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.

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.

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.

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.

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.

Triptolide evaluates DNA methylation level of matrix metalloproteinase 9 gene in human fibrosarcoma HT-1080 cells / 中国中药杂志

<p><b>OBJECTIVE</b>The effect of triptolide on the DNA methylation level of MMP-9 gene and the mRNA expression of tissue inhibitors of met-alloproteinases (TIMPs) were examined in human fibrosarcoma HT-1080 cells to explore the molecular mechanisms involved in the anticancer activity of triptolide.</p><p><b>METHOD</b>HT-1080 cells were cultured in MEM containing 10% newborn calf serum and 1% penicillin-streptomycin. Triptolide was dissolved in dimethyl sulfoxide (DMSO) at a concentration of 1 goL-1 and stored at -20 degrees C. Triptolide was freshly diluted with culture medium perior to use and directly added to cell cultures at the indicated concentration, and incubated for 72 hours at 37 degrees C in a humidified atmosphere with 5% CO2, with changes of reagents every 24 hours. Methylation specific PCR (MSP)was applied to assess the methylation status of MMP-9 gene promoter, and semi-quantitative reverse transcription polymerase chain reaction (RT-PCR) was employed to measure the mRNA expression of tissue inhibitors of metalloproteinases (TIMPs) in human fibrosarcoma HT-1080 cells after 72 hours of treatment with 6 nmol x L(-1), 12 nmol x L(-1) or 18 nmol x L(-1) triptolide, respectively.</p><p><b>RESULTS</b>The methylation index of MMP-9 gene promoter was statistically elevated in HT-1080 cells after 72 hours of treatment with 18 nmol L(-1) triptolide, compared with those in controls (0.61 +/- 0.10 vs 0.39 +/- 0.10, P < 0.05), while no significant difference was noted between 6 nmol x L(-1) or 12 nmol x L(-1) triptolide treated HT-1080 cells and controls (0.40 +/- 0.15 vs 0.39 +/- 0.10, 0.46 +/- 0.20 vs 0.39 +/- 0.10, respectively, both P > 0.05). The mRNA expression of TIMP-1, -2, -3 or -4 was not significantly changed in HT-1080 cells after 72 hours of treatment with the indicated concentrations of triptolide, respectively compared with those in controls (all P > 0.05).</p><p><b>CONCLUSION</b>The results demonstrated that triptolide upregulates the methylation level of MMP-9 gene in HT-1080 cells in vitro.</p>

Expression and methylation status of IL-13 receptor α1 gene in peripheral T lymphocytes of patients with systemic lupus erythematosus / 中华皮肤科杂志

Objective To investigate the mRNA expression and methylation status of IL-13 receptor(IL-13R)α1 gene in peripheral T lymphocytes of patients with systemic lupus erythematosus(SLE).Methods Venous blood samples were obtained from 10 SLE patients(5 in active phase,5 in inactive phase)and 6 normal human controls.CD4+ and CD8+ T cells were isolated from these samples via magnetic activated cell sorting(MACS).Real-time quantitative PCR was used to test the mRNA expression of IL-13Rα1 gene,and methylation specific PCR to detect the methylation status.Results The expression level of IL-13Rα1 mRNA was 2.224±0.251,1.712±0.132.and 1.104±0.044 in CD4+ T cells of active SLE patients,inactive SLE patients and controls,respectively;the difference between the three groups was statistically significant(all P＜0.05).The expression level of IL-13Rα1 mRNA in CD8+T cells was significantly higher in active SLE patients than that in the normal controls(1.672±0.142 vs 1.238±0.106,P＜0.05),while no difference was noted between inactive and active SLE patients or normal controls.The methylation index of IL-13Rα1 gene was 0.454±0.023.0.635±0.065.0.844±0.097 in CD4+T cells of active SLE patients,inactive SLE patients and normal controls,respectively,and the difference between the three groups was significant(all P＜0.05),while no significant difference was observed in the methylation index in CD8+T cells among these groups(P＞0.05).The IL-13Rα1 mRNA expression in CD4+T and CD8+T cells was positively correlated with SLE disease activity index(SLEDAI)score(r=0.79,0.76,P=0.007,0.02 respectively).A negative correlation was found between the methylation level Of IL-13Rα1 in CD4+T cells and SLEDAI score(r=-0.89.P＜0.0 1).as well as between the IL-13Rα1 mRNA expression and its methylation level(r=-0.84,P＜0.0 1).Conclusion The development of SLE may be related to the overexpression of IL-13Rα1 gene induced by DNA hypomethylation in T cells.