ABSTRACT
Abstract Mucopolysaccharidosis (MPS) is a group of metabolic disorders caused by the deficiency or complete absence of certain lysosomal enzymes responsible for the breakdown of mucopolysaccharides, causing an accumulation of glycosaminoglycans (GAGs) throughout the body. Mucopolysaccharidosis type I (MPS I), also called Hurler syndrome, is an autosomal recessive lysosomal storage disorder resulting from a deficiency of the enzyme α-L-iduronidase. This report aims to present the clinical findings and diagnosis of a 21-month-old female with no history of similar cases in their previous generations. The diagnosis was considered based on the clinical and radiological characteristics of Hurler syndrome (HS) and confirmed biochemically, becoming the first confirmed case in the Dominican Republic.
ABSTRACT
Objective:To investigate clinical efficacy of mucopolysaccharide polysulfate cream combined with sertaconazole nitrate cream in the treatment of scaly hyperkeratotic tinea pedis.Methods:From March 2019 to January 2020, 100 patients with scaly hyperkeratotic tinea pedis were enrolled into this study, and randomly and equally divided into 2 groups by using a random number table: control group treated with topical sertaconazole nitrate cream alone at a dose of 0.5-1 g twice a day; combined group treated with topical mucopolysaccharide polysulfate cream at a dose of 0.5-1 g followed by topical sertaconazole nitrate cream at a dose of 0.5-1 g 30 minutes later, which were performed twice a day. The treatment lasted 4 weeks. The time to clinical symptom relief, efficacy and incidence of adverse reactions were compared between the two groups. Dermatology life quality index (DLQI) was assessed at 0, 2 and 4 weeks after the start of treatment. Two-independent-sample t test, repeated measures analysis of variance and chi-square test were used for statistical analysis. Results:After treatment, the time to pruritus relief and that to desquamation improvement were 6.05 ± 1.98 and 12.03 ± 3.92 days respectively in the combined group, which were significantly shorter than those in the control group (8.39 ± 2.11, 15.11 ± 4.05 days, t = 5.72, 3.86, respectively, both P < 0.001) . During the 4 weeks of treatment, DLQI scores gradually decreased in both the 2 groups (all P < 0.001) , which were significantly lower in the combined group than in the control group at weeks 2 and 4 (both P < 0.001) . After 4-week treatment, the total response rate was 98% (49/50) in the combined group, significantly higher than that in the control group (82%, 41/50; χ2= 7.11, P= 0.007) . There was no significant difference in the incidence of adverse reactions between the two groups ( P > 0.05) . Conclusion:Mucopolysaccharide polysulfate cream can improve the efficacy of sertaconazole nitrate cream in the treatment of scaly hyperkeratotic tinea pedis.
ABSTRACT
Objective:To investigate the inhibitory effect and mechanisms of action of mucopolysaccharide polysulfate cream on hypertrophic scar formation.Methods:Circular full-thickness wounds with a diameter of 6 mm were made in both ears of 16 New Zealand white rabbits to establish a rabbit ear model of hypertrophic scar. There were 3 hypertrophic scars in each rabbit ear. About 14 days after the operation, scars on the left ear were topically treated with mucopolysaccharide polysulfate cream, and served as the experimental group; scars on the right ear were topically treated with the cream vehicle, and served as vehicle control group. The dosage of topical agents for one rabbit ear was approximately 0.4 g, which were given twice a day for 6 consecutive weeks. Scar tissues were collected on days 0, 14 and 42, that is, 14, 28 and 56 after operation respectively, and subjected to hematoxylin and eosin (HE) staining, Masson staining and immunohistochemical study, so as to evaluate histopathological scores, measure the scar thickness and collagen fiber density, and determine the expression of type Ⅰ and Ⅲ collagen and the ratio of type Ⅰ/Ⅲ collagen. The t test and one-way analysis of variance were used to compare the indices between groups. Results:Compared with pretreatment histopathological manifestations, HE staining showed extensive extracellular matrix deposition, inflammatory cell infiltration and local hyperemia in the control group after 42-day treatment, but no obvious changes in the experimental group. The pathological scores of scar tissues on the rabbit ears significantly increased over time in the control group (days 0, 14 and 42: 4.16 ± 1.61, 6.50 ± 1.46, 6.53 ± 1.34, respectively; F = 13.69, P = 0.001) , while there was no significant change in the experimental group (days 0, 14 and 42: 4.65 ± 1.52, 5.13 ± 1.83, 5.38 ± 1.60, respectively; F = 0.78, P > 0.05) . Masson staining showed extremely high content of dark blue-dyed collagen fibers in the control group on day 42, but there was a decrease in the content of collagen fibers in the experimental group; with the increase in treatment duration, the thickness of scar tissues significantly increased in the control group compared with that before treatment ( F = 5.64, P = 0.007) , while there was no significant change in the experimental group ( F = 1.48, P > 0.05) . Immunohistochemical study revealed no significant change in the expression of type Ⅲ collagen in either the experimental group or the control group at any of the above posttreatment time points compared with that on day 0 ( F = 0.22, 0.92, respectively, both P > 0.05) , but the expression of type Ⅰ collagen and the ratio of type Ⅰ/Ⅲ collagen significantly increased in the control group ( F = 7.47, P < 0.001; F = 4.70, P = 0.005, respectively) . On day 42, the expression of type Ⅰ collagen and the ratio of type Ⅰ/Ⅲ collagen significantly decreased in the experimental group compared with the control group ( t = 3.04, P = 0.007; t = 2.35, P = 0.030, respectively) . Conclusion:Topical mucopolysaccharide polysulfate cream is effective in preventing and inhibiting scar hypertrophy by reducing the scar thickness and inhibiting the collagen fiber hyperplasia and type I collagen expression.
ABSTRACT
Objective@#To investigate whether topical mucopolysaccharide polysulfate (MPS) cream can reduce the incidence of eczema and skin atrophy in patients with moderate- or low-risk infantile hemangioma after the treatment with topical beta-blockers or 595-nm pulsed dye laser (PDL) , and to analyze factors influencing the occurrence of eczema and skin atrophy.@*Methods@#A total of 722 patients aged 0- 1 years with moderate- or low-risk infantile hemangioma were enrolled from 5 Children′s Hospitals in China. According to the disease condition and therapy acceptability, these patients were divided into 6 groups to be treated with topical beta-blockers and MPS cream (blocker+ MPS group) , topical beta-blockers (blocker group) , 595-nm PDL and topical MPS cream (PDL+ MPS group) , 595-nm PDL (PDL group) , 595-nm PDL combined with topical beta-blockers and MPS cream (PDL+ blocker+ MPS group) , and 595-nm PDL and topical beta-blockers (PDL+ blocker group) , respectively. All the externally applied agents were applied twice a day, and PDL was performed once every 4 weeks. Efficacy and adverse reactions were evaluated after 3-month treatment. Univariate and multivariate Logistic regression analyses were carried out to analyze factors influencing the incidence of eczema and skin atrophy in patients with infantile hemangioma after treatment, and chi-square test was carried out to compare efficacy among the groups.@*Results@#After 3-month treatment, multivariate Logistic regression analysis for comparing the blocker+ MPS group with blocker group showed that the risk factor for eczema on the surface of hemangiomas was no topical treatment with MPS cream (P= 0.007, OR= 3.887, 95% CI: 1.439-10.493) , while no correlations were observed between the occurrence of skin atrophy on the surface of hemangiomas and analyzed factors. Multivariate Logistic regression analysis for comparing the PDL+MPS group with PDL group showed that no topical treatment with MPS cream (P < 0.001, OR= 7.402, 95% CI: 2.604-21.042) and northern areas (P < 0.001, OR= 67.048, 95% CI: 7.977-563.518) were risk factors for eczema on the surface of hemangiomas, and risk factors for skin atrophy on the surface of hemangiomas included no topical treatment with MPS cream (P = 0.001, OR = 9.371, 95 CI: 2.590 - 33.900) and abundant blood supply of hemangiomas (P = 0.036, OR = 2.971, 95% CI: 1.075-8.208) . Multivariate Logistic regression analysis for comparing the PDL + blocker+ MPS group with PDL+ blocker group showed that risk factors for eczema on the surface of hemangiomas were no topical treatment with MPS cream (P= 0.005, OR= 3.426, 95% CI: 1.446-8.119) and northern areas (P < 0.001, OR= 31.704, 95% CI: 6.924-145.158) , and risk factors for skin atrophy on the surface of hemangiomas included no topical treatment with MPS cream (P < 0.001, OR= 6.011, 95% CI: 2.558-14.126) and southern areas (P= 0.022, OR= 3.021, 95% CI: 1.177-7.753) . After 3-month treatment, the response rate was significantly higher in the PDL group than in the PDL+ MPS group (χ2= 4.531, P= 0.033) , and significantly higher in the blocker group than in the blocker+ MPS group (χ2= 4.344, P= 0.037) . There were no significant differences in the response rate or cure rate among the other groups (all P > 0.05) .@*Conclusion@#During the treatment of moderate- or low-risk infantile hemangioma with topical beta-blockers or 595-nm PDL, the combination with topical MPS cream can reduce the occurrence of eczema and skin atrophy without affecting the therapeutic effect.
ABSTRACT
Objective To investigate whether topical mucopolysaccharide polysulfate (MPS)cream can reduce the incidence of eczema and skin atrophy in patients with moderate-or low-risk infantile hemangioma after the treatment with topical beta-blockers or 595-nm pulsed dye laser(PDL), and to analyze factors influencing the occurrence of eczema and skin atrophy. Methods A total of 722 patients aged 0-1 years with moderate-or low-risk infantile hemangioma were enrolled from 5 Children' s Hospitals in China. According to the disease condition and therapy acceptability, these patients were divided into 6 groups to be treated with topical beta-blockers and MPS cream(blocker+MPS group), topical beta-blockers(blocker group), 595-nm PDL and topical MPS cream(PDL+MPS group), 595-nm PDL(PDL group), 595-nm PDL combined with topical beta-blockers and MPS cream(PDL+blocker+MPS group), and 595-nm PDL and topical beta-blockers(PDL+blocker group), respectively. All the externally applied agents were applied twice a day, and PDL was performed once every 4 weeks. Efficacy and adverse reactions were evaluated after 3-month treatment. Univariate and multivariate Logistic regression analyses were carried out to analyze factors influencing the incidence of eczema and skin atrophy in patients with infantile hemangioma after treatment, and chi-square test was carried out to compare efficacy among the groups. Results After 3-month treatment, multivariate Logistic regression analysis for comparing the blocker + MPS group with blocker group showed that the risk factor for eczema on the surface of hemangiomas was no topical treatment with MPS cream(P = 0.007, OR = 3.887, 95％ CI: 1.439- 10.493), while no correlations were observed between the occurrence of skin atrophy on the surface of hemangiomas and analyzed factors. Multivariate Logistic regression analysis for comparing the PDL + MPS group with PDL group showed that no topical treatment with MPS cream(P < 0.001, OR = 7.402, 95％ CI: 2.604- 21.042)and northern areas(P <0.001, OR=67.048, 95％CI:7.977-563.518)were risk factors for eczema on the surface of hemangiomas, and risk factors for skin atrophy on the surface of hemangiomas included no topical treatment with MPS cream(P=0.001, OR=9.371, 95 CI:2.590-33.900)and abundant blood supply of hemangiomas(P=0.036, OR=2.971, 95％CI:1.075-8.208). Multivariate Logistic regression analysis for comparing the PDL+ blocker + MPS group with PDL + blocker group showed that risk factors for eczema on the surface of hemangiomas were no topical treatment with MPS cream(P=0.005, OR=3.426, 95％CI:1.446-8.119) and northern areas(P<0.001, OR=31.704, 95％CI:6.924-145.158), and risk factors for skin atrophy on the surface of hemangiomas included no topical treatment with MPS cream(P<0.001, OR=6.011, 95％CI:2.558- 14.126) and southern areas (P = 0.022, OR = 3.021, 95％ CI: 1.177- 7.753). After 3-month treatment, the response rate was significantly higher in the PDL group than in the PDL+MPS group(χ2=4.531, P = 0.033), and significantly higher in the blocker group than in the blocker + MPS group (χ2 =4.344, P=0.037). There were no significant differences in the response rate or cure rate among the other groups(all P>0.05). Conclusion During the treatment of moderate-or low-risk infantile hemangioma with topical beta-blockers or 595-nm PDL, the combination with topical MPS cream can reduce the occurrence of eczema and skin atrophy without affecting the therapeutic effect.
ABSTRACT
Objective:To assess the therapeutic effect of mucopolysaccharide polysulfate cream in prevention of postoperative scars.Methods:One hundred postoperative patients were divided into an experimental group and a control group (each n=50).After stitch removal,the experimental group wiped mucopolysaccharide polysulfate cream,and the control group wiped urea cream.The scars of the two groups were evaluated by Vancouver Scar Scale (VSS) on the day of stitch removal and in the 4th,8th,12th,16th,20th,24th,28th,and 32th weeks during the treat process.Results:At the beginning of treatment,the total VSS scores in the experimental group were always lower than that in the control group (P＜0.05).There was no difference between the two groups in the color scores at each time point of follow-up (P＞0.05).Form the 20th week,the vascular distribution scores in the experimental group were lower than that in the control group (P＜0.05).And the thickness and flexibility scores in the experimental group were lower at each time point of follow-up (P＜0.05).There were no differences between the two groups in wounds in head,face,or neck in the total VSS scores and all index scores (P＞0.05),and the total VSS scores in the experimental group,who had wounds in chest,shoulder,or back,or had wounds in waist,abdomen,or hip,or had wounds in extremity,were lower than that in the control group (P＜0.05).The vascular distribution and thickness scores in the experimental group,who had wounds in chest,shoulder,or back,were better than that in the control group (P＜0.05).Conclusion:Wiping mucopolysaccharide polysulfate cream after operation as soon as possible can effectively prevent scar hyperplasia,and it is worth to be widely applied in clinic.
ABSTRACT
Objective Through studying the apoptosis induced by stichopus japonicus acid mucopoly saccharide in the hepatocellular carcinoma cell line HepG2 in vitro, analysing the expression of Bcl-2 and nm-23in HepG2, to provide the theory foundation and its feasibility on whether it can be used for the chemotherapy of hepetocellular carcinoma. Methods The cells of HepG2 were cultured in vitro and treated with SJAMP at different doses(0.25,0. 5,1.0,2.0,4.0 g/L). MTT was used to observe the inhibitory effects of SJAMP on cell growth, Western blotting was used to detect apoptosis, and the apoptosis related change of expression of protein Bcl-2 and nm23-H1. Results (1) MTT identified that SJAMP produced an obvious time-and-dose-dependent inhibitory effect on the HlepG2 cells. (2) Western blot showed that SJAMP could induce the apoptosis of HepG2 cells through changing the expression of the protein of Bcl-2 and nn23-H1 (P<0.05). Conclusion (1)SJAMP produced obvious inhibitory effects on HepG2 cells and induce HepG2 apoptosis. (2)SJAMP can enduce the anti-tumor function in the method of changing the expression of protein Bcl-2 and nm23-H1.
ABSTRACT
Objective To investigate the effects of Stichopus variegatus mucopolysaccharide on plasma nitric oxide (NO) and serum endothelin(ET) after angioplasty in rabbits. Methods 50 New Zealand rabbits were randomly divided into four groups: Stichopus group, simvastatin group, model group and normal group. Endothelium of iliac arteries in Stichopus group, simvastatin group and model group were denuded by balloon catheter and the rabbits were fed with 2%cholesterol, 3%lard and 3%yolk mixed forage for six weeks, and then, atherosclerotic stenosis was showed by iliac angiography.When transluminal balloon angioplasty was done, from then on the feeds was changed from high cholesterol to ordinary forage. Stichopus (0.5g?kg -1?d -1) and simvastatin (0.5mg?kg -1?d -1) were given by gastric canal. While NaCl solution (0.9%) was given in model group. All rabbits were fed in separate cages and free drinking. After four weeks since transluminal balloon angioplasty, blood was collected from carotid artery to measure the changes of concentrations of endothelin(ET) and nitric oxide(NO). Examination of pathology by optical and electron microscope were done at same time. Results After four weeks since transluminal angioplasty, the concentration of plasm ET in model group increased significantly compared with the normal group(114.86?24.89 ng?L -1 vs 75.62?9.68 ng?L -1) and serum NO decreased significantly(37.50? 12.79?mol?L -1 vs 106.37?18.01 ?mol?L -1).The concentration of plasm ET in model group was higher than that in the simvastatin groups and Stichopus groups(114.86?24.89 ng?L -1 , 81.08?14.71 ng?L -1 and 83.34?11.41 ng?L -1 ,respectively),and the concentration of serum NO decreased obviously(95.63?8.62 ?mol?L -1,98.80?9.06 ?mol?L -1 and 37.50?12.79 ?mol?L -1,respectively). Conclusion After iliac arterial TA the concentrations of plasm ET were increased and the serum NO were decreased, the endothelial function fell into disorder. The Stichopus variegatus mucopolysaccharide had the effects of improving the endothelial function after angioplasty in rabbits by adjustment of plasm ET and serum NO.
ABSTRACT
The technique of extracting and refining mucopolysaccharide from Dasyatis akajei cartilage obtained was studied and the content of mucopolysaccharide was measured.The results showed that when the content of NaOH was 6%,temperature 40℃, extracting time 6h,followed neutral protein enzyme dissolution for 7h under 40℃,protein precipitation with trichloroacetic acid and 4 times volume 95% C_(2)H_(5)OH for polysaccharide precipitation.the extraction rate was over 21%, the product was white powder and its purity was about (84.53%).Physical and chemical properties of the mucopolysaccharide were identified.
ABSTRACT
The etiology and pathogenesis of lumbar disc herniation is not clearly proved although biochemical change, physical stress, genetic factor, autoimmune phenomenon and psychophysiologic phenomenon have been considered by many investigators. The most important one is a result of pathologic acceleration of normal aging of the tissue. The present study is aimed to investigate the changes of mucosubstances in normal aging and herniated disc which might have possible role on the disc herniation. The materials used in this study consisted of 123 cases of surgically removed herniated disc and 7 cases of normal control disc obtained from autopsy. Sections were cut and stained with periodic acid-schiff (P.A.S.)-alcian blue (pH 2.5) for the detection of neutral mucosubstances, alcian blue at pH 2.5 for nonsulfated acid mucosubstances, and alcian blue at pH 1.0 and Spicers diamine method (HID) for sulfated mucosubstances. The results were as follows. l. Of the 123 cases of lumbar disc herniation, there was a slight male preponderance over female with a ratio of 1.5:1.0. The most commonly affected level was L4-5 comprising 69 cases (56.1%), and up to three quarters of the patients the age was from 3rd to 5th decades in which it was rather evenly distributed. 2. The time interval between the appearance of symptoms and operation was less than 1 year in 77 cases (62.6%), in 32 cases (26.0%) being within 3 months. 3. Histochemical studies of the normal discs showed increase of neutral mucopolysaccharides with reduction of acid and sulfated mucopolysaccharides in the nucleus pulposus, and increase of all the mucosubstances in the annulus fibrosus by aging. 4. Histochemical studies of herniated disc showed an acceleration of the changes seen in normal changes. 5. Cartilaginous end plate formation was noted in 46,3% of herniated discs. The results obtained by the present investigation suggest that herniation of lumbar diosc is closely associated with accelerated or premature degeneration of the disc.