (0.631) exhibited good predictive power for the occurrence of PCL avulsion fracture. Conclusion:The larger coronal notch angle and medial posterior tibial slope were positively correlated with the incidence of PCL avulsion fracture.
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Objective To evaluate the efficacy of in-situ split liver transplantation (ISSLT) in children.Methods From June 2015 to August 2018,10 liver grafts from DBD were split in-situ.All the donors were male,and the median age of the donors was 28.5 year old (18-48 year).One left half graft and 9 left lateral lobe grafts (including 2 reduced size grafts) were transplanted to 10 pediatric recipients.Four grafts were transplanted in our center,and the rest 6 grafts were shared to other two transplant center.The primary diseases of the recipients included biliary atresia (8/10),hepatic sinus obstruction syndrome (1/10) and Alagille syndrome (1/10).The median age of the recipients was 10 month (7 month-11 year),and the mean body weight was 9.8 ± 6.6 kg (5-28 kg).Results All liver grafts were split in-situ.The mean split time of liver grafts was 88.5 ± 18.9 min.The mean weight of split grafts was 336.7-± 85.4 g.All recipients were subjected to piggyback liver transplantation.Operation time was 542.5 ± 112.1 min.Anhepatic time was 52.0 ±-13.5 min.GRWR was (3.98 ±0.96)％.GRWR of two cases was more than 5％,so segment Ⅲ was partially reduced.During the follow-up period,9 cases were alive and 1 case died due to multiple organ failure 1 day after liver transplantation.Conclusions ISSLT can enlarge the graft pool for children and achieve good results.
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Objective To investigate the clinical characteristics of patients with advanced hepatic alveolar echinococcosis, so as to provide more evidence for the diagnosis and treatment of advanced hepatic alveolar echinococcosis. Methods The clinical records of 175 cases with hepatic alveolar echinococcosis admitted to the Affiliated Hospital of Qinghai University from 2012 to 2017 were retrospectively reviewed. Results Among the 175 cases with hepatic alveolar echinococcosis, the male to female ratio was 1∶1.6, and the mean age was (38.6 ± 12.9) years (range, 11 to 69 years). 93.7% of the patients (164 cases) were Tibetan, and 79.4% (139 cases) were from Guoluo Prefecture. 73.7% (129 cases) were herdsmen, and 58.3% (102 cases) were illiterate. The major clinical symptoms included upper abdominal pain, yellowing of skin and mucosa, and scleral icterus. 69.1% of the lesions were located in bilateral livers, and 63.4% of the cases have 2 and more lesions. 53.7% of the cases have their inferior vena cava involved by the lesions. In addition, the most affected neighboring organ was diaphragmatic muscle (21.7%), and lung was the most affected distant metastatic site (40.6%). Conclusions Advanced hepatic alveolar echinococcosis is highly prevalent in Guoluo Prefecture, Qinghai Province. Screening of this disease is required in this region and rational treatment scheme should be developed.