Objective To investigate the relationship between the genotype and phenotype of erythropoietic protoporphyria (EPP) in a family.Methods Venous blood samples were collected from two patients with EPP as well as their asymptomatic parents and grandmother.PCR was performed to assess the mutation of FECH gene,and real-time quantitative PCR to detect the expression of FECH gene.Results A mutation IVS3 + 1G ＞ A was detected in the two patients and their mother.Haplotype analysis showed that both patients with photosensitivity carried the wild-type low-expressed allele IVS3-48C,while their mother,the asymptomatic carrier,harbored the normal allele IVS3-48T.As real-time PCR showed,the expression intensity of FECH gene gradually increased from patients,asymptomatic patients to normal individuals.Conclusion The difference in expression intensity of FECH gene may contribute to the variability in clinical presentation of EPP.

Objective To investigate the establishment and application effect of Evaluation Form for Cancer Pain Nursing Quality Control. Methods Nursing quality control team was founded on account of the leadership of the superintendent of nursing department and the head nurse from department of pain clinic. The team consisted of eight nurses containing the head nurse from model ward of the standardized treatment for cancer pain and nurses from department of pain clinic. According to the criteria and content of on- site assessment of cancer pain standardized treatment demonstration unit, indexes for cancer pain nursing quality was made, combining with the management practice of cancer pain nursing. The quality indexes was divided into five one- class indexes which had a total score of 100 and 20 for each including pain assessment, cancer pain treatment, patient education, nursing ability and others. Each one- class index followed with two - level index. And the table was applied to the cancer pain standardized treatment demonstration unit. Results After application of the Evaluation Form for Cancer Pain Nursing Quality Control, the following items were improved compared with those before application: accuracy of pain assessment [97.22%(70/72)vs.90.28%(65/72)], correctness of nursing record [98.61%(71/72)vs.88.89%(64/72)], satisfactory rate of pain control [97.22%(70/72)vs.84.72%(61/72)], accurate usage rate of cancer drugs [100.00%(72/72)vs.88.89%(64/72)] , understanding rate of health knowledge of patients and their family members[100.00%(72/72)vs.80.56%(58/72)], satisfactory degree of patients and their family members with nurses [100.00%(72/72)vs. 88.89%(64/72)], χ2=4.71, 8.87, 10.86, 8.87, 18.00, 8.87, P<0.05 or 0.01. Conclusions Application of Evaluation Form for Cancer Pain Nursing Quality Control in management of cancer pain can improve the nursing quality of cancer patients.

Objective To characterize the inheritance of erythropoietic protoporphyria (EPP) by detecting the mutations of ferroehelatase (FECH) gene in a Chinese family with EPP. Methods Peripheral blood samples were obtained from 4 patients and 3 unaffected individuals in a family with EPP, as well as from 50 unrelated healthy human controls. PCR was performed to amplify all the 11 exons and flanking sequence of FECH gene followed by direct sequencing. Results A splicing mutation,I.e., IVS3+1G→A, was identified in the proband as well as his symptomatic sister, cousin, grandfather and asymptomatic mother, but not in his asymptomatic father, grandmother, or unrelated healthy controls. The genotypes IVS1-23 T/C and IVS3-48 C/T were noted in the proband, his father, sister, cousin and grandfather, but absent in his mother or grandmother who carried IVS1-23 C/C and IVS3-48 T/T genotypes. Conclusions A novel splicing mutation is found in the FECH gene in a Chinese EPP family, which, together with two lowly expressed alleles IVS1-23T and IVS3-48C, is likely to be responsible for the clinical phenotype of EPP in this family.

Objective To observe the histological change of different waves in treating SD rats of the long-pulse 1064nm Nd:YAG laser and the 560～1200 nm intense pulse light,in order to provide the theory bases of non-ablative rejuvenation.Methods Two waves were used on experimental mice.The dermic thickness and the expression of collagen typesⅠand Ⅲwere detected by HE stain and immunohistochemical methods. Semiquantitative analysis was used to determine the mean of absorbance.Results Thedermal thicknesses and the mean of absorbance of collagen typesⅠandⅢin two different waves were higher than those in common control groups(P＜0.05).The effect of Nd:YAG laser groups were higher than IPL groups(P＜0.05).The expression of collagen typeⅠwas higher than that of collagen type Ⅲ(P＜0.001).Conclusion After Nd:YAG laser or IPL irradiation,the dermal thickness and collagen typesⅠandⅢof SD rats are increased.The effects of Nd:YAG laser are better than those of 560～1 200 nm IPL.The expression of collagen type Ⅲ is obviously more than that of collagen typeⅠin the early,whereas the expression of collagen typeⅠis obviously more than that of collagen type Ⅲin the later.It proves that the mechanism of dermal remodeling of non-ablative skin rejuvenation is mainly correlation with raising range and time of collagen typeⅠ.

Objective To evaluate the imaging quality of the non-contrast enhanced MR angiography of sampling perfection with application optimized contrasts using different flip angle evolutions (SPACE)in showing portal system and compared it with that of the contrast enhanced MR angiography of volumetric interpolated breath-hold examination (VIBE),and study its diagnostic ability in the detection of portosystemic and portohepatic collaterals.Methods Thirty consecutively cirrhotic patients with suspected of portosystemic and portohepatic collaterals were enrolled,and underwent SPACE followed by VIBE at 1.5 T MR scanner.The diagnostic accuracy of SPACE for the portal vein disease was evaluated by two doctors and compared it with that of VIBE.The contrast-to-noise ratio(CNR) and signal-to-noise ratio(SNR) of two MRA techniques were compared by using the Wilcoxon signed rank test.The quality assessment including scores of the portal vein segments and overall image quality were used the paired t test.Results Twenty-one patients were diagnosed as portal hypertension,including five types of portosystemic collaterals: esophageal varices (n =5),gastric fundic varices (n =11),splenic varices (n =5),paraumbilical varices (n =5) and cavemous transformation (n =2),and one patient was diagnosed as portal vein tumor thrombus.The diagnostic efficiency of SPACE was equivalent to that of VIBE.In SPACE,the SNR were 291 ± 57,301 ± 74,344 ±76 and the CNR were 231 ±59,242 ±73,286 ±76 at main portal vein,the left branch of portal vein and the right branch of portal vein,respectively.However in VIBE,the SNR were 185 ± 56,176 ± 52,182 ±52 and the CNR were 57 ±23,50 ±21,57± 19 at,respectively.Both SNR and CNR of portal vein segments in the former were better than those in the latter (t values were 7.691,7.418,7.946,15.746,13.508 and 13.880,respectively,P ＜ 0.05).There were no significant difference for the scores of displaying main,left branch and right branch portal vein and overall image quality in VIBE and SPACE (Z values were -1.496,-1.895,-1.496,-2.138,-2.324 and-1.328,respectively,P ＞ 0.05).The scores of displaying the distal branches of left and right portal vein were 2.08 ± 0.78,2.08 ± 0.78 in SPACE,and 1.75 ± 0.53,1.71 ± 0.55 in VIBE,respectively.It was better (Z =-2.138,-2.324,P ＜ 0.05) in SPACE than that in VIBE.Conclusion The SPACE has better visualization of portal vein distal branches than VIBE,and it can be applied for the diagnosis of the portal vein disease.

Inflammatory reaction dominated by defense response will arise against infection and trauma. As an important proinflammatory cytokine, high mobility group box 1 (HMGB1) is widely expressed in all nuclear cells to mediate the inflammatory response. However, the biological functions of HMGB1 in inflammation vary depending on the type of HMGB1 protein modification and the localization in the cell. HMGB1 protein will be modified as acetylation of lysine residues, methylation of lysine residues, oxidation of cysteine residues, phosphorylation of serine residues, glycosylation of asparagine residues, adenosine diphosphate-ribosylation and lactylation of the protein in the nucleus, migrate from the nucleus to the cytoplasm, and release into the extracellular compartment. Extracellular HMGB1 can bind to receptors for advanced glycation end products (RAGE) and Toll-like receptors, activate cells and regulate inflammatory responses. The authors review the research progress in regulatory mechanism of HMGB1 in inflammation response from aspects of its post-translational modifications, releases, biological roles and binding receptors, hoping to provide theoretical basis for finding the targets of inflammation intervention.

Animals ; Antibodies, Neutralizing/biosynthesis* ; Antibodies, Viral/biosynthesis* ; Body Weight/immunology* ; COVID-19/virology* ; Disease Models, Animal ; Disease Progression ; Humans ; Immunohistochemistry ; Lung/virology* ; Male ; Mesocricetus ; Nasal Cavity/virology* ; RNA, Viral/immunology* ; SARS-CoV-2/pathogenicity* ; Severity of Illness Index ; Viral Load