Rat lung microvascular endothelial cells (LMVEC) were cocultured with rat pulmonary arterial smooth muscle cells (PASMC). The cultures were divided into 5 groups: normal group (N), lipopolysaccharide (LPS) 2h (L 2), LPS 8h (L 8), LPS 16h (L 16 ), and LPS 24h (L 24 ). The activity of IL 6 in LMVEC and PASMC supernatants was determined by radio immunity method, and the expression level of IL 6 mRNA in LMVEC was detected with RT PCR. The results showed that the activity of IL 6 in LMVEC and PASMC was enhanced by LPS at L 2, reaching the higher level after 8 hour stimulation. Furthermore, the activity of IL 6 was stronger in homotypic groups than that of coculture groups. The expression of IL 6 mRNA in LMVEC was increased in L2 group, and highest in L6 group, but lowered in L12 group, though it was still stronger than that of N. It suggest that IL 6 mRNA and the activity of IL 6 in LMVEC and PASMC were enhanced in both homotypic and coculture groups by LPS. And the mRNA level and activity of IL 6 were higher in homotypic groups than that of coculture groups.

[Objective] To study the protection and mechanism of mica granules to rats stomach mucosa injury caused by non-water ethanol.[Method] Randomly divide 48 healthy SD rats into high-,middle-and low-dosed groups of mica granules,Simida group,model control group and blank control group as well,i.e.6 groups.After fasting for 24h,separately make stomach perfusion of mica granules and Simida to all rats in advance;after 3h,make stomach perfusion of non-water ethanol 1ml/100g for stomach mucosa injury;45m later,take proper blood of lower vena cava,sacrifice them for stomach,then respectively test all mucosa injury index,SOD,MDA,and GSH-Px content in serum and mucosa,observe pathological changes to evaluate mucosa injury degree and medical function.[Result] In mica groups,the SOD and GSH-Px were markedly higher than that in model control group,but MDA was lower than later(P

Objective To study the effects of electro-acupuncturing points of Yangming meridians on hemiplegia.Methods45 early hemiplegic patients were divided into Groups A,B and C(18 cases in each group).Physical therapy was performed in each group,those in Group B were electro-acupunctured at points of Yangming meridians,while those in Group C at the points according to balancing muscular tension.They were assessed with Fugl-Meyer Assessment and Ashworth Spasm Rating before and 2,4 weeks after treatment.Results2 weeks after treatment,motor function and muscular tension were significantly improved in all the groups,while the efficacy in Groups B and C was superior to Group A(P<0.05).4 weeks after treatment,the Ashworth score of lower extremity in Group B was superior to Groups C and A(P<0.05).ConclusionElectro-acupuncturing at points of Yangming meridians can improve the motor function especially muscular tension in hemiplegic patients,which may be helpful for flaccid paralysis,but adverse for spastic paralysis.

Objective To explore the effect on traditional experiment and case teaching method in regional anatomy study. Methods 80 students from 2014 medical students were randomly selected as the teaching subjects and divided into traditional group and case teaching group. The traditional group con-tained 40 students, using the traditional teaching method, while case teaching group had also 40 students with case teaching method. In the process of teaching, three clinical cases were introduced, including thesubtotal thyroidectomy thoracic outlet syndrome andpancreatic cancer. After the end of the course, the students conducted a unified questionnaire and examination. SPSS 18.0 was used for data line t test or chi square test between the two groups. Results The scores of the students in the case group in the selection questions, blanks and essay questions in the final exam were higher than those of the traditional group; The average total score of the case group was (85.69 ±11.61), while the traditional group was (73.19 ±18.66), and the difference was statistically significant (t=3.597, P=0.002). The results of the questionnaire showed that the students in the case group were higher than the traditional group, and the difference was statistically significant ( χ2=14.753, P=0.001). Conclusion The effect on regional anatomy study with case teaching method is better than the traditional teaching method, and it is a promising teaching reform for the med-ical students.

Objective To investigate the clinical features,diagnosis,treatment,and prognosis of patients with liver injury caused by Periploca forrestii Schltr.Methods A retrospective analysis was performed for the general data,clinical manifestations,and laboratory examinations of 22 patients who were diagnosed with liver injury caused by Periploca forrestii Schltr.from November 2014 to December 2015,and their clinical type was determined according to the classification criteria of drug-induced liver injury recommended by the Council for Intemational Organizations of Medical Sciences.Results There were 12 female and 10 male patients.The mean medication time ranged from 1 week to 2 months,and as for biochemical markers,there were mainly abnormalities in alanine aminotransferase (ALT),aspartate aminotransferase (AST),total bilirubin (TBil),alkaline phosphatase (ALP),and gamma-glutamyl transpeptidase (GGT).ALT and AST increased in all the patients,with mean levels of 676.68±481.11 U/L and 527.36±361.14 U/L,respectively;TBil increased to a mean level of 170.26±147.30 μmol/L in 19 patients;ALP increased to a mean level of 135.61±59.26 U/L in 13 patients;GGT increased to a mean level of 195.65±138.48 U/L in 20 patients.As for clinical typing,18 patients had liver cell injury,none had cholestasis,3 had a mixed type,and 1 had an unclassified type.One patient died and all the other patients fully recovered.Conclusion Periploca forrestii Schltr.had complex constituents,and liver injury caused by this drug is mainly liver cell injury.The pathogenesis of liver injury caused by Periploca forrestii Schltr.is presumed to be related to patients' idiosyncratic reaction to its constituents.

Objective To summarize the clinical features of 9 cases with mutations in PKHD1 gene for a better understanding of its phenotype.Methods Clinical data of nine cases with mutations in PKHD1 gene were summarized from January 2011 to December 2016 in our center,including clinical manifestations,laboratory findings,imaging data and family investigation.Next generation sequencing was used to screen 4000 genes in case 1 to 4 and whole exons in case 5 to 9.Significant variants detected by next generation sequencing were confirmed by conventional Sanger sequencing.Segregation analysis was performed using parental DNA samples.Relevant literature was reviewed.Results Among these 9 cases,5 are male,4 are female.The average age of onset was 2.6 years old (ranging from 0.5-5.2 years).Renal ultrasound revealed that all 9 cases had cysts in bilateral kidney,7 cases with enlarged kidney,1 case with normal size kidney,1 case with normal size kidney,and 1 case with bilateral renal atrophy.Two cases with renal artery stenosis,1 case with focal narrowing in left main branch and 1 case with vesico-ureteral reflux were found.Among the 9 cases,3 cases had homozygous mutations,and 6 cases had compound heterozygous mutations,including 1 nonsense mutation,1 frameshift mutation and 15 missense mutations.There were 2 cases with 3 heterozygous mutations,2 c.5935C ＞ T mutations and 2 eases with C.5869G ＞ A mutations.A total of 10 new mutations were identified.Conclusion Patients with mutations in the PKHD1 gene had normal size kidney,or even atrophic kidney.Renal artery stenosis,vesicoureteral reflux and bronchial stenosis were all first reported in patients with mutations in PKHD1 gene.The novel mutations,c.274C ＞ T,c.9059T ＞ C,c.8996delG,c.281C ＞ T,c.10424T ＞ A,c.7092T ＞ G,c.4949T ＞ C,c.5869G ＞ A,c.6197A ＞ G and c.1877A ＞ G further expanded the mutation spectrum of PKHD1 gene.

Objective: To summarize the clinical manifestation and molecular characteristics of COQ6 mutation induced nephrotic syndrome, and to evaluate efficacy of CoQ₁₀ therapy. Method: Clinical data of the case with infantile nephrotic syndrome was summarized, including clinical manifestations, laboratory findings and family investigation. The patient received CoQ₁₀ 30 mg/(kg·d) therapy. Urine protein/creatinine ratio, serum albumin and creatinine were detected to assess the efficacy of the therapy. Result: (1) The 10 months old boy was presented with nephrotic level proteinuria and hypoalbuminemia. Extra-renal manifestations included cardiovascular abnormality, motor and mental retardation and unilateral ptosis. The patient had no consanguinity. A novel homozygous p. R360W mutation in COQ6 gene was identified and confirmed by next-generation sequencing and Sanger sequencing, respectively. Family analysis showed that homozygous p. R360W mutation in COQ6 gene was inherited from his parents. Missense p. R360W mutation was damaging by prediction online PolyPhen and SIFT software. After 2 months of CoQ₁₀ complementary therapy, the patient′s urine protein/creatinine ratio declined from 7.2 to 1.3, and decreased further to 0.01 mg/mg with normal albumin level and renal function within 3 months. Nephropathy remission was maintained and growth retardation improved significantly during the last follow-up. Nevertheless, the patient manifested with sensorineural deafness at the age of 2 years. (2) There were 6 different mutations in coenzyme Q₁₀ biosynthesis monooxygenase 6 (COQ6) in 13 individuals from 7 families by homozygosity mapping in the whole world. Each mutation was linked to early-onset SRNS with sensorineural deafness. Renal biopsy revealed FSGS in 7 cases and DMS in 1 case. Other manifestations included ataxia, seizures, facial dysmorphism, nephrolithiasis and growth retardation. Four patients received CoQ₁₀ supplementation and responded to the treatment. Conclusion Renal disease caused by recessive COQ6 gene mutation was nephrotic syndrome. The patient benefited from early CoQ₁₀ complement and reached nephropathy remission.

Objective: To investigate the clinical and genetic character of Chinese children with the aarF domain containing kinase 4 (ADCK4)-associated glomerulopathy. Methods: Applying next generation sequencing to detect possible gene mutation(renal disease associated monogene was pooled as one panel) in 69 children with steroid-resistant nephrotic syndrome (SRNS) or persistent proteinuria of unknown origin. Sanger sequencing was used to confirm the significant mutations found in the children and to validate these mutation sites in their patients. Using online software (PolyPhen2, SIFT, Mutation Taster) to predict whether the detected missense mutations were disease causing or not. Collecting and analyzing clinical data of children with ADCK4-associated glomerulopathy, which included onset age, clinical manifestation, and renal pathology. Results: The ADCK4 gene mutation was detected in 8 children with a positive rate of 11.6% (8 out of 69), among which 3 patients carried homozygous c.748G>C mutation, 3 patients carried homologous c.737G>A mutation, 1 patient carried compound heterozygous mutation(c.748G>C and c.737G>A), and 1 patient carried compound heterologous mutation(c.551A>G and c.737G>A). Collectively, there were only 3 mutation sites found in total 8 patients, in which the mutation sites of c.748G>C and c.737G>A had high detection frequency in these 8 patients. These 3 mutation sites were all missense mutation which were predicted to be disease causing by online software and not reported before. The average onset age was 6.5 years (2 years-11.75 years). Four patients presented with SRNS and the other 4 presented with persistent proteinuria. All 8 patients had no extrarenal manifestation, renal biopsy revealed focal segmental glomerulosclerosis (FSGS) in most patients, among which 3 cases had gone to end-stage renal disease (ESRD) at disease onset, and 2 cases progressed to ESRD 2 and 5 years after onset respectively. Seven patients had received glucocorticoid and/or immunosuppressive drug while only one patient getting partial response. All 8 patients were treated with large amount of coenzyme Q₁₀ (15 mg·kg^-1·d^-1) after definite diagnosis of ADCK4 mutation-some patients had acquired encouraging curative effect. Conclusions ADCK4-associated glomerulopathy is not rare especially in the children with SRNS. The onset age is relatively old and the extrarenal manifestation is less common. FSGS is a main pathology type. Patients usually have no response to immunosuppressive therapy, but may benefit from addition of large amount of coenzyme Q₁₀. Some patients may only manifest with insidious proteinuria, causing the early diagnosis to be difficult, which deserves more attention. Three new missense mutations expand disease causing mutation repertoire of ADCK4 gene, among which the two sites of c.748G>C and c.737G>A may be mutation hotspot of ADCK4-associated glomerulopathy in Chinese population, and need further study.

Objective To summarize the clinical manifestation and molecular characteristics of COQ6 mutation induced nephrotic syndrome , and to evaluate efficacy of CoQ 10 therapy.Method Clinical data of the case with infantile nephrotic syndrome was summarized , including clinical manifestations , laboratory findings and family investigation .The patient received CoQ 10 30 mg/( kg? d ) therapy.Urine protein/creatinine ratio , serum albumin and creatinine were detected to assess the efficacy of the therapy . Result (1) The 10 months old boy was presented with nephrotic level proteinuria and hypoalbuminemia . Extra-renal manifestations included cardiovascular abnormality , motor and mental retardation and unilateral ptosis.The patient had no consanguinity .A novel homozygous p.R360W mutation in COQ6 gene was identified and confirmed by next-generation sequencing and Sanger sequencing , respectively.Family analysis showed that homozygous p.R360W mutation in COQ6 gene was inherited from his parents.Missense p.R360W mutation was damaging by prediction online PolyPhen and SIFT software .After 2 months of CoQ10 complementary therapy , the patient′s urine protein/creatinine ratio declined from 7.2 to 1.3, and decreased further to 0.01 mg/mg with normal albumin level and renal function within 3 months.Nephropathy remission was maintained and growth retardation improved significantly during the last follow-up.Nevertheless, the patient manifested with sensorineural deafness at the age of 2 years.(2) There were 6 different mutations in coenzyme Q10 biosynthesis monooxygenase 6 ( COQ6 ) in 13 individuals from 7 families by homozygosity mapping in the whole world.Each mutation was linked to early-onset SRNS with sensorineural deafness . Renal biopsy revealed FSGS in 7 cases and DMS in 1 case.Other manifestations included ataxia , seizures, facial dysmorphism , nephrolithiasis and growth retardation .Four patients received CoQ 10 supplementation and responded to the treatment .Conclusion Renal disease caused by recessive COQ 6 gene mutation was nephrotic syndrome .The patient benefited from early CoQ 10 complement and reached nephropathy remission .

Objective:To analyze the disease spectrum among children who were using hydroxychloroquine (HCQ), and evaluate the drug′s safety and compliance.Methods:From January 2008 to December 2019, children from Children′s Hospital of Fudan University who used HCQ were selected as subjects, the disease spectrum of HCQ was analyzed, and the drug safety and compliance were evaluated for the patients who were followed up for more than 6 months. Demographic information, diagnosis, initial dose, time of continuous use, cumulative dosage and related adverse reactions report, project and the results of eye test were collected.Results:A total of 528 cases used HCQ during the 12 years, with 156 male cases and 372 female cases, and age at initial medication was (10.5±3.2) years. Among them, 514 cases (97.3%) had rheumatic disease, 5 had pulmonary interstitial lesions and 9 had other system diseases. The top three of the rheumatic diseases were systemic lupus erythematosus (SLE) in 316 cases (316/514,61.5%), juvenile idiopathic arthritis in 69 cases (69/514,13.4%), and juvenile dermatomyositis in 56 cases (56/514,10.9%). During the same period, 397 cases were diagnosed with SLE, and the utilization rate was 79.6% (316/397), which was the highest compared with other diseases and increased year by year. Pulmonary interstitial lesions included 4 cases with SFTPC gene defect related interstitial lung disease. Of the 528 ceses who were treated with HCQ, 397 cases were included for evaluating HCQ′s safety and compliance, the initial dose was (4.2±1.0) mg/kg, duration was 29.6 (14.9, 48.8) months, the longest usage time was 127 months, the largest cumulative dosage was 566.8 g. The continuous usage duration ( Z=-3.191, P=0.001) of SLE was significantly higher than those of other diseases, as well as cumulative dosage ( Z=-5.355, P=0.001). All cases received comprehensive eye exams before medication, 354 cases (354/397, 89.2%) were followed up in the ophthalmological department, and 65.5% (232/354) of them could be reviewed regularly at least 1 time per year. One case suffered from severe skin adverse reactions when the drug was used for 32.7 months, and no other serious adverse reactions were reported. HCQ related retinopathy was not seen during the follow-up period. There were 5 cases stopped HCQ on their own. Conclusions:HCQ was widely used in rheumatic disease in children, especially in those with SLE. It was safe for long-time usage in children, and the medication compliance and the ophthalmic follow-up was good.