Erythropoietic protoporphyria （EPP） is a rare inherited metabolic disease that often involves skin， blood， and nervous systems， and EPP with the main manifestations of severe liver damage and acute abdominal pain is extremely rare. By reviewing the clinical data and genetic testing results of a patient with EPP， this article discusses the clinical features and pathogenic genes of this disease， in order to improve the understanding of the disease among hepatologists and achieve early diagnosis and treatment.

Objective To explore the prevention and treatment of vasovagal reflex during and after operation in diseases of urinary system.Methods From February 2020 to April 2023,1436 patients who completed inpatient surgery in Department of Urology,Songshan Hospital,Qingdao University Medical College were selected to analyze the emergency management measures of vasovagal reflex during and after operation and summarize the diagnosis and treatment experience.Results Among 1436 patients,vasovagal reflex occurred in 4 cases during operation and 14 cases after operation,with an incidence of 1.25%.Most patients showed simultaneous decrease in blood pressure and heart rate.After intravenous injection of atropine and dopamine,blood pressure and heart rate returned to normal,and various concomitant symptoms disappeared,and no death cases were reported.Conclusion Urological specialists should pay attention to vasovagal reflex,sum up experience,do early identification,timely treatment to ensure the safety of patients.

Rhamnogalacturonan Ⅱ(RG-Ⅱ)is one of the structural domains of pectin whose structure is highly conserved among species.The main chain of RG-Ⅱ consists of approximately nine galacturonic acids linked by α-1,4 glycosidic bonds,with six well-defined side chains replacing them(A-F).The structures of the disaccharide side chains C and D and the monosaccharide side chains E and F in RG-Ⅱ from different sources remain essentially the same.In contrast,the oligosaccharide side chains A and B showed slight variability.Structural characterization of RG-Ⅱ can be achieved by molecular weight,monosaccharide composition,and mass spectrometry.The polysaccharides containing RG-Ⅱ structural domains in traditional Chinese medicines(TCMs)have high medicinal value.Isolation of RG-Ⅱ can be achieved using endo-polygalacturonase(Endo-PG)and Penicillium oxalicum.A substantial number of RG-Ⅱ standards can be rapidly prepared from red wine for the development of new quantitative methods to realize the quality control of active polysaccharides from traditional Chinese medicines and to promote the research process of polysaccharides from traditional Chinese medicines.

OBJECTIVE: To study the anatomical characteristics of blood vessels in the lateral segment of the vertebral body through the surgical approach of oblique lumbar interbody fusion (OLIF) using MRI imaging, and evaluate its potential vascular safety zone. METHODS: The lumbar MRI data of 107 patients with low back and leg pain who met the selection criteria between October 2019 and November 2022 were retrospectively analyzed. The vascular emanation angles, vascular travel angles, and the length of vessels in the lateral segments of the left vertebral body of L ₁-L ₅, as well as the distance between the segmental vessels in different Moro junctions of the vertebral body and their distances from the edges of the vertebrae in the same sequence (bottom marked as I, top as S) were measured. The gap between the large abdominal vessels and the lateral vessels of the vertebral body was set as the lateral vascular safe zones of the lumbar spine, and the extent of the safe zones (namely the area between the vessels) was measured. The anterior 1/3 of the lumbar intervertebral disc was taken as the simulated puncture center, and the area with a diameter of 22 mm around it as the simulated channel area. The proportion of vessels in the channel was further counted. In addition, the proportions of segmental vessels at L ₅ without a clear travel and with an emanation angel less than 90° were calculated. RESULTS: Except for the differences in the vascular emanation angles between L ₄ and L ₅, the vascular travel angles between L ₁, L ₂ and L ₄, L ₅, and the length of vessels in the lateral segments of the vertebral body among L ₁-L ₄ were not significant ( P>0.05), the differences in the vascular emanation angles, vascular travel angles, and the length of vessels between the rest segments were all significant ( P<0.05). There was no significant difference in the distance between vessels of L ₁, L ₂ and L ₂, L ₃ at Moro Ⅰ-Ⅳ junctions ( P>0.05), in L ₃, L ₄ and L ₄, L ₅ at Ⅱ and Ⅲ junction ( P>0.05). There was no significant difference in the vascular distance of L ₂, L ₃ between Ⅱ, Ⅲ junction and Ⅲ, Ⅳ junction, and the vascular distance of L ₃, L ₄ between Ⅰ, Ⅱ junction and Ⅲ, Ⅳ junction ( P>0.05). The vascular distance of the other adjacent vertebral bodies was significant different between different Moro junctions ( P<0.05). Except that there was no significant difference in the distance between L ₂I and L ₃S at Ⅰ, Ⅱ junction, L ₃I and L ₄S at Ⅱ, Ⅲ junction, and L ₂I and L ₃S at Ⅲ, Ⅳ junction ( P>0.05), there was significant difference of the vascular distance between the bottom of one segment and the top of the next in the other segments ( P<0.05). Comparison between junctions: Except for the L ₃S between Ⅰ, Ⅱ junction and Ⅱ, Ⅲ junction, and L ₅S between Ⅰ, Ⅱ junction and Ⅱ, Ⅲ and Ⅲ, Ⅳ junctions had no significant difference ( P>0.05), there were significant differences in the distance between the other segmental vessels and the vertebral edge of the same sequence in different Moro junctions ( P<0.05). The overall proportion of vessels in the simulated channels was 40.19% (43/107), and the proportion of vessels in L ₁ (41.12%, 44/107) and L ₅ (18.69%, 20/107) was higher than that in the other segments. The proportion of vessels in the channel of Moro zone Ⅰ (46.73%, 50/107) and zone Ⅱ (32.71%, 35/107) was higher than that in the zone Ⅲ, while no segmental vessels in L ₁ and L ₂ were found in the channel of zone Ⅲ ( χ ²=74.950, P<0.001). Moreover, 26.17% (28/107) of the segmental vessels of lateral L ₅ showed no movement, and 27.10% (29/107) vascular emanation angles of lateral L ₅ were less than 90°. CONCLUSION L ₁ and L ₅ segmental vessels are most likely to be injured in Moro zones Ⅰ and Ⅱ, and the placement of OLIF channels in L _{4, 5} at Ⅲ, Ⅳ junction should be avoided. It is usually safe to place fixation pins at the vertebral body edge on the cephalic side of the intervertebral space, but it is safer to place them on the caudal side in L _{1, 2} (Ⅰ, Ⅱ junction), L _{3, 4} (Ⅲ, Ⅳ junction), and L _{4, 5} (Ⅱ, Ⅲ, Ⅳ junctions).
Humans ; Retrospective Studies ; Spinal Puncture ; Magnetic Resonance Imaging ; Anticoagulants ; Bone Nails

OBJECTIVE: To investigate the feasibility of a dual-crosslinked injectable hydrogel derived from acellular musclar matrix (AMM) for promoting myoblasts proliferation and myogenic differentiation. METHODS: Firstly, hyaluronic acid was oxidized with NaIO ₄ and methylated to prepare methacrylamidated oxidized hyaluronic acid (MOHA). Then, AMM obtained by washing enzymatically treated muscle tissue was aminolyzed to prepare aminated AMM (AAMM). MOHA hydrogel and AAMM were crosslinked using Schiff based reaction and UV radiation to prepare a dual-crosslinked MOHA/AAMM injectable hydrogel. Fourier transform infrared spectroscopy (FTIR) was used to characterize MOHA, AAMM, and MOHA/AAMM hydrogels. The injectability of MOHA/AAMM hydrogel were evaluated by manual injection, and the gelation performance was assessed by UV crosslinking. The rheological properties and Young's modulus of the hydrogel were examined through mechanical tests. The degradation rate of the hydrogel was assessed by immersing it in PBS. The active components of the hydrogel were verified using immunofluorescence staining and ELISA assay kits. The promotion of cell proliferation by the hydrogel was tested using live/dead staining and cell counting kit 8 (CCK-8) assays after co-culturing with C2C12 myoblasts for 9 days. The effect of the hydrogel on myogenic differentiation was evaluated by immunofluorescence staining and real time quantitative polymerase chain reaction (RT-qPCR). RESULTS: FTIR spectra confirmed the successful preparation of MOHA/AAMM hydrogel. The hydrogel exhibited good injectability and gelation ability. Compared to MOHA hydrogel, MOHA/AAMM hydrogel exhibited higher viscosity and Young's modulus, a reduced degradation rate, and contained a higher amount of collagen (including collagen type Ⅰ and collagen type Ⅲ) as well as bioactive factors (including epidermal growth factor, fibroblast growth factor 2, vascular endothelial growth factor, and insulin-like growth factor 1). The live/dead cell staining and CCK-8 assay indicated that with prolonged incubation time, there was a significant increase in viable cells and a decrease in dead cells in the C2C12 myoblasts within the MOHA/AAMM hydrogel. Compared with MOHA hydrogel, the difference was significant at each time point ( P<0.05). Immunofluorescence staining and RT-qPCR analysis demonstrated that the deposition of IGF-1 and expression levels of myogenic-related genes (including Myogenin, Troponin T, and myosin heavy chain) in the MOHA/AAMM group were significantly higher than those in the MOHA group ( P<0.05). CONCLUSION The MOHA/AAMM hydrogel prepared based on AMM can promote myoblasts proliferation and myogenic differentiation, providing a novel dual-crosslinked injectable hydrogel for muscle tissue engineering.
Hydrogels ; Hyaluronic Acid/pharmacology* ; Vascular Endothelial Growth Factor A/metabolism* ; Tissue Engineering/methods* ; Cell Differentiation ; Myoblasts/metabolism* ; Cell Proliferation

Objective    To analyze the occurrence of postoperative pulmonary complications (PPC) and the risk factors in patients with spontaneous pneumothorax who underwent micro single-port video-assisted thoracoscopic surgery (VATS). Methods    A total of 158 patients with spontaneous pneumothorax who underwent micro single-port VATS in our hospital from April 2017 to December 2019 were retrospectively included, including 99 males and 59 females, with an average age of 40.53±9.97 years. The patients were divided into a PPC group (n=21) and a non-PPC group (n=137) according to whether PPC occurred after the operation, and the risk factors for the occurrence of PPC were analyzed. Results    All 158 patients successfully completed the micro single-port VATS, and there was no intraoperative death. The postoperative chest tightness, chest pain, and dyspnea symptoms basically disappeared. During the postoperative period, there were 3 patients of pulmonary infection, 7 patients of atelectasis, 4 patients of pulmonary leak, 6 patients of pleural effusion, 1 patient of atelectasis and pleural effusion, and the incidence of PPC was 13.29% (21/158). Multivariate logistic regression analysis showed that lung disease [OR=32.404, 95%CI (2.717, 386.452), P=0.006], preoperative albumin level≤35 g/L [OR=14.912, 95%CI (1.719, 129.353), P=0.014], severe pleural adhesions [OR=26.023, 95%CI (3.294, 205.557), P=0.002], pain grade Ⅱ-Ⅲ 24 hours after the surgery [OR=64.024, 95%CI (3.606, 1 136.677), P=0.005] , age [OR=1.195, 95%CI (1.065, 1.342), P=0.002], intraoperative blood loss [OR=1.087, 95%CI (1.018, 1.162), P=0.013] were the risk factors for PPC after micro single-port VATS. Conclusion    There is a close relationship between PPC after micro single-port VATS and perioperative indexes in patients with spontaneous pneumothorax. Clinically, targeted prevention and treatment can be implemented according to the age, pulmonary disease, preoperative albumin level, intraoperative blood loss, degree of pleural adhesion and pain grading 24 hours after surgery.

Objective:To observe and analyze the clinical features, treatment methods and efficacy of patients with retinopathy associated with incontinentia pigmenti (IP).Methods:A retrospective case study. Twelve clinical confirmed IP patients (24 eyes) in Zhongshan Ophthalmic Center of Sun Yat-sen University from January 2015 to December 2018 were included in this study. The best corrected visual acuity and intraocular pressure examination were performed in patients (>4 years old). All patients were examined on the anterior segment, vitreous body, and fundus under topical anesthesia or general anesthesia. Eight cases underwent genetic testing. Patients with active disease should be given anti-vascular endothelial growth factor (VEGF) drug treatment, retinal laser photocoagulation or vitrectomy, those without active disease should be observed. All patients were followed up for 1 to 3 months, with an average follow-up time of 18.7 months.Results:All patients were all female, with an average age of 6.3±9.8 years old at the first ophthalmology visit. According to the recommendations of the pediatrician, 3 cases were actively screened for ophthalmology (referrals), with an average age of 0.4±0.5 years (median age: 2 months). A total of 9 cases were not recommended for referrals (non-referrals), including 3 cases of ophthalmology who were diagnosed for the first time due to visual impairment, and 6 cases of undiagnosed IP before the ophthalmology visit, the average age of their first visit was 8.2±10.8 years (medium age: 3 years old). The age of the first visit for non-referred patients was larger than that of referrals, and the difference was statistically significant ( Z=-2.141, P=0.036). Among the 24 eyes of 12 cases, there were no obvious fundus abnormalities in 1 case or 2 eyes, 11 cases of IP-related retinopathy in 22 eyes (91.7%, 22/24), 8 cases of binocular asymmetry (66.7%, 8/12). There were active lesions on the fundus in 7 eyes (29.2%, 7/24). Patients underwent simple retinal laser photocoagulation and/or anti-VEGF drug therapy. During the follow-up, retinal neovascularization recurred in 1 eye. Among the 8 cases that underwent genetic testing, 3 cases (37.5%, 3/8) were deleted in exons 4-10 of the IKBKG gene. Conclusions:IP is more common in women. IP-associated retinopathy is noted with early-onset, asymmetrical retinopathy, which is identified with retinal neovascularization and vitreous proliferation. Early detection and timely treatment are essential.

Objective To explore the neuroprotective effect and mechanism of picroside II on p38 mitogen activated protein kinase (p38 MAPK) signal transduction pathway after cerebral ischemia/reperfusion injury in rats. Methods A total of 150 healthy male Wistar rats were subject to establish middle cerebral artery occlusion/reperfusion (MCAO/R) models by inserting a monofilament thread. All rats were randomly divided into sham group, model group, picroside (Pier) group, anisomycin (Anis, agonist of p38 MAPK) group, Anis+Picr group, SB203580 (SB, inhibitor of p38 MAPK) group and SB+Picr group. The neurobehavioral function was evaluated by modified neurological severity score points (mNSS) test. The structure of neuron was observed using HE staining. The apoptotic cells were counted using TUNEL assay. The expression of phosphorylated p38 MAPK (p-p38 MAPK) in cortex was determined using the immunohistochemistry. And the expressions of p-p38 MAPK, phosphorylated MAPK activated protein kinase-2 (p-MK2), phosphorylated cytoplasm phospholipase A2 (p-cPLA2), interleukin-6 (IL-6) and tumor necrotic factor a (TNF-α) were determined by Western blotting. Results No neurological behavioral malfunction was found in sham group. In model group, the damage of neuron was worsened, while the neurobehavioral function score, apoptotic cell index and the expressions of p-p38 MAPK, p-MK2, p-cPLA2,IL-6 and TNF-α increased significantly than those in control group. No significant difference was found in TNF-α. In Pier group, SB group and SB+Picr group, the damage of neuron was lighter, the neurological behavioral function was improved, the number of apoptotic cells and the expressions of p-p38 MAPK, p- MK2, p-cPLA2 and IL-6 decreased significantly than those in model group. In Anis group and Anis + Pier group, the damage was worsen, the cerebral infarction was larger, and the expressions of p-p38 MAPK, p-MK2, p-cPLA2 and IL-6 increased significantly than those in control group. Conclusion Picroside II can protect the neuron from the apoptosis and inflammation reaction after MCAO/R by inhibiting p38 MAPK signal transduction pathway in rats.

Objective:To study the effect of spleen and marrow strengthening method combined with CAG regimen on the quality of life(QOL) of elderly patients with acute myeloid leukemia with spleen-kidney Yang deficiency syndrome.Methods:From June 2017 to October 2018, 50 elderly patients with acute myeloid leukemia with spleen-kidney Yang deficiency were randomly divided into treatment group and control group by random number table method, with 25 patients in each group.The patients in the control group were treated with CAG regimen(Ara-C+ Acla+ G-CSF), while the patients in the treatment group were treated with CAG regimen and leukemia prescription I, which was the empirical prescription for spleen and kidney Yang deficiency syndrome in elderly patients with acute myeloid leukemia.The patients were treated for two courses.The traditional Chinese medicine (TCM) syndromes and QOL scores of patients in two groups were compared and observed.Results:Before treatment, there was no statistically significant difference in the score of TCM syndromes between the two groups ( P>0.05). After treatment, the improvement of TCM syndromes in the treatment group had statistically significant difference compared with before treatment[before treatment (9.29±4.22)points, after treatment (5.04±3.83)points, t=3.656, P=0.001], but that in the control group had no statistically significant difference ( P>0.05). The treatment group was better than the control group ( t=-2.081, P=0.044). The total effective rate of the treatment group was 58.33% (14/24), which was significantly higher than that of the control group[26.32% (5/19)], and the difference was statistically significant (χ 2=5.831, P<0.05). Before treatment, there was no statistically significant difference in the total score of QOL between the two groups ( P>0.05). After treatment, the scores of QOL in both two groups were improved, the differences were statistically significant[the control group: (40.37±2.93)points vs.(38.21±2.76)points, t=2.337, P=0.025; the treatment group: (41.46±2.57)points vs.(36.54±2.34)points, t=6.929, P=0.000], and the QOL score of the treatment group was better than that of the control group ( t=-2.145, P=0.038). Conclusion:The improvement of TCM syndromes and QOL of elderly patients with acute myeloid leukemia with spleen-kidney Yang deficiency syndrome treated by spleen and marrow strengthening method combined with CAG regimen is better than that treated by CAG chemotherapy alone.

Objective: To explore the relationship among sexual assault experience,interpersonal trust and psychosexual health in middle school girls,and to provide a reference for psychological counseling for the diagnosis of sexually assaulted individuals. Methods: A sample of 499 middle school girls from 5 middle schools in Shaoguan city of Hunan Province were assessed with the Sexual Assault Questionnaire, Interpersonal Trust Scale and Adolescent Psychosexual Health Scale. Results: The rate of sexual assault was 36.87% while the non-contact sexual assault was 34.87%, and the rate of sexual touching was 8.82% whlie the rate of rape was 1.20%. There were negative correlations among sexual assault experience, sexual values, sexual adaptation and psychosexual health(r=-0.21,-0.10,-0.13,P<0.01).The interpersonal trust modified the relationship between sexual assault experience and sexual awareness, sexual values, sexual adaptation, psychosexual health (95%CI=-0.05--0.01,-0.05--0.01,-0.04-0.00,-0.04--0.02,P<0.05). Conclusion Interpersonal trust plays a moderating role in the association between sexual assault experience and psychosexual health.The adverse effects of sexual assault on psychosexual health might be relieved through improving interpersonal trust among middle school girls.