Objective To report two cases of familial Gitelman syndrome and literature review regarding the updates of relevant genes,classification,treatment,and prognosis.Methods The clinical data of two sisters with Gitelman syndrome were retrospectively analyzed.Results Their blood pressures were within normal range.Hypokalaemic alkalosis,hypomagnesemia,and hypocalciuria were corrected almost completely after three days of intravenous magnesium and potassium infusion,spirolactone and indometacin.However,the maintenance of normal potassium was unsuccessful over one year.Conclusion Hypokalaemic alkalosis,hypomagnesemia,and hypocalciuria were normalized in Gitelman syndrome.There was some debate in regard to using PGE2 synthetase inhibitors.Tolerance of long-term medication will be the big challenge for curative effect.

Objective To evaluate the feasilibility of ultrasonic cholangiography(USC)with contrast agent SonoVue in evaluating the obstructive bile duct diseases.Methods Twenty patients with obstructive jaundice(choledocholithiasis in 4,post-operative biliary strictures in 2 and malignant tumors in 14)underwent uhrasound-guided percutaneous transhepatic cholangiodrainge drainage(PTCD).Afterward,USC with contrast agent SonoVue administrated from the PTCD tube and percutaneous transhepatic cholangiography(PTC)were carried out in each patient.By using the surgical or PTC findings as reference standard,accuracy of USC in determining the obstructive level and cause of bstructive was evaluated.Results ①The visualization of contrast agent SonoVue was successfully obtained in all 20 patients,three order of bile duct was visualized in 19 patients.The visualization of the ultrasound contrast agent persisted more than 6 min.There was no side effect occurred during the procedure and no uncomfortable complaint after the procedure.②USC determined correctly in 18 cases.Compared with surgical or PTC findings,the accuracy of USC in determining obstructive levels was 90.0%(18/20),and the accuracy in determining the obstructive cause was 85.0%(17/20).Conclusions As a new and safe technique for cholangiography,USC is comparable to PTC in displaying the intrahepatic bite duct and determining the obstructive levels in patients with obstructive jaundice.

A method of complete acid hydrolysis combined with high performance anion exchange chromatography and pulsed amperometric detection was developed for the monosaccharide composition analysis of arabinoxylan from the seeds of Plantago asiatica L. The parameters including hydrolysis methods, acid types, acid concentration, hydrolysis temperature, hydrolysis time and placement time, which would affect the hydrolysis process, were optimized. The results showed that it would have a better hydrolysis effect for polysaccharide from the seeds of Plantago asiatica L. with 2 mol/L H2 SO4 in an atmospheric oil bath at 120℃for 2 hours. However, the placement time for diluted solution of the hydrolyzed polysaccharide should be less than 6 hours. The polysaccharide was mainly composed of Arabinose (8. 89%) and Xylose (41. 52%) and Galacturonic acid (0. 73%). Glcuronic acid (3. 44%) was detected simultaneously, and there were also trace amounts of Galatose and Glucose. The results were reproducible. Other arabinoxylans from Panicummiliaceum L. shell, Avena sativa L. bran and Hordeum vulgare L. were taken for monosaccharide compositions analysis under the optimal hydrolysis conditions and the analysis results were good. This study would provide a good reference for monosaccharides composition analysis of arabinoxylans from various sources.

Objective To investigate the risk factors for preeclampsia (PE) in pregnancies complicated with chronic aplastic anemia ( CAA ) by analyzing the obstetric management and pregnancy outcome.Methods Retrospectively review the clinical data including the obstetric management,the laboratory findings and the pregnancy outcome of 41 pregnant women complicated with CAA,all of whom were hospitalized in Peking University People's Hospital from May 2002 to February 2011.Multiple logistic regression was used to explore the risk factors associated with PE.Results ( 1 ) Twenty-eight patients were diagnosed before conception while 13 were diagnosed during gestation.Eleven patients including all the 7 who were categorized as severe CAA presented with mild bleeding in the third trimester.( 2 ) The medians of white blood cell counts,hemoglobin concentrations and platelet counts were 5.0 × 109/L,66.0 g/L and 12.0 × 109/L respectively.(3) The obstetric management consisted of strict assessment, intensive surveillance and follow-up,appropriate supportive measures,timely recognition of complications,and delivery when necessary.Twenty-one patients received supportive transfusions.Ten patients developed PE,all of whom were diagnosed as severe PE( SPE).Twelve patients suffered postpartum hemorrhage,and 3 of them had blood loss more than 1000 mL All were conservatively treated in success.(4) The median gestational age of delivery was 37 weeks.Sixteen cases delivered before 37 weeks and 5 delivered before 34 weeks.Two patients developed SPE at 29 weeks and 30 weeks respectively,and both of the neonates died for severe asphyxia.The birth weight of the live neonates ranged from 1500 to 3660 g.(5) The postpartum follow-up period ranged from 6 months to 7 years.Thirty-three patients got improvement without dependence on transfusions.Four achieved no remission and still needed intermittent transfusions.Four were lost in followup.(6) Significant differences were found in the bleeding tendency,the platelet counts and the delivery weeks when comparing the patients developing PE and those without PE.No differences were found with regard to the age,the gestational age of first visit,the percentage of patients diagnosed before conception,the percentage of severe CAA,the choice of treatment,the white blood cell counts and the hemoglobin level.The Multiple logistic regression showed that the platelet count less than 10 × 109/L was an independent risk factor for CAA patients developing PE (P =0.006).Conclusions Most pregnancies complicated with CAA could achieve good maternal and fetal outcome, when intensive prenatal care and supportive management are provided SPE is the most common complication.The platelet count less than 10 × 109/L is perhaps an independent risk factor for CAA patients developing PE.

Objective:To assess the changes of left ventricular systolic function and global myocardial work in heart failure with preserved ejection fraction (HFpEF) patients by speckle tracking layer-specific strain combined with myocardial work technique and explore the diagnostic value of each parameter for HFpEF.Methods:From December 2019 to December 2020, 38 HFpEF patients (HFpEF group) and 38 healthy individuals with age- and sex-matched (control group) were enrolled consecutively in the Fourth Affiliated Hospital of Harbin Medical University. Conventional ultrasound parameters were collected. Layer-specific strain and myocardial work techniques were used to obtain the global longitudinal strain (GLS) of the left ventricular endocardium, mid-myocardium, and epicardium (GLSendo, GLSmid, GLSepi), global myocardial work index (GWI), global myocardial work efficiency (GWE), global constructive work (GCW), and global wasted work (GWW). The absolute difference of GLS(ΔGLS) between endocardium and epicardium were calculate.All parameters were analyzed statistically. ROC curves were plotted to compare the effectiveness of layer-specific strain and myocardial work parameters in predicting left ventricular systolic function impairment in HFpEF patients.Results:①Left atrial diameter, interventricular septum at end-diastole, left ventricular posterior wall at end-diastole, relative wall thickness, left ventricular mass index, and average early diastolic peak velocity (E)/early diastolic tissue velocity (e′) in HFpEF group were significantly higher compared with control subjects, while late diastolic peak velocity (A), E/A, and e′ were significantly lower (all P<0.05); E, left ventricular end-diastolic diameter, left ventricular end-diastolic volume, left ventricular end-systolic volume, fraction shortening, and left ventricular ejection fraction were not different between HFpEF and control groups (all P>0.05). ②The global longitudinal strain of the left ventricule was highest in the endocardium and lowest in the epicardium. ③Compared with control subjects, HFpEF patients demonstrated significantly decreased GLSendo, GLSmid, GLSepi, ΔGLS, GWI, GWE, GCW and increased GWW (all P<0.01). ④The ROC results showed that the area under the curve of ΔGLS and GWE for predicting left ventricular contractile function impairment in HFpEF group, was 0.884 and 0.882, respectively; The cutoff values were -5.8% and 95%; The sensitivity were 84.2% and 71.1%, and the specificity was 84.2% and 89.5%, respectively. ⑤The ROC curve of combining the two technologies showed that the maximum area under the curve of the ΔGLS in tandem with GWE was 0.944, the sensitivity was 81.6%, and the specificity was 97.4%. Conclusions:Both speckle tracking layer-specific strain and myocardial work techniques can sensitively detect left ventricular myocardial function impairment in HFpEF patients at an early stage. ΔGLS and GWE are more reliable indexes for predicting left ventricular systolic function damage in HFpEF patients. Combining the two techniques can improve the diagnostic performance in HFpEF patients.