Objective:To explore the application effect of optimizing diet management in patients with hyperphosphatemia.Methods:Seventy-seven patients who underwent regular hemodialysis in the blood purification department of the First Affiliated Hospital of Guangxi Medical University from September 2018 to June 2019 were selected. Patients were randomly divided into control group (39 cases) and intervention group (38 cases) by the method of random number table. The control group received routine nursing, while the intervention group received optimized dietary management intervention. The blood phosphorus, blood calcium, hemoglobin, albumin, dietary phosphorus related knowledge level and phosphorus control diet compliance of the two groups before and after the intervention were compared respectively.Results:After 3 months of intervention, the scores of knowledge about food phosphorus, compliance of phosphorus control diet and total score of the intervention group were (22.00±3.92), (34.82±4.69) and (56.82±7.48) points, which were higher than (18.46±3.57), (30.54±3.52) and (49.00±6.13) points of the control group, the difference was statistically significant ( tvalues were 4.146, 4.536 and 5.022, P<0.05). After 6 months of intervention, the scores of knowledge about food phosphorus, compliance of phosphorus control diet and total score of the intervention group were (25.74±3.36), (41.63±5.27) and (67.37±7.67) points, which were higher than (20.97±3.81), (32.36±4.38) and (53.33±6.80) points of the control group, the difference was statistically significant ( tvalues were 5.815, 8.403 and 8.504, P<0.05). After 3 months of intervention, the blood phosphorus level in the intervention group was 1.81 (1.67, 2.10) mmol/L, which was lower than 2.13 (1.87, 2.32) mmol/L in the control group, the difference was statistically significant ( Zvalue was-3.237, P<0.05). After 6 months of intervention, the blood phosphorus level in the intervention group was 1.75 (1.63, 1.91) mmol/L, which was lower than that in the control group 1.90 (1.83, 2.13) mmol/L, and the difference was statistically significant ( Zvalue was-3.343, P<0.01). Conclusion:Optimizing dietary management can improve patients' knowledge level of food phosphorus and dietary compliance of phosphorus control, effectively reduce blood phosphorus level, and have no obvious effect on nutritional status.

OBJECTIVE: To study the microanatomy by endoscope-assisted via retrolabyrinthine approach. METHOD: Nineteen cadaveric heads fixed with formalin were dissected in our study. The data that endoscope could be extent and the distance between the important point were measured. By simulated the retrolabyrinthine approach, endoscope was placed to observe the nerves and vessels, the distance between nerves and the central point of the anterior edge of sigmoid sinus were measured. RESULT: The distance from the inferior margin of petrous ridge to the upper bound of endolymphatic sac was (9.93+/-1.52)mm; and from superior margin of petrous ridge to the inferior margin common bony crus was (4.64+/-0.91)mm;and from the intersection of posterior semicircular canal to the anterior wall of sigmoid was (7.85+/-1.47)mm on the left, and (5.69+/-1.68)mm on the right. The distance from inferior margin common bony crus to the anterior wall of sigmoid was (13.9+/-1.71)mm on the left, and (11.31+/-2.03)mm on the right. The trochlear nerve and abducent nerve could be observed under endoscope. The relationship between the trigeminal nerve, acoustic nerve and the vessels could be identified clearly. The distance from the central point of the anterior edge of sigmoid to the roots of the trigeminal nerve, facial nerve, vestibulocochlear nerve and glossopharyngeal nerve were (29.88+/-2.77) mm, (32.04+/-2.04) mm, (29.17+/-1.65) mm, (35.49+/-1.53) mm respectively. CONCLUSION The visual field of the cerebellopontine angle appear wider by the endoscope assisted retrolabyrinthine approach. Nerves, vessel, internal acoustic pore, jugular foramen region can be clearly seen. Some minimal invasive surgery can be done by this approach.
Adult ; Cerebellopontine Angle ; anatomy & histology ; surgery ; Ear, Inner ; surgery ; Endoscopes ; Female ; Humans ; Male

OBJECTIVE: To explore the molecular basis for a pedigree affected with Darier-White disease. METHODS: Genomic DNA was isolated from 3 patients and 1 unaffected member from the pedigree, as well as 80 healthy controls. Targeted sequence capture and next-generation sequencing were used to screen mutations of skin disease-related genes. Candidate mutations were verified by Sanger sequencing, and co-segregation analysis was carried out to confirm the pathogenicity of mutation. Conservation analysis and protein structure and function were also predicted with Bioinformatic tools. RESULTS: A heterozygous mutation c.2246G>T (p.G749V) was identified in exon 15 of ATP2A2 gene in all 3 patients from the pedigree, but not in the unaffected member or 80 healthy controls. The corresponding amino acid was highly conserved, and mutation of which can lead to structural and functional changes of the protein. CONCLUSION The c.2246G>T missense mutation of the ATP2A2 gene probably underlies the Darier-White disease in this pedigree by causing damages to the structure and function of sarcoplasmic/endoplasmic reticulum calcium ATPase 2 (SERCA2).
Darier Disease ; genetics ; Heterozygote ; Humans ; Mutation, Missense ; Pedigree ; Sarcoplasmic Reticulum Calcium-Transporting ATPases ; genetics

Objective To observe the clinical therapeutic effect of Qingxuan Jiangya decoction on patients with primary hypertension (PH) and its influence on their rennin-angiotensin-aldosterone system (RAAS). Methods A prospective study was conducted, including 178 patients with definite diagnosis of PH (Ⅰ-Ⅱ grade) who came from Guanggu district to the third Hospital of Wuhan City from April 2016 to March 2017, and they were divided into a control group and an observation group according to the method of taking medicine, each group 89 cases. The control group received 5 mg amlodipine besylate, orally once a day; the observation group on the basis of treatment in the control group, the Qingxuan Jiangya decoction (gastrodia rhizome 20 g, uncaria 20 g, ligusticum rhizome 15 g, salviae miltiorrhizae 10 g, puerarin 20 g, radix ophiopogonis 20 g, fructus lycii 25 g, chrysanthemum 10 g, root poria 15 g, poria cocos 10 g, radix glycyrrhizae 5 g) was added, a dose (400 mL) divided into two parts, one part taken twice daily, in the morning and in the evening after meals; the two groups took the treatment continuously for 4 weeks. The blood pressure dynamic changes and plasma levels of renin, angiotensin Ⅱ (Ang Ⅱ), aldosterone (ALD) and clinical efficacy were observed, and the safety of the drugs was evaluated by measuring the changes of liver and kidney functions. Results After treatment in the two groups, the daytime systolic pressure (D-SBP) and diastolic pressure (D-DBP) and nighttime SBP (N-SBP) and DBP (N-DBP), the mean of 24 hours-SBP and 24 hours-DBP were all lower than those before treatment, and the D-DBP, N-SBP and 24 hours-DBP of the observation group were significantly lower than those of control group [D-DBP (mmHg, 1 mmHg = 0.133 kPa): 78.24±5.35 vs. 81.56±6.23, N-SBP (mmHg):117.35±5.42 vs. 122.63±7.23, 24 hours-DBP (mmHg): 74.15±5.26 vs. 80.51±6.52, all P < 0.05]; after treatment, the levels of renin, Ang Ⅱ and ALD in two groups were also declined, and the degrees of decrease in the observation group were more significant than those in the control group [renin (ng/L): 5.46±0.41 vs. 7.82±0.36, ALD (ng/L):128.48±31.42 vs. 168.48±28.32, Ang Ⅱ (ng/L): 52.35±14.27 vs. 75.38±15.65, all P < 0.05]; there were no significant changes in the levels of alanine aminotransferase (ALT), aspartate transaminase (AST), urea and creatinine (Cr) before and after treatment in the two groups, showing that the treatment was safe. Conclusion Qingxuan Jiangya decoction can effectively and safely reduce the blood pressure in PH patients, and its mechanism is possibly related to its influence on RAAS.

Objective:To compare the risk assessment capability of model for end-stage liver disease (MELD), glasgow-blatchford score (GBS), and the AIMS65 scoring system for liver cirrhosis patients with esophageal and gastric variceal bleeding (EGVB).Methods:A retrospective analysis was made on data of 182 cirrhosis patients with EGVB admitted to the Department of Gastroenterology, General Hospital of Tianjin Medical University from January 1, 2015 to March 1, 2018. According to the MELD, GBS and AIMS65 scoring system, the corresponding scores of each patient were calculated to evaluate the ability of the three scoring systems to correctly classify EGVB as a " high-risk patient" . The receiver operating characteristic curve was drawn to compare the predictive value of three scoring systems for different clinical outcomes (blood transfusion, rebleeding, and death). The area under curve (AUC)>0.7 was believed to have higher accuracy.Results:The clinical outcomes of 182 patients included blood transfusion in 113 (62.1%) cases, rebleeding in 31 (17.0%) cases, and death of 11 (6.0%) cases. The MELD score was 7-25, GBS was 3-16, and AIMS65 score was 0-3. There were 4 (2.2%) patients with MELD score < 9, 139 (76.4%) patients with AIMS65 score 0-1, including 68 patients with AIMS65 score of 0 and 71 patients with AIMS65 score of 1. The AUC of MELD, GBS and AIMS65 for predicting blood transfusion was 0.514 (95% CI: 0.439-0.589), 0.681 (95% CI: 0.608-0.748), and 0.669 (95% CI: 0.596-0.737), respectively. When predicting rebleeding, the AUC of MELD, GBS and AIMS65 was 0.525 (95% CI: 0.449-0.599), 0.528 (95% CI: 0.453-0.602) and 0.580 (95% CI: 0.505-0.652), respectively. When predicting in-hospital mortality, the AUC of MELD, GBS and AIMS65 was 0.642 (95% CI: 0.567-0.711), 0.581 (95% CI: 0.505-0.653) and 0.786 (95% CI: 0.719-0.843), respectively. AIMS65 was superior to MELD ( P=0.083 6) and GBS ( P=0.047 0). Conclusion:GBS can correctly classify cirrhosis patients with EGVB as " high-risk group" , and is better than AIMS65 and MELD scoring system. MELD, GBS and AIMS65 all have poor accuracy in predicting blood transfusion and rebleeding, AIMS65 has a higher predictive value for death.

Objective To investigate the effects of Larotrectinib(Lar)on autophagy,proliferation and migration of colon cancer cells and its molecular mechanism.Methods Human colon cancer cell lines COLO 205,HCT 116 and human colonic epithelial cell line CP-H040 were treated with different concentrations of Lar(0,100,200,400,800,1600 and 3 200 nmol/L).CCK-8 assay was used to detect the cell viability of COLO 205,HCT 116 and CP-H040 cells.COLO 205 and HCT 116 cells were randomly divided into control group(Con group),Lar group,Chloroquine group(CQ group)and Lar+CQ group.Cell invasion was detected by Transwell assay.Scratch test was used to detect cell migration ability.Ki67 immunofluorescence staining was used to detect cell proliferation.Real-time quantitative polymerase chain reaction was used to detect the mRNA expression of epithelial-mesenchymal transition related markers in colon cancer cells.Autophagy was detected by adenovirus transfection experiment and transmission electron microscopy.Western blot was used to detect the expression of adenosine monophosphate-activated protein kinase/mammalian target of rapamycin(AMPK/mTOR)pathway related proteins.Results Lar significantly inhibited the viability of COLO 205 and HCT 116 cells in a concentration-dependent manner(F=355.181,403.758,all P<0.001).Compared with the Con group,the number of invasive cells,Ki67 fluorescence intensity and scratch healing rate of colon cancer cells in the Lar group were decreased,the expression of E-cadherin mRNA was increased,the expressions of Vimentin and MMP2 mRNA were decreased,the formation of autophagosomes and autophagic flow,the ratio of microtubule-associated protein light chain 3(LC3)Ⅱ/Ⅰ and p-AMPK/AMPK were increased,and the expression of p62 protein and p-mtor/mTOR ratio were decreased,with significant differences(t=4.399～54.214,all P<0.05).Compared with the Lar group,the formation of autophagosome was decreased and the expression of p62 protein was increased in the Lar+CQ group,and the difference was statistically significant(t=2.755～24.784,all P<0.05).Conclusion Lar can inhibit the proliferation and migration of colon cancer cells,and the underlying mechanism is related to activation of the AMPK/mTOR signaling pathway and thus inducts autophagy.

Objective To evaluate the effect of γ-aminobutyric acid (GABA) and its receptors upon the proliferation of CD8+T cells.Methods The splenic CD8+T cells of Balb/c mice were obtained by CD8+f cell magnetic bead sorting kit.Under the effect of CD3/CD28-activated magnetic bead,CD8+T cells were stimulated by different concentrations of GABA.5-bromo-2-deoxyuridine (BrdU) labeling and flow cytometry were performed to detect the proliferation of CD8+T cells.The expression levels of GABA-A and GABA-B receptor before and after CD8+T cell activation were compared by fluorescent quantitative real-time polymerase chain reaction (PCR).Result Flow cytometry result revealed that GABA could inhibit the proliferation of activated CD8+T cells,manifested as significant decrease in the quantity of CD152+CD8+T cells.Fluorescent quantitative real-time PCR demonstrated that GABA-A receptor subtypes α2,α6 and GABA-B receptor subtype 1a were expressed only when the CD8+T cells were activated.After CD8+T cell activation,the quantity of GABA-A receptor subtypes α3,αs,β2,β3,γ1,γ2 and θ were significantly increased,whereas the quantity of GABA-B2R and GABA-B1b did not significantly differ before and after CD8+T cell activation.Conclusions GABA can suppress the proliferation of activated CD8+T cells.The activation of CD8+T cells is regulated by GABA receptors.However,the underlying mechanism remains to be elucidated.

Objective To investigate the application value of Multi-Latex polygranular technique joint detection of kidney injury-related urinary microproteins in noninvasive diagnosis after renal transplantation. Methods Clinical data of 72 recipients undergoing renal transplantation were retrospectively analyzed. According to the level of serum creatinine (Scr), the recipients were divided into normal renal function group (group A, n=14), mild kidney injury (group B, n=37), and severe kidney injury group (group C, n=21). 20 healthy volunteers were selected as the healthy control group (HC group). The contents of urinary retinol binding protein (RBP), microalbumin (mAlb), IgG, transferrin (TRF), α₁-microglobulin (MG), and β₂-MG of subjects in each group were detected using the Multi-Latex polygranular technique. The correlation between urinary microproteins and Scr, blood urea nitrogen (BUN) was analyzed. The differences of urinary microproteins in each group were compared. And the diagnostic value of single and joint detection of urinary microproteins was evaluated. Results Six kinds of urinary microproteins in HC group and group A were significantly lower than those in group B and group C, and six kinds of urinary microproteins in group B were significantly lower than those in group C (all P < 0.01). Six kinds of urinary microproteins in renal transplant recipients were positively correlated with BUN. RBP, mAlb, α₁-MG, and β₂-MG were positively correlated with Scr. The correlations were statistically significant (P < 0.001-0.05). The diagnostic value of joint detection of urinary microproteins is better than the detection of single index, among which TRF+mAlb+RBP+α₁-MG quadruple detection had the highest diagnostic value. Conclusions Six kinds of urinary microproteins can be used as specific indicators to reflect graft renal function. The polygranular technique can simultaneously detect its contents and achieve noninvasive diagnosis. The diagnosis based on TRF+mAlb+RBP+α₁-MG quadruple detection is expected to further improve the noninvasive diagnosis system after renal transplantation.

Objective:To study the relationship between white matter injury (WMI) and brain maturity in preterm infants at full-term corrected gestational age (cGA).Methods:A retrospective study was performed in preterm infants [GA≤32 weeks or birth weight (BW) ≤1 500 g] admitted to the neonatal intensive care unit of the First Affiliated Hospital of Xi'an Jiaotong University from January 2017 to August 2018 and the Northwest Women and Children's Hospital from January 2017 to June 2017. The infants received conventional magnetic resonance imaging (MRI) at cGA 37~42 weeks. The infants were assigned into the WMI group and the control group according to the WMI scoring system, including the total maturation scores (TMS) and four sub-item scores.Results:A total of 118 premature infants were enrolled in this study (17 cases in the WMI group and 101 cases in the control group). The GA was (30.3±1.7) weeks, and BW was (1 356±268) g. The proportion of delayed TMS in the WMI group was significantly higher than the control group [58.8%(10/17) vs. 31.7%(32/101), P<0.05]. The TMS of the WMI group were significantly lower than the control group [(10.7±1.8) vs. (11.8±1.5), P<0.05]. The sub-item scores of TMS showed that the myelination [(2.8±0.6) vs. (3.1±0.4), P<0.05] and glial cell migration bands of the WMI group [(1.6±0.4) vs. (2.1±0.6), P=0.004] were significantly lower than the control group and no significant differences existed in cortical folding and involution of germinal matrix tissue scores between the two groups. Conclusions:The brain maturity of preterm infants with WMI is substantially delayed than those without WMI, including delayed myelination and delayed disappearance of glial cell migration bands.

Objective To investigate the dynamic changes of peripheral blood lymphocyte subsets and their correlation with renal function in recipients with stable graft status after renal transplantation. Methods Forty-five recipients who underwent renal transplantation for the first time and had stable graft function within postoperative 6 months were selected. The proportion and absolute value of lymphocyte subsets were detected by flow cytometry (FCM) in 180 peripheral blood samples from recipients at 15 d, 1, 3 and 6 months after renal transplantation. The dynamic changes of lymphocyte subsets with the extension of postoperative time and their correlation with serum creatinine (Scr) and blood urea nitrogen (BUN) were analyzed. Results The Scr levels did not significantly differ at 4 time points after renal transplantation (all P > 0.05). The BUN levels significantly differed between 15 d and 1 month after renal transplantation, and between 1 and 3 months after renal transplantation (P=0.002, P=0.001). The proportion of CD3⁺CD8⁺T cells, CD3⁺CD4⁺T cells, natural killer (NK) cells and CD4/CD8 ratio at postoperative 15 d significantly differed from those at 1 month after operation (P=0.009, P=0.004, P < 0.001, P=0.004). The proportion of B cells significantly differed between 15 d and 1 month, and between 1 and 3 months after renal transplantation (both P < 0.001). The absolute values of CD3⁺T cells, CD3⁺CD8⁺T cells, CD3⁺CD4⁺T cells and NK cells at postoperative 15 d significantly differed from those at 1 month after renal transplantation (P=0.001, P=0.002, P=0.003, P < 0.001). The absolute values of CD3⁺CD8⁺T cells significantly differed between 3 and 6 months after operation (P=0.015). The absolute value of B cells at 1 month after renal transplantation significantly differed from that at 3 months after renal transplantation (P=0.001). The proportion and absolute value of lymphocyte subsets were not significantly correlated with the Scr level (both P > 0.05). The proportion and absolute value of CD3⁺CD8⁺T cells and NK cells were negatively correlated with BUN (P < 0.001-0.05), whereas the proportion of CD3⁺CD4⁺T cells and B cells was positively correlated with the BUN level (P < 0.001-0.05). The absolute value of CD3⁺T cells was negatively associated with the BUN level (P < 0.05). Conclusions T cells and NK cells in the lymphocyte subsets of stable recipients raise to the stable state within 1 month after renal transplantation, whereas B cells decrease to stable state within 3 months renal transplantation. The dynamic changes of lymphocyte subsets are correlated with the BUN level.