Objective: To analyze the clinical characteristics of Henoch-Schonlein purpura nephritis(HSPN).Methods: The clinical data of 90 HSPN patients were analyzed.Results: The patients with proteinuria over 1 gram per day and those with nephritic proteinuria accounted for 64.44% and 27.78%,respectively.Persistent gross hematuria often accompanied severe proteinuria even nephritic syndrome.Leucocyturia prevalence increased while the proteinuria became severe.Conclusion: Adolescent and adult HSPN have many clinical presentations with high index of gastral bleeding.The clinical presentations are getting worse with the increasing profile of proteinuria.Persistent gross hematuria and leucocyturia may reflect the severity and activity of HSPN.

Objective To explore the correlation between maternal serum total bile acid (TBA) of pregnant women with intrahepatic cholestasis of pregnancy (ICP) and varying degrees of neonatal lung injury.Methods A total of 52 cases of pregnant women with ICP and their corresponding newborns were enrolled into the ICP group from March 2014 to December 2015 in the People's Hospital of Shapingba District.Other 52 cases of pregnant women received cesarean delivery whose gestational age and birth weight of newborns were close to the ICP group and their corresponding newborns were selected as the control group.The conditions of neonatal lung injury were recorded,and the correlations of maternal serum level of TBA and exposure time of high serum level of TBA to degrees of lung injury were analyzed as well.Results The incidence rate of neonatal lung injury in the ICP group (67.3 ％) was higher than that in the control group (17.3 ％),there was statistically significant difference (P＜0.05).The degree of lung injury was positively correlated with maternal serum level of TBA and exposure time of high serum level of TBA (r=0.687,P=0.000;r=0.523,P=0.001).Conclusion The probability of neonatal lung injury of corresponding pregnant women with ICP is significantly increased,and the extent of lung injury is positively correlated with concentration of maternal serum TBA and exposure time of high serum level of TBA.

Objective To investigate the clinical characteristics of 31 patients suffering from ANCA associated vasculitis(AAV).Methods The clinical data of 31 patients with ANCA positive profiles diagnosed as AAV were analyzed,including ANCA spectrum,renal and other organs' clinical features.Results There were 16 males and 15 females,average admission age(54.19?20.00)years(18 to 84 years).Totally 27 MPA and 4 WG were diagnosed;onset symptoms of renal involvement were in 15 cases and others in 16 cases;18 patients had respiratory system involvements including 8 cases with pulmonary hemorrhage.In admission 27 MPA patients with average SCr(460.42?354.55)?mol/L,and WG group with(659.62?535.1)?mol/L.ANCA spectrum showed 24 P-ANCA cases and 7 C-ANCA cases,while ELISA method showed 25 anti-MPO cases and 6 anti-PR3 cases.Conclusion AAV has many kinds of manifestations and progresses in many variable ways.Kidney and respiratory system are most vulnerable in AAV.The treatment is very limited in advanced stage of AAV.The key to improving the outcome of AAV is early detection of ANCA and early diagnosis.

On May 12,2008,a disastrous earthquake scaled 8.0 Richter hit Wenchuan,Sichuan province in China.Treating the acute kidney injury caused by crush syndrome in survivals of the earthquake has been a big challenge to the nephrologists.In this paper,we shared our experiences on the multi-disciplinary collaboration in management of acute kidney injury caused by crush syndrome.In addition to surgical therapy for crush injury and compartment syndrome and the renal replacement therapy for acute renal injury and its related complications,the early multi-disciplinary collaboration including rehabilitation,mental health care,infection control and ICU also contributed greatly to the successful treatment of the victims of the earthquake.

Objective To explore the effects of intra-jugular vein dual lumen catheter lock heparin in different concentrations on the coagulation function,hemorrhagic tendency and catheter thrombosis risk in hemodialysis patients,and to investigate the reasonable lock heparin cuncentration. Method Ninety end stage renal disease (ESRD)patients receiving regular hemodialysis were enrolled and randomly assigned into 3 groups(n=30):Group A(pure heparin lock solution,6250 U/m1),GrouP B(medium heparin lock solution,1040 U/ml)and Group C(low hepafin lock solution,625 U/ml).The coagulation indexes were determined in short term.Complications such as bleeding,thrombosis,infection and thrombocytopenia were monitored.Results Prothrombin time(PT),actiwtted partial thromboplastin time(APTT)and thrombin time (TT)were significantly prolonged in Group A(P＜0.01);only APTT was signifieanlly prolonged in Group B:however,no significant changes were observed in Group C.Hemorrhage risk was much higher in Group A than that in Group B and C (26.7%vs 10%and 0.P＜0.05).Catheter thrombosis incidence was significantly higher in Group C than that in Group A and B(23.3%vs 0and 10%,P＜0.05).Only 1 suspected catheter related infection was found in Group C,and 2cases of moderated thrombocytopenia in Group A. Concltrsion Moderate concentration of lock heparin solution has the best balance of hemorrhagic and thrombotic risk,and should be recommended to most of regular hemodialysis patients.

OBJECTIVETo analyze the clinicopathologic features and genetic mutation in a patient diagnosed with focal segmental glomerulosclerosis (FSGS).

METHODSClinicopathologic data of the patient, who was diagnosed with primary FSGS by renal biopsy, was collected. Mutations of FSGS-related genes were screened with next-generation sequencing. Suspected pathogenic mutation was verified with Sanger sequencing.

RESULTSNext-generation sequencing detected a missense mutation (c.2215C to G, p.P739A) in exon 28 of the COL4A5 gene in the patient. The same mutation was also detected in his mother who was asymptomatic. Another missense mutation (c.2215C to T, p.P739S) in the same codon has been related with Alport syndrome.

CONCLUSIONThe c.2215C to G (p.P739A) mutation may be one of pathogenic mutations underlying FSGS. This has provided further evidence for the phenotypic heterogeneity of COL4A5 gene mutations.

Adult ; Base Sequence ; Collagen Type IV ; genetics ; Exons ; Female ; Glomerulosclerosis, Focal Segmental ; genetics ; High-Throughput Nucleotide Sequencing ; Humans ; Male ; Molecular Sequence Data ; Mutation ; Young Adult

Objective To detect variant of APOE gene in a Chinese Tibetan patient with lipoprotein glomerulopathy (LPG) confirmed by renal biopsy and to explore its pathogenesis.Methods Clinical and pathological data was collected.DNA was extracted from peripheral blood sample of the patient and subjected to PCR and Sanger sequencing.Pathogenicity of the variant was analyzed by bioinformatics software.Results Renal biopsy of the patient has confirmed the diagnosis of LPG.DNA sequencing suggested that the patient has carried a heterozygous c.527G＞C (p.R176P) variant of the APOE gene (APOE Osaka/Kurashiki).Four cases of LPG have been found to carry the same variant,and the encoded amino acid (p.176R) is highly conserved during evolution.Bioinformatic analysis using SIFT,PolyPhen2 and PANTHER software all predicted the variant to be pathogenic.Conclusion The discovery of author's patient provided further evidence for the pathogenicity of APOE Osaka/Kurashiki and,more importantly,provide new evidence for the multiracial origin of LPG-related APOE variants.

Objective:To explore the genetic basis of a Chinese patient with Bardet-Biedl syndrome (BBS).Methods:Clinical data of the patient was analyzed. Next-generation sequencing was carry out to screen pathogenic variants, and candidate variants were verified by Sanger sequencing and subjected to bioinformatic analysis based on the guidelines from the American College of Medical Genetics and Genomics.Results:Next-generation sequencing revealed that the proband has harbored two pathogenic variants of the MKKS gene (c.635C>T and c. 1664C>G) and one likely pathogenic variant of the TMEM67 gene (c.2498T>C). Sanger sequencing of the proband and her mother confirmed that the proband has harbored two compound heterozygous MKKS variants and a heterozygous TMEM67 variant. Both of the MKKS variants were previously unreported and located in a highly conserved domain and predicted to be disease-causing by bioinformatic analysis. Conclusion:The two novel MKKS/BBS6 variants probably underlay the BBS in the proband. The TMEM67 variant may have an epistatic effect on mutations of BBS-associated loci.

Objective To explore the assessment value of serum albumin(ALB) to the severity and prognosis of very low birth weight(VLBW).Methods The quality of birth weight,gestational age,serum albumin,critically ill scores and prognosis were analyzed retrospectively from October 2015 to September 2016 in this hospital.Results The mean birth weight was(1.32±0.15) kg,the overall survival rate was 80.21 ％ and the serum albumin was (27.39±3.80)g/L in 96 cases of patients.The occurrence rate of neonatal respiratory distress syndrome,sepsis,mechanical ventilation and critical cases were higher in albumin＜28 g/L group than that of albumin≥28 g/L group,and the survival rate was lower,the difference were statistically significant(P＜0.05).The area under ROC curve(AUC)showed that ALB and neonatal critical illness score(NCIS) had a predictive value for the survival of VLBW(AUC=0.848,0.917,P＜0.5),but the predicted value of ALB was less than NCIS.According to Logistic analysis,ALB was the risk factors for survival rate in VLBW.Conclusion Serum ALB has a certain predictive value for the severity and prognosis of VLBW,but subgroups of NCIS.

Objective:To investigate the application value of a new clinical probation log designed based on the subjective-objective-assessment-plan (SOAP) structured medical record.Methods:Quantitative and qualitative studies were conducted among 97 students in the autumn semester to evaluate the effect of the new clinical probation log based on the SOAP structured medical record on their academic performance and clinical thinking. SPSS 26.0 was used to perform the t-test and ANOVA. Results:The students using the new version had a significantly better score (96.29±1.38) than those using the old version (93.53±1.60) ( P<0.001), while the multivariate analysis showed that the students who first used the old version and then switched to the new version had a significantly better improvement in the score compared with those who first used the new version and then switched to the old version ( P<0.001). The qualitative interview showed that it was necessary to use handwritten internship log, and compared with the old version, the new version could better promote the ordered clinical thinking of students. The teacher comments could give feedbacks, and all the students interviewed thought that the new version held promise for clinical application. Conclusions:The clinical probation log based on the SOAP structured medical record can help to improve the effectiveness of probation and cultivate clinical thinking ability, and thus it holds promise for application in clinical probation teaching.