Rifabutin(RBT) is a rifamycin derivative,like rifampicin(RIF),registered for the prophylaxis and treatment of mycobacterium avium complex (MAC)in patients with AIDS by FDA in 1992.Subsequently,the drug was approved by many other countries.But now,it is used not only in the prophlaxis and treatment of mycobacterium avium complex but also in the treatment of pulmonary tuberculosis and Helicobacter pylori.For its high lipophilic characteristic and weak inducing properties compare to other rifamycin derivative,it can be applied in treatment with many diseases successfully,especially when combine with other antibiotics,and can solve the problem of traditional antibiotics resistance and increase the clinical safety of combined medical treatment.This paper just shows the progress of clinical pharmacological study and related aspects on rifabutin in order to instruct prescription.

Objective To assess effects of stress dynamic CT myocardial perfusion imaging (CT-MPI) combined with coronary CT angiography (CCTA) on the diagnosis of myocardial perfusion defects in coronary artery disease (CAD). Methods Patients with CAD diagnosed by CCTA underwent ATP stress CT-MPI examination. Single-photon emission computed tomography (SPECT) myocardial perfusion imaging (SPECT-MPI) was performed within one week and set as the reference standard. CT-MPI results were qualitatively analyzed, and myocardial blood flow (MBF), myocardial blood volume (MBV) as well as time to peak (TTP) were quantified according to CT-MPI. Effects of CCTA, CT-MPI, and CT-MPI combined with CCTA on predicting myocardial perfusion defects were assessed in comparison with NMPI. Results Thirty patients [(54.8±8.4)years] were enrolled in our study, 20 were men (68%). MBF [(79.3±18.0) versus (135.1± 35.2) ml·100 ml-1·min-1] and MBV [(8.9±2.9) versus (13.8±8.9) ml/100 ml] were significantly decreased in hypoperfused segments compared with normal segments, while TTP was increased in hypoperfused segments [(13.9 ± 2.5)s] compared with normal segments [(9.1 ± 2.1)s] (t=0.302, 0.866 and 0.024 respectively, all P values<0.01). The sensitivity, specificity of CT-MPI for identifying segments with perfusion defects were 91.3%(147/161), 84.6%(281/332), respectively. On a per-vessel basis, the area under the receiver operating characteristic curve for predicting myocardial perfusion defects were 0.635(95%CI:0.517—0.753) for CCTA, 0.709(95%CI:0.599—0.819)for CT-MPI, and 0.837(95%CI:0.749—0.925)for CT-MPI combined with CCTA, respectively. Conclusions The performance of stress dynamic CT-MPI in the diagnosis of myocardial perfusion defects in CAD was good. One-stop examination of CT-MPI combined with CCTA improves the diagnostic accuracy for identifying flow-obstructing stenosis.

Objective To assess the diagnostic value of 3.0T magnetic resonance imaging (MRI) and ultrasonic cardiography in women with high altitude pulmonary arterial hypertension (PAH). Methods Seventy-six women with high altitude PAH treated at our hospital were divided into either an MRI group (group A) or an ultrasonic cardiography group (group B), with 38 cases in each group. Fifty healthy women from high altitude areas were enrolled as a control group (group C). Group A underwent MRI examination alone, Group B underwent ultrasonic cardiography examination alone, and Group C underwent concomitant MRI and ultrasonic cardiography examinations. Diagnosis accuracy and diagnostic results were compared among different groups. Results Compared with group B, diagnosis accuracy significantly rose in group A (P < 0.05). MRI showed that except right ventricular end diastolic transverse diameter,left atrial diameter, aortic diameter, and right ventricular end systolic transverse diameter, other indexes differed significantly between groups A and C (P < 0.05). Ultrasonic cardiography showed that the SPAP of group B was (44.5 ± 8.6) mmHg. Right ventricular outflow tract, pulmonary artery, right ventricular inner diameter, right atrial inner diameter, right ventricle anterior wall, interventricular septal thickness, right ventricular Tei index, and right ventricular ejection fraction differed significantly between groups B and C (P<0.05), although there was no significant difference in LVEF or LV-Tei between the two groups (P>0.05). Conclusion Both MRI and ultrasonic cardiography can diagnoses high altitude PAH in women effectively. MRI can accurately evaluate the heart structure and function in women with high altitude PAH, representing a more efficient diagnostic method.

Objective To analyze the differences of coronary artery anomalies between Han and Tibetan nationality living in middle and high altitude.Methods A total of 7 028 adults living in the Qinghai Plateau (1 800-7 720 m altitude),who underwent coronary CT angiography in Qinghai Cardiocerebro-vascular Disease Special Hospital between 2010 to 2015,were included in this study.There were 6 391 cases of the Han nationality and 637 cases of the Tibetan nationality.The differences of coronary artery anomalies between Han and Tibetan nationality were analyzed retrospectively.Results (1) Incidence of coronary artery anomalies was lower in Han nationality than in the Tibetan nationality (1.596％ (102/6 391) vs.4.239％ (27/637),P ＜0.001).(2) There was 64.7％ (66/102) male residents with coronary artery anomalies in Han nationality,and 74.1％ (20/27) male residents with coronary artery anomalies in Tibetan nationality (P =0.359).(3) Left side coronary artery anomalies in Han nationality was similar as in Tibetan nationality (64.4％ (67/104) vs.55.6％ (15/27),P =0.396).(4) Incidence of benign coronary artery anomalies was significantly lower in Han nationality than in Tibetan nationality (0.720％ (46/6 391) vs.2.200％ (14/637),P ＜ 0.001).Incidence of potentially dangerous coronary artery anomalies was also significantly lower in Han nationality than in Tibetan nationality (0.876％ (56/6 391) vs.2.041％ (13/637),P =0.004).(5)Ten kinds of coronary artery anomalies were found in this study.There were significant differences between Han and Tibetan nationality in left coronary artery originated from right coronary sinus(0.046％ (3/6 391) vs.0.471％ (3/637),P =0.012),in left circumflex branch originated from right coronary sinus (0.046％ (3/6 391) vs.0.471％ (3/637),P =0.012),and opening of right coronary artery in left coronary sinus or left anterior descending (0.704％ (45/6 391) vs.1.570％ (10/637),P =0.018).Conclusion The incidences of coronary artery anomalies and benign coronary artery anomalies were significantly lower in Han nationality residents than that of the in Tibetan nationality residents living in middle and high altitude.

OBJECTIVE: To explore the molecular basis for a Chinese family affected with neurofibromatosis type I. METHODS: Peripheral blood samples were collected from the proband and his parents. Potential mutations of NF1 gene were screened by PCR and Sanger sequencing. Pathogenicity of candidate mutations was analyzed using Polyphen-2 and Provean software. RESULTS: Two mutations of the NF1 gene, including c.702G>A (synonymous mutation) and c.1733T>G (missense mutation), were discovered in the proband. Neither mutation was found in his parents and 50 healthy controls. Bioinformatics analysis indicated that the c.1733T>G mutation (p.Leu578Arg) was probably damaging. The affected codon L578 is highly conserved across various species. CONCLUSION The c.1733T>C mutation of the NF1 gene probably underlies the neurofibromatosis type I in this family.
Asian Continental Ancestry Group ; Genes, Neurofibromatosis 1 ; Humans ; Mutation ; Neurofibromatosis 1 ; genetics ; Neurofibromin 1 ; genetics ; Pedigree

Objective To explore the predictive value of artificial intelligence(AI)plaque quantitative analysis combined with CT derived fractional flow reserve(CT-FFR)and pericoronary fat attenuation index(FAI)in coronary heart disease(CHD)in Qinghai.Methods A total of 118 suspected CHD patients were selected,and were divided into a stenosis group(n=76)and a non-stenosis group(n=42)based on whether their vascular stenosis rate was＞50％.The plaque volume,load,CT-FFR value,and pericoronary FAI of the two groups were measured and compared,and their predictive value in CHD was further analyzed.Results The plaque total volume,calcified and non-calcified plaque volume,lipid plaque volume,and corresponding volume load of the stenosis group were significantly higher than those of the non-stenosis group(P＜0.05),the percentage of napkin ring sign and pericoronary FAI were significantly higher than those of the non-stenosis group,and the CT-FFR was significantly lower than that of the non-stenosis group(P＜0.05).Logistic regression analysis found that the volume load of calcified and lipid plaques,napkin ring sign,CT-FFR,and pericoronary FAI were independent risk factors for CHD(P＜0.05).The combination of AI plaque quantitative analysis,CT-FFR,and pericoronary FAI had high sensitivity and specificity,with high value in CHD diagnosis.Conclusion The combination of AI plaque quantitative analysis,CT-FFR,and pericoronary FAI has high value in the diagnosis of CHD,and may be worthy of clinical promotion application.

The human Shwachman-Diamond syndrome (SDS) is an autosomal recessive disease caused by mutations in a highly conserved ribosome assembly factor SBDS. The functional role of SBDS is to cooperate with another assembly factor, elongation factor 1-like (Efl1), to promote the release of eukaryotic initiation factor 6 (eIF6) from the late-stage cytoplasmic 60S precursors. In the present work, we characterized, both biochemically and structurally, the interaction between the 60S subunit and SBDS protein (Sdo1p) from yeast. Our data show that Sdo1p interacts tightly with the mature 60S subunit in vitro through its domain I and II, and is capable of bridging two 60S subunits to form a stable 2:2 dimer. Structural analysis indicates that Sdo1p bind to the ribosomal P-site, in the proximity of uL16 and uL5, and with direct contact to H69 and H38. The dynamic nature of Sdo1p on the 60S subunit, together with its strategic binding position, suggests a surveillance role of Sdo1p in monitoring the conformational maturation of the ribosomal P-site. Altogether, our data support a conformational signal-relay cascade during late-stage 60S maturation, involving uL16, Sdo1p, and Efl1p, which interrogates the functional P-site to control the departure of the anti-association factor eIF6.
Crystallography, X-Ray ; GTP Phosphohydrolases ; chemistry ; metabolism ; Humans ; Protein Domains ; Ribosome Subunits, Large, Eukaryotic ; chemistry ; metabolism ; Saccharomyces cerevisiae ; chemistry ; metabolism ; Saccharomyces cerevisiae Proteins ; chemistry ; metabolism