To explore the role of the "Clinical Practice Guidelines" column and others in the Medical Joumnal of Peking Union Medical College Hospital (Med J PUMCH) in promoting diseiplinary development.

Objective To report a pedigree with tyrosinemia type Ⅱ,and to analyze its causative mutations.Methods Clinical data were obtained from a 10-year-old male proband with tyrosinemia type Ⅱ,and analyzed retrospectively.Blood and urine samples were collected from 19 persons in 3 generations of the pedigree,and the amino acid level was detected in these samples.Genomic DNA was extracted from all of the 19 family members,and mutations in the tyrosine aminotransferase (TAT) gene were detected.Results The patient developed photophobia at 2 months after birth,and the symptom was gradually aggravated after that.At the age of 6 years,ocular pain and photophobia occurred.At the age of 8 years,linear keratotic plaques occurred on his fingertips and soles of both feet,with obvious tenderness.Ophthalmic examination showed no obvious abnormalities in corneal staining or ocular fundus.Skin examination showed multiple linear keratotic plaques on the fingers and soles of both feet.The serum tyrosine level was 825.64 μmol/L,and the level of p-hydroxyphenyllactic acid in urine was 161.4 μmol/L.Genetic testing showed 2 novel mutations,including c.236G ＞ A at position 236 in exon 2 of the TAT gene causing the substitution of glycine by glutamic acid (p.Gly79Glu),and c.1141G ＞ T at position 1141 in exon 10 of the TAT gene leading to the formation of a premature termination codon instead of glutamic acid (p.Glu381*).The proband was the only patient in the family.Some members in the patrilineal family carried the mutation c.1141G ＞ T (p.Glu381*),and some in the maternal family carried the mutation c.236G ＞ A (p.Gly79Glu).Conclusion This is the first case of tyrosinemia type Ⅱ reported in the domestic population,and 2 novel heterozygous mutations were identified in the TAT gene,which may lead to the occurrence of tyrosinemia type Ⅱ in the patient.

Objective To evaluate systematic reviews or Meta-analyses related to ventilator-associated pneumonia (VAP) in closed suction system or open suction system.Methods CNKI, CBM, Cochrane Library, Web of Science, MEDLIN and Embase databases were searched for systematic reviews or meta-analyses in closed suction system and open suction system. Two reviewers independently evaluated the articles, and then extracted the data. QQAQ evaluation sheet was used to assess and cross-check reporting characteristics and methodological quality of included literature respectively, and divergence would be resolved by discussion. Results Nine systematic reviews or Meta-analysis were included, which involved three aspects: incidence rate of VAP, airway colonization bacteria and the duration of mechanical ventilation.Conclusions This revaluation of systemic evaluation provides high-quality evidence resources and provides reliable evidence support for the development of clinical practice.

Objective To explore the curative effect of insulin combined with An Er Shu in treatment ofⅡtoⅢ degree pressure sores of elderly patients with diabetes mellitus. Methods A total of 120 cases of elderly diabetes mellitus patients with Ⅱ to Ⅲ degree pressure sores were randomly divided into the control group (30 cases, conventional nursing treatment), the experimental group 1 (30 cases, insulin spray coating), the experimental group 2 (30 cases, An Er Shu brushing besmear), and the experimental group 3(30 cases, insulin combined An Er Shu). The curative effect and the healing time were observed. Results After four weeks treatment total effective rate was 60.0%(18/30) in the control group, 66.7%(20/30) in the experimental group 1, 76.7%(23/30) in the experimental group 2, 100.0%(30/30) in the experimental group 3, and there was significant difference in the 3 experimental groups comparing with the control group, the experimental group 1 and the experimental group 2, respectively (χ2=15.000, 12.000, 5.822, P<0.05 or 0.01). The healing time ofⅡdegree pressure sores was (19.03 ± 0.85) d in the control group, (18.90 ± 0.92) d in the experimental group 1, (18.43 ± 0.82) d in the experimental group 2, and (16.97 ± 1.25) d in the experimental group 3, and there was significant difference in the experimental group 3 comparing with the control group, experimental group 1 and experimental group 2, respectively (t=8.013, 7.918, 8.930, P<0.01), and in the experimental group 2 comparing with the control group (t=3.525, P<0.01). The healing time ofⅢdegree pressure sores was (24.17 ± 1.51) d in the control group, (23.63 ± 1.33) d in the experimental group 1, (23.47 ± 1.25) d in the experimental group 2, and (21.07 ± 1.46) d in the experimental group 3, and there was significant difference in the experimental group 3 comparing with the control group, experimental group 1 and experimental group 2, respectively (t=6.918, 7.048, 9.200, P<0.01). Pressure sores area reduction was (44.47 ± 37.63)%in the control group, (56.50 ± 39.64)%in the experimental group 1, (66.23 ± 37.54)%in the experimental group 2, and (96.52 ± 7.71) % in the experimental group 3, and there was significant difference in the experimental group 3 comparing with the control group, the experimental group 1 and the experimental group 2, respectively (Z=-4.274,-4.274,-3.400, P<0.01). Conclusions Insulin combined An Er Shu in treatment of pressure sores in elderly patients with diabetes mellitus can improve curative effect and shorten the healing time.

Mytilin-derived-peptide-1 (MDP-1) and mytilin-derived-peptide-2 (MDP-2) are two truncated decapeptides with reversed sequence synthesized corresponding to the residues 20-29 of mytilin-1 (GenBank Accession No. FJ973154) from M. coruscus. The objective of this study is to characterize the structural basis of these two peptides for their antimicrobial activities and functional differences, and to investigate the inhibitory mechanism of MDPs on Escherichia coli and Sarcina lutea. The structures of MDP-1 and MDP-2 in solution were determined by 1H 2D NMR methods; the antibactericidal effects of MDPs on E. coli and S. lutea were observed by transmitted electron microscopy (TEM). Both MDP-1 and MDP-2 have a well-defined loop structure stabilized by two additional disulfide bridges, which resemble the-hairpin structure of mytilin-1 model. The surface profile of MDPs' structures was characterized by protruding charged residues surrounded by hydrophobic residues. TEM analysis showed that MDPs destroyed cytoplasmic membrane and cell wall of bacteria and the interface between the cell wall and membrane was blurred. Furthermore, some holes were observed in treated bacteria, which resulted in cell death. Structural comparison between MDP-1 and MDP-2 shows that the distribution of positively charged amino acids on the loop of MDPs is topologically different significantly, which might be the reason why MDP-2 has higher activity than MDP-1. Furthermore, TEM results suggested that the bactericidal mechanisms of MDPs against E. coli and S. lutea were similar. Both MDP-1 and MDP-2 could attach to the negatively charged bacterial wall by positively charged amino acid residues and destroy the bacteria membrane in a pore-forming manner, thus cause the contents of the cells to release and eventually cell death.
Animals ; Anti-Infective Agents ; chemical synthesis ; pharmacology ; Antimicrobial Cationic Peptides ; chemical synthesis ; chemistry ; pharmacology ; Cell Wall ; drug effects ; Escherichia coli ; drug effects ; Mytilus ; chemistry ; Sarcina ; drug effects