A 20-year-old male patient presented to the Department of Dermatology of Peking Union Medical College Hospital with complaints of an 8-year history of facial scarring, swelling of the lower limbs, and a 4-year history of scalp thickening. Physical examination showed thickening furrowing wrinkling of the skin on the face and behind the ears, ciliary body hirsutism, blepharoptosis, and cutis verticis gyrate. Both lower limbs were swollen, especially the knees and ankles. The skin of the palms and soles of the feet was keratinized and thickened. Laboratory examination using bone and joint X-ray showed periostosis of the proximal middle phalanges and metacarpals of both hands, distal ulna and radius, tibia and fibula, distal femurs, and metatarsals.Genetic testing revealed two variants in SLCO2A1, c.1658T > A and c.96+4A > C.Through multidisciplinary consultation, we confirmed the diagnosis of pachydermoperiostosis.

ObjectiveTo construct an outcome set for clinical evaluation of traditional Chinese medicine （TCM） for chronic pulmonary heart disease， and to provide consensus outcomes for the evaluation of the clinical effectiveness of TCM for chronic pulmonary heart disease. MethodsWe searched randomised controlled trials of TCM for chronic pulmonary heart disease on China National Knowledge Infrastructure （CNKI）， Wanfang Data Knowledge Service Platform （WF）， VIP Chinese Science Journals Database （VIP）， Chinese Biomedical Literature Service Database （SinoMed）， PubMed， Cochrane Library， and Embase. We also searched Chinese Clinical Trial Registry Platform and the U.S. Clinical Trial Registry database to obtain the outcome indicators reported in the clinical research protocols of TCM for chronic pulmonary heart disease. The outcome indicators were also collected through semi-structured interviews of clinicians and patients. Then integrated the outcome indicators collected by the above methods to construct the indicator pool. Through two rounds of Delphi surveys and a consensus conference， the core outcome set for clinical evaluation of TCM for chronic pulmonary heart disease was determined. ResultsAfter screening， there were 1313 literature meeting the criteria， and 595 outcome indicators were extracted， then combined with the outcomes from semi-structured interviews which clinicians and patients concerned， finally an indicator item pool containing 369 outcome indicators were formed. After the initial screening of indicators in the pool by the steering committee， 58 indicators were established into the initial list of indicator entries. In the first round of Delphi survey， the expert coordination coefficient for the results was 0.401， and the Cronbach coefficient was 0.989. A total of 35 indicators that did not meet the criteria ［＜70% of the participants rated the outcome as 7~9 （critical） and the mean of the expert ratings ＜7］ were deleted， and 23 were retained， with 7 new indicators added that were open to supplementation by the experts， resulting in a total of 30 indicators that were included in the second round of Delphi survey. In the second round of Delphi survey， the expert coordination coefficient was 0.303， and the Cronbach coefficient was 0.974， with a total of 7 indicators that did not meet the criteria being deleted， and 21 indicators being retained for the consensus conference. After the consensus meeting， the core outcome set for clinical evaluation of chronic pulmonary heart disease in two major categories， acute exacerbation stage and stable stage， was finally determined， in which there were four indicators in acute exacerbation stage： N-terminal B-type natriuretic peptide precursor （NT-proBNP）， blood qi analysis， all-cause mortality rate， and complication rate； and there were eight indicators in the stable stage： pulmonary function index， six-minute walk test distance， New York cardiac function classification， all-cause mortality rate， re-hospitalisation rate， Chronic Obstructive Pulmonary Disease Assessment Test （CAT） score， Short Form 36 Health Survey （SF-36）， and TCM syndrome score. ConclusionThe core outcome sets of TCM clinical evaluation in the acute exacerbation stage and stable stage are constructed， which is helpful to improve the practicability， comparability and transparency of TCM clinical research results in pulmonary heart disease.

Objective This study aimed to explore the prognostic value of 18F-FDG PET/CT Metabolic and Heterogeneity Parameters Combined with Clinical Features Before Definitive Chemoradiotherapy(D-CRT)in predicting the prognosis of esophageal squamous cell carcinoma(ESCC)Patients.Methods A retrospective analysis was conducted on clinical data from 106 patients with ESCC who received D-CRT at the first affiliated Hospital of University of Science and Technology of China between January 2017 and December 2021.All patients underwent 18F-FDG PET/CT examination before the treatment.The primary tumor′s metabolic and heterogeneity parameters were obtained through data processing.All patients were followed up for overall survival.The Kaplan-Meier method and Cox proportional hazards models were used to analyze the association between clinical features,tumor metabo-lism and heterogeneity parameters and patient prognosis.Results The 1-and 1.5-year overall survival rates of all patients were 77.4%and 51.9%.The median survival time was 20 months.Univariate analysis showed that N stage,M stage,metabolic tumor volume,total lesion glycolysis,heterogeneity index-2(HI-2),and coefficient of variation with a threshold of 40%maximum standard uptake value(CV40%)were correlated with the prognosis of ESCC(all P<0.05).Multivariate analysis showed that N stage and CV40%were independent predictors of prognosis in patients with ESCC(P = 0.039 and P<0.001,respectively).Conclusion N stage and tumor metabolic heterogeneity parameter CV40%,which offering a degree of predictive value,are closely related to the prognosis of patients with ESCC treated with D-CRT.

Objective:To study the current status of longitudinal extrauterine growth restriction (EUGR) in extremely preterm infants (EPIs) and to develop a prediction model based on clinical data from multiple NICUs.Methods:From January 2017 to December 2018, EPIs admitted to 32 NICUs in North China were retrospectively studied. Their general conditions, nutritional support, complications during hospitalization and weight changes were reviewed. Weight loss between birth and discharge > 1SD was defined as longitudinal EUGR. The EPIs were assigned into longitudinal EUGR group and non-EUGR group and their nutritional support and weight changes were compared. The EPIs were randomly assigned into the training dataset and the validation dataset with a ratio of 7∶3. Univariate Cox regression analysis and multiple regression analysis were used in the training dataset to select the independent predictive factors. The best-fitting Nomogram model predicting longitudinal EUGR was established based on Akaike Information Criterion. The model was evaluated for discrimination efficacy, calibration and clinical decision curve analysis.Results:A total of 436 EPIs were included in this study, with a mean gestational age of (26.9±0.9) weeks and a birth weight of (989±171) g. The incidence of longitudinal EUGR was 82.3%(359/436). Seven variables (birth weight Z-score, weight loss, weight growth velocity, the proportion of breast milk ≥75% within 3 d before discharge, invasive mechanical ventilation ≥7 d, maternal antenatal corticosteroids use and bronchopulmonary dysplasia) were selected to establish the prediction model. The area under the receiver operating characteristic curve of the training dataset and the validation dataset were 0.870 (95% CI 0.820-0.920) and 0.879 (95% CI 0.815-0.942), suggesting good discrimination efficacy. The calibration curve indicated a good fit of the model ( P>0.05). The decision curve analysis showed positive net benefits at all thresholds. Conclusions:Currently, EPIs have a high incidence of longitudinal EUGR. The prediction model is helpful for early identification and intervention for EPIs with higher risks of longitudinal EUGR. It is necessary to expand the sample size and conduct prospective studies to optimize and validate the prediction model in the future.

Objective:To report the results of national surveillance on the distribution and antimicrobial resistance profile of clinical Gram-negative bacteria isolates from bloodstream infections in China in 2022.Methods:The clinical isolates of Gram-negative bacteria from blood cultures in member hospitals of national bloodstream infection Bacterial Resistant Investigation Collaborative System（BRICS）were collected during January 2022 to December 2022. Antibiotic susceptibility tests were conducted by agar dilution or broth dilution methods recommended by Clinical and Laboratory Standards Institute（CLSI）. WHONET 5.6 and SPSS 25.0 software were used to analyze the data.Results:During the study period，9 035 strains of Gram-negative bacteria were collected from 51 hospitals，of which 7 895（87.4%）were Enterobacteriaceae and 1 140（12.6%）were non-fermenting bacteria. The top 5 bacterial species were Escherichia coli（ n=4 510，49.9%）， Klebsiella pneumoniae（ n=2 340，25.9%）， Pseudomonas aeruginosa（ n=534，5.9%）， Acinetobacter baumannii complex（ n=405，4.5%）and Enterobacter cloacae（ n=327，3.6%）. The ESBLs-producing rates in Escherichia coli， Klebsiella pneumoniae and Proteus spp. were 47.1%（2 095/4 452），21.0%（427/2 033）and 41.1%（58/141），respectively. The prevalence of carbapenem-resistant Escherichia coli（CREC）and carbapenem-resistant Klebsiella pneumoniae（CRKP）were 1.3%（58/4 510）and 13.1%（307/2 340）；62.1%（36/58）and 9.8%（30/307）of CREC and CRKP were resistant to ceftazidime/avibactam combination，respectively. The prevalence of carbapenem-resistant Acinetobacter baumannii（CRAB）complex was 59.5%（241/405），while less than 5% of Acinetobacter baumannii complex was resistant to tigecycline and polymyxin B. The prevalence of carbapenem-resistant Pseudomonas aeruginosa（CRPA）was 18.4%（98/534）. There were differences in the composition ratio of Gram-negative bacteria in bloodstream infections and the prevalence of main Gram-negative bacteria resistance among different regions，with statistically significant differences in the prevalence of CRKP and CRPA（ χ2=20.489 and 20.252， P<0.001）. The prevalence of CREC，CRKP，CRPA，CRAB，ESBLs-producing Escherichia coli and Klebsiella pneumoniae were higher in provinicial hospitals than those in municipal hospitals（ χ2=11.953，81.183，10.404，5.915，12.415 and 6.459， P<0.01 or <0.05），while the prevalence of CRPA was higher in economically developed regions（per capita GDP ≥ 92 059 Yuan）than that in economically less-developed regions（per capita GDP <92 059 Yuan）（ χ2=6.240， P=0.012）. Conclusions:The proportion of Gram-negative bacteria in bloodstream infections shows an increasing trend，and Escherichia coli is ranked in the top，while the trend of CRKP decreases continuously with time. Decreasing trends are noted in ESBLs-producing Escherichia coli and Klebsiella pneumoniae. Low prevalence of carbapenem resistance in Escherichia coli and high prevalence in CRAB complex have been observed. The composition ratio and antibacterial spectrum of bloodstream infections in different regions of China are slightly different，and the proportion of main drug resistant bacteria in provincial hospitals is higher than those in municipal hospitals.

Objective:To report the results of national surveillance on the distribution and antimicrobial resistance profile of clinical Gram-positive bacteria isolates from bloodstream infections in China in 2022.Methods:The clinical isolates of Gram-positive bacteria from blood cultures in member hospitals of National Bloodstream Infection Bacterial Resistant Investigation Collaborative System（BRICS）were collected during January 2022 to December 2022. Antibiotic susceptibility tests were conducted by agar dilution or broth dilution methods recommended by Clinical and Laboratory Standards Institute（CLSI）. WHONET 5.6 and SPSS 25.0 software were used to analyze the data.Results:A total of 3 163 strains of Gram-positive pathogens were collected from 51 member units，and the top five bacteria were Staphylococcus aureus（ n=1 147，36.3%），coagulase-negative Staphylococci（ n=928，29.3%）， Enterococcus faecalis（ n=369，11.7%）， Enterococcus faecium（ n=296，9.4%）and alpha-hemolyticus Streptococci（ n=192，6.1%）. The detection rates of methicillin-resistant Staphylococcus aureus（MRSA）and methicillin-resistant coagulase-negative Staphylococci（MRCNS）were 26.4%（303/1 147）and 66.7%（619/928），respectively. No glycopeptide and daptomycin-resistant Staphylococci were detected. The sensitivity rates of Staphylococcus aureus to cefpirome，rifampin，compound sulfamethoxazole，linezolid，minocycline and tigecycline were all >95.0%. Enterococcus faecium was more prevalent than Enterococcus faecalis. The resistance rates of Enterococcus faecium to vancomycin and teicoplanin were both 0.5%（2/369），and no vancomycin-resistant Enterococcus faecium was detected. The detection rate of MRSA in southern China was significantly lower than that in other regions（ χ2=14.578， P=0.002），while the detection rate of MRCNS in northern China was significantly higher than that in other regions（ χ2=15.195， P=0.002）. The detection rates of MRSA and MRCNS in provincial hospitals were higher than those in municipal hospitals（ χ2=13.519 and 12.136， P<0.001）. The detection rates of MRSA and MRCNS in economically more advanced regions（per capita GDP≥92 059 Yuan in 2022）were higher than those in economically less advanced regions（per capita GDP<92 059 Yuan）（ χ2=9.969 and 7.606， P=0.002和0.006）. Conclusions:Among the Gram-positive pathogens causing bloodstream infections in China， Staphylococci is the most common while the MRSA incidence decreases continuously with time；the detection rate of Enterococcus faecium exceeds that of Enterococcus faecalis. The overall prevalence of vancomycin-resistant Enterococci is still at a low level. The composition ratio of Gram-positive pathogens and resistant profiles varies slightly across regions of China，with the prevalence of MRSA and MRCNS being more pronounced in provincial hospitals and areas with a per capita GDP≥92 059 yuan.

Abstract: Objective　To detect the antibody levels of hantavirus in serum samples from patients suspected with hemorrhagic fever with renal syndrome (HFRS) in Heilongjiang Province from 2019 to 2021, and to provide scientific basis for the prevention and control of disease. Methods　Enzyme-linked immunosorbent assays (ELISA) were used to detect the IgM antibodies to hantavirus in serum samples collected from suspected patients with HFRS in the acute-phase, and IgM and IgG antibody in convalescent-phase serum samples. The positive rate of IgM antibody in acute-phase serum samples of patients in different years was analyzed with χ2 test by SPSS 19.0, and the data were sorted out and analyzed about patients' gender, occupation, age, date of onset and interval from onset to initial diagnosis by EpiData 3.1, Excel 2003 software. Results　A total of 351 acute-phase serum samples and 208 convalescent-phase serum samples were detected in patients suspected with HFRS, respectively. There were 317 positive IgM antibodies of serum samples in the acute stage, with the positive rate of 90.31%. There was no significant difference in the positive rate of IgM antibodies in the acute stage between different years (χ2=0.895, P=0.639). T The IgM antibodies and IgG antibodies were positive in 32 (15.39%) and 28 (13.46%) of the convalescent-phase serum samples, respectively. Moreover, 148 patients (71.15%) were double-positive for IgM and IgG antibodies at the convalescent stage. The ratio of male to female patients was 4.56∶1, for which male patients were much more than female patients. Occupation was dominated by farmers (253 cases, 79.81%), followed by workers (19 cases, 5.99%) and the unemployed (17 cases, 5.36%), respectively. The age of patients ranged from 10 to 88 years old, with a median age of 49 years old. Most of the patients were in the age group from 30 years old to 60 years old (209 cases, 65.93%), among which the age group from 40 years old to 50 years old (86 cases, 27.13%) had the highest proportion, and the age group from 60 years old to 90 years old had a proportion of 20.18% (19 cases). May and November were the peak periods of HFRS in Heilongjiang Province. The median interval between onset and initial diagnosis was 4 days. Conclusions　There is a gap of about 10% between the clinical diagnosis of HFRS cases and the confirmed cases detected by laboratory in Heilongjiang Province from 2019 to 2021. The virus-specific detection results are important for confirming the diagnosis of local patients with HFRS.

OBJECTIVE: To explore a surgical method for the reconstruction of volar soft tissue defect and sensory and vascular repair in middle and far phalangeal digits. METHODS: From January 2016 to January 2020, a total of 14 patients , 9 males and 5 females, ages ranging from 22 to 69 years old, and with volar soft tissue defects in the middle and distal digits 2 to 4, underwent surgical reconstruction using the V-Y shaped flap with digital artery and nerve at the metacarpophalangeal joint. The defect area was (2.0~2.5) cm×(1.5 ~2.0) cm. The procedure involved the harvest of a V-Y shaped flap with the digital artery and nerve from the metacarpophalangeal joint. Flap design, dissection of blood vessels and nerves, and anastomosis with the digital artery and nerve were performed according to a standardized protocol., Functional exercise of affected finger was initiated 3 weeks postoperatively. Subsequent assessments were conducted to evaluate finger pulp sensation, shape and other relevant parameters. According to the upper extremity functional evaluation standard set up by Hand Surgery Branch of Chinese Medical Association, the surgical outcomes were evaluated. RESULTS: All 14 cases demonstrated successful tissue transplantation, , with immediate recovery of sensation observed in 10 cases with distal finger pulp defects. Four patients with middle phalangeal defects experienced gradual sensory recovery within 2 to 3 months postoperatively. Thirteen patients were followed up for a mean duration of (8.8 ± 4.49) months, during which satisfactory outcomes were observed. The average two-point resolution of the finger pulp was 4-6mm, and sensory function evaluation yielded a score of S3 or above. Patients exhibited realistic finger shape, normal skin color and temperature, good wear resistance, and cold resistance. Furthermore, finger joint function was essentially normal. CONCLUSION The V-Y shaped flap with digital artery and nerve at the metacarpophalangeal joint offers a suitable solution for repairing the defect of the middle or distal phalangeal finger. This technique is characterized by its simplicity, low risk, and favorable outcomes, including restored finger shape, blood supply and sensation. Moreover, high patient satisfaction was achieved.
Male ; Female ; Humans ; Young Adult ; Adult ; Middle Aged ; Aged ; Plastic Surgery Procedures ; Skin Transplantation ; Finger Injuries/surgery* ; Treatment Outcome ; Soft Tissue Injuries/surgery* ; Fingers/surgery* ; Ulnar Artery/surgery* ; Metacarpophalangeal Joint/surgery*

OBJECTIVE: To analyze the clinical characteristics of the patients with B-cell chronic lymphoproliferative disease（B-CLPD） in the new drug era and the effect of new drug treatment on efficacy and survival. METHODS: The clinical and laboratory data of 200 cases B-CLPD patients diagnosed between April 2015 and August 2021 were analyzed retrospectively. The clinical efficacy and survival of the patients under different treatments including Bruton tyrosine kinase（BTK） inhibitors， rituximab， and chemotherapy alone were analyzed. The prognostic factors affecting the survival of patients were analyzed by univarite analysis and multivariate analysis. RESULTS: There were 119 male（59.5％） and 81 female（40.5%） in 200 cases B-CLPD patients， the sex ratio(male/female) was 1.5∶1 with median age of 61（30- 91） years old. The distribution of subtypes were as fallows: 51 cases (25.5%) of chronic lymphocytic leukemia/small lymphocytic lymphoma（CLL/SLL）， 64（32.0%） cases of follicular lymphoma（FL）， 40（20.0%） cases mantle cell lymphoma（MCL）， 30（15.0%） cases of marginal zone lymphoma（MZL）， 10（5%） cases of lymphoplasmacytic lymphoma/waldenstrom macroglobulinemia（LPL/WM）， 5（2.5%） cases of B cell chronic lymphoproliferative disorders unclassified（B-CLPD-U） . The main clinical manifestation of 102 patients was lymph node enlargement, 32 cases were complicated with B symptoms. Among CLL/SLL patients， there were 12（23.5%） cases in Binet A and 39（76.5%） cases in Binet B/C. There were 29 patients（20.9%） in Ann Arbor or Lugano stage I-II and 110 cases（79.1%） in stage III-IV of other subtypes. The complete remission（CR） rate was 43.1%（25/58）， 40.2%（39/97）， 7.1%（1/14）， and overaIl response rate（ORR） was 87.9%（51/58）， 62.9%（61/97）， 28.6%（4/14） in the groups of BTK inhibitors， rituximab-based therapy， and chemotherapy alone. The 3-year OS rate and PFS rate in all patients was 79.2% and 72.4% respectively. The 3-year OS rate of patient with MZL, CLL/SLL, FL,WM was 94.7%, 87.7%, 86.8% and 83.3% respectively, while the 3-year OS rate of MCL was only 40.6%, which was significantly lower than other subtypes. The median OS of patients treated with BTK inhibitors and rituximab-based therapy was 20.5 and 18.5 months respectively， and the 3-year OS rate was 97.4% and 90.7%. However， the median PFS of patients receiving chemotherapy alone was 4 months， and the 1-year OS rate was 52.7%， which was statistically significant compared with the other two groups（P<0.05）. Univarite analysis showed that anemia， elevated lactate dehydrogenase， elevated β2-microglobulin， and splenomegaly were the poor prognostic factors for OS（P<0.05）， elevated lactate dehydrogenase was also poor prognostic factors for PFS（P<0.05）. Multifactor analysis showed that anemia and elevated lactate dehydrogenase were the independent poor prognostic factors for survival（P<0.05）. CONCLUSION The clinical features of B-CLPD was various， anemia and elevated lactate dehydrogenase are the prognostic factors for poor survival. BTK inhibitors and new immunotherapy can improve the survival and prognosis of patients in the new drug era.
Humans ; Adult ; Female ; Male ; Middle Aged ; Aged ; Aged, 80 and over ; Rituximab/therapeutic use* ; Leukemia, Lymphocytic, Chronic, B-Cell/drug therapy* ; Retrospective Studies ; Lymphoma, Mantle-Cell ; Prognosis ; Lymphoma, B-Cell, Marginal Zone ; Lactate Dehydrogenases

Objective: To evaluate the long-term efficacy and safety of the implantable ventricular assist system EVAHEART I in clinical use. Methods: Fifteen consecutive patients with end-stage heart failure who received left ventricular assist device therapy in Fuwai Hospital from January 2018 to December 2021 were enrolled in this study, their clinical data were retrospectively analyzed. Cardiac function, liver and kidney function, New York Heart Association (NYHA) classification, 6-minute walk distance and quality of life were evaluated before implantation and at 1, 6, 12, 24 and 36 months after device implantation. Drive cable infection, hemolysis, cerebrovascular events, mechanical failure, abnormally high-power consumption and abnormal pump flow were recorded during follow up. Results: All 15 patients were male, mean average age was (43.0±7.5) years, including 11 cases of dilated cardiomyopathy, 2 cases of ischemic cardiomyopathy, and 2 cases of valvular heart disease. All patients were hemodynamically stable on more than one intravenous vasoactive drugs, and 3 patients were supported by preoperative intra aortic balloon pump (IABP). Compared with before device implantation, left ventricular end-diastolic dimension (LVEDD) was significantly decreased ((80.93±6.69) mm vs. (63.73±6.31) mm, P<0.05), brain natriuretic peptide (BNP), total bilirubin and creatinine were also significantly decreased ((3 544.85±1 723.77) ng/L vs. (770.80±406.39) ng/L; (21.28±10.51) μmol/L vs. (17.39±7.68) μmol/L; (95.82±34.88) μmol/L vs. (77.32±43.81) μmol/L; P<0.05) at 1 week after device implantation. All patients in this group were in NYHA class Ⅳ before implantation, and 9 patients could recover to NYHA class Ⅲ, 3 to class Ⅱ, and 3 to class Ⅰ at 1 month after operation. All patients recovered to class Ⅰ-Ⅱ at 6 months after operation. The 6-minute walk distance, total quality of life and visual analogue scale were significantly increased and improved at 1 month after implantation compared with those before operation (P<0.05). All patients were implanted with EVAHEART I at speeds between 1 700-1 950 rpm, flow rates between 3.2-4.5 L/min, power consumption of 3-9 W. The 1-year, 2-year, and 3-year survival rates were 100%, 87%, and 80%, respectively. Three patients died of multiple organ failure at 412, 610, and 872 d after surgery, respectively. During long-term device carrying, 3 patients developed drive cable infection on 170, 220, and 475 d after surgery, respectively, and were cured by dressing change. One patient underwent heart transplantation at 155 d after surgery due to bacteremia. Three patients developed transient ischemic attack and 1 patient developed hemorrhagic stroke events, all cured without sequelae. Conclusion: EVAHEART I implantable left heart assist system can effectively treat critically ill patients with end-stage heart failure, can be carried for long-term life and significantly improve the survival rate, with clear clinical efficacy.
Humans ; Male ; Adult ; Middle Aged ; Female ; Heart Failure/complications* ; Follow-Up Studies ; Retrospective Studies ; Heart-Assist Devices ; Quality of Life