OBJECTIVE To analyze the dose-adjusted concentrations of Posaconazole oral suspension in patients undergoing hematopoietic stem cell transplantation （HSCT） and their influential factors. METHODS Data were collected from hospitalized HSCT patients admitted to the First Affiliated Hospital of Shandong First Medical University （Shandong Provincial Qianfoshan Hospital） from January 2021 to April whtwhm@yeah.net 2023 who took Posaconazole oral suspension for the prevention of invasive fungal disease （IFD） and received blood concentration of posaconazole. The rate of concentration attainment and clinical failure rate of posaconazole for the prevention of IFD were evaluated， and one-way and multiple linear regression analyses were performed for the influential factors of dose-adjusted concentrations （C0/D） of posaconazole. RESULTS A total of 44 patients were enrolled； the mean C0 of posaconazole in patients was （0.99±0.94） µg/mL， and 20 patients had a C0≥0.7 μg/mL， with a concentration attainment rate of 45.45% for the prevention of IFD； 13 cases were clinical failures， with a clinical failure rate of 29.55%. Of 24 patients who did not achieve C0/D of posaconazole for IFD prophylaxis， one patient was a clinical failure despite timely dose adjustment of posaconazole in seven patients； seven of the thirteen patients who did not undergo dose adjustment were clinical failures； and the remaining four patients were switched to other antifungal agents. The results of univariate analysis showed that gender， body mass index （BMI）， renal function， combined use of sodium phenytoin， omeprazole and metoclopramide had a significant effect on the C0/D of posaconazole （P＜0.05）； the results of multivariate linear regression analysis showed that gender， BMI and combined use of sodium phenytoin were the independent factors affecting the C0/D of posaconazole （P＜0.05）. CONCLUSIONS Significant individual differences are reflected in the blood concentration of Posaconazole oral suspension； gender， BMI and combined use of sodium phenytoin are independent factors affecting the C0/D of posaconazole.

Acute respiratory distress syndrome (ARDS) is a common cause of death in critically ill patients. At present, the pathogenesis of ARDS has not been clarified, which is mainly related to excessive inflammatory response, increased endothelial and epithelial permeability, and decreased alveolar surfactant. In recent years, many studies have shown that mitochondrial DNA (mtDNA) is involved in the occurrence and development of ARDS by inducing inflammation and activating immune response, and has the potential to be used as biomarkers for ARDS. This article reviews the role of mtDNA in the pathogenesis of ARDS, aiming to provide new strategies for the treatment of ARDS, and ultimately reduce the mortality of ARDS patients.
Humans ; DNA, Mitochondrial ; Mitochondria ; Inflammation ; Permeability ; Respiratory Distress Syndrome

Objective:To explore the characteristics, clinical manifestations and gene mutation types of Cornelia de Lange syndrome (CdLs), and to improve the understanding of the disease.Methods:Clinical data and gene test results of a pediatric case of CdLs diagnosed in the First Affiliated Hospital of Xinxiang Medical University in August 2019 were analyzed retrospectively.Results:A female patient with 2 years and 8 months old presented a special appearance with a low and flat nose, a wide eye distance, audition ears, a downward inclination of the mouth corner, a high arch of the jaw and a small jaw deformity, who had recurrent seizures, speech and mental retardation.The result of gene test showed the mutation of SMC1A gene c. 2923C > T, and thus the patient was diagnosed as type 2 CdLs. Conclusions:CdLs is a rare genetic metabolic disease with special facial features and physical signs.There is only one case of CdLs with gene mutation of SMC1A in China through literature review.The mutation of SMC1A gene c. 2923C>T in CdLs cases has not been reported at home and abroad, which expands the variation spectrum of the SMC1A gene.

Objective:To study the predictive value of total serum bilirubin (TSB) and the ratio of bilirubin to albumin (B/A) in neonatal acute bilirubin encephalopathy (ABE).Methods:Neonates with extremely severe hyperbilirubinemia (TSB≥425 μmol/L) treated in the Nanjing Maternal and Child Health Hospital, Maternity and Child Health Care of Guangxi Zhuang Autonomous Region, Northwest Women and Children's Hospital, Yinchuan Maternal and Child Health Hospital and Liaocheng People's Hospital from March 2018 to August 2019 were selected as prospective subjects for this study. According to the score of brain injury induced by bilirubin, the subjects were divided into ABE group and non-ABE group, and the predictive value of TSB peak and B/A for neonatal ABE were analyzed.Results:A total of 194 infants with extremely severe hyperbilirubinemia were recruited in this study, including 20 in ABE group and 174 in non-ABE group. The peak value of bilirubin ranged from 427 to 979 μmol/L. The optimal critical values of TSB peak value and B/A for ABE prediction were 530 μmol/L and 9.48, respectively. The sensitivity and specificity of ABE prediction were 85.0% and 92.8% when combined with TSB peak and B/A values.Conclusions:TSB peak combined with B/A value can effectively identify neonatal ABE. When the TSB peak value was greater than 530 μmol/L and the B/A value was greater than 9.48, the neonates had a higher risk of neonatal ABE.

Gallstone is one of the most common diseases in hepatobiliary, cholesterol gallstone is the most common type of gallstone. One of the important causes of gallstone formation is the precipitation of cholesterol crystals caused by cholesterin supersaturation. Scavenger receptor type BI (SR-BI) is a kind of multifunctional membrane receptor protein, which can mediate the selective uptake of cholesterol in liver and then affect the content of cholesterol in bile. Its role in the formation of gallstone has been initially revealed. In this paper, the relationship between the occurrence of cholesterol gallstones and scavenger receptor type B type I was summarized in order to provide new ideas for the further study of the pathogenesis of gallstone.

Objective:To investigate the clinicopathological features of pulmonary epithelioid hemangioendothelioma (PEHE).Methods:Eighteen cases of PEHE were collected from August 2011 to December 2018 at the First Affiliated Hospital of Zhengzhou University. All cases were retrospectively studied by hematoxylin and eosin staining and immunohistochemistry (IHC). The clinicopathological features were reviewed; the status of CAMTA1 and TFE3 gene was analyzed and patients′ outcome was followed up.Results:Of the 18 cases, there were 11 males and 7 females with a male to female ratio of 1.6 to 1.0. The patients′ age ranged from 36 to 68 years (mean 52 years). Twelve cases (12/18) showed a single nodule and six cases (6/18) showed multiple bilateral nodules. Seven cases (7/18) involved other organs besides lung. Seventeen (17/18) patients presented with respiratory symptoms and one patient (1/18) presented with abdominal pain. Grossly, the tumors were greyish-white nodules with indistinct borders. Microscopically the tumor cells were epithelioid and arranged in strands and nests, and cytoplasmic vacuoles were commonly noted. The stroma was myxochondroid or hyaline. By IHC, the tumor cells were positive for CD31(18/18), CD34 (16/18), ERG (18/18) and Fli-1 (18/18); CKpan was focally positive in 5 cases (5/18). TFE3 was positive in 3 cases (3/18), and Ki-67 index ranged from 5% to 30%. FISH analysis showed seventeen cases (17/18) had CAMAT1 rearrangement, one case had TFE3 rearrangement displaying a split signal. Eight patients (8/18) had surgical excision, three patients (3/18) had surgery and chemotherapy, and seven patients (7/18) had chemotherapy only. Four patients (4/18) died of the disease.Conclusions:Patients with PEHE have non-specific symptoms, and correct diagnosis depends on pathologic biopsy and the exclusion of other tumors with epithelioid morphology. Some patients with PEHE have poor prognosis, particularly in those who have multiple nodules, peripheral invasion or metastasis.

Objective To explore the effect of Omaha system nursing model on nutritional status and disease condition of patients with low body mass and chronic obstructive pulmonary disease (COPD). Methods Sixty COPD patients with low body mass who were admitted to the First Affiliated Hospital of Huzhou University from January 2015 to November 2016 were enrolled, and they were randomly divided into an Omaha system nursing model group and a routine care intervention group, with 30 cases in each group. The Delphi expert consultation method was used to construct the Omaha nursing problem system and intervention system for COPD patients with low body mass. The patients in the routine care intervention group were given routine care intervention; the patients in the Omaha system nursing model group underwent nutritional intervention according to the Omaha system nursing model. The Mini Nutritional Assessment (MNA), COPD Assessment Test (CAT) and the modified Medical Research Council Dyspnea Scale (mMRC) were used to be the evaluation system of the Omaha system nursing model, the changes of MNA, CAT and mMRC evaluation scores on admission and in 1, 3, and 6 months after discharge in the two groups were recorded, and the correlations between MNA score and CAT, mMRC scores were analyzed. Results Omaha system analysis showed that the main health problems of the Omaha system nursing model group were distributed in the environmental (17.86%), social psychological (8.93%), physiological (19.64%), and health-related behavioral (53.57%) aspects, among which health-related behaviors were mostly common. There were no statistical significant differences in the scores of CAT, mMRC, and MNA between the two groups on admission (all P > 0.05). The CAT and mMRC scores of the Omaha system nursing model group in 1, 3, and 6 months after discharge were significantly lower than those on admission (CAT score: 16.98±2.39, 16.67±2.55, 15.36±2.17 vs. 25.76±3.67; mMRC score: 2.35±0.57, 1.97±0.52, 1.49±0.51 vs. 3.07±0.55, all P < 0.05), MNA scores were significantly higher than those on admission (11.89±3.57, 13.97±3.52, 15.49±3.51 vs. 10.33±3.02, all P < 0.05), and along with the prolongation of time the decrease and increase in scores were more significant. The scores of the Omaha system nursing model group were improved more significantly in 1, 3 and 6 months after discharge than those of the routine care intervention group (CAT score: 16.98±2.39, 16.67±2.55, 15.36±2.17 vs. 23.01±2.67, 21.15±2.79, 19.06±2.61; mMRC score: 2.35±0.57, 1.97±0.52, 1.49±0.51 vs. 3.06±0.65, 3.06±0.61, 2.65±0.67;MNA score: 11.89±3.57, 13.97±3.52, 15.49±3.51 vs. 9.96±3.15, 10.06±3.09, 8.55±3.17, all P < 0.05]. Pearson correlation analyses showed that MNA score was significantly negatively correlated with CAT score (r = -0.493, P = 0.001) and with mMRC score (r = -0.594, P = 0.001) respectively. Conclusion Using the Omaha system nursing model for nutrition intervention in COPD patients with low body mass can significantly improve their nutritional status and disease condition as well as quality of life.

Objective: To identify the key pathogenic genes of leptin regulating gallbladder contraction and secretion in mice and to reveal the potential molecular mechanism by comprehensive bioinformatics. Methods: The expression profile of GSE3293 was downloaded from Gene Expression Omnibus (GEO) database. The data contained 8 samples, including 4 leptin-treated gallbladder samples and 4 saline-treated gallbladder samples. The most valuable 250 differentially expressed genes (DEGs) were obtained by grouping analysis of GEO online GEO 2 R-TOP 250 software or tools, and further analyzed by bioinformatics. The GO function and KEGG pathway enrichment of DEGs were analyzed by DAVID online software. The protein-protein interaction (PPI) network of DEGs was constructed from STRING database. Results: A total of 250 differentially expressed genes were identified from the GSE3293 dataset, of which 197 genes were up-regulated and 53 genes were down-regulated. GO analysis showed that the biological functions of DEGs were mainly concentrated on MHC class II protein complexes, plasma membrane, extracellular exosome. KEGG pathway analysis showed that these DEGs were mainly involved in tuberculosis, leishmaniasis, cell adhesion molecules, bacteriophages, infection and other signaling pathways. PPI network showed that these DEGs coded proteins interacted strongly, and the first five pairs of DEGs with the strongest correlation were screened out. Conclusions The molecular mechanism of cholelithiasis is predicted from gene level by bioinformatics analysis of function enrichment and PPI of DEGs in mouse gallbladder. However, the function of DEGs still needs a lot of clinical and molecular biological experiments to confirm.

Objective? To compare the effects of constant pressure urination and timed urination during indwelling catheterization in elderly patients with coma in the emergency intensive care unit (EICU). Methods? Totally 100 elderly patients with coma admitted in EICU, the First Affiliated Hospital of Huzhou University from January 2015 to June 2017 were selected by convenient sampling. The 49 patients admitted from January 2015 to March 2016 were included into the control group, while the 51 patients admitted between April 2016 and June 2017 were included into the treatment group. Patients in the control group were allowed urination every 3-4 hours, while patients in the treatment group were monitored for continuous bladder pressure using a

Objective To investigate the function of serum and bile leptin in the formation of gallstones.Methods Patients were divided into two groups:gallbladder cholesterol gallstone group (group A,n =58) and non-gallstone group (group B,n =33).With a body mass index of 24 kg/m2 for the standard,the group A was also divided into the group A1 (body mass index ≥24 kg/m2,n =30) and group A2 (body mass index ＜ 24 kg/m2,n =28).Group B was divided into group B1 (body mass index≥24 kg/m2,n =18) and group B2 (body mass index ＜24 kg/m2,n =15).Fasting blood samples from all study participants were assayed for total bile acid,total cholesterol,triglyceride,low density lipoprotein,high density lipoprotein,apolipoprotein AI,apolipoprotein B,lipoprotein (a).Serum leptin and bile leptin were measured using an enzyme-linked immunosorbent assay.Results A total of 58 patients with gallbladder cholesterol gallstone and 33 controls were included in the study.The serum level of leptin,total cholesterol,low density lipoprotein,apolipoprotein B and triglyceride were significantly increased,high density lipoprotein and apolipoprotein AI significantly decreased in patients with gallbladder cholesterol gallstone compared with controls(P ＜0.05).While bile leptin,total Bile acid,lipoprotein(a) were no significant difference between group A and group B (P ＞ 0.05).Serum leptin in patients with gallbladder cholesterol gallstone were significantly positively correlated with Body Mass Index(r =0.65,P =0.01),Especially when Body Mass Index ≥ 24 kg/m2 (r =0.73,P ＜ 0.01).Conclusions Changes in levels of serum leptin may affect levels of blood lipid,some lipoprotein and bile leptin,then promote the formation of cholesterol stones.