AIM: To study the effect of Qingdaipowder Gel (QDPG) on mice specific dermatitis (AD) model and the antibacterial effect of the ethanol extract of Qingdaipowder. METHODS: AD model of mice was established by repeated skin induction with 2,4-dinitrochlorobenzene (DNCB). Fifty-six mice were randomly divided into blank group, model group, Hydrocortisone Butyrate Cream group (Hyd, 1.5 mg/cm

Polymyxin B, which is a last-line antibiotic for extensively drug-resistant Gram-negative bacterial infections, became available in China in Dec. 2017. As dose adjustments are based solely on clinical experience of risk toxicity, treatment failure, and emergence of resistance, there is an urgent clinical need to perform therapeutic drug monitoring (TDM) to optimize the use of polymyxin B. It is thus necessary to standardize operating procedures to ensure the accuracy of TDM and provide evidence for their rational use. We report a consensus on TDM guidelines for polymyxin B, as endorsed by the Infection and Chemotherapy Committee of the Shanghai Medical Association and the Therapeutic Drug Monitoring Committee of the Chinese Pharmacological Society. The consensus panel was composed of clinicians, pharmacists, and microbiologists from different provinces in China and Australia who made recommendations regarding target concentrations, sample collection, reporting, and explanation of TDM results. The guidelines provide the first-ever consensus on conducting TDM of polymyxin B, and are intended to guide optimal clinical use.
Humans ; Anti-Bacterial Agents/therapeutic use* ; China ; Drug Monitoring/methods* ; Polymyxin B ; Practice Guidelines as Topic

PURPOSE: The maximum width between the mesial and distal labial transitional line angles, described as “esthetic width” herein, could significantly influence the visual perception of the teeth and smile. This study aimed to conduct biometric research on esthetic width and to explore whether regular distribution exists in the esthetic width of human teeth. MATERIALS AND METHODS: A total of 4,264 maxillary and mandibular anterior teeth were measured using the Geomagic studio software program. The proportions of maxillary to mandibular homonymous teeth and proportions between the adjacent teeth were calculated. Bilateral symmetry and the correlation between the esthetic and mesiodistal widths were both accounted for during the measurement procedures. RESULTS: The mean esthetic widths were 6.773 ± 0.518 mm and 4.329 ± 0.331 mm for maxillary and mandibular central incisors, respectively, 5.451 ± 0.487 mm and 5.008 ± 0.351 mm for maxillary and mandibular lateral incisors, respectively, and 3.340 ± 0.353 mm and 5.958 ± 0.415 mm for maxillary and mandibular canines, respectively. Except for the mandibular canines, no significant difference in esthetic width was found among homonymous teeth from the same jaw. A high linear correlation was found between the esthetic and mesiodistal widths of the same tooth, except for the maxillary canines. Esthetic width proportions among different tooth categories showed some regular patterns, which were similar to those of the mesiodistal width. CONCLUSION Esthetic width is regularly distributed among the teeth in the Chinese population. This could provide an important reference for anterior dental restorations and dimension recovery in esthetic reconstruction of anterior teeth.

OBJECTIVE: To explore the genetic basis for a pedigree affected with Alport syndrome. METHODS: Next generation sequencing and Sanger sequencing was carried out to detect potential variant of the COL4A5 gene among members from the pedigree and 100 unrelated healthy controls. RESULTS: A novel missense c.3293G>T (p.Gly1098Val) variant was found in the COL4A5 gene among 6 affected members but not the unaffected members of the pedigree or the 100 healthy controls. According to the American College of Medical Genetics and Genomics standards and guidelines, the c.3293G>T variant was classified as pathogenic (PP1-strong+PM1+PM2+PP3+PP4). CONCLUSION By destructing the Gly-X-Y structure of its protein product, the c.3293G>T variant of the COL4A5 gene probably underlies the Alport syndrome in this pedigree. Above finding has enriched the spectrum of COL4A5 variants.

OBJECTIVE: To analyze variant of IDS gene in a pedigree affected with mucopolysaccharidosis type II (MPS II). METHODS: The proband was subjected to next generation sequencing and Sanger sequencing to identify potential variants. Suspected variant was analyzed by its co-segregation with the disease in the pedigree. Its impact on mRNA splicing was analyzed by using reverse transcription PCR (RT-PCR). RESULTS: A hemizygous IVS1-3T>G variant was found in the IDS gene in the proband. RT-PCR results revealed two abnormal cDNA fragments of 600 bp and 300 bp. The 600 bp fragment had inserted 216 nucleotides at the 3' end of intron 1, while the 300 bp fragment had lost 109 nucleotides at the 5' end of exon 2, which resulted in two truncated proteins comprising 38 and 92 amino acids, respectively, instead of the normal product (550 amino acids). The proband and his mother were respectively hemizygous and heterozygous for the variant. The same variant was not found among 100 normal controls. CONCLUSION The IVS1-3T>G variant of the IDS gene probably underlies the MPS II in this pedigree by causing reduction or elimination of the IDS protein.

Objective: To explore the characteristics of the default memory network (DMN) and working memory network (WMN) at resting state brain functional network of exercise addiction people. Methods: Twenty-nine sports addicts and 26 non-sports addicts matched by sex, age, average education level and sports dependence were screened by the exercise addiction index (EAI). Resting status brain scanning was performed with 3.0T magnetic resonance scanner.Sparse approximation coefficients independent component analysis (SACICA) model was used to analyze the independent components of brain networks. Results: Compared with the DMN template, four features were extracted, including " basic conformity" , " less frontal lobe" , " more frontal lobe" and " less occipitoparietal lobe" . Compared with the parameters of " basic conformity" , the proportion of exercise addiction group (33.3%, 9/27) was higher than that of control group (18.2%, 4/22). In the other three parameters, the proportion of exercise addiction group (37.0%, 10/27; 3.7%, 1/27; 22.2%, 6/27) was lower than those of control group (45.5%, 10/22; 22.7%, 5/22; 27.3%, 6/22). But Chi-square test showed that there was no significant difference between the two groups(all P>0.05). Compared with the WMN template, six features were extracted, including " basic conformity" , " more frontal and parietal lobes" , " more parietal lobes" , " more frontal lobes" , " less frontal lobes" and " less parietal lobes" . The percentages of the first three features in exercise addiction group (22.2%, 6/27; 7.4%, 2/27; 7.4%, 2/27) were less than those in the control group (45.5%, 10/22; 22.7%, 5/22; 9.1%, 2/22), while the percentages of the last three features in the exercise addiction group (7.4%, 2/27; 37.0%, 10/27; 14.8%, 4/27) were higher than those in the control group (4.5%, 1/22; 13.6%, 3/22; 0, 0). Chi-square test showed that there was no significant difference in all features between the two groups was statistically(P>0.05). Conclusion No significant characteristic changes are found in DMN and WMN networks of exercise addiction population.

Objective To study the use of laparoscopy in the diagnosis and treatment of obstructive infantile cholestasis.Methods The clinical data of 106 patients with obstructive infantile cholestasis from January 2012 to June 2017 were studied retrospectively.After two weeks of conservative treatments which failed to decrease the bilirubin levels significantly,these patients were subjected to laparoscopic diagnosis and treatment.Results A correct diagnosis was established in all these 106 patients by laparoscopic biliary tract exploration and cholangiography.Eighty-eight patients were diagnosed to have biliary atresia (83.0％),16 patients inspissated bile syndrome (15.1％) and 2 patients biliary hypoplasia (1.9％).Thirty-eight of the 88 biliary atresia patients gave up operative treatment after laparoscopic biliary tract exploration and cholangiography.The remaining 50 biliary atresia patients were treated with open Kasai portoenterostomy.The prognosis of the biliary atresia patients were different from the non-biliary atresia patients.On follow-up for 4 months to 5 years,all the 18 non-biliary atresia patients were in good condition and there was no recurrence of jaundice after laparoscopic cholecystostomy and biliary tract irrigation.Conclusions The laparoscopic minimally invasive technique helped to establish diagnosis and treatment in patients with obstructive infantile cholestasis.For patients with biliary atresia,this procedure gave a definitive diagnosis and offered an opportunity for surgery.For patients with inspissated bile syndrome and biliary hypoplasia patients,laparoscopic cholecystostomy and biliary tract irrigation established the correct diagnosis and reduced liver damage resulted by cholestasis.

PURPOSE: Rituximab with cyclophosphamide, doxorubicin, vincristine, and prednisone administered every 3 weeks (R-CHOP-21) is the standard care for diffuse large B-cell lymphoma (DLBCL). It is unknown whether the dose-dense R-CHOP (R-CHOP-14) could improve the outcome of the disease in Asian population. MATERIALS AND METHODS: Newly diagnosed DLBCL patients were centrally, randomly assigned (1:1) to receive R-CHOP-14 or R-CHOP-21. R-CHOP-14 was administered every 2 weeks, and R-CHOP-21 was administered every 3 weeks. Primary end point was disease-free survival (DFS). Secondary end points included overall survival (OS), progression-free survival (PFS), response rate and toxicities. RESULTS: Seven hundred and two patients were randomly assigned to receive R-CHOP-14 (n=349) or R-CHOP-21 (n=353). With a median follow-up of 45.6 months, the two groups did not differ significantly in 3-year DFS (79.6% for R-CHOP-14 vs. 83.2% for R-CHOP-21, p=0.311), 3-year OS (77.5% for R-CHOP-14 vs. 77.6% for R-CHOP-21, p=0.903), or 3-year PFS (63.2% for R-CHOP-14 vs. 66.1% for R-CHOP-21, p=0.447). Patients with an International Prognostic Index (IPI) score ≥ 2 had a poorer prognosis compared to those with an IPI score < 2. Grade 3/4 hematologic and non-hematologic toxicities were manageable and similar between R-CHOP-14 and R-CHOP-21. CONCLUSION: R-CHOP-14 did not improve the outcome of DLBCL compared to R-CHOP-21 in Asian population. With manageable and similar toxicities, both of the two regimens were suitable for Asian DLBCL patients. For high-risk patients with IPI ≥ 2, new combination regimens based on R-CHOP deserve further investigation to improve efficacy.
Asian Continental Ancestry Group ; B-Lymphocytes ; Cyclophosphamide ; Disease-Free Survival ; Doxorubicin ; Follow-Up Studies ; Humans ; Lymphoma, B-Cell ; Prednisone ; Prognosis ; Rituximab ; Vincristine

OBJECTIVETo detect mutations of the XPC (XPC complex subunit, DNA damage recognition and repair factor) gene in a family affected with xeroderma pigmentosum group C (XP-C).

METHODSThe patient was subjected to next-generation sequencing and Sanger sequencing. Suspected mutations were validated by Sanger sequencing. Effect of splicing mutation was confirmed by reverse transcription-PCR (RT-PCR).

RESULTSCompound heterozygous mutations of c.2098G to T and c.2034-7_2040del were found in the XPC gene in the proband. Among these, c.2098G to T (p.G700X) is a nonsense mutation resulting in a truncated XPC protein. C.2034-7_2040del involves the -1 position, which may alter the splice donor site of the intron 11 of XPC and result in a truncated XPC protein with loss of amino acids from 940 to 679 positions. The two mutations were not detected among 100 unrelated healthy controls.

CONCLUSIONMutations of c.2098 G to T and c.2034-7_2040del of the XPC gene may lead to abnormal XPC expression and reduction or elimination of normal XPC functions, which may underlie the disease in this family.

OBJECTIVETo assess the value of droplet digital PCR (ddPCR) for non-invasive prenatal diagnosis of single gene disease in two families.

METHODSPaternal mutation in cell-free DNA derived from the maternal blood and amniotic fluid DNA was detected by ddPCR. Suspected mutation in the amniotic fluid DNA was verified with Sanger sequencing.

RESULTSThe result of ddPCR and Sanger sequencing indicated that the fetuses have carried pathogenic mutations from the paternal side in both families.

CONCLUSIONDroplet digital PCR can accurately detect paternal mutation carried by the fetus, and it is sensitive and reliable for analyzing trace samples. This method may be applied for the diagnosis of single gene diseases caused by paternal mutation using peripheral blood sample derived from the mother.

Fathers ; Female ; Genetic Diseases, Inborn ; diagnosis ; Humans ; Male ; Maternal Serum Screening Tests ; Mutation ; Polymerase Chain Reaction ; methods ; Prenatal Diagnosis ; methods ; Sequence Analysis, DNA