Objective To observe the clinical effect of recombinant human growth hormone(rhGH) in the treatment of Turner syndrome(TS).Methods The clinical data of 40 children with TS who were treated with rhGH were retrospectively analyzed.Results After treatment for 6 months,the GV,IGF-1 levels [(9.4 ± 2.7) cm/a,(433.9 ± 164.9)μg/L] were significantly higher than those before treatment [(2.5 ± 0.4)cm/a,(186.7 ± 7.33) μg/L],the differences were statistically significant(t =45.562 5,5.061 0,all P ＜ 0.05).The treatment dose of rhGH,initial treatment age had no significant relationship with GV,the GH peak before treatment had no significant correlation with GV.There was no obvious adverse reactions in the treatment process.Conclusion For children with TS,rhGH can improve the height,improve the therapeutic effect,reduce the burden of family and society.Low dose of rhGH in the treatment of TS can achieve better curative effect,the initial treatment age and growth rate in children had no obvious relationship,the insulin growth factor is an important index during the treatment,and the changes of insulin growth should be closely monitored,and adjusting the dosage of the drug in order to achieve the best therapeutic effect.

Objective To study the effect of growth hormone on serum levels of Ghrelin, leptin(LP), insulin like growth factor-1(IGF-1) and insulin-like growth factor binding protein 3(IGFBP3) in children with idiopathic short stature(ISS).Methods 86 patients of ISS who received therapy from September 2013 to September 2015 were selected.According to random number table,those patients were divided into observation group ( n=43) and the control group (n=43).Control group was treated with routine nutrition therapy, while the observation group was added with recombinant human growth hormone.The Ghrelin, LP, IGF-1, IGFBP3 levels and height pre-and post-treatment were compared.Results After treatment, the level of Ghrelin in observation group [ ( 4.85 ±0.41 ) ng/mL ] was lower than that of control group [ ( 5.63 ±0.62 ) ng/mL ] , the level of LP [ ( 6.53 ± 0.78)mg/L] was higher than that of control group[(5.49 ±0.62)mg/L](all P<0.05); the levels of IGF-1[(387.94 ±47.38)ng/mL] and IGFBP3 [(6.74 ±0.73)μg/mL]in the observation group were higher than that of the control group[(243.54 ±36.82)ng/mL,(4.85 ±0.61)μg/mL](all P<0.05); the height in the observation group[(110.17 ±8.12)cm] was higher than that of control group[(114.58 ±9.40)cm](P<0.05).Conclusion Recombinant human growth hormone is well for ISS, which can effectively improve Ghrelin, LP, promote physical growth IGF-1, IGFBP3 levels, promote growth.

Humans ; Molecular Biology ; Nephrotic Syndrome ; genetics

Objective:To observe the clinical efficacy of buccal acupuncture plus exercise therapy in treating scapulohumeral periarthritis (SP). Methods:Sixty-three SP patients were intervened by buccal acupuncture plus exercise therapy. The treatment was given once every day, 5 sessions as a course, with a 2-3 d interval between two courses. The therapeutic efficacy was observed after 3 courses of treatment. Results:Thirty-five patients were recovered, 23 cases were improved, while 5 cases showed ineffective, and the total effective rate was 92.1%. Conclusion:Buccal acupuncture plus exercise therapy can produce a significant efficacy in treating SP, and thus is worth promotion in clinic.

Objective In this study,the phenotype heterogeneity of 2 male patients with X-linked Alport syndrome from one family was analyzed and the likely reasons were discussed by reviewing the literature.Methods The clinical data at the time of diagnosis and during 5 years follow-up of 2-male patients with X-linked Alport syndrome from one family were collected.The α5 (Ⅳ) chain expression in the epidermal basement membrane was detected by indirect immunofluorescence method.COL4A5 gene mutations in skin fibroblasts and genomic DNA were detected by using reverse transcription polymerase chain reaction and direct sequencing and PCR sequencing methods from skin fibroblasts and genomic DNA,respectively.Results The diagnostic age of patient Ⅲ 1 was 14 years old.He had only microscopic hematuria,and proteinuria was negative.A negative α5 (Ⅳ) chain staining pattern was detected in his epidermal basement membrane.After 5 years follow-up without drug treatment,he was 19 years old,had persistent microscopic hematuria and normal renal function.The urinary microalbumin was 19.2-31.8 mg/L.The diagnosis age of patient Ⅱ 4 was 29 years old.The hematuria and proteinuria were found at 22 years old.He was treated with tripterygium wilfordii for 1 year.His disease progressed to an end stage of renal disease and he received hemodialysis therapy at 24 years old.He had the renal transplantation surgery at 29 years old,just 2 months before he came to hospital.And his renal function was restored.After 5 years follow-up,his urine examination and renal function were normal.Both patients had a missense mutation c.3650G ＞ A(p.G1217D) in exon 41 in COL4A5 gene.Conclusions The different phenotypes of 2 male patients from one family with X-linked Alport syndrome were reported.The most possible reason for this is somatic mosaic variants in COL4A5 gene based on literature review.Physicians should be alert to phenotype heterogeneity in male X-linked Alport syndrome despite having the same gene mutation.

Objective To investigate the efficacy of steroid spray nose treated in child with chronic nasosi- nusitis.Methods 161 cases who were examined a patient of the 5～14 years old child of a chronicⅠtype nasosi- nusitis were divided into two groups,A group 92 cases,spray with the steroid spray nose+the complex treatment.B group 69 cases,only uses the complex treatment,a month of the course of treatment,two groups of all match with the nasal sinuses to douche.Results All cases were followed-up for at least 6 months.In A group,71 cases were cured,13 cases turned for the better,8 was invalid.Total efficient rate was 91.3 %.In 13 group,40 cases were cured, 13 cases turned for the better,16 was invalid.The efficient rate was 76.8 %.The A group obviously surpassed the B group(P

Objective To explore the correlation between phenotypes in female with X-linked Alport syndrome(XLAS) and X-inactivation of different tissues.Methods Thirty-six female diagnosed as XLAS were studied,and proteinuria was taken as a marker of the severity of clinical phenotypes.X-inactivation patterns were analyzed in peripheral blood cells of 36 XLAS female and in skin fibroblasts of 12 XLAS female.The X-inactivation analysis was performed by using Hpa Ⅱ predigestion of DNA followed by polymerase chain reaction(PCR) of the highly polymorphic CAG repeat of the androgen receptor gene.Results The average X-inactivation levels of the mutant allele decreased while the degree of proteinuria increased,so there was a negative correlation between the degree of proteinuria and the X-inactivation ratios of the mutant allele in blood cells(r=-0.543,P=0.002).However,there was no correlation between the degree of proteinuria and the X-inactivation ratios of the mutant allele in skin fibroblasts(r=-0.131,P=0.701).Conclusions X-inactivation ratios might explain partially the diverse phenotypes in XLAS female patients,which suggested that the prognosis of XLAS female might be predicted via analysis of the X-inactivation in peripheral blood cells.

Objective To search for the normal value and developmental regular rule of motor norve conduction in children.Methods One hundred and fourty-nine children aged from 0 to 14 years old were divided into 7 groups in accordance with age. There were 19 pecrsons aged from 20 to 35 years old in adult group. The motor nerve conduction function of median nerve, ulnar perve, tibial nerve and peroneal nerve of every suhject was determined by nerve evoked potential meter.Results The terminal latency of action potential of every nerve is decreased along with growing up of age before 6 years old and increased after 6 years old. The conduction velocity of ulnar nerve is the fastest,then the peroneal nerve and median nerve, that of tibial nerve is the slowest. Median nerve development quicken after 3 months of birth and approch ulnar nerve at the time of 3 years old. Tibial nerye get into fast development period from 3 months to 1 years old and catch up peroneal nerve. The conduction velocity of every nerve extend to adult level in th period of 3~6 years old.Conclusion The every parameler of nervous conduction has a great difference of age in the period of child,particularly in that of infancy. therefore, the normal values should be set up in accordance with proper age groups. This study shows that it's suitable to divide groups in accordance with new-born, 3 and 6 months, 1, 3 and 6 years old. Adult criterion should be used in the children dbove 6 years old.

Collagen Type IV ; genetics ; Female ; Fetal Development ; Humans ; Nephritis, Hereditary ; diagnosis ; genetics ; Pregnancy ; Prenatal Diagnosis ; methods

Mesenchymal stem cells (MSCs)have inherent tumor-trophic migratory properties and low immunogenicity,which allow them to serve as vehicles for delivering effective,targeted therapy to tumors.MSC plays an anti-tumor role by releasing some cytokines,which can be strengthened by oncolytic virus,antiangio-genesis agent,tumor necrosis factor guided apoptosis ligand,IL,IFN and pro-drug.In addition,there are some advantages in combination treatment of MSC and others therapies.