Diagnostic criteria and treatment progress of incontinentia pigmenti / 中国医师杂志

Incontinentia pigmenti (IP) is an X-linked dominant disease affecting the skin, teeth, eyes and central nervous system caused by mutations in the IKBKG gene. 95% of patients are female. Skin rash is the prominent manifestation and main diagnostic basis of IP, while external skin damagesare often the factors affecting the prognosis of IP. The diagnostic criteria for IP have been updated in recent years, and Sanger sequencing remains the gold standard for the detection and analysis of IKBKG mutations. IP patients should be fully evaluated, and active treatment should be given if eye retinopathy and nervous system damage are found.

DNA methylation status of the forkhead box protein 3 gene in CD4+ T cells of patients with Henoch-Sch(o)nlein purpura and its correlation with regulatory T cells / 中华皮肤科杂志

Objective To determine the proportion of CD4+ CD25+ regulatory T (Treg) cells,mRNA expression of the forkhead box protein 3 (Foxp3) gene,and DNA methylation status of the Foxp3 promoter in peripheral CD4+ T cells from patients with Henoch-Sch(o)nlein purpura.Methods Totally,20 inpatients with Henoch-Sch(o)nlein purpura and 20 healthy controls were enrolled from Department of Dermatology,the Second Xiangya Hospital of Central South University between 2015 and 2016,and there were no significant differences in the gender and age between the two groups (both P ＞ 0.05).CD4+ T cells were isolated from the peripheral blood samples of these subjects.Real-time fluorescence-based quantitative PCR was performed to detect the mRNA expression of the Foxp3 gene,flow cytometry to determine the proportion of CD4 + CD25+ Treg cells,and sodium bisulfite sequencing PCR (BSP) to determine the DNA methylation status of the Foxp3 promoter.Statistical analysis was carried out with SPSS16.0 software by using two-sample t test for the comparison between the two groups,and linear correlation analysis for evaluating the correlations of the DNA methylation status of the Foxp3 promoter with clinical severity scores and the proportion of CD4+CD25+ Treg cells.Results Compared with the healthy control group,the Henoch-Sch(o)nlein purpura group showed significantly decreased mRNA expression of the Foxp3 gene in CD4+ T cells (0.380 ± 0.226 vs.1,t =9.503,P ＜ 0.01),proportion of CD4+CD25+ Treg cells (1.668％ ± 0.959％ vs.2.741％ ± 1.131％,t =2.552,P ＜ 0.05),but significantly increased DNA methylation status of the Foxp3 promoter (0.712 ± 0.164 vs.0.453 ± 0.147,t =3.610,P ＜ 0.01).In the Henoch-Sch(o)nlein purpura group,the DNA methylation status of the Foxp3 promoter was negatively correlated with the percentage of CD4+CD25+ Treg cells (r =-0.490,P ＜ 0.05),but positively correlated with the clinical severity scores (r =0.486,P ＜ 0.05).The DNA methylation level of the Foxp3 promoter was significantly higher in the patients with renal impairment than in those without renal impairment (P ＜0.05).Conclusion The patients with Henoch-Sch(o)nlein purpura showed increased DNA methylation status of the Foxp3 promoter in CD4+ T cells,decreased mRNA expression of the Foxp3 gene and proportion of CD4+CD25+ Treg cells,which may be related to the occurrence of Henoch-Sch(o)nlein purpura,and affect disease development and prognosis.

Kaposi varicelliform eruption with severe complications:a case report / 临床儿科杂志

Objective To investigate the clinical features and prognosis of Kaposi varicelliform eruption with severe complications. Methods The clinical data of one child with Kaposi varicelliform eruption with severe complications was retrospectively analyzed. The related literatures were reviewed. Results A 5-month-old boy presented with recurrent rash on the head and face for 3 months and aggravated for 3 days. The skin lesions showed a characteristic of typical dome-shaped blisters with hemorrhagic crusting. At admission, the boy suffered with severe hypoproteinemia, hypocalcemia, and electrolyte disorder. The hypocalcemia was aggravated gradually. On the fifth day of admission, the boy had fever, convulsions, and tachycardia. Blood culture showed methicillin-resistant Staphylococcus aureus (MASA) infection. The diagnosis of sepsis was confirmed. At that very day, the boy started to have coagulopathy, so Fusidic and Vancomycin for anti-infection, Acyclovir for antivirus, intravenous infusion immunoglobulin, albumin, cryoprecipitate, plasma and calcium gluconate were administered, supplied with albumin and blood coagulation factor. The boy's condition gradually became stable and discharged on the 19th day after admission. Conclusions When Kaposi varicelliform eruption is complicated with hypoproteinemia and hypocalcemia, the critical illness is indicated. Clinicians should be alerted to the existence of sepsis, coagulation disorders, even septic shock and disseminated intravascular coagulation.

Mesenchymal stem cells inhibit the activation and proliferation of CD4+ T cells in Sj(o)gren syndrome by promoting prostaglandin E2, hepatocyte growth factor and indoleamine 2, 3-dioxygenase secretion / 中华风湿病学杂志

Objective To investigate the suppressive effect of prostaglandin E2 (PGE2),hepatocyte growth factor (HGF) and indoleamine 2,3-dioxygenase (IDO) whose secretion was promoted by human umbilical cord mesenchymal stem cells(MSCs) on the activated peripheral blood CD4+ T cells in primary Sj(o)gren syndrome (pSS) in vitro.Methods Primary cultured umbilical cord MSCs were identified by flow cytometry,and peripheral blood CD4+ T cells were sorted in pSS patients.CD4+ T cells were cultured with CD3,CD28 antibody for 72 h to be the activated(control group);the activated CD4+ T cells were co-cultured with MSCs for 72 h(MSCs group) or MSCs were pre-stimulated with interferon-γ (IFN-γ),then the activated CD4+ T cells were co-cultured with pre-stimulated MSCs for 72 h (pre-stimulated group).The suspension of CD4+ T cells were collected and counted.PGE2,HGF and IDO in the supernatants were detected by ELISA.Mean in groups were compared using ANOVA,and multiple comparisons were used with LSD method.Results The concentrations of PGE2 in the supernataut of the control group,MSCs group and pre-stimulated group were (111 ±4) pg/ml,(2 814±6) pg/ml and (2 716±8) pg/ml (F=167 292.12,P＜0.01) respectively.The concentrations of HGF in the above groups were (597±9) pg/ml,(383±9) pg/ml and (727±12) pg/ml(F=878.61,P＜0.01) respectively.The concentrations of IDO in the above groups were (143±4) pg/ml,(835±5) pg/ml and (588±3) pg/ml (F=21 104.41,P＜0.01) respectively.Compared with the control group,levels of PGE2 significantly increased in the MSCs group and the pre-stimulated group that CD4+ T cells were co-cultured with MSCs (t=509.88,P＜0.01 and t=491.48,P＜0.01),and levels of IDO also significantly increased (t=202.69,P＜0.01 and t=130.39,P＜0.01),while the activation and proliferation of CD4+T cells were inhibited (t=-16.20,P＜0.01 and t=-31.48,P＜0.01).Compared with MSCs group,levels of PGE2 and IDO significantly decreased in pre-stimulated group (t=-18.40,P＜0.01 and t=-72.30,P＜0.01),and levels of HGF significantly increased in pre-stimulated group(t=41.51,P＜0.01),while the activation and proliferation of CD4+ T cells were further inhibited (t=-15.28,P＜0.01).Conclusion MSCs can inhibit the activation and proliferation of CD4+ T cells in pSS in vitro.The suppressive effect of MSCs may be achieved by promoting secretion of cytokines such as PGE2,HGF and IDO.HGF plays a more important role in the suppressive effect of MSCs pre-stimulated with IFN-γ.Too much PGE2 or IDO propably results in negative feedback regulation of MSCs.

Effect of Yupingfeng granule on Th17/Treg balance in children with milk protein allergy / 中国医师杂志

Objective To observe Thl7 cells,Treg cells and their related factors in peripheral blood of children with milk protein allergy and explore the influence of Yupingfeng granule on Th17 / Treg imbalance and its clinical effect.Methods 40 children with milk protein allergy were divided into two groups randomly:the conventional treatment group (n =20),Yupingfeng granule group (n =20).The conventional treatment group received conventional treatment for 2 months.On the basis of routine treatment,Yupingfeng granule group was additionally treated with Yuping feng granule.The serum Th17,Treg cell counts,interleukin (IL)-17 and transforming growth factor-β1 (TGF-β1) levels were detected,and the eczema area and severity index (EASI) score of rash in children was recorded.Results The levels of Th17 cells and IL-17 in allergy children were obviously increased compared with those of the normal children,while the Treg cells,and the TGF-β1 level were lower than those of the normal children (P ＜ 0.05).After treatment,the Thl7 cells,the IL-17 levels and ESAI scores of the conventional treatment group and the Yupingfeng granule group were lowered,while the Treg cells and the TGF-β1 levels were increased (P ＜ 0.05).Compared with the conventional treatment group,these indexes increased and decreased more significantly in the Yupingfeng granule group (P ＜ 0.05).Conclusions Milk allergy children have obvious imbalance of Thl7/Treg;the Yupingfeng granule can adjust this imbalance and alleviate the allergic symptoms of milk protein.

Prevalence of skin diseases in pre-school children aged 0-7 years in 12 cities of China / 中华皮肤科杂志

Objective To investigate the prevalence of skin diseases in pre-school children aged 0-7 years in cities of China.Methods From November 2014 to April 2015,12 cities were chosen as survey spots,and pre-school children aged 0-7 years served as respondents.A population-based study was conducted,and 40 vaccination clinics and 80 kindergartens were selected by stratified random sampling.A questionnaire survey and dermatological examination were performed by trained dermatologists.Results A total of 20 033 pre-school children received questionnaires,whose age was 2.41 ± 1.82 years (range,0.08-6.83 years).Among these respondents,7 823 children were found to have skin diseases,with the total prevalence of skin diseases being 39.05％ (7 823/20 033).Additionally,the prevalence of skin diseases reported in the 12 cities from high to low was as follows:66.96％ (612/914,Dalian),56.73％ (2 310/4 072,Shanghai),55.49％ (556/1 002,Wuhan),49.18％ (390/793,Taiyuan),47.16％ (316/670,Chengdu),41.93 ％ (566/1 350,Nanjing),41.03％ (318/775,Chongqing),35.98％ (240/667,Hefei),33.87％ (677/1999,Shenzhen),31.37％ (554/1 766,Changsha),23.52％ (1 107/4 706,Beijing),13.42％ (177/1 319,Shenyang).Totally,40 kinds of skin diseases were investigated,and the 10 most common skin diseases were eczema/infantile eczema/atopic dermatitis (18.71％,3 749/20 033),ichthyosis vulgaris(6.25％,1 253/20 033),lichen pilaris (5.73％,1 148/20 033),diaper dermatitis (5.29％,1 059/20 033),papular urticaria(5.25％,1 052/20 033),hemangioma/vascular malformation (3.86％,774/20 033),pityriasis alba (3.45％,691/20 033),infectious skin diseases (2.59％,519/20 033),urticaria(1.71％,344/20 033)and contact dermatitis (0.5％,100/20 033).Conclusion The total prevalence of skin diseases among pre-school children in cities of China is 39.05％,and eczema/atopic dermatitis is the most common skin disease.

Effects of propranolol on in vitro cultured hemangioma endothelial cells and their mechanisms / 中华皮肤科杂志

Objective To evaluate effects of propranolol on the proliferation and apoptosis of in vitro cultured hemangioma endothelial cells (HemEC),and to explore their molecular mechanisms.Methods Hemangioma tissues were resected from 7 children with proliferative hemangioma,and used for in vitro culture of HemEC.Meanwhile,cultured human umbilical vein endothelial cells (HUVEC) served as controls.The 2 kinds of cells were treated with propranolol at different concentrations of 0,25,50,75,100,125 and 150 μmol/L for 24,48 and 72 hours separately.Methyl thiazolyl tetrazolium (MTT) assay was performed to evaluate cellular proliferative activity,and flow cytometry to determine the apoptosis rate.Some cultured HemEC were divided into 2 groups to be treated with 100 μmol/L propranolol-containing culture medium (propranolol group) and culture medium alone (blank control group),respectively,for 18 hours.Total RNA in the 2 groups was extracted separately.Differentially expressed genes in HemEC between the above 2 groups were identified by DNA microarray technology,and verified by real-time quantitative PCR.Results The treatment with 25 μmol/L propranolol for 24 and 48 hours caused a slight proliferation of HemEC (P ＜ 0.05).The survival rate of HemEC was decreased after the treatment with propranolol at the concentration of ≥ 100 μmol/L for more than 24 hours,while the proliferation of HUVEC was inhibited by the treatment with propranolol at the concentration of ≥ 100 μ mol/L for more than 48 hours.During 24-72 hours of treatment with 100-150 μmol/L propranolol,the survival rates of HemEC were significantly lower than those of HUVEC (P ＜ 0.05).After the treatment with 100-150 μmol/L propranolol,the apoptosis rate of HemEC gradually increased with the increase in treatment duration and concentrations of propranolol (all P ＜ 0.05).Compared with the blank control group,186 differentially expressed genes (＞ 1.5-fold changes) were screened out by DNA microarray technology,including 128 upregulated genes and 58 down-regulated genes.Real-time quantitative PCR showed that the mRNA expression of proprotein convertase subtilisin/kexin type 9 (PCSK9) and fatty acid binding protein 3 (FABP3) in the propranolol group were (9.88 ± 2.19) and (21.90 ± 8.18) times that in the blank control group respectively (t =7.028,4.427 respectively,P ＜ 0.05).Conclusions Propranolol at high concentrations can inhibit the proliferation of HemEC and HUVEC,and its inhibitory effect on HemEC is stronger than that on HUVEC.The inhibitory effect of propranolol on HemEC may be related to the inhibition of HemEC proliferation and promotion of HemEC apoptosis.

A case of pediatric drug-induced hypersensitivity syndrome / 中华皮肤科杂志

A 10-year and 9-month-old female patient presented with skin rashes all over the body,fever and superficial lymphadenectasis for 18 days after an intravenous drip of fosfomycin.Skin examination showed generalized swollen erythema all over the body,whose surfaces were covered with a large number of sticky furfuraceous grey-white scales.Laboratory examination revealed markedly increased levels of alanine aminotransferase and aspartate aminotransferase,as well as an increased number of eosinophils.Histopathological examination of skin lesions showed infiltration of scattered lymphocytes in the superficial dermis,as well as around skin appendages.Immunohistochemical study demonstrated that the infiltrating lymphocytes mainly included T lymphocytes,and no atypical cells were observed.The patient was diagnosed with druginduced hypersensitivity syndrome.After the treatment with intravenous glucocorticoids,immunoglobulin and oral cyclosporine,favorable therapeutic effects were achieved.

Efficacy and safety of Chushizhiyang ointment for the treatment of mild atopic dermatitis in infants: a multicenter clinical study / 中华皮肤科杂志

Objective To evaluate the efficacy and safety of Chushizhiyang ointment for the treatment of mild atopic dermatitis in infants.Methods A multicenter,randomized,open,active-controlled clinical trial was conducted.A total of 204 infants with atopic dermatitis were enrolled and randomly divided into 2 groups to be topically treated with Chushizhiyang ointment (test group,n =103) and hydrocortisone butyrate cream (control group,n =101),respectively,for 2 weeks.The improvement of eczema area and severity index (EASI) scores and quality of life was evaluated at 7 days and 14 days after the treatment,so was the incidence of adverse events and adverse reactions.Results Ninety-eight infants in the test group and 101 in the control group were included in the full analysis set,which revealed that the disease severity significantly decreased after the treatment in both groups.The EASI scores at the baseline and on days 7 and 14 were 2.47 ± 4.04,0.92 ± 1.25 and 0.39 ± 1.04 respectively in the test group,as well as 2.13 ± 2.01,0.85 ± 1.58 and 0.45 ± 1.65 respectively in the control group.Furthermore,the test group and control group both showed that EASI scores on days 7 and 14 significantly decreased compared with those at the baseline (the test group:T =-1 666,-1 793,respectively,both P ＜ 0.001;the control group:T =-1 951,-1 941,respectively,both P ＜ 0.001).No significant differences in EASI scores at the baseline or on days 7 and 14 were observed between the test group and control group (all P ＞ 0.05).The response rates in the test group and control group were 47.96％ (47/98) and 55.44％ (56/101) respectively on day 7,as well as 79.59％ (78/98) and 84.16％ (85/101) respectively on day 14,and there were also no significant differences between the two groups (both P ＞ 0.05).The adverse reactions mainly manifested as erythema,itching and scaling in the test group,as well as hypopigmentation,telangiectasia,scaling and hyperpigmentation in the control group.No significant difference in the incidence of adverse events was found between the test group (2.9％,3/103) and control group (6.9％,7/101).Conclusion Chushizhiyang ointment shows definite efficacy for mild atopic dermatitis in infants with good safety and tolerability,and can be a teatment option for mild atopic dermatitis in infants.

The application of the cloning and expression of SMCY gender-specific fusion antigens with the corresponding polyclonal antibodies / 中国法医学杂志

Objective with the advantages of rapidity in detection protein, We selected the gender-specific amino acid sequence based on human SMCY and SMCX, cloned and expressed SMCY gender-specific fusion antigens. The rabbits were immunized with purified antigens to obtain the polyclonal antibodies. A new method was established for rapidly sex identification of forensic evidence samples by detecting SMCY antigens with the corresponding polyclonal antibodies. Methods We found three differential fragments by analyzing the sequence of human SMCY and SMCX. Then we cloned this three fragments and ligated as a new recombinant.This SMCY gender-specific fusion antigen gene was sub-cloned into pET-28a and expressed in Escherichia coli. The fusion antigen was purified by Ni-NTA column. The rabbits were immunized with purified antigen to produce the specific polyclonal antibodies.The reactivity of the polyclonal antibody was evaluated by ELISA and Western blotting. We developed a colloidal gold test strip for detecting the gender of human samples. Results We successfully selected gender-specific amino acid sequence, the SMCY gender-specific fusion antigen was expressed by prokaryotic expression and the polyclonal antibody was prepared by immunizing rabbit. The results of colloidal gold strip tests showed that there is a significant difference between male and female serums. Conclusion The results showed that the SMCY gender-specific fusion antigen could be recognized by the polyclonal antibody.The colloidal gold strip tests made by SMCY gender-specific fusion antigens and the corresponding polyclonal antibodies could be used for rapidly determining the gender of forensic evidence samples.

The significance of serological markers and European League Against Rheumatism SS Disease Activity Index score in patients with primary Sj(o)gren's syndrome / 中华风湿病学杂志

Objective To explore the relationship between the clinical features,serological markers and European League Against Rheumatism SS Disease Activity Index (ESSDAI) scores of primary Sj(o)gren's syndrome (SS).Methods We enrolled 106 patients,who fulfilled the 2002 classification criteria for primary SS from December 2008 to January 2015,to evaluate the relationship among the clinical characteristics,laboratory features,serological variables and ESSDAI scores.According to serological variables,the prognosis was subdivided into three distinct groups:favourable (no serological markers),intermediate (one serological marker) and poor (two or more serological markers).These data were analyzed by Chi-square test and variance analysis.Results The mean ESSDAI score of 106 pSS patients was (11±7).ESSDAI score was categorized according to the EULAR-SS recommendations as low activity,moderate activity and high activity (scores of 0-4,5-13 and ≥14,respectively),and the positive rate of antinuclear antibody (ANA) 1:100 (6 cases,37.5％;37 cases,66.1％;32 cases,94.1％) in three different ESSDAI levels was statistically different (x2＝18.110,P＜0.01).Those with positive ANA 1:100[positive (13±7) and negative (7±4)],anti-SSA antibody postive (12±7) and negative (9±7),anti-RNP antibody (positive 16±9 and negative 10±6) had higher ESSDAI scores than those with negative ones (F=8.812,P=0.0001;F=3.862,P=0.024;F=5.786,P=0.004).No statistical difference in ESSDAI means were found between patients with positive anti-SSB antibody,rheumatoid factor (RF),FS level,dry mouth,Raynoud's phenomenon and psychosomatic diseases.The ESSDAI scores of favourable group,intermediate group and poor group were significantly different (8±5,10±7,14±7,F=8.715,P=0.000 1).In comparison with the other two groups,the poor pSS patients had a higher frequency of positive ANA 1:100 (15 cases,55.6％;20 cases,57.1％;40 cases,90.9％),anti-SSA antibody(11 cases,0.7％;23 cases,41.1％;36 cases,81.8％),anti-SSB antibody (6 cases,2 2.2％;13 cases,37.1％;23 cases,52.3％),anti-RNP antibody (0 case,0;2 cases,5.7％;9 cases,20.5％) (x2=17.408,P=0.002;x2=14.306,P=0.006;x2=12.330,P=0.015;x2=1 1.482,P=0.022).Conclusion Patients with two or more serological markers may have higher ESSDAI score,and which in turn may associate with poor prognosis.

Pathogenic Bacteria and Drug Sensitivity of 374 Cases of Children with Staphylococcal Scalded Skin Syn-drome / 中国药师

Objective:To understand the pathogen bacteria detection and drug sensitivity results in the children with staphylococ -cal scalded skin syndrome to provide data for the clinical treatment .Methods: Totally 374 children with staphylococcal scalded skin syndrome treated from January 2010 to June 2015 were selected .The children's wound secretion and blood samples were collected , and bacterial culture and drug sensitivity test were carried out .Results:Totally 223 pathogenic bacteria were detected out in the wound se-cretion samples;17 cases of blood culture were positive with the positive rate of 4.55%;187 strains of staphylococcus aureus were de-tected out;64 strains of MRSA were found out with the MRSA detection rate of 34.22% (64/187).The sensitivities of MSSA and MRSA to common antibacterial drugs were different .The susceptibility rates of MSSA and MRSA to vancomycin , teicoplanin , teicopla-nin and linezolid were all 100.00%.The sensitivity rates of MRSA to penicillin , oxacillin, piperacillin, piperacillin, cefoperazone so-dium, cefazolin, cefuroxime, cefoxitin, azithromycin and clindamycin were all zero .Conclusion: The pathogenic examination of staphylococcal scalded skin syndrome is very important , and antibiotics should be used reasonably according to the results of drug sensi-tivity.

The effect of human umbilical cord mesenchymal stem cells on miRNA profile of activated peripheral ;blood CD4+ T cells from patient with primary Sj?gren's syndrome / 中华风湿病学杂志

Objective To investigate how human umbilical cord mesenchymal stem cells (MSCs) in vitro regulate the miRNA profile of activated peripheral blood CD4+T cells from patient with primary Sj?gren's syndrome (pSS). Methods Peripheral blood CD4+T cells from patient with pSS were sorted and divided into healthy naive group, pSS naive group, pSS activated group, MSC treatment group and MSC (pre-stimulated by IFN-γ) treatment group. CD4+ T cells were counted. MiRNA microarray technology was used to detect the expression profile of CD4+T cells, and the expression of miRNA125b and miRNA155 was verified by real time quantification-polymerase chain reaction (RT-PCR). Mean in groups were compared using ANOVA, and multiple comparisons were used with LSD method. Results Both MSCs and IFN-γ-MSCs could inhibit the proliferation of activated CD4+ T cells in a MSC-dependent manner, but there was no significant difference between two groups. Microarray analysis found that the differentially enriched miRNAs in pSS na?ve (vs healthy na?ve), pSS activation (vs pSS na?ve), MSC treatment (vs pSS activation) and pre-IFN-γ MSC treatment (vs pSS activation) were 42 miRNAs, 56 miRNAs, 21 miRNAs and 24 miRNAs, respectively. Furthermore, the expressions of miRNA125b and miRNA155 were verified by RT-PCR and found that miRNA125b relative level in 5 groups was 1.02 ±0.13, 0.80 ±0.11, 0.44 ±0.17, 0.76 ±0.17 and 0.81 ±0.15 (F=18.32, P<0.01), and miRNA155 was 1.5 ±0.8, 3.9 ±1.3, 8.4 ±2.6, 10.1 ±4.2 and 11.2 ±5.0 (F=26.65, P<0.01). Conclusion MSCs can regulate miRNA profile of activated CD4+ T cells in peripheral blood of patient with pSS, and partially reverse down-regulated miR-125b in activated CD4+T cells, which may play a regulatory role in inhibiting the activation of CD4+T cells by MSCs.

A case of Parkes-Weber syndrome accompanied by pseudo-Kaposi's sarcoma / 中华皮肤科杂志

A girl who aged eight years and seven months presented with prunosus patches on the right buttock for 8 years,gradual unilateral enlargement of the right lower limb for more than 7 years,and multiple vegetations for 1 year.Dermatological examination showed nevus flammeus and multiple malodorous vegetations over the right lower limb with high skin temperature.The right lower limb was thicker and longer than the left lower limb.X-ray examination,magnetic resonance imaging and Doppler ultrasound examination revealed high-flow vascular malformations.Pathological examination of the vegetations showed vascular proliferation,fibroblast proliferation and erythrocyte extravasation.She was diagnosed as Parkes-Weber syndrome accompanied by pseudo-Kaposi's sarcoma.

Treatment of Kasabach-Merritt syndrome in three infants by percutaneous selective digital subtraction angiography combined with transarterial hardened embolization / 中华皮肤科杂志

Objective To develop a new treament strategy for Kasabach-Merritt syndrome.Methods Three infants who were diagnosed with Kasabach-Merritt syndrome and suffered from thrombocytopenia as well as bleeding and clotting disorders were treated with percutaneous selective digital subtraction angiography combined with transarterial hardened embolization under general anesthesia.Sclerosing agents included bleomycin A5 (4.0 mg),iodized oil (1.5 ml),dexamethasone (2.5 mg) and iopamidol (3 ml).Polyvinyl alcohol mixed with iopamidol (at a volume fraction of 0.5) served as the embolic material.Results All the three patients were successfully treated by the minimally invasive surgery.The amount of blood platelet returned to normal within 24 hours after the operation.On the fourth day,all the patients were discharged from hospital with the restoration of coagulation function.Revisits at one month and three months after the operation showed that hemangiomas markedly shrank and even subsided,and blood platelet count was maintained within normal range.Conclusions Percutaneous selective digital subtraction angiography combined with transarterial hardened embolization can result in a recovery of blood platelet count and shrinkage of hemangioma,and may serve as a minimally invasive treatment option for Kasabach-Merritt syndrome.

Efficacy and safety of tacrolimus 0.03 ％ ointment for the treatment of 2-year-old patients with moderate to severe atopic dermatitis / 中华皮肤科杂志

Objective To evaluate the efficacy and safety of tacrolimus 0.03％ ointment for the treatment of 2-year-old patients with moderate to severe AD.Methods An open-labeled,non-comparative,multi-center study was carried out,which included 59 2-year-old children with moderate to severe AD.All the patients were given topical tacrolimus 0.03％ ointment twice daily for 3 weeks.The evaluation of patients was scheduled at the baseline,1,2,and 3 weeks after the start of treatment.Clinical outcome parameters included the total response rate,eczema area and severity index score (EASI score),the percentage of body surface area (BSA％) affected,physician's global evaluation (PGE),children's dermatology life quality index (CDLQI),visual analog scale (VAS) pruritus score.Safety was assessed based on adverse events reported by patients or observed by the physicians.Results At the end of the treatment,the total response rate was 65.85％ with an EASI score of 4.18,and BSA％ of 16.41％.Of these patients,85.10％ achieved a satisfactory outcome,2.13％ achieved a complete cure,and all achieved an improvement,with no exacerbation observed.The 3-week treatment also resulted in a significant decrease in VAS pruritus score (from 6.80 to 3.21) and CDLQI (frown 7.06 to 2.91).Side effects mainly manifested as temporary burning sensation at the application site,and no severe adverse events associated with tacrolimus were observed.Conclusion Tacrolimus 0.03％ ointment seems safe and effective for the treatment of 2-year-old patients with moderate to severe AD.

Langerhans cell histiocytosis: clinical analysis of 126 cases / 中华皮肤科杂志

Objective To analyze lesional and clinical characteristics of Langerhans cell histiocytosis in children.Methods A clinical retrospective study was performed on 126 patients with Langerhans cell histiocytosis collected from 2006 to 2011 at the Hunan Children's Hospital.Results Of the 126 patients,the youngest was 2months old,and the oldest was 9 years old.The ratio of male to female was 2.5 ∶ 1.Clinical manifestations included eczematid,seborrheic lesions,hemorrhagic maculopapules,yellow nodules and white macules.Of the three clinical phenotypes of Langerhans cell histiocytosis,Letter-Siwe disease was the most prevalent,and most cases of LetterSiwe disease were associated with hepatosplenomegaly,abnormal chest X-ray,impaired hematopoietic function and multifocal bone injuries.The clinical grade was mainly Ⅲ and Ⅳ in patients with Letter-Siwe disease,Ⅰ in patients with eosinophilic granuloma,and varied from Ⅰ to Ⅳ in patients with Hand-Schuller-Christian disease with Ⅱ as the most common.Of these patients,those with eosinophilic granuloma had the oldest average age with bone as the only affected organ,while those with Letter-Siwe disease had the youngest average age with the greatest number of affected organs.The treatment of Langerhans cell histiocytosis included surgical operation and combined chemotherapy.Conclusions Langerhans cell histiocytosis has characteristic skin lesions and diverse clinical manifestations.Pathology has diagnostic significance to Langerhans cell histiocytosis.Therapy strategies and curative effects are dependent on the severity of,and the organs affected by Langerhans cell histiocytosis.

Over-expression of signal transducer and activator of transcription 2 gene in the peripheral blood cells is correlated with active disease in patients with systemic lupus erythematosus / 中华风湿病学杂志

Objective To investigate the expression of Signal transducer and activator of transcription 2 (STAT2) gene in the peripheral blood mononuclear cells of patients with systemic lupus erythematosus, and to evaluate the possible connections between STAT2 gene expression levels and clinical features. Methods One hundred and forty-four SLE patients, 27 non-SLE patients with other rheumatisms and 58 normal controls were recruited for this research, and the subjects were surveyed for clinical data collection. SYBR Green Dye based real-time quantitative PCR method was used to compare the expression levels of STAT2 in patients with SLE and those in the controls. The correlation of the gene expression levels and disease activity and specificity was studied. Results STAT2 expression levels (5.2±1.7) in SLE patients were remarkably higher than those in non-SLE patients and normal controls (4.3±1.1, 4.5±1.2, P<0.01 in both). The expression levels of STAT1 were increased in active SLE patients(5.2±1.5), comparing with those observed in inactive SLE patients (4.8±2.9, P<0.01), and expression levels of STAT1 in SLE patients were negatively correlated with C3 levels in sera (r=-0.449, P<0.01) whereas were positively correlated with SLEDAI-2K score and 24 hour urine protein (r=0.317, 0.309, P<0.01 in both). Conclusion Over-expression of STAT2 gene in the peripheral blood cells is linked with the pathogenesis of systemic lupus erythematosus, and the elevated expression level of STAT2 is correlated with SEE disease activity.

Th17-related cytokine profiles in plasma of patients with systemic lupus erythemtosus / 中华风湿病学杂志

Objective To analyze the levels of T helper(Th)17-related cytokines interleukin(IL)-17,IL-22,IL-23 and IL-27 in plasma of patients with systemic lupus erythematosus(SLE).Methods Plasma IL-17,IL-22,IL-23 and IL-27 levels were measured by enzyme-linked immunosorbent assay in 45SLE patients and 32 healthy controls and their associations with each other,disease activity and clinical features were evaluated.Results Plasma levels of IL-17 and IL-23 were significantly higher in SLE patients than in controls[77.8(25.4～487.6)pg/ml vs 36.4(15.7～338.2)pg/ml;14.7(<7.8～247.5) pg/ml vs <7.8(<7.8～81.7)pg/ml.both P<0.01].with no difference between active and inactive disease.In contrast,IL-22 levels were markedly decreased in SLE patients compared with the controls[77.4(<15.6～559.7)pg/ml vs 378.8(21.8～1154.2)pg/ml,P<0.01]and were lower in active disease than in inactive disease(P<0.01).IL-27 levels tended to be higher in SLE patients compared with controls,but the difference was not significant (P>0.05).A strong and positive correlation was found between IL-17 and IL-23 levels(P<0.01)in SLE patients.IL-22 levels were negatively correlated with SLEDAI score,erythrocyte sedimentation rate and antidsDNA antibody titers(all P<0.01),and positively correlated with C3 levels (P<0.05).Each cytokine levels were not related to specific manifestations and treatments.Conclusion Th17 cytokine response in peripheral blood of patients with SLE is abnormal.and IL-17 and IL-22 appear to play different roles in SLE pathophysiology.IL-23/IL-27 imbalance may contribute to the development of Th17-mediated inflammation in SLE.

Clinical analysis of 272 patients with Kawasald disease / 中华皮肤科杂志

Objective To investigate the clinical features of Kawasaki disease.Methods A retrospective analysis was performed in 272 children diagnosed as Kawasaki disease from 2002 to 2006.Clinical data,laboratory findings and auxiliary examination results were collected for these patients.Results The male-to-female ratio Was 2.58:1.Onset ages between 1 to 3 years accounted for 59.2%of patients.Of these patients,100%had a fever for more than 5 days,76.1%transient polymorphous exanthema,74.6% bilateral conjunctival hyperemia,47.8%flare and fissure on the oral lip,58.5%strawberry tongue,22.8% firm swelling of hands and feet as well as flushing of palms and soles,3 1.2%subacute desquamation at the junctional site between nail bed and skin,36%cervical lymphadenopathy.Laboratory findings showed a significant increase in the count of peripheral blood leukocytes and pefipheral blood platelets as well as erythrocyte sedimentation rate in 80.5%,87.5%and 96.2% of Patients,respectively.Additionally,81.6%of these patients were positive for C reactive protein and the frequency of coronary aaery involvement was 54.3%.All patients were treated with aspirin,and high-dose intravenous immunoglobulin was given to 258 patients.Fever relieved and the condition was controlled in all patients with an average hospitalization period of 8.9 days.Conclusions Kawasaki disease should be suspected in Patients with exanthematous lesions,fever lasting for more than 5 days and poor response to antibiotic therapy.Peripheral blood platelet count and cardiac ultrasound are of great value in the diagnosis of Kawasaki disease.Aspirin iS the first choice in treating Kawasaki disease,and adjunctive high-dose intravennous immunoglobulin treatment may facilitate the quick control offever.