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1.
Chinese Journal of Dermatology ; (12): 117-120, 2020.
Article in Chinese | WPRIM | ID: wpr-870233

ABSTRACT

Objective To analyze the disease constitution,accuracy of clinical and pathological diagnoses of skin biopsy samples in Peking Union Medical College Hospital.Methods A total of 29987 patients subjected to skin biopsy were collected from Department of Dermatology,Peking Union Medical College Hospital from June 2010 to November 2018,and clinical and histopathological diagnoses of these skin biopsy samples were analyzed retrospectively.Results According to the results of histopathological diagnosis,confirmed diagnoses of these patients could be classified into 33 categories and 242 kinds.Common disease categories included epidermal tumors (2931 cases,9.77%),connective tissue diseases (2809 cases,9.37%),melanocytic tumors (2078 cases,6.93%),erythematous scaly pustular dermatoses (1376 cases,4.59%),lichenoid dermatoses (1291cases,4.31%),allergic or eczematous skin diseases (1282 cases,4.28%)and infectious skin diseases (1156 cases,3.86%).Common skin diseases included scleroderma (1887 cases,6.29%),pigmented nevus (1755 cases,5.85%),seborrheic keratosis (1136 cases,3.79%),eczema (1089 cases,3.63%),psoriasis (881 cases,2.94%),lichen planus (867 cases,2.89%),lupus erythematosus (638 cases,2.13%),pemphigus (549 cases,1.83%),and basal cell carcinoma (501 cases,1.67%).Poor consistency was observed between clinical diagnosis and histopathological diagnosis of lichen planus,bullous pemphigoid,granuloma annulare and hypereosinophilic dermatitis.Conclusions Common disease categories of the skin biopsy samples in Peking Union Medical College Hospital were epidermal tumors,connective tissue diseases,melanocytic tumors,erythematous scaly pustular dermatoses,lichenoid dermatoses,and allergic or eczematous skin diseases.Poor consistency was observed between clinical and pathological diagnosis in some skin diseases,and understanding of these diseases should be improved.

3.
Journal of Medical Biomechanics ; (6): E064-E068, 2019.
Article in Chinese | WPRIM | ID: wpr-802506

ABSTRACT

Objective To investigate the effect of different fluid shear stress (FSS) on the regulation of planar cell polarity (PCP) signaling, and further to explore the relationship among FSS, PCP signaling pathway and ciliogenesis. Methods The hydrodynamic cell model of adjustable FSS was established. qPCR and immunofluorescence were used to detect the mRNA expression of PCP signaling pathway core protein Dvl2 and cilia assembly protein IFT88, cell targeting and co-localization under different FSS. Western blot (WB) was used to detect the protein expression of Dvl2 at 18 h under different FSS. Results The qPCR result showed that compared with 1.5 Pa FSS, under 0.1 Pa FSS, the mRNA expression of Dvl2 was higher at 6 h and 18 h (P<0.05), significantly higher at 12 h (P<0.01); the mRNA expression of IFT88 was significantly higher at 18 h (P<0.01). The WB result showed that compared with 0 h, under 0.1 Pa FSS, the protein expression of Dvl2 was higher at 18 h (P<0.05), significantly lower under 1.5 Pa FSS (P<0.01); compared with 1.5 Pa FSS, the protein expression of Dvl2 was higher at 18 h under 0.1 Pa FSS (P<0.05). The immunofluorescence result showed that the positive expression of Dvl2 increased with the loading time on FSS increasing, and gradually aggregated at a point around the nucleus; the positive expression of IFT88 was gradually transferred from the nucleus to the cytoplasm and aggregated at a point under 0.1 Pa FSS, and gradually decreased and depolymerized under 1.5 Pa FSS. Protein Dvl2 and IFT88 were located in the same position in cells under 0.1 Pa FSS and before 18 h under 1.5 Pa FSS, and colocalization of proteins Dvl2 and IFT88 was not observed after 18 h under 1.5 Pa FSS due to IFT88 depolymerization. Conclusions Laminar FSS played an inhibition on the transduction of PCP signaling pathway and a hindrance on the process of ciliogenesis, while low FSS played a promotion on the transduction. PCP signaling pathway might regulate FSS-induced ciliogenesis by Dvl2.

4.
Chinese Journal of Microsurgery ; (6): 442-445, 2019.
Article in Chinese | WPRIM | ID: wpr-792084

ABSTRACT

To explore the clinical effect of microsurgery in the treatment of the tumor which was diagnosed with the plexiform neurofibroma (PN) of the forearm and palm. Methods From January, 2014 to June, 2017, 6 cases of the PN in the forearm and palm were removed by microsurgery such as neurovascular transplantation, separation and anastomosis under microscope, etc. There were 4 males and 2 females, with an average age of 9.2 (range, 2-18 )years. There was 1 case with PN of the median nerve, ulnar nerve and their branches in the right fore-arm and palm, 2 cases with PN of the median nerve and its branches in the right forearm and palm, 2 cases with PN of the median nerve and its branches in the left forearm and palm, and 1 case with PN of the ulnar nerve and its branches in the left forearm and palm.The postoperative function and feeling of the patients were evaluated by outpa-tient followed-up. Results The pathological results of 6 patients all showed PN, and their incisions healed primari-ly.The patients were followed-up for 6 to 36 months, with an average of 18 months. No obvious scar formation was observed in all incisions. Among them, PN of the palmar of the youngest patient recurred after the operation, and it was resected in a second operation.The remaining 5 patients had no recurrence during follow-up.The 2 point resolu-tion of each fingertip of the affected limb of the patients who had median and ulnar PN was 2-5 mm, with an average of 3.30 mm; the 2 point resolution of the thumb, indicator, middle and ring fingers of the affected limbs of the patients who had median PN was 2-5 mm, with an average of 2.95 mm; the 2 point resolution of the ring and little fingers of the affected limbs of the patients who had ulnar PN was 3-4 mm, with an average of 3.50 mm.According to the related evalu-ation criteria made by the American Orthopedic Foot and Ankle Surgery Society (AOFAS), the results of the forearm and hand functions were excellent in 5 cases, good in 1 case. Conclusion The application of microsurgical techniques in the treatment of PN in the forearm and palm can be effective separation of tumor and nerve fibers, effectively protect the branches of the median nerve and ulnar nerve and their blood circulation, prevent recurrence and reduce nerve damage after operation.

5.
Chinese Journal of Dermatology ; (12): 815-819, 2018.
Article in Chinese | WPRIM | ID: wpr-710467

ABSTRACT

Objective To describe high-frequency skin ultrasonographic and dermoscopic features of seborrheic keratosis (SK).Methods Between August and December in 2017,46 outpatients with SK were enrolled from the Department of Dermatology of Peking Union Medical College Hospital,and 50 SK lesions were observed by 50-and 20-MHz high-frequency skin ultrasonography and dermoscopy.The highfrequency ultrasonographic and dermoscopic features of SK lesions were summarized and analyzed.Statistical analysis was done by using chi-square test for comparisons of evaluation results of SK lesions between 50-and 20-MHz ultrasonography,and the correspondence between high-frequency skin ultrasonographic and dermoscopic features of SK was analyzed by calculating the simple matching coefficient.Results As 50-and 20-MHz ultrasonography showed,ultrasonographic features of SK lesions from top to bottom were enhanced hyperecho (48/50 vs.39/50,respectively,P =0.007),enhanced hyperechoic masses or spots in the stratum corneum (22/50 vs.11/50,respectively,P =0.019),sound shadows behind the epidermis (34/50 vs.13/50,respectively,P < 0.001),skin lesions with regular shapes and clear borders (46/50 vs.41/50,respectively,P =0.137),heterogeneous hypoechoic areas in the lesions (50/50 vs.47/50,respectively,P =0.079) and internal hyperechoic spots (25/50 vs.2/50,respectively,P <0.001),the lesional bottom being at the same level (40/50 vs.36/50,respectively,P =0.349),and reduced dermal echogenicity below the lesion (50/50 vs.28/50,respectively,P < 0.001).In regard to the overall evaluation of the above 8 ultrasonographic characteristics,50-MHz ultrasonography was superior to 20-MHz ultrasonography (P =0.002).The common dermoscopic features of the 50 SK lesions were clear borders (n =50),comedo-like openings (n =45),ridge or cerebriform pattern (n =31),hairpin-like vessels (n =30),multiple milia-like cysts (n =24),moth-eaten border (n =21) and shiny white streaks (n =3).Under 50-and 20-MHz ultrasonography,the simple matching coefficients between enhanced hyperechoic masses or spots in the stratum corneum and comedo-like openings under dermoscopy were 42% (21) and 20% (10) respectively,and the simple matching coefficients between internal hyperechoic spots and multiple milialike cysts (> 3) under dermoscopy were 58% (29) and 48% (24) respectively.Conclusion Highfrequency skin ultrasonography and dermoscopy both are of great value to the assessment of SK lesions,and 50-MHz ultrasonography is superior to 20-MHz ultrasonography for imaging skin lesions.

6.
Chinese Journal of Geriatrics ; (12): 702-706, 2018.
Article in Chinese | WPRIM | ID: wpr-709339

ABSTRACT

Objective To perform a systematic review of the literature on the cost-effectiveness of stroke rehabilitation care ,and to provide evidence for medical insurance payment and clinical decision making on stroke rehabilitation care. Methods We included published randomized controlled trails (RCTs)which compared rehabilitation care (RC)and usual care(UC)on stroke ,and summarized clinical outcome ,resource use ,costs ,and other indexes as outcomes of interest. Results Among included eight RCT studies ,the results of four models of stroke care were found as following. (1)Four studies involving early supported discharge(ESD)showed lower costs in rehabilitation care ,but similar(n=3) or better(n=1)clinical outcome. (2)Two studies involving home-based rehabilitation showed similar costs in one and lower costs in the other ,with no difference in clinical outcome between two trials. (3) One study involving stroke unit care showed higher costs and better clinical outcome. (4)One trial involving integration stroke service was associated with lower costs ,but no difference in the effectiveness. Conclusions For patients with stroke ,rehabilitation care leads to significant improvements on cost-effectiveness. Promoting the clinical use of rehabilitation care will bring about clinical benefits ,increase the efficiency of medical resources use ,and improve patient's quality of life.

7.
Chinese Journal of Radiology ; (12): 204-208, 2018.
Article in Chinese | WPRIM | ID: wpr-707918

ABSTRACT

Objective To investigate the correlation between dynamic contrast enhancement magnetic resonance imaging (DCE-MRI) quantitative parameters and pathological grading in esophageal squamous cell carcinoma(SCC). Methods Prospective analysis of esophageal squamous cell carcinoma confirmed by electronic gastrointestinal endoscopy was performed. Thirty nine patients who underwent radical resection of esophageal carcinoma with MRI examination one weeks before operation were included. All patients underwent routine chest MRI and DCE-MRI scans, and DCE-MRI quantitative parameters including volume transfer constant (Ktrans), exchange rate constant (Kep) and extravascular extracellular volume fraction(Ve)were measured.Pathological analysis of postoperative specimens,including pathological grading(highly differentiated,moderately differentiated,poorly differentiated,undifferentiated),gross tumor pathological type(ulcerative type,medullary type,fungating type,sclerotic type)and local infiltration degree (T staging) were performed. Kruskal-Wallis H test was used to compare the differences of quantitative parameters between different pathological T staging,gross tumor pathological types and pathological grades of DCE-MRI,and a Dunn-Bonferroni test for post hoc comparisons.Spearman rank correlation analysis was used to evaluate the correlation between DCE-MRI parameters and pathological grading of esophageal squamous cell carcinoma.The ROC curves was used to evaluate the efficiency of different parameters in the diagnosis of poorly differentiated esophageal squamous cell carcinoma. Result Among the thirty nine patients, they were divided into three group according to pathological findings: well differentiated (12 patients),moderately differentiated(15 patients)and poorly differentiated group(12 patients);ulcerative type (19 patients), fungating type(10 patients), medullary type(10 patients);T1, 2 stage(16 patients), T3 stage(14 patients), and T4 stage(9 patients). There was no significant difference in the value of Ktrans, Kepand Ve between different T staging groups and different tumor pathological types groups(all P>0.05).The differences of Ktrans, Kepand Vebetween different pathological grading groups were statistically significant (all P<0.05). There were positive correlation between Ktrans, Kep, Veand the pathological grading, rs value were 0.874, 0.672, 0.578 respectively, all P<0.01. The ROC curve area of Ktrans, Kepand Vein the diagnosis of poorly differentiated esophageal squamous cell carcinoma was 0.941,0.809 and 0.773 respectively.The diagnostic efficiency of Ktranswas the best.Conclusions The quantitative parameters of DCE-MRI are correlated with the pathological grading of esophageal squamous cell carcinoma. Ktrans, Kepand Vecan reflect the perfusion characteristics of esophageal squamous cell carcinoma.

8.
Article in Chinese | WPRIM | ID: wpr-608699

ABSTRACT

Objective To investigate the expression of microRNA-106b(miR-106b)in the placentas of patients with pre-eclampsia and its relationship with matrix metallopeptidase(MMP)-2,and its effect on the invasion and proliferation of trophoblasts. Methods (1) Placental tissues were collected from patients with mild pre-eclampsia (mPE, n=30) , severe pre-eclampsia (sPE, n=30) and normal pregnant women (n=40). Human choriocarcinoma cell lines JAR and JEG3 were assigned to the miR-106b mimics group, the mimics negative control group, the miR-106b inhibitor group and the inhibitor negative control group, respectively. (2) The target gene of miR-106b(such as MMP-2) was predicted by bioinformatics. Dual-luciferase reporting system was used to verify the regulation of miR-106b on the expression of MMP-2. (3) The expressions of miR-106b and MMP-2 were measured by quantitative real-time PCR (qRT-PCR) and western blot. (4) Cell proliferation was determined by MTT assay. (5) Invasive activities in each group were assessed by cell transwell invasion assays. Results (1) Predicting result of bioinformatics indicated that MMP-2 was one of the target genes of miR-106b. Dual-luciferase activity assay demonstrated that MMP-2 was the direct target of miR-106b(P<0.01).(2) The results of qRT-PCR.①The expression of miR-106b in the placentas of mPE, sPE, normal pregnant women were 2.89±0.04, 1.96±0.03, 1.01±0.03, respectively (P<0.05). And the expression of MMP-2 mRNA in the placentas of mPE, sPE, normal pregnant women were 1.87±0.05, 0.69±0.03, 2.78±0.03, respectively (P<0.05).②The expression of miR-106b in the JAR cell line in the miR-106b mimics group, the mimics negative control group, the miR-106b inhibitor group and the inhibitor negative control group were 2.39 ± 0.03, 1.03 ± 0.04, 0.73 ± 0.03, 1.11 ± 0.04, respectively (P<0.05). And its expression in the JEG3 cell line were 2.17±0.04, 1.18±0.04, 0.61±0.03 and 1.22±0.03, respectively (P<0.05). ③The expression of MMP-2 mRNA in the JAR cell line in the miR-106b mimics group, the mimics negative control group, the miR-106b inhibitor group and the inhibitor negative control group were 0.45±0.15, 1.02±0.03, 2.28±0.03, 1.11±0.03, respectively (P<0.05). And its expression in the JEG3 cell line were 0.58±0.03, 1.25±0.15, 2.25±0.03, 1.21±0.03, respectively (P<0.05). (3) The results of western blot.①The expression of MMP-2 protein in the placentas of mPE, sPE, normal pregnant women were 1.63 ± 0.04, 0.55±0.03, 2.82±0.03, respectively (P<0.05).②The expression of MMP-2 protein in the JAR cell line in the miR-106b mimics group, the mimics negative control group, the miR-106b inhibitor group and the inhibitor negative control group were 0.41 ± 0.03, 0.97 ± 0.03, 2.25 ± 0.03, 1.01 ± 0.03, respectively (P<0.05). And its expression in the JEG3 cell line were 0.53±0.03, 1.20±0.03, 2.31±0.04, 1.19±0.03, respectively (P<0.05). (4) miR-106b could inhibit the proliferation of JAR and JEG3 cells, cell proliferation rates in the miR-106b mimics group were lower than that in the mimics negative control group (P<0.05). And cell proliferation rate in the miR-106b inhibitor group was higher than the inhibitor negative control group (P<0.05) . (5) The numbers of JAR cell that passed the membrane in the miR-106b mimics group, the mimics negative control group. The miR-106b inhibitor group and the inhibitor negative control group were 61±15, 79±13, 134±13, 80±12, respectively( P<0.05). And the numbers of JEG3 cell that passed were 57±12, 71±15, 128±15, 70± 14, respectively (P<0.05). Conclusion The miR-106b could inhibit the invasion and proliferation of JAR and JEG3 cells through targeting MMP-2, and have a relationship with the pathogenesis of pre-eclampsia.

9.
Article in Chinese | WPRIM | ID: wpr-663864

ABSTRACT

Objective To investigate the role of microRNA-26a (miR-26a) in cell migration and invasion in cervical cancer and its regulatory effects on high mobility group protein A1(HMGA1). Methods Both HeLa and SiHa cells were divided into four groups:miR-26a mimic group,mimic control group,miR-26a inhibitor group and inhibitor control group. MiR-26a expression was evaluated by quantitative real-time polymerase chain reaction (qRT-PCR). Target genes for miR-26a-5p were predicted by bioinformatics and verified by dual-luciferase reporting system. HMGA1 expression was evaluated by Western blot and qRT-PCR. Cell migration and invasion were analyzed by in vitro assays. Results (1) HMGA1 was predicted as one of the potential targets of miR-26a by TargetScan. Results of dual-luciferase activity assay further con-firmed that HMGA1 was directly regulated by miR-26a. (2) Expression of miR-26a and HMGA1 at mRNA level was respectively enhanced and inhibited in miR-26a mimic-transfected HeLa as well as SiHa cells as compared with those in the corresponding mimic control groups (P<0.05). On the contrary, expression of miR-26a and HMGA1 at mRNA level was respectively reduced and increased in miR-26a inhibitor groups as compared with those in the corresponding inhibitor control groups(P<0.05). (3) Expression of HMGA1 in miR-26a mimic-transfected HeLa and SiHa cells was lower than that in the corresponding mimic control groups(P<0.05). But expression of HMGA1 in miR-26a inhibitor groups was higher than that in the corre-sponding inhibitor control groups(P<0.05). (4) Abilities of cell migration and invasion were suppressed in miR-26a mimic groups as compared with those in the corresponding mimic control groups,but were enhanced in miR-26a inhibitor groups as compared with those in the corresponding inhibitor control groups(P<0.05). Conclusion MiR-26a can inhibit the proliferation and invasion of HeLa and SiHa cells through targeting HMGA1,suggesting that miR-26a is closely related to cervical cancer and might be associated with chemo-therapy resistance in cervical cancer. This study might provide a new strategy for the prevention and treat-ment of cervical cancer.

10.
Chinese Journal of Dermatology ; (12): 478-481, 2017.
Article in Chinese | WPRIM | ID: wpr-616667

ABSTRACT

Objective To describe common dermoscopic features of primary cutaneous amyloidosis (PCA),and to explore the application value of dermoscopy in assisting the diagnosis of PCA.Methods Seventeen patients with PCA were collected from Department of Dermatology of Peking Union Medical College Hospital between April 2014 and December 2016.A total of 45 skin lesions were subjected to dermoscopy,and their dermoscopic features were analyzed.Results The common dermoscopic features of PCA included central hubs,which could be white,brown or scar-like areas,various pigment structures and shiny white streaks.Dermoscopy showed that central hubs could be observed in all the lesions (100%),while white center hubs alone were observed in 14 (31%) lichenoid lesions,both white center hubs and scar -like areas in 5 (11%) lichenoid lesions,brown center hubs alone in 8 (18%) macular lesions,both white and brown center hubs in 6 (13%) lichenoid lesions and 17 (38%) macular lesions.All the lesions showed various pigment structures.Shiny white streaks were observed in 4 (9%) lichenoid lesions.Conclusion Dermoscopy has good application value in assisting the diagnosis of PCA.

11.
Chinese Health Economics ; (12): 12-15, 2017.
Article in Chinese | WPRIM | ID: wpr-614159

ABSTRACT

Objective:To explain the supplier induced demand theory paradigm,track the empirical research progress,so as to provide references for health care reform in China.Methods:The new classical theory,game theory and behaviorism school were launched to process theoretical derivation of induced demand.The classical empirical research and the latest research progress were introduced to systemically review on supplier induced demand.Results:The generation of supplier induced demand was related with incentive system,the type of diseases and the crowd of research.Conclusion:It could effectively reduce the induced demand for health care that constraining hospital internal incentive,conducting medical supply side structural reform,developing the “Internet +medical” and building Diagnosis Related Groups (DRGs) payment system.

12.
Chinese Health Economics ; (12): 54-57, 2017.
Article in Chinese | WPRIM | ID: wpr-612083

ABSTRACT

Objective:To investigate the health resources utilization efficiency from the perspective of the end-of-life medical costs in advanced cancer patients,estimate the method of calculating the price range for medical service package in community health service center.Methods:Taking the medical cost of cancer patients in final year of the end of life in Hubei under the dual-referal policy of high leveled hospitals and community medical institutions,it estimated the pricing range for palliative care medical package and designed the medical payment policy for hierarchy diagnosis.Results:With the new medical service package policy,maintained the same level of medical service quantity,the total medical cost would save a great amount.Conclusion:Based on the accurate budget calculation of end-of-life costs,it had great significance for the implementation of classified diagnosis policy,rationally design the link and pricing range between hospitals and community medical service package.

13.
Article in English | WPRIM | ID: wpr-277876

ABSTRACT

Objective To investigate the features of Langerhans cell histiocytosis (LCH). <b>Method</b> Skin lesions,systemic involvement,imaging characteristics,laboratory tests,immunophenotying,treatment response,and survival of 122 LCH patients treated at our center from February 1983 to August 2013 were retrospectively analyzed. Results LCH was associated with diverse skin lesions. Lung was the most involved organ,followed by bone,skin,lymph nodes,liver,spleen,oral cavity,and thyroid. Multisystem LCH was more common than single-system LCH. Single-system LCH was mostly treated by surgery,whereas multisystem LCH by combined chemotherapy. Conclusion LCH has diverse clinical manifestations,with lungs being the most often involved organ. Surgery or chemotherapy is the mainstream treatment.

14.
Chongqing Medicine ; (36): 1588-1591, 2017.
Article in Chinese | WPRIM | ID: wpr-511948

ABSTRACT

Objective To investigate the effects of rabbit carotid artery cannula on atherosclerosis formation and β-catenin expression.Methods Fifteen 2-month-old New Zealand rabbits weighing(2.0±0.2)kg were randomly divided into three groups,high-fat diet group,left common carotid artery cannula group and left common carotid artery cannula plus high-fat diet group,5 cases in each group,and taking the right blood vessels in the left common carotid artery cannula group served as the control group.The animals were sacrificed after 9-week feeding,and the total rabbit carotid artery in each group was taken;the real-time PCR and immunohistochemistry were used to detect β-catenin mRNA and protein expression targeting in rabbit common carotid artery tissue.Results The real-time PCR results showed that the β-catenin mRNA expression in the left common carotid artery cannula group was higher than that in the control group,high fat diet group and the left common carotid artery cannula + high-fat diet group.The immunohistochemistry results showed that,except for the control group,various groups had the β-catenin protein location in the cytoplasma,moreover which in the left carotid artery cannula group and left common carotid artery cannula +high-fat diet group mainly located in the area of intimal hyperplasia.Conclusion β-catenin is highly expressed in the atherosclerotic vessel wall caused by rabbit carotid artery cannula.

15.
Chinese Journal of Dermatology ; (12): 702-705, 2016.
Article in Chinese | WPRIM | ID: wpr-503739

ABSTRACT

Objective To report a pedigree with X?linked dominant protoporphyria(XLDPP), and to detect 5?aminolevulinic acid synthetase 2(ALAS2)gene mutations in this pedigree. Methods A clinical investigation was performed in a pedigree with XLDPP, and relevant data were collected from family members. A next?generation sequencing method was applied to screen possible mutation sites, and Sanger sequencing was performed to determine pathogenic gene mutations. Dermoscopy was conducted to observe skin lesions in the patients with XLDPP, and the Fotofinder system and very high frequency (VHF) ultrasound system were utilized to assess the severity of photodamage. Liver and gallbladder ultrasonography as well as blood examination were performed for all the family members. Results A deletion mutation, c.1706?1709ΔAGTG, was detected in the ALAS2 gene on the X chromosomes of all the patients in this family, which led to replacement or loss of 19-20 C?terminal residues through transcriptional frameshifting, and eventually caused an increase in ALAS2 activity. In the patients with XLDPP, skin photodamage was relatively severe;protoporphyrin?induced hepatobiliary damage was observed and aggravated with age;anemia and iron deficiency occurred sometimes. Conclusion The deletion mutation c.1706?1709ΔAGTG of the ALAS2 gene may be the underlying cause of XLDPP in this pedigree.

16.
Chinese Journal of Dermatology ; (12): 651-653, 2016.
Article in Chinese | WPRIM | ID: wpr-502499

ABSTRACT

Objective To evaluate the prognostic accuracy of the score of toxic epidermal necrolysis (SCORTEN) scoring system in patients with toxic epidermal necrolysis (TEN) or Stevens-Johnson syndrome (SJS).Methods Clinical data were collected from 39 patients with SJS/TEN hospitalized in Peking Union Medical College Hospital during April 1992 and March 2014,and retrospectively analyzed.Among the 39 patients,13 had died,and the other 26 patients,who were matched to the dead patients in a ratio of 2:1 for age,all had a definite diagnosis and were discharged with improved conditions.The SCORTEN scoring system was used to evaluate the 39 patients with SJS/TEN and calculate expected mortality.The expected mortality and actual mortality were compared between different groups stratified by age in the 39 patients.The receiver operating characteristic (ROC) curve was drawn to assess the prognostic accuracy of the SCORTEN scoring system.Results According to the SCORTEN scoring system,15 out of the 39 patients scored 1 point,14 scored 2 points,6 scored 3 points,and 4 scored 4 points.The total number of expected deaths was 6.808,while that of actual deaths was 13.There was no significant difference between the expected mortality and actual mortality in every SCORTEN score-based group.The area under curve (AUC) was 0.832 8,indicating a good predictive ability of the SCORTEN scoring system.Conclusion The SCORTEN scoring system can predict mortality in TEN/SJS patients at early stage.

17.
Article in Chinese | WPRIM | ID: wpr-478186

ABSTRACT

BACKGROUND:Peripheral facial nerve injury first involves the retrograde reactions of central nervous system axons, and nerve regeneration wil depend on the survival and functional status of neuronal cel bodies. OBJECTIVE:To explore the expression of neuronal cadherin and placental cadherin in facial nuclei after facial nerve injury. METHODS:New Zealand white rabbits were randomly divided into model group (n=48) and control group (n=8). In the model group, every eight rabbits were used to carry out the test respectively at 1, 4, 7, 14, 21 days after the model of facial nerve injury (right side) was established. SP and real-time quantitative PCR methods were taken to test the expression of neuronal cadherin and placental cadherin in the facial nerve nucleus at mRNA and RESULTS AND CONCLUSION:The control group had neuronal cadherin-and placental cadherin-positive neurons. In the model group, neuronal cadherin and placental cadherin positively expressed in the facial motorneurons (right side), and their expressions were peaked at 14 days. Compared with the control group, the mRNA expression of neuronal cadherin in the facial motorneurons was increased significantly at 4-28 days after injury;the mRNA expression of placental cadherin in the facial motorneurons was decreased significantly at 1 day after injury, and then increased significantly at 7-28 days. It is suggested that the expression of neuronal cadherin and placental cadherin is positive in the early stage of facial nerve injury, and the expression of placental cadherin is always present, while the expression of neuronal cadherin relatively lasts for a short time. After facial nerve injury, the expression of neuronal cadherin and placental cadherin in the facial nerve nucleus is both increased, which indicates that the facial nerve regeneration may be related to the high expression of adhesion molecules.

18.
Article in Chinese | WPRIM | ID: wpr-746873

ABSTRACT

OBJECTIVE@#To explore the clinical repair value of facial skin and tissue defect caused by tumour, trauma and infection with the axial flap.@*METHOD@#The clinical data of 38 patients with facial skin and tissue defect were analyzed retrospectively. All of them were repaired by the axial flap.@*RESULT@#The axial flap was alive in all patients, and all incision healed in the first stage. All patients had a satisfied result after the second stage of flap surgery.@*CONCLUSION@#The method of the axial flap in face can be clinically applied in the facial skin and tissue defects caused by tumour, trauma injury and infection. Because the axial flap cotained named blood vessel, the survival rate of it is high.


Subject(s)
Dermatologic Surgical Procedures , Face , General Surgery , Humans , Reconstructive Surgical Procedures , Retrospective Studies , Skin Transplantation , Surgical Flaps , Wound Healing
19.
Chinese Journal of Oncology ; (12): 859-862, 2015.
Article in Chinese | WPRIM | ID: wpr-286708

ABSTRACT

<p><b>OBJECTIVE</b>To evaluate the efficacy and safety of topical PUVA treatment of refractory lesions of mycosis fungoides.</p><p><b>METHODS</b>From January 2008 to 2014, a total of 10 patients (4 males and 6 females) with mycosis fungoides were treated with topical PUVA in Peking Union Medical College Hospital, including 7 cases in plaque stage and 3 cases in tumor stage. The average number of lesions were 1.9±0.9. The median age of these patients was (46.0±9.4) years. The average course of disease was (12.4±7.7) years. Psoralen was applied topically on treatment area 30 min before total body UVA irradiation treatment, 3 times a week. And the efficiency and safety of the therapy were evaluated.</p><p><b>RESULTS</b>All the patients were treated with topical PUVA with a median total dose of (161.60±135.96) J/cm2 in an average of (18.10±14.61) fractions. Total dose of UVA was (1 953.25±829.73) J/cm2, and total number of treatment was (261.90±116.79) fractions. The total treatment time was (45.80±26.64) months. Complete clinical response (CR) rate was 60.0%, partial response (PR) rate was 30.0%, and the overall response rate (CR+PR) was 90.0%. One patient showed no response. No severe acute or chronic side effects were observed.</p><p><b>CONCLUSION</b>Topical PUVA therapy is effective in the treatment of refractory lesions of mycosis fungoides with little severe side effects.</p>


Subject(s)
Adult , Female , Ficusin , Therapeutic Uses , Humans , Male , Middle Aged , Mycosis Fungoides , Drug Therapy , Pathology , PUVA Therapy , Photosensitizing Agents , Therapeutic Uses , Treatment Outcome
20.
Chinese Journal of Oncology ; (12): 626-628, 2014.
Article in Chinese | WPRIM | ID: wpr-272322

ABSTRACT

<p><b>OBJECTIVE</b>Only a few clinical reports in the treatment of early mycosis fungoides (MF)(IA, IB, IIA stage) are available in the literature. The purpose of this study was to compare the efficacy and safety of narrow-band UVB and psoralen plus ultraviolet A (PUVA) photochemoterapy in 24 patients with early-stage MF, and explore a new approach for the treatment of early mycosis fungoides.</p><p><b>METHODS</b>A total of 24 identified early mycosis fungoides patients received PUVA, NB-UVB and a combined therapy of PUVA followed by NB-UVB (n = 9/6/9) irradiation. A retrospective study was carried out to analyze the sex, age of onset, TNM stage, treatment, and duration of treatment, and times of treatment, duration of maintenance treatment, effective and recurrence in these patients. The data were analyzed using SPSS 17.0 and a two-sided test at the α = 0.05 level of significance was conducted.</p><p><b>RESULTS</b>Of the 24 patients studied, the average treatment was 104.5 (95% CI, 75.71-133.29) times. The average duration of treatment was 12.88 (95% CI, 9.90-15.85) months. The average maintenance treatment time was 11.08 (95% CI, 2.13-20.04) months. The effective rate (CR+PR) of PUVA treatment was 88.9%, recurrence rate was 11.1% (n = 9). In the NB-UVB treatment group, the effective rate was 100.0%, and the recurrence rate was 33.3% (n = 6). In the PUVA followed by NB-UVB (combination therapy) treatment group, the effective rate was 77.8% and the recurrence rate was 55.6% (n = 9). There were no significant differences among the three groups in terms of number of treatments, treatment duration, maintenance treatment duration, effective rate and recurrence rate (P > 0.05).</p><p><b>CONCLUSIONS</b>PUVA and NB-UVB are effective and safe in the targeted therapy of early stage mycosis fungoides. The combined therapy of PUVA followed by NB-UVB can reduce the total PUVA dose and risk of developing skin cancer.</p>


Subject(s)
Combined Modality Therapy , Methods , Ficusin , Humans , Mycosis Fungoides , Therapeutics , Neoplasm Recurrence, Local , PUVA Therapy , Photochemotherapy , Physical Examination , Retrospective Studies , Treatment Outcome , Ultraviolet Therapy
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