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ObjectiveTo detect the mutations of GJB3 and GJB4 genes in two sporadic cases of erythrokeratodermia variabilis(EKV).MethodsGenomic DNA was extracted from two sporadic patients with EKV,their family members,and 100 normal human controls.All the exons and adjacent splice sites of GJB3 and GJB4 genes were amplified by PCR.Mutation scanning was carried out via direct bidirectional DNA sequencing.ResultsA G134C mutation was found at the GJB3 gene in patient 1,which caused a substitution of glycine by alanine at codon 45 (G45A).No mutation was found in the GJB4 gene in case 1 or GJB3 and GJB4 genes in case 2.ConclusionA missence mutation G45A in GJB3 gene is found in a patient with EKV.
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Objective To investigate the effect of acitretin on the histopathology and ultrastructure of lesions from patients with bullous ichthyosiform erythrodermia (BIE), and to explore mechanisms underlying the modulation of keratinization process by acitretin. Methods Lesional tissue was obtained from the back of 4 patients with BIE before and after the treatment with acitretin. Light microscopy and transmission electron microscopy were performed to observe histopathological and ultrastructural changes in these lesions. Results After treatment, the improvement in clinical manifestations was more than 75% in all the 4 patients, and reached 90% in 1 of the 4 patients. As histopathology and ultrastructural study showed, there was an obvious improvement in hyperkeratosis and continuity of extra cellular lamellar membrane, and a decrease in keratin deposition in prickle and granular layer, but no remarkable changes were observed for the proliferation of prickle cells or acantholysis. Conclusions Acitretin shows a favorable efficacy in clinical treatment of BIE,with histopathological and ultrastructural improvement mainly located in the stratum corneum. The modulation of keratinization process in keratinocytes by acitretin appears more apparent in granular and corneum layers.
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Objective To explore the best formula of the 0 5% liposome podophyllotoxin chitosan film. Methods Chitosan,acetic acid and gelatin were selected as three factors to prepare film, each factor including three levels, and 0 5% liposome podophyllotoxin served as blank factor. The films of different formula were prepared according to orthogonal design. The evaluation was made on the basis of the characteristics of conglutination and dissolution, then the best formula was determined finally. Results The results showed that the conglutination and dissolution of the film which was made of 2% chitosan, 1%acetic acid, 2% gelatin and 0 5% liposome podophyllotoxin chitosan was the most perfect. Conclusion The film designed according to orthogonal experiment was coincidenced with the requirements in aspect of conglutination and dissolution, and its appearance was perfect.
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Objective To search the clinical manifestations,data of laboratory examination, features of CT and MRI, therapy, prognosis in order to evaluate the progress in therapy of patients with crypotococcus meningitis(CM).Methods The findings of clinical features were analyzed and summarized for therapy of 43 CM cases identified with India-ink capsule staining.Results Primary positive rates of India-ink capsule staining were 58 1%(25/43), secondary positive rates were 30 2%(13/43),7 0%(3/43),patients diagnosed with cultivate positive. Clinical cure was achieved in 22 of 32 patients (68 7%) treated with amphotericin B, 4 patients of without efficacy using amphotericin B were cured by liposomal amphotericin B treatment.6 patients was died from cerebral hernia.Conclusions It is indicated that amphotericin B and fluconazole are effective and well tolerated in treatment of crypotococcus meningitis. If amphotericin B should inefficiency therapy of CM,liposomal amphotericin B would be consider.
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OBJECTIVE: To observe the release mode and the amount of retention in skin of podophyllotoxin liposomes in topical application. METHODS: Two kinds of podophyllotoxin liposome suspension (DPPC liposome and bean lecithin liposome) and podophyllotoxin tincture in same concentration were topically applied on the skin of porkets and the amounts of retention of drug in skin of different preparations were detected with confocal laser scanning microscope.RESULTS: The quantity of retention of podophyllotoxin DPPC liposome in epidermis and superficial layer of dermis was larger than those of other two preparations and the drug concentration in applying DPPC liposome kept higher for 48 hours.CONCLUSION:Podophyllotoxin liposome coated by DPPC is much more targetable to skin and is a fairly good topical preparation for applying on skin.
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Objective To study the distribution pattern of liposome podophyllotoxin(LP)in rat skin.Methods The rats were divided into two groups:0.5%liposome podophyllotoxin suspension was ap-plied to LP group,0.5%podophyllotoxin tincture was applied to control group.The skin specimens were ob-tained1?2?4?6?12and24h after drug application,the amount of fluorescent stain was observed under con-focal laser scanning microscope and converted to the values of area under the curve(AUC).Results The epidermal AUC of fluorescent amount in LP group was1.5-fold than that in control group,dermal AUC was2.3-fold higher.The unit area fluorescent amount in both epidermis and dermis was highest2hours after topical medication in control group(1585.52/?m 2 and2005.66?m 2 ),and quickly reduced after4hours.But the epidermal and dermal unit area fluorescent amount in LP group was rather low in4hours after topi-cal medication,and gradually increased after6hours,and peaked after12hours(750.28/?m 2 and1073.08/?m 2 ).Conclusion Liposome preparation of podophyllotoxin can be slowly released and lasts longer in the skin.