Objective To investigate the effects of Chinese drugs for nourishing Yin and clearing the heat on regulation of sex hormones and clinical efficacy patients with SLE.Methods Fifty four active SLE patients with Yin deficiency were randomly divided into 2groups.Thirty patients in tr eatment group were treated with Pill of Wind-weed Phellodendron and Rehman nia(Zhibai Dihuang Wan)and Bolus for Replenishing Vital Ess ence(Da Buyin Wan)combined with western medicine for 2months,while 24patients in control group were treated with western medicine alone.Before and a fter treatment,the serum levels of f ollicle-stimulating hormone(FSH),luteinizing hormone,estradiol,pr ogesterone,testosterone,prolactin were measured by ELISA in 54patien ts,respec-tively.Before treatment the serum l evels of FSH,luteinizing hormone,estradiol,progesterone,testosterone,prolactin were measured in 30active SLEpatien ts without Yin deficiency and 25age-matched healthy controls simultaneously.Results The mean levels of FSH,luteinizing h ormone,estradiol were significant ly higher in patients with Yin de-ficiency than those without Yin deficiency and those in heathy controls(P

Objective To investigate the risk factors of flare in systemic lupus erythematosus(SLE).Methods In this retrospective cohort study132cases of SLE were followed up for1～5years.The risk factors of SLE flare were analyzed by univariate and multivariate logistic regression with computer software,SPSS.Results Of the132patients,82cases had more than one flare,the total number of flares was115.The incidence of flare was0.22per patient-year.The frequency of flares within5years after diagnosis of SLE was higher than that in more than5years.By univariate logistic regression analysis the age younger than40years at the beginning of the study,age of disease onset younger than32years,stress,depression,overwork,pregnancy,suffering from infectious disease,and quick decreasing of prednisone dosage significantly increased the risk of flare(P

Objective To analyze clinical and pathological features of dermatomyositis with panniculitis as a skin manifestation.Methods Clinical data were collected from 9 cases of dermatomyositis with panniculitis as a skin manifestation in Department of Dermatology of Zhongshan Hospital affiliated to Fudan University from October 2012 to July 2016,and their clinical and pathological features were analyzed.Results Of the 9 cases,6 were female and 3 were male,and the age ranged from 28 to 73 years.Panniculitis lesions of the 9 patients all manifested as painful indurated plaques or nodules on the buttock,thigh,waist,back,abdomen,upper extremities and cheeks.These lesions occurred before,after or simultaneously with the onset of characteristic skin and muscle lesions of dermatomyositis,especially preceded the onset of characteristic lesions of dermatomyositis by 30 years in 1 case.Histopathological examination of lesions showed liquefaction degeneration of basal cells,inflammatory infiltration of lymphocytes and plasma cells around blood vessels,in the fat lobules as well as between the lobules and septa in the dermis.The necrosis and calcification of lipocytes,lipomembranous changes,fibrinoid necrosis of damaged vessel walls and microvascular occlusion were observed in some cases.Because panniculitis preceded the onset of characteristic lesions of dermatomyositis,2 patients were misdiagnosed with lupus panniculitis and morphea profunda for several times.Most patients had good response to systemic glucocorticoids combined with immunosuppressive agents,while the patients with lipomembranous fat necrosis had poor response to the combination therapy.Conclusions Panniculitis lesions of dermatomyositis are histologically characteristic,and may do not coincide with the onset of characteristic lesions of dermatomyositis.If panniculitis lesions precede characteristic lesions of dermatomyositis,patients will be easily misdiagnosed.Thus,persistent follow-up visit will be of great importance for the diagnosis.

Objective To observe the effect of the 8-word bandage on the walking ability of stroke patients with knee hyperextension. Methods Fifty patients with stroke combined with knee hyperextension were randomly divided into the observation group and the control group with 25 cases in each group. The control group was treated with conventional methods, including Bobath technology, Brunnstrom therapy and motor relearning primarily rehabilitation training. In addition to the conventional methods, patients in the observation group used 8-word bandage to fix knee joint in walking training. Before and after 8 weeks of treatment, Holden walking function classification, 10-meter maximum walking speed and improved Barthel index were adopted to evaluate the walking ability, maximum walking speed and the life ability of the patients. Results There were no significant differences in the scores before treatment between the two groups. The Holden walking function classification, the 10-meter maximum walking speed and the Barthel index scores were significantly improved after 8-week treatment in both two groups (P<0.05), and patient conditions were more significantly improved in the observation group than those of the control group (P<0.05). Conclusion Using 8-word bandage to fix knee joint can significantly improve knee hyperextension in patients with stroke, so as to improve the walking ability and activities of daily living.

Objectives To explore the relationship between illness perceptions and health behavior in the subjects. Methods Revised illness perception questionnaire and health promoting lifestyle profileⅡ (HPLPⅡ) were used to measure 101 patients′illness perception and health behavior between the groups. Results The HPLPⅡscore of LEAD was(2.17 ± 0.29) with the score of dimension. The course of disease, prognosis and perception of emotion were negatively related to health behaviour; personal control and primaty congtion of drsease were postively related to health behaviour (all P<0.01). Conclusion Medical personnel should pay attention to the relationship between illness perceptions and health behavior in patients with LEAD and effective measures should be taken to improve the patients′illness perceptions, promote their choices and persistence in health behavior and improve the quality of life.

Objective To firstly report 4 cases of dermatomyositis characterized by painful palmar eruptions complicated by fatal rapidly progressive interstitial lung disease (RP-ILD) in China.Methods Four patients with dermatomyositis with painful palmar eruptions complicated by fatal RP-ILD were enrolled from the Department of Dermatology,Zhongshan Hospital,Fudan University between December 2014 and April 2017,and their clinical and pathological features were analyzed.Results Among these patients,3 were female and 1 was male.Their age ranged from 47 to 59 years.Of the 4 patients,3 had no muscular involvement.All of the 4 patients had multiple solid red papules or nodules on the bilateral palms,palmar and lateral surfaces of fingers,which preceded,followed or concurred with the onset of other skin lesions of dermatomyositis.The occurrence of type Ⅰ respiratory failure was preceded by 3 weeks to 5 months of painful palmar eruptions in the 4 patients.Early-stage palmar eruptions were easily misdiagnosed as contact dermatitis,eczema or erythema multiforme.Histopathological examination of the skin lesions on the finger palmar surface showed perivascular infiltration of a few lymphocytes in the dermis,and deposition of varying amounts of mucin-like substances around blood vessels and appendages.Of the 4 patients,3 showed positive staining for anti-melanoma differentiation-associated gene 5 antibody.Although the 4 patients received anti-inflammatory and immunosuppressive therapies,they all finally died of respiratory failure.Conclusions Dermatomyositis with painful palmar eruptions may indicate the occurrence of fatal RP-ILD,and early biopsy of skin lesions is needed to help to identify the disease.Immunosuppressive treatment should be performed timely to improve the prognosis in these patients.

Objective:To determine classification and clinical features of morphea.Methods:A retrospective analysis was conducted on epidemiological information about clinical manifestations of and laboratory data from 180 patients with morphea, who visited Zhongshan Hospital, Fudan University from January 2010 to July 2021. Two-independent-sample t test was used to compare the age at onset between genders, and chi-square test to analyze differences in clinical characteristics between different genders and subtypes. Results:Among the 180 patients, 123 were females and 57 were males, with a male-to-female ratio of 1∶2.16. The age at onset of morphea was 28.69 ± 17.97 years for female patients, and 29.90 ± 20.67 years for male patients. Among them, linear morphea was the most common type in this study (68 cases, 37.78%), followed by plaque morphea (63 cases, 35.00%), mixed morphea (28 cases, 15.56%) and deep morphea (21 cases, 11.67%). The disease occurred in all age groups, but the age at onset significantly varied among different clinical subtypes ( F = 5.95, P < 0.001). No significant difference was observed in the age at onset or proportion of clinical subtypes between genders ( F = 0.15, P = 0.696; χ2 =2.88, P = 0.410). Atrophoderma of Pasini and Pierini (APP) was very common (62 cases, 34.44%) in the 180 patients, which mainly manifested as plaques or linear lesions, and 26 out of 45 patients with plaque APP and 11 out of 17 with linear APP were both accompanied by other subtypes of morphea. Among the 75 patients tested for autoantibody profiles, 34 (45.33%) presented with positive results. More diverse types of autoantibodies were found in female patients compared with male patients, and antinuclear antibodies, anti-SSA and anti-SSB antibodies were the most common types. There were various types of comorbidities in female patients, but lichen sclerosus et atrophicus and vitiligo were the most common comorbidities in both genders. Conclusion:High incidence and frequent co-occurrence with other subtypes of APP may be the characteristics of Chinese patients with morphea, and it is recommended to classify morphea into plaque, linear, deep and mixed subtypes.

Objective:To study the effect of Sanjie Xiaoliu Recipe on transplanted tumors in breast cancer mice through in vivo experiments, in order to explore the efficacy and mechanism of Sanjie Xiaoliu Recipe on breast cancer patients. Methods:45 BaL B/c female mice were selected to establish the transplanted tumor model of breast cancer. All the transplanted tumor models of breast cancer mice were randomly divided into five groups: the control group (intragastric administration of normal saline), the low, medium and high dose group of Sanjie Xiaoliu Recipe (intragastric administration of different doses of Sanjie Xiaoliu Decoction), and the paclitaxel group (intraperitoneal injection of paclitaxel). After 24 days of continuous administration, the diet and activities of mice were observed; the body weight, weight and volume changes of transplanted tumor mice were recorded, and the tumor inhibition rate was calculated. Western blot was used to detect the expression of epithelial mesenchymal transformation related proteins (E-cadherin, N-cadherin, Vimentin) in the transplanted tumor tissues of mice in each group.Results:(1) The food intake and activity status of mice treated with Sanjie Xiaoliu Recipe were less affected by the transplanted tumor of breast cancer. (2) The volume and weight of transplanted tumor in the treat groups were smaller than those in the control group (all P<0.01), and the volume of transplanted tumor in the middle dose group of Sanjie Xiaoliu Recipe was smaller than that in the low dose group ( P<0.05). The tumor inhibition rates among the treatment groups were: Sanjie Xiaoliu Recipe medium dose group 52.4%, paclitaxel group 40.3%, Sanjie Xiaoliu Recipe low dose group 39.5%, Sanjie Xiaoliu Recipe high dose group 34.1%. (3) The results of Western blot showed that the expression level of E-cadherin in the transplanted tumor tissue of the treat groups was higher than that in the control group, and the expression levels of N-cadherin and Vimentin were lower than that in the control group, with statistically significant difference (all P<0.05). Conclusions:The Sanjie Xiaoliu Recipe can improve the weakness and reduced consumption of breast cancer mice, which can inhibit the tumor mass growth in mice to a certain extent. Its mechanism may be that Sanjie Xiaoliu Recipe can inhibit the epithelial mesenchymal transformation of breast cancer and the invasion and metastasis of breast cancer.

Objective To explore the effects of improvements on setup error ( SE ) and clinical target ( CTV ) margin of supraclavicular field ( SCF ) by using moisture-cured resin cushion and breast bracket for lower neck fixation in breast cancer patients who underwent post-mastectomy radiotherapy. Methods Totally 13 patients with breast cancer who underwent post-mastectomy radiotherapy were enrolled. All patients were immobilized by breast bracket and moisture-cured resin cushion. Firstly, each patient′s lower neck and head was fixed well by moisture-cured resin cushion, filling the gap between the neck and breast bracket. Secondly,each patient underwent the cone-beam CT ( CBCT) at the first, tenth and twentieth treatment after positioning. Then these CBCT images were registered to the planning CT to determine setup errors in translational and rotational directions, and SCF CTV margins by the systematic and random errors were evaluated. Results The setup errors in x (left-right), y (superior-inferior), z (anterior-posterior) translational directions were (2. 16 ± 1. 25), (1. 50 ± 1. 28), (1. 94 ± 1. 12) mm and (1. 76 ±1. 87)°, (1. 82 ±1. 12)°, (0. 99 ±0. 58)°, respectively in θ (pitch degree),Ф(roll degree),ψ( yaw degree) rotational directions. Non-parametric rank test ( Mann-Whitney U test) was performed with previous data, the differences of the setup error in y, z,θ directions were statistically significant ( Z =4. 152, 3. 415, 2. 053, P<0. 05). The margins from CTV were 4. 07, 4. 03 and 3. 73 mm in x, y and z directions, respectively. Compared with the previous study on SCF, CTV margin required 8, 8 and 6 mm in x, y and z axis directions, the volume of CTV to PTV were decreased by 32. 73% on average. Conclusions Compared with plastic circular pillow alone, moisture-cured resin cushion with breast bracket locating method could reduce setup errors in SCF target region. The margins from CTV to PTV weredecreased to 4. 07, 4. 03 and 3. 73 mm in x, y and z axis directions at least.

OBJECTIVE: To report on a case with severe hemophilia A (HA) due to a large duplication of F8 gene. METHODS: Inversion detection, Sanger sequencing, and multiplex ligation-dependent probe amplification (MLPA) were used to detect the mutation in the proband and his mother. RESULTS: The patient, a 7-year-old boy, was diagnosed with severe HA at 8 months. No inhibitor was developed over 150 exposure days. Intronic inversion detection and Sanger sequencing have failed to identify pathogenic variants, while MLPA revealed a large duplication [Ex 1_22 dup (2 copies)] in the proband, for which his mother was a carrier [Ex 1_22 dup (3 copies)]. Large duplications of the F8 gene have so far been found in 24 HA patients, all of whom had a severe phenotype, only one had a history of inhibitors. CONCLUSION Large duplications of F8 gene are associated with severe HA. The diagnostic rate for HA may be increased by MLPA.
Child ; Factor VIII/genetics* ; Gene Duplication ; Hemophilia A/genetics* ; Humans ; Introns ; Male ; Mutation ; Phenotype