Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal hematopoietic stem cell disease caused by abnormal expression of glycosylphosphatidylinositol (GPI) on the cell membrane due to mutations in the phosphatidylinositol glycan class A(PIGA) gene. It is commonly characterized by intravascular hemolysis, repeated thrombosis, and bone marrow failure, as well as multiple systemic involvement symptoms such as renal dysfunction, pulmonary hypertension, swallowing difficulties, chest pain, abdominal pain, and erectile dysfunction. Due to the rarity of PNH and its strong heterogeneity in clinical manifestations, multidisciplinary collaboration is often required for diagnosis and treatment. Peking Union Medical College Hospital, relying on the rare disease diagnosis and treatment platform, has invited multidisciplinary clinical experts to form a unified opinion on the diagnosis and treatment of PNH, and formulated the Expert Consensus of Multidisciplinary Diagnosis and Treatment for Paroxysmal Nocturnal Hemoglobinuria (2024), with the hope of promoting the standardization of PNH diagnosis and treatment.

Background::The conversion of adenosine (A) to inosine (I) through deamination is the prevailing form of RNA editing, impacting numerous nuclear and cytoplasmic transcripts across various eukaryotic species. Millions of high-confidence RNA editing sites have been identified and integrated into various RNA databases, providing a convenient platform for the rapid identification of key drivers of cancer and potential therapeutic targets. However, the available database for integration of RNA editing in hematopoietic cells and hematopoietic malignancies is still lacking.Methods::We downloaded RNA sequencing (RNA-seq) data of 29 leukemia patients and 19 healthy donors from National Center for Biotechnology Information (NCBI) Gene Expression Omnibus (GEO) database, and RNA-seq data of 12 mouse hematopoietic cell populations obtained from our previous research were also used. We performed sequence alignment, identified RNA editing sites, and obtained characteristic editing sites related to normal hematopoietic development and abnormal editing sites associated with hematologic diseases.Results::We established a new database, "REDH", represents RNA editome in hematopoietic differentiation and malignancy. REDH is a curated database of associations between RNA editome and hematopoiesis. REDH integrates 30,796 editing sites from 12 murine adult hematopoietic cell populations and systematically characterizes more than 400,000 edited events in malignant hematopoietic samples from 48 cohorts (human). Through the Differentiation, Disease, Enrichment, and knowledge modules, each A-to-I editing site is systematically integrated, including its distribution throughout the genome, its clinical information (human sample), and functional editing sites under physiological and pathological conditions. Furthermore, REDH compares the similarities and differences of editing sites between different hematologic malignancies and healthy control.Conclusions::REDH is accessible at http://www.redhdatabase.com/. This user-friendly database would aid in understanding the mechanisms of RNA editing in hematopoietic differentiation and malignancies. It provides a set of data related to the maintenance of hematopoietic homeostasis and identifying potential therapeutic targets in malignancies.

Objective To establish a gas chromatography for simultaneous determination of camphor residue and borneolum content in Qingchang Suppository. Methods Gas chromatograph method was used. The chromatographic column was Agilent capillary column(30 m×0.25 mm×0.25 µm). The column temperature was 140 ℃. The sample injection temperature was 250 ℃. The FID detector temperature was 250 ℃. Results Camphor，borneol and isoborneol content showed good linear in the extent of 0.0299~1.497(r=1.000), 0.0205~1.025(r=1.000), 0.0097~0.4830 µg (r=1.000). RSDs of precision，stability and repeatability test results were less than 2%. The recovery was 99.7%, 101.0%, 102.5%. Conclusion This method is simple and quick with accurate result, which could be used for the content determination of Borneol in Qingchang Suppository.

Objective To improve the quality of prescriptions and promote the rational drug application of Dingqing Tablets by investigating the outpatient prescriptions in a tertiary A hospital. Methods A total of 4 796 prescriptions of outpatient pharmacy patients from August 1, 2020 to August 1, 2021 were extracted from the hospital information system by the hospital information software, focusing on the analysis of indications, usage and dosage, drug interaction, etc. Results 10 departments including hematology department and geriatrics department were used Dingqing Tablets, and the irrationality was mainly manifested in the superposition of drug flavors and drug interactions. Conclusion Dingqing tablets were widely used in clinic and had remarkable curative effect. However, there are certain risks in the use of Dingqing tablets. It is necessary to add medication education and supervision to promote the safe and rational use of drugs in clinic.

Peri-implantitis, characterized by inflammation of tissues around implants and gradual loss of supporting bone tissue, has become one of the main causes for implant failure. Thoroughly removing the plaque biofilm on the implant surface is the first principle in the treatment of peri-implantitis. For this reason, various decontamination methods have been proposed, which can be divided into 2 categories: Removing biofilm and killing microorganisms according to the effect of plaque biofilm on the implant surface. However, at present, there is no decontamination method that can completely remove the plaque biofilm on the implant surface, and it lacks of clinical recommended guidelines. To understand the advantages and disadvantages, effectiveness and safety for different implant surface decontamination methods is of great significance to guide the clinical selection for peri-implantitis treatment.
Bone and Bones ; Decontamination ; Dental Implants ; Humans ; Inflammation ; Peri-Implantitis/therapy* ; Prostheses and Implants

Psoriatic arthritis (PsA) is a complicated psoriasis comorbidity with manifestations of psoriatic skin and arthritic joints, and tailoring specific treatment strategies for simultaneously delivering different drugs to different action sites in PsA remains challenging. We developed a need-based layered dissolving microneedle (MN) system loading immunosuppressant tacrolimus (TAC) and anti-inflammatory diclofenac (DIC) in different layers of MNs,

Abdominal aortic aneurysm (AAA) and atherosclerosis (AS) have considerable similarities in clinical risk factors and molecular pathogenesis. The aim of our study was to investigate the differences between AAA and AS from the perspective of metabolomics, and to explore the potential mechanisms of differential metabolites via integration analysis with transcriptomics. Plasma samples from 32 AAA and 32 AS patients were applied to characterize the metabolite profiles using untargeted liquid chromatography-mass spectrometry (LC-MS). A total of 18 remarkably different metabolites were identified, and a combination of seven metabolites could potentially serve as a biomarker to distinguish AAA and AS, with an area under the curve (AUC) of 0.93. Subsequently, we analyzed both the metabolomics and transcriptomics data and found that seven metabolites, especially 2'-deoxy-D-ribose (2dDR), were significantly correlated with differentially expressed genes. In conclusion, our study presents a comprehensive landscape of plasma metabolites in AAA and AS patients, and provides a research direction for pathogenetic mechanisms in atherosclerotic AAA.

Objective To explore the genetic pathogen of patients with non-syndromic hearing impairment and to provide prenatal diagnosis for the families of hereditary deafness. Methods Mutation screening of GJB2, SLC26A4, GJB3 and mitochondrial 12 S rRNA genes was performed in 208 patients with non-syndromic hearing impairment by gene chip. Then direct sequencing was used in 41 patients who were found one mutation of GJB2 or SLC26A4 gene. And prenatal diagnosis was carried out in two families by direct sequencing. Results Eighty-six patients (41.35％) were found at least one mutation by gene chip. Among them, 40 patients were found to carry two mutations and 46 patients were found to carry one mutation. The most frequent mutation was 235delC, which was found in 46 patients. And 12 cases were found the second mutation through direct sequencing. A total of 52 (25.00％) patients were detected two mutations. Prenatal diagnosis showed that one fetus carried compound mutations of 299-300delAT and 235delC, and another one carried heterozygous mutation of IVS7-2A>G. Conclusion Patients with non-syndromic hearing impairment can be accurately diagnosed by gene chip and Sanger sequencing. The prenatal diagnosis is primary means for high-risk fetuses.

Objective Comparative study of gastric tube implantation at different times in patients with invasive mechanical ventilation. Methods Random sampling was used to divide 160 patients who were incubated with mechanical ventilation at the hospital into two groups,There were 80 patients in each group. Two groups of patients were routinely placed gastric tube by a stationary nurse.The observation group was treated with ordinary silica gel stomach tube at early stage(immediately)after trachea cannula, the control group still used the ordinary silica gel stomach tube after the trachea cannula late (24 hours later).The one-time successful rate,the total successful rate,the operation time and the result of the two Methods were observed in the two groups. Results The observation group Placing stomach tube one-time successful rate and the total successful rate was 75% (60/80), 97.5% (78/80), significantly higher than the control group 27.5%(22/80),72.5%(58/80),the difference was statistically significant(χ2=36.12, 19.61,P<0.01).The operation time of gastric tube in observation group was(1.49 ± 0.45)min,which was significantly lower than that of control group(3.07 ± 0.32)min,the difference was statistically significant (t=25.48,P<0.01).The observation group only heard gurgling not pumped to the gastric juice of 10 cases (12.50%), only smoke into the gastric juice does not hear the gurgling in 0 cases, both in 70 cases (87.50%),both in 0 cases,the control group were 37 cases(46.25%),0 cases,40 cases(50.00%),3 cases (3.75%). There were significant differences between the 2 groups in determining the success of gastric tube placement,and the difference was statistically significant(χ2=39.36,P<0.01). Conclusions After the trachea cannula,the ordinary silica gel gastric tube was placed in the early stage(immediately),which improved the successful rate of gastric tube placement in the mechanical ventilation patients, Shortened the operation time,improved the work efficiency and alleviated the patient's pain.

The aim of this study is to prepare and characterize cardiac troponin T (cTnT) monoclonal antibodies (mAb), and further develop a chemiluminescence quantitative detection assay for cTnT. BALB/c mice were immunized with recombinant cTnT antigen, and specific mAbs were prepared using conventional hybridoma technique and screened by indirect ELISA method. To identify the epitopes, several cTnT peptide fragments were synthesized or expressed by genetic engineering. A double antibody sandwich ELISA method was used to screen the mAb pairs for cTnT detection, and the automatic chemiluminescence detection assay for cTnT was developed. In total 220 clinical specimens were used for system comparison between our assay and Roche cTnT assay; further performance characteristics was evaluated by testing 238 clinical samples and 784 physical examination samples. We successfully screened 33 strains of hybridoms against cTnT, and the mAbs' epitopes were identified. Mab E16H8 and C8G11 with a detection limit of 10 pg/mL cTnT antigen were selected to develop the full automatic chemiluminescence quantitative assay. The correlation coefficient of our reagent with Roche's was 0.959 9, with a coincidence rate of 95%. The assay presented a sensitivity of 97.5%, and a specificity of 99.15% in detection of clinical samples. The cTnT concentration was less than 0.080 6 ng/mL in 99% of general population, which agrees with the definition of WHO on patients with acute myocardial infarction (AMI). In summary, we developed monoclonal antibodies against predominant epitopes for diagnostics of cTnT, and an automatic tubular chemiluminescence quantitative detection assay was further developed, which presents a high coincidence rate with Roche's.
Antibodies, Monoclonal ; immunology ; Enzyme-Linked Immunosorbent Assay ; Epitopes ; immunology ; Humans ; Hybridomas ; Luminescent Measurements ; Myocardial Infarction ; Peptide Fragments ; Sensitivity and Specificity ; Troponin T ; immunology