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Nephrotic syndrome(NS)is the most frequent cause of glomerular disease in children,it can deve-lop many complications,including infection,thrombosis and acute kidney injury,while acute adrenocortical suppression is a complication of some drugs administration. This review focuses on the pathophysiology and management of common complications in patients with NS.
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Objective To investigate the clinical features and gene mutation of a newborn with neonatal-onset ornithine transcarbamylase deficiency (OTCD) and report the multidisciplinary perinatal management of the mother with late-onset OTCD.Methods The clinical features,biochemical data and the treatment of a newborn boy with OTCD and his mother admitted by Xin Hua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine in April,2013,were collected.The ornithine transcarbamylase (OTC) gene in the family was analyzed.Results Serum ammonium in the male newborn gradually increased to 1 020 μ mol/L at 48 h after birth.His blood amino acids level and urine organic acid level showed a pattern indicative of OTCD [blood arginine (97.43 μ mol/L,reference 1.00-25.00 μ mol/L),citrulline (27.43 μ mol/L,reference 4.00-30.00 μ mol/L),ornithine (161.66 μ mol/L,reference 10.00-120.00 μ mol/L) and methionine (70.45 μ mol/L,reference 10.00-50.00 μ mol/L); urine uracil (67.11 μ mol/mol Crea,reference 0.00-7.00 μ mol/mol Crea) and orotic acid (1 372.66 μ mol/mol Crea,reference 0.00-1.50 μ mol/mol Crea)].DNA studies revealed a c.583G ＞ A (G195R) homozygous mutation of the OTC gene.His mother was heterozygous for OTCD and developed acute hyperammonemia during pregnancy.Her blood showed a normal-leveled arginine (8.44 μ mol/L,reference 1.50-25.00 μ mol/L),a normal-leveled citrulline(8.41 μ mol/L,reference 7.00-35.00 μ mol/L),an elevated glutamate(279.15 μ mol/L,reference 45.00-200.00 μ mol/L).Her urine uracil (51.55 μ mol/mol Crea,reference 0.00-7.00 μ mol/mol Crea) and orotic acid (38.75 μ mol/mol Crea,reference 0.00-1.50 μ mol/mol) were elevated.Successful management of her prenatal and postpartum blood ammonia level was achieved after administration of pharmacologic nitrogen scavengers and protein limitation.DNA studies revealed a c.583G ＞ A (G195R) heterozygous mutation in the newborn's mother and grandmother.Conclusions General management on pregnant OTCD women is effective.Male newborn patients often have a poor prognosis.
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Objective To investigate the clinical efficacy of Q-switch 1064 nm wavelength laser combining with microneedle technology for the treatment of melasma.Methods 70 melasma patients were selected in the Out-patient Department of Wuhan General Hospital of Guangzhou Military Command from July 2012 to Sept.2013.The patients were randomly divided into 3 groups:the combining therapy group (patients in this group received Q-switch 1064 nm wavelength laser combining with microneedles technology,the treatment interval was 2 or 3 weeks,and the whole course was 2-6 months);the microneedle group and the Q-switch 1064 nm wavelength laser group.The effect and the adverse reaction were observed and recorded during the treatment,and the reduced area of the pigment patch was also measured to judge the total efficacy after the course.Results The total effective rate was 78.6％ (22/28) in the combining therapy group and 40.0％ (8/20) in the microneedle group,45.5％ (10/22) in the Q-switch 1064 nm wavelength laser group.There were statistically significant differences between the combining therapy group and the other groups (P1 ＜0.05,P2 ＜0.05),without obvious adverse reaction.Conclusions It is significantly better to use American Medlite C6 Q-switch 1064 nm wavelength laser combining with microneedles technology than that of the single use of Q-switch 1064 nm wavelength laser or microneedles in the treatment for the patients with melasma.The method is also simple to handle and suitable for clinical application.
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Objective To investigate the clinical efficacy of fractionated ultrapulse carbon dioxide laser combined with microneedle for depressed acne scars.Methods Totally 86 patients with depressed acne scar were randomly divided into 2 groups:the treatment group received fractionated ultrapulse carbon dioxide laser combined with microneedle technology and the control group only received fractionated ultrapulse carbon dioxide laser.The treatment interval was 1 to 3 months,and the whole course was 2-8 times.The total effective rate and the adverse reaction were analyzed in the facial acne scar.Results The surface of depressed acne scars was significantly improved.ECCA score of all patients declined obviously after treatment,the score of the treatment group declined from 61± 11 to 45± 15,and the control group declined from 57± 14 to 40± 16.There were significantly statistical differences in the results of each group (P＜0.05).More treatment times resulted in better surface improvement.The total effective rate were 81.8％ (18/22) and 100％ (24/24) in the treatment group,and 80.0％ (16/20) and 100％ (20/22) in the control group.The course was 6.8± 1.6 months in the treatment group and 20.8±4.6 months in the control group (P＜0.05) to get a similar satisfied result.There were no obvious adverse reactions except two cases of erythema and pigmentation in the treatment group and 5 cases in the control group.The former was with less adverse reactions.Conclusions Ultrapulse carbon dioxide laser combined with microneedle technique is effective for facial acne scars,with less complications and shorten treatment course.
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Objective To evaluate the efficiency and safety of hair removal in different areas with Lightsheer Duet diode laser and to compare clinical effect and safety in axillae hair removal between the two models,high-speed and standard model.Methods Lightsheer Duet diode laser was used to remove hairs from different areas in 200 cases,for 4-8 weeks treatment 1 time,4 times for 1course.The curative effect and adverse reaction were observed after the treatment.Thirty axillae hair removal subjects were chosen randamly and received treatments using the different models,high-speed hand piece on one side and the standard hand piece on the other side.Hair removal efficiency and the immediate pain were compared and evulated on each subject.Results The hair was significantly reduced after 4 times treatments in 200 cases.The total effective rate was 95.54 ％ and positively related with the treatment times.Pigmentation was found in 3 cases,but it disappeared after 1-3 months.The total effective rate was 100 ％ after the 3 times treatments in axillae hair removal subjects both using the high-speed model or the standard model in 30 cases.There was no statistical difference between the two models except less pain in the high-speed model.Conclusions Lightsheer Duet diode laser is a safe and efficient method in hair removal.The high-speed model has more advantage on speed and causing pain.
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Objective To investigate the modulation of ET-3 on the nuclear factor (NF)-κB/Bfl-1 antiapoptotic pathway in a malignant melanoma cell line A375. Methods Flow cytometry was performed to detect the apoptosis in cultured A375 cells after treatment with ET-3 of 100 nmol/L for 24 hours. ET-3 of various concentrations (0, 1, 10, 100 nmol/L) was used to treat some A375 cells with or without the pretreatment with the ETRB antagonist BQ788; after another 24-hour culture, RT-PCR and Western blot were conducted to examine the mRNA expression of Bfl-1 and protein expressions of Bfl-1 and ETRB, respectively. Results The 24-hour treatment with ET-3 of 100 nmol/L significantly reduced the apoptosis rate of A375 cells (F = 10.68, P ＜0.05). The mRNA and protein expressions of Bfl-1 were up-regulated by ET-3 in a concentration dependent manner (both P ＜ 0.01 ), while BQ788 significantly blocked the ET-3-induced up-regulation (F = 420.38,229.49, both P ＜ 0.01 ). The protein expression of pNF-κB in A375 cells was also enhanced by ET-3 of different concentrations (all P ＜ 0.05), but the enhancement was suppressed by BQ788, and there was a significant difference in the protein expression of pNF-κB between cells treated with ET-3 of 100 nmol/L and those treated with the combination of ET-3 of 100 nmol/L and BQ788 (F = 255.46, P ＜ 0.01 ). Conclusion ET-3/ETRB inhibits the apoptosis in A375 cells likely by activating the NF-κB/Bfl-1 anti-apoptotic pathway.
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Objective To investigate the expression and significance of the human telomeruse gene (hTERC)in the cytologic specimens of cervix for early cervical lesion screening.Methods Cytologic samples of cervix including 120 normal persons,86 CIN patients and 14 SCC patients were analyzed for aberrations of 3q26 using a commercially available two-color FISH probe.Results Positive expression rates of hTERC gene in normal,CIN Ⅰ ,CIN Ⅱ,CIN Ⅲ and SCC samples were 1.7%(2/120),2.6%(1/38),55.6%(10/118),87.5%(14/16),100.0%(14/14),100.0%(14/14),respectively.Compared with CIN Ⅰ/CIN Ⅱ group,hTERC gene positive expressions in SCC/CIN Ⅲ group were higher significantly(P=0.01),and them were also difference between CIN Ⅰ-CIN Ⅲ group and normal controls(X~2=113.52,P ＜0.01).With progression from CIN to carcinoma,polyploidization resulting in aneuploidy and high TERC gene copy numbers in SCC group ascended significantly.Among the total abnormal cells,the number of the cells percentage which hTERC amplification signals was more than 3:3 in CIN Ⅰ ,CIN Ⅱ,CIN Ⅲ and SCC groups were 6.12%,7.98%,28.07% and 33.97%,respectively.Condusion The hTERC gene is increasingly gained with progression of CIN and may appear to be another screening testmarker for early cervical cancer screening and accessory diagnosis.
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In order to investigate the expression of endothelin receptor B (ETR-B) in human malignant melanoma (MM) cells A375 and SK-mel-1 and the proliferative effects of endothelin 3 (ET3) on A375 cells, RT-PCR was applied to detect the expression of ETR-B gene in human MM cells A375 and SK-mel-1. MTT method was used to evaluate the growth enhancing effects of ET3 on A375 cell line in vitro. The results showed that ETR-B gene was expressed in both MM A375 and SK-mel-1 cells. ET3 had stronger ability to enhance the proliferation of A375 cells in vitro in a concentration-dependent manner. It was suggested that ET3/ETR-B might play an important proliferative role in MM.
Subject(s)
Cell Line, Tumor , Cell Proliferation/drug effects , Endothelin-3/pharmacology , Melanoma/metabolism , Melanoma/pathology , Receptor, Endothelin B/metabolism , Reverse Transcriptase Polymerase Chain ReactionABSTRACT
In order to compare the clinical characteristics of familial and non-familial bullous lichen planus (BLP), the archival data of 36 BLP patients with positive family history and 21 BLP patients with negative family history diagnosed according to the clinical features and histopathology were collected in our department from 1956 to 2003. The clinical features were analyzed and compared. There were significant differences between familial and non-familial BLP in age of onset, duration of disease and extension of eruption (P<0.01). It was concluded that familial BLP appeared to differ from the non-familial form in its earlier age of onset, longer duration of the disease, more extensive eruption and more tendency to involve nails. Hereditary factors may play a role in the pathogenesis of familial BLP.
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In order to compare the clinical characteristics of familial and non-familial bullous lichen planus (BLP), the archival data of 36 BLP patients with positive family history and 21 BLP patients with negative family history diagnosed according to the clinical features and histopathology were collected in our department from 1956 to 2003. The clinical features were analyzed and compared. There were significant differences between familial and non-familial BLP in age of onset, duration of disease and extension of eruption (P＜0.01). It was concluded that familial BLP appeared to differ from the non-familial form in its earlier age of onset, longer duration of the disease, more extensive eruption and more tendency to involve nails. Hereditary factors may play a role in the pathogenesis of familial BLP.
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In order to investigate the expression of endothelin receptor B (ETR-B) in human ma-lignant melanoma (MM) cells A375 and SK-mel-1 and the proliferative effects of endothelin 3 (ET3) on A375 cells, RT-PCR was applied to detect the expression of ETR-B gene in human MM cells A375 and SK-mel-1. MTT method was used to evaluate the growth enhancing effects of ET3 on A375 cell line in vitro. The results showed that ETR-B gene was expressed in both MM A375 and SK-mel-1 cells. ET3 had stronger ability to enhance the proliferation of A375 cells in vitro in a con- centration-dependent manner. It was suggested that ET3/ETR-B might play an important proliferative role in MM.
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OBJECTIVE:To study the actions of seabuckthorn oil emulsion against chronic atrophic gastritis and gastic ulcer in rats.METHODS:Chronic atrophic gastritis and gastric ulcer models were established in rats and mice and the antiulcer and anti-chronic atrophic gastritis actions of seabuckthorn oil emulsion were determined.RESULTS:Seabuckthorn oil emulsion could obviously prevent gastric ulcer and chronic atrophic gastritis induced by deoxycholic acid and stress and could decrease the index of acute gastric ulcer caused by ligature of pylorus in rats and could oppose the ulcer induced by indomethacin-ethanol significantly in mice.CONCLUSION:The results suggest that the antiulcer and anti-chronic atrophic gastritis actions of seabuckthorn oil emulsion may be related with its protection of the gastric mucosal barrier and neutralization of acid.