OBJECTIVE: To dynamically observe the levels and activities of von Willebrand factor (vWF) and ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) in plasma of children with congenital ventricular septal defect (VSD) during perioperative period, and explore the value of plasma vWF antigen (vWF:Ag) and ADAMTS-13 activity (ADAMTS-13: AC) in evaluating vascular endothelial injury and prognosis in children with VSD. METHODS: In this cross-sectional study, a total of 74 children with VSD who underwent surgical treatment in TEDA International Cardiovascular Hospital from September 2018 to March 2019 were enrolled in the observation group. Among them, there were 28 cases of pure VSD, 32 cases of VSD combined with pulmonary hypertension, and 14 cases of VSD combined with valvular heart disease. 31 healthy children who underwent physical examination in Tianjin Children's Hospital during the same period were collected as the control group. The biochemical indexes of the children at admission were recorded. Peripheral plasma was collected at admission, postsurgery day 0 and day 1, respectively, and the levels of vWF activity (vWF:AC), vWF:Ag, ADAMTS-13 antigen (ADAMTS-13:Ag) and ADAMTS-13:AC were detected. RESULTS: The level of plasma vWF:Ag and vWF:AC in the observation group before surgery were significantly lower than those in the control group (P<0.001), and increased continuously, on postsurgery day 0 and day 1 (P<0.001). The level of ADAMTS-13:Ag in the observation group before surgery was significantly higher than that in the control group (P<0.001), which decreased significantly on postsurgery day 0 (P<0.001), and increased significantly on postsurgery day 1 compared with postsurgery day 0 (P=0.033). The level of ADAMTS-13:AC in the observation group before surgery was significantly lower than that in the control group (P=0.015), which decreased significantly on postsurgery day 0 (P=0.037), and increased on postsurgery day 1, but the difference was not statistically significant (P=0.051). The changes of vWF and ADAMTS-13 in the three subgroups were basically similar to the observation group. vWF: Ag/ADAMTS-13: AC ratio on postsurgery day 0 and day 1 had high diagnostic value in vascular endothelial injury (AUC=0.80, P<0.001; AUC=0.93, P<0.001). Preoperative vWF and ADAMTS-13 levels, and related baseline indicators were not correlated with postoperative infection, bleeding, thrombosis,etc. CONCLUSION Preoperative vWF: Ag, vWF: AC and ADAMTS-13: AC levels in children with VSD are low, while the level of ADAMTS-13: Ag is high. After surgery, the levels of vWF: Ag and vWF: AC are increased and the level of ADAMTS-13: Ag is decreased. The postoperative vWF: Ag/ADAMTS-13: AC ratio shows high diagnostic value in evaluating vascular endothelial injury. There is no correlation between preoperative vWF and ADAMTS-13 levels with perioperative clinical events.
Child ; Humans ; ADAMTS13 Protein ; Cross-Sectional Studies ; Heart Septal Defects, Ventricular ; Prognosis ; von Willebrand Factor

Non-alcoholic fatty liver disease (NAFLD) is considered to be a manifestation of metabolic syndrome and has become one of the chronic diseases that endanger health around the world. There is still a lack of effective therapeutic drugs in clinical practice. Farnesoid X receptor (FXR) has been a popular target for NAFLD research in recent years. Fexaramine (Fex) is a potent and selective agonist of FXR, and its mechanism of action to improve NAFLD is unclear. Therefore, in this study, a mouse model of NAFLD was constructed using a high-fat, high-cholesterol diet and treated with Fex orally for 6 weeks. We evaluated the ameliorative effect of Fex on disorders of glucolipid metabolism in NAFLD mice, and preliminarily explored its potential mechanism of action. The animal experiments were approved by the Animal Ethics Committee of Shanghai University of Traditional Chinese Medicine (approval number: PZSHUTCM210913011). In this study, it was found that 100 mg·kg^-1 Fex significantly inhibited body weight gain, alleviated insulin resistance, improved liver injury and lipid accumulation in NAFLD mice. The effect of Fex on the expression of hepatic intestinal FXR and its target genes in NAFLD mice was further examined. Analysis of serum and hepatic bile acid profiles and expression related to hepatic lipid metabolism. It was found that Fex could stimulate intestinal FXR, promote fibroblast growth factor 15 (FGF15) secretion, inhibit the expression of cytochrome P450 family 7 subfamily A member 1 (CYP7A1), the rate-limiting enzyme of bile acid synthesis in liver, regulate bile acid synthesis by negative feedback, and improve the disorder of bile acid metabolism. At the same time, Fex reduces liver lipid synthesis and absorption, increases fatty acid oxidation, thus improving liver lipid metabolism. This study shows that Fex can improve NAFLD by activating intestinal FXR-FGF15 signal pathway and regulating liver lipid metabolism.

OBJECTIVES: To investigate the risk factors for neonatal asphyxia in Hubei Enshi Tujia and Miao Autonomous Prefecture and establish a nomogram model for predicting the risk of neonatal asphyxia. METHODS: A retrospective study was conducted with 613 cases of neonatal asphyxia treated in 20 cooperative hospitals in Enshi Tujia and Miao Autonomous Prefecture from January to December 2019 as the asphyxia group, and 988 randomly selected non-asphyxia neonates born and admitted to the neonatology department of these hospitals during the same period as the control group. Univariate and multivariate analyses were used to identify risk factors for neonatal asphyxia. R software (4.2.2) was used to establish a nomogram model. Receiver operator characteristic curve, calibration curve, and decision curve analysis were used to assess the discrimination, calibration, and clinical usefulness of the model for predicting the risk of neonatal asphyxia, respectively. RESULTS: Multivariate logistic regression analysis showed that minority (Tujia), male sex, premature birth, congenital malformations, abnormal fetal position, intrauterine distress, maternal occupation as a farmer, education level below high school, fewer than 9 prenatal check-ups, threatened abortion, abnormal umbilical cord, abnormal amniotic fluid, placenta previa, abruptio placentae, emergency caesarean section, and assisted delivery were independent risk factors for neonatal asphyxia (P<0.05). The area under the curve of the model for predicting the risk of neonatal asphyxia based on these risk factors was 0.748 (95%CI: 0.723-0.772). The calibration curve indicated high accuracy of the model for predicting the risk of neonatal asphyxia. The decision curve analysis showed that the model could provide a higher net benefit for neonates at risk of asphyxia. CONCLUSIONS The risk factors for neonatal asphyxia in Hubei Enshi Tujia and Miao Autonomous Prefecture are multifactorial, and the nomogram model based on these factors has good value in predicting the risk of neonatal asphyxia, which can help clinicians identify neonates at high risk of asphyxia early, and reduce the incidence of neonatal asphyxia.
Infant, Newborn ; Humans ; Male ; Pregnancy ; Female ; Nomograms ; Retrospective Studies ; Cesarean Section ; Risk Factors ; Asphyxia Neonatorum/etiology*

Non-puerperal mastitis(NPM)is a group of chronic inflammatory diseases with breast pain,lumps,abscesses and sinus tracts/fistulas as the main clinical manifestations,which is easily confused with breast cancer or other benign breast diseases.NPM always leads to a long treatment cycle and high recurrence rate,which may cause a large economic and psychological burden to patients.At present,the etiology and pathogenesis of NPM are still unclear,but it has a certain correlation with immune abnormality,bacterial infection,hormone disorder and other factors.Although several diagnostic methods available,the diagnosis of NPM relies on histopathological examination mainly.The treatment methods of the disease include observation and follow-up,pharmacotherapy,surgical treatment,etc.,but there is still no unified standard for specific treatment timing and treatment selection.In view of the controversy over etiology and treatment selection of NPM,this paper comprehensively summarizes the latest research progress in disease characteristics,clinical diagnosis and treatment of NPM based on domestic and foreign literature,aiming to provide reference and inspiration for the selection of reasonable clinical diagnosis and treatment.

Purpose: Capecitabine is an extensively used oral prodrug of 5-fluorouracil in treatment of colon cancer and is known to cause hand-foot syndrome (HFS). As the target enzyme for capecitabine, thymidylate synthase (TYMS) plays a key role for 5-fluorouracil metabolism and has been associated with some side effects caused by capecitabine. The aim of our study is to identify the possible genetic predictors of capecitabine-induced HFS (CAP-HFS) in Chinese colorectal cancer patients. Materials and Methods: Whole exons of TYMS were sequenced for 288 extreme phenotype HFS patients, including 144 severe or early-onset (first 2 cycles) moderate HFS extreme cases and 144 extreme controls with no reported HFS. The associations between polymorphisms and CAP-HFS were analyzed using logistic regression under an additive model. Results: We identified a novel risk mutation (c.1A>G, chr18:657743), was associated with severe HFS in an extreme case who was affected during the first cycle of treatment. Moreover, we identified three new variants, rs3786362, rs699517, rs2790, and two previously reported variants, 5’VNTR 2R/3R and 3′-untranslated region 6-bp ins-del, which were significantly associated with CAP-HFS (p < 0.05). In silico analysis revealed that the effect of these polymorphisms in the TYMS region on the development of HFS might not be restricted solely to the regulation of TYMS expression, but also the TYMS catalytic activity through the indirect effect on ENOSF1 expression. Conclusion This study identified new polymorphisms in TYMS gene significantly associated with CAP-HFS, which may serve as useful genetic predictors for CAP-HFS and help to elucidate the underlying mechanism of HFS.

Objective:To investigate the clinical characteristics of old patients with spinal cord injury. Methods:From January 1, 2013 to December 31, 2019, totally 386 old (≥ 60 years) patients with spinal cord injury were enrolled. Their gender, age, etiology, American Spinal Injury Association Impairment Scale (AIS) and complications were analyzed. Results:In the old patients with spinal cord injury, traumatic spinal cord injury was more common in males (71.17%) and non-traumatic spinal cord injury was more common in females (56.19%). Fall on level surface was the most important cause of spinal cord injury both in old men (28.83%) and women (24.76%). Tumor (19.05%) was the most common non-traumatic cause of spinal cord injury in old female patients. Cervical segment (78.46%) was the most common site of injury in old traumatic spinal cord injury, while thoracic segment (52.14%) was the most common site of injury in non-traumatic spinal cord injury. Grade D (38.08%) was the most common AIS grade, followed by grades C (28.76%), A (21.50%), and B (11.66%). Spinal canal stenosis (23.31%) played an important role in the etiology of old spinal cord injury. Neuralgia, venous thrombosis of lower extremities and urinary tract infection were the most common complications in old patients with spinal cord injury. Conclusion:Fall on level surface is the leading cause of spinal cord injury in old patients, and the proportion of fall in the etiology of old spinal cord injury tends to increase with age. It is important to take effective measures to avoid falling in the old adults to prevent spinal cord injury.

Objective:To investigate the outcome of neurological function and the clinical characteristics of complications in children with spinal cord injury. Methods:From 2011 to 2019, children under 15 years old with spinal cord injury were selected in our hospital. Their level of injury and American Spinal Injury Association Impairment Scale (AIS) at one month, three months and one year were recorded. And the complications such as pressure ulcers/scald, urinary tract infection, hydronephrosis/vesicoureteral reflux, constipation, osteoporosis/fracture, deep vein thrombosis, neurodynia, heterotopic ossification, scoliosis and hip dysplasia were analyzed. Results:Of 159 individuals, 41 were boys and 118 were girls, the average age at injury was (6.08±2.57) years. The main cause of spinal cord injury was sports accidents (47.8%), and the main injury sites were thoracic spinal cord injury (89.3%). The cause of spinal cord injury was correlated with age at injury (r = -0.160, P = 0.044), gender (r = -0.458, P < 0.001) and injury sites (r = -0.249, P = 0.002). Complete spinal cord injury counted for 71.7%, and the AIS grade at one month was correlated with that at twelve months (r = 0.984, P < 0.001). The main complications were urinary tract infection (69.2%), constipation (67.9%), hydronephrosis/vesicoureteral reflux (37.7%), scoliosis (25.8%) and hip dysplasia (25.2%). The incidence of ulcers/scald was correlated with injury site (r = 0.179, P = 0.024). The AIS grade three months after injury was significantly correlated with urinary tract infection, constipation, scoliosis and hip dysplasia (|r| > 0.227, P < 0.01). The incidence of ulcers/scald was correlated with osteoporosis/fracture (r = 0.208, P < 0.01). The incidence of urinary tract infection was significantly correlated with hydronephrosis/vesicoureteral reflux, constipation, scoliosis and hip dysplasia (r > 0.261, P < 0.001), as well as osteoporosis/fracture (r = 0.195, P < 0.05). The incidence of hydronephrosis/vesicoureteral reflux was significantly correlated with constipation, osteoporosis/fracture, scoliosis and hip dysplasia (r > 0.146, P < 0.01). The incidence of constipation was significantly correlated with scoliosis and hip dysplasia (r > 0.313, P < 0.01), as well as osteoporosis/fracture (r = 0.160, P < 0.05). The incidence of osteoporosis/fracture was significantly correlated with scoliosis and hip dysplasia (r > 0.342, P < 0.01). The incidence of scoliosis was significantly correlated with hip dysplasia (r = 0.818, P < 0.001). Conclusion:The recovery of neurological function after spinal cord injury in children is closely correlated to AIS. The outcome of complete spinal cord injury is poor. The common complications after spinal cord injury in children demonstrate specific age characteristics, and the incidence of urinary tract infection, constipation, hydronephrosis/vesicoureteral reflux, scoliosis and hip dysplasia are common complications, which need more attention.

Objective:To explore the clinical manifestations and MRI features of pediatric spinal cord injury (SCI) after back bend. Methods:A retrospective study was performed. All the medical records and MRI images of children with SCI after back bend were identified in Beijing Bo'ai Hospital from January 1st, 2002 to August 31st, 2020. Results:A total of 120 SCI children after back bend were reviewed, out of whom 119 cases were girls, one case was boy. The age ranged from 38 to 162 months, with the median age of 76 months. More cases were discovered in July and September every year (32 cases, 26.7%), as well as in weekends (67 cases, 55.8%). The main clinical manifestations were sensory and motor dysfunction of both lower limbs (120 cases, 100%), bladder and bowl incontinence (120 cases, 100%). The common first symptoms included sudden attack of lumbar pain (39 cases, 32.5%), lower limbs paralysis (30cases, 25.0%) and leg pain (10 cases, 8.3%). The peak time of symptoms ranged from five minutes to two days, with the median time of 50 minutes. The MRI features of 104 children with SCI within one week after back bend were as follows: the abnormal signals of MRI in spinal cord involved lower cervical and all the following segments of spinal cord. The number of the segments of spinal cord with abnormal signals ranged from two to 15, with the median of seven segments. The most common segments with abnormal signals were T₉ (96 cases, 92.3%), T₁₀ (96 cases, 92.3%) and T₁₁ (90 cases, 86.5%). Among the cases followed up, 48 cases with complete injury demonstrated a vast and serous spinal cord atrophy (SCA) below the injury segments as early as 37 days after the injury, the SCA would become worse at the chronic stage and maybe involve the spinal cord above the injury segments. In 31 cases with incomplete injury, the abnormal signals of MRI in spinal cord were limited in the lumbar enlargement, with a various degree of SCA at the late stage. All the cases were diagnosed as SCI without radiologic abnormality, out of whom 89 (74.2%) cases suffered from thoracic complete SCI, 31 (25.8%) cases suffered thoracic or lumbar incomplete SCI. The common complications included scoliosis, hip joint dysplasia, urinary tract infection, hydronephrosis, osteoporosis, pathological fracture of lower limbs and valgus knee. Conclusion:The main clinical symptoms of pediatric SCI after back bend were sudden lumbar pain, sensory and motor dysfunction of both lower limbs, and bladder and bowl incontinence. Most of the cases were thoracic complete SCI, the MRI features at the early stage were multiple segments of abnormal signals of spinal cord around T₉ and T₁₀, and later an extensive severe SCA below the injury segments to the conus medullaris, accompanied by the SCA above the injury segments.

Objective:To investigate the occurrence and related factors of autonomic dysreflexia (AD) during intermittent catheterization in patients with spinal cord injury (SCI). Methods:Case control study was used in this study. Intermittent catheterization was performed on 44 SCI patients hospitalized from April, 2019 to April, 2020, The data of age, gender, time after injury, segment of injury, degree of injury, resting blood pressure, immediate blood pressure after catheterization, catheterization numbers, catheterization volume and duration of catheterization were collected. Descriptive analysis and binary Logistic regression analysis were used to analyze the occurrence and related factors of AD. Results:Totally, AD happened in 26 (59.1%) patients. Urethral catheterization was done 1738 times, out of which AD accounted for 187 times (10.8%). The risk of AD increased with the time after injury and age (P < 0.05). The probability of AD was lower in T₇ SCI and below than in T₆ SCI and above (P = 0.002). Catheterization numbers, gender, degree of injury, catheterization volume and duration of catheterization were not influencing factors of AD (P > 0.05). Conclusion:It is necessary to have a full understanding for the occurrence of AD in patients with SCI during intermittent catheterization. For patients with SCI in T₆ and above, long time after injury and elderly patients, routine monitoring of blood pressure during intermittent catheterization is recommended to detect and deal with AD in time.

Objective:To observe the relationship between bone metabolism biochemical markers and clinic features in patients with spinal cord injury. Methods:From July, 2018 to December, 2019, totally 135 patients with spinal cord injury were enrolled. They were assessed with American Spinal Injury Association Impairment Scale (AIS). β-collagen type I C-terminal telopeptide (β-CTX), total N-terminal propeptide of type I precollagen (TP1NP), 25-hydroxyvitamin D [25(OH)D] and parathyroid hormone (PTH), serum calcium and serum phosphorus were measured. The level of TP1NP, β-CTX, 25(OH)D and PTH among clinical characteristics (gender, age, disease course, AIS grade and so on) were analyzed. Results:The levels of β-CTX and 25(OH)D were lower in women than in men (|t| > 2.044, P < 0.01). There was difference in the level of 25(OH)D among different ages (F = 3.156, P < 0.05). The levels of β-CTX and TP1NP increased in the first four months after spinal cord injury, and decreased then; while the level of PTH decreased in the first four months, and increased then (P < 0.001). The level of β-CTX was lower in patients of AIS D than in patients of AIS A and C (t >2.679, P < 0.05). The level of TP1NP was higher in paraplegics than in quadriplegics (Z = -2.035, P < 0.05). The level of β-CTX was higher in patients with fractures or surgeries involving bone than in patients without fractures or surgeries involving bone (t = 2.169, P < 0.05). There was no difference in all the bone metabolism markers between patients with and without lower extremity motor function (t < 0.839, Z < 1.822, P > 0.05). The ratio of 25(OH)D deficience was 85.19%. Conclusion:Bone conversion was active in the first four months after spinal cord injury, and decreased gradually then, which may be related to fractures of spine or surgeries involving spine after injury. The effect of spinal cord injury on bone metabolism markers is not clear. Most of patients with spinal cord injury were lack of vitamin D.