Objective To summarize the clinical characteristics of patients with connective tissue diseases (CTD) and autonomic neuropathy. Methods The medical records of inpatients with CTD and autonomic neuropathy from 2005 to 2015 were retrospectively analyzed including clinical manifestations, laboratory examinations, treatment and outcome. Categorical data were expressed in percentages. Kolmogorov-Smirnov test was used to examine normal distribution. Continuous data of normal distribution were expressed as x ±s deviation, while data without a normal distribution were described as median and interquartile range (P25, P75). Results Among the nine patients included in this study, all were female, and the median age was 42 years (32~50 years old). Four patients (4/9) were systemic lupus erythematosus (SLE), three patients (3/9) were primary Sj?gren's syndrome (pSS), two patients (2/9) were rheumatic arthritis (RA), and four patients were secondary Sj?gren's syndrome (SS) (two with SLE and two with RA). Five patients (5/9) had autonomic nervous dysfunction before they were diagnosed of CTD, while four patients (4/9) developed autonomic nervous dysfunction after diagnosis of CTD. The most common symptom of autonomic nervous dysfunction was postural hypotension (9 patients, 9/9), followed by hypohidrosis (4 patients, 4/9), urinary retention (2 patients, 2/9), gastrointestinal dysmotility (2 patients, 2/9) and tonic pupil (1 patient, 11%). After treatment of CTD, autonomic symptoms of three patients improved, while the others didn't. Four of the remaining 6 patients improved after receiving other assistant treatments including vasoconstrictor, pyri-dostigmine bromide, and plasma exchange. Conclusion Patients with CTD could present with autonomic neuropathy, which is mainly characterized with postural hypotension. If patients had related symptoms, clinicians should pay more attention to whether CTD exists. If treatment for CTD couldn't improve patients' condition, other assistant treatment might be considered.

Objective To explore the prevalence and independent influencing factors of arthritis in Chinese patients with systemic sclerosis(SSc).Methods A total of 248 SSc patients were enrolled in this prospective cross-sectional study in Peking Union Medical College Hospital.Demographic,clinical and laboratory data were compared in arthritis group and non-arthritis group.Results Of 248 Chinese SSc patients,mean age of onset was (37.1 ± 13.7) years old and disease duration was (7.4 ± 5.2) years.Overall prevalence of arthritis was 41.1％ (102/248).The presence of arthralgia(95.1％ vs 15.1％),muscle weakness(38.2％ vs 24.7％),short of breath (60.8％ vs 45.2％),and intestinal lung disease (64.7％ vs 49.3％) occurred more frequently in patients with arthritis than those without arthritis (P ＜0.05).The elevation of C reactive protein,anti-Sm and anti-cyclic citrullinated peptide were more common in arthritis group than in non-arthritis group(61.4％ vs 43.9％,11.4％ vs 2.5％ and 28.1％ vs 2/16,respectively).There were no obvious differences in the Rodnan score,digital ulcers,pulmonary arterial hypertension,and six-minute walk test between two groups(P ＞ 0.05).Conclusion This study reveals the significant prevalence of arthritis in a large cohort of SSc patients in China.Compared to non-arthritis patients,the SSc patients with arthritis have a more severe inflammation reaction and a higher rate of intestinal lung disease.

Objective To investigate the changes of major causes of death in patient with dermatomyositis/polymyositis (DM/PM) and the factors affecting the mortality of DM/PM in the past 28 years.Methods Death cases with DM/PM from 1985 to 2013 were retrospectively analyzed.x2 test and Fisher exact test was used for count data analysis;independent samples t-test measurement was used for data analysis;P＜0.05 was consi-dered statistically significant.Results Out of 1 443 patients with DM/PM, 74 died, in whom 48 were female and 26 were male.The mortality rate was 4.7％ among female and 6.3％ among male cases and the total in-hospital mortality of DM/PM was 5.1％.The two most common causes of death in patients with DM/PM were infection and respiratory involvement, accounting for 62.2％ (46/74) and 14.9％ (11/74) respectively.Death occurred most commonly in the first year after disease onset, accounting for 79.7％(59/74).The leading causes of death in the first year were infection and respiratory involvement.The mortality of DM/PM patients was peaked 12.3％(18/146) from 1996 to 2000;infection was the most frequent cause of death.Conclusion Infection and respiratory involvement are the main causes of death in DM/PM.Death occurrs most commonly in the first year after disease onset.

Objective To evaluate clinical application value of polymerase chain reaction (PCR) detection for bacteria in peritoneal dialysis associated peritonitis (PDAP).Methods Peritoneal dialysis fluid specimens were collected from January 2014 to December 2014 in The First Affiliated Hospital of Anhui Medical University.Conventional bacterial culture and PCR detection were used respectively.According to the bacterial 16S rRNA gene, universal primers were devised and designed, based on reference, the specific primers of 17 kinds of experimental bacteria.Real-time fluorescent PCR (Real-time PCR, qPCR) amplification was implemented.The establishment of standard strain DNA extract was used as positive control;sterile double distilled water was used as negative control.Results (1) The traditional bacterial culture results showed that positive proportion was 26/40 in specimen of 40 cases, gram-positive strains accounting for 18/26.Main species were epidermis staphylococcus (5/26), hemolysis staphylococcus (4/26), escherichia coli (4/26), and streptococcus viridans (3/26).(2) The PCR detection results showed that total positive rate was 33/40 in 40 patients specimens, among which 2 cases of positive samples ended up with no specific strains being detected;the main bacteria strains in PCR were not different from ordinary culture results.(3) With bacterial culture as the gold standard, the detection sensitivity of PCR technology for PDAP pathogenic bacteria was 96.15％ and specificity was 42.86％;the detection positive rate was significantly higher than ordinary culture method.(4) PCR technology for detecting pathogenic bacteria could produce results within 4-6 hours, while reported positive results in the traditional bacterial culture would take (77.88±15.53) hours, which was significantly longer than PCR.Conclusion Compared with traditional bacteria culture method, PCR method is more sensitive, simple, and quick.Bacteria detection using PCR technique is of clinic applied value in PDRP.

OBJECTIVETo synthesize a novel nano-antibacterial inorganic filler and provide a new way to give dental composite resin antibacterial property.

METHODSQuaternary ammonium iodide N,N,N-trimethyl-3-(trimethoxysilyl) propan-1-aminium iodide were organically synthesized firstly and then the N,N,N-trimethyl-3-(trimethoxysilyl) propan-1-aminium iodide was grafted to the nano-silica particle to synthesize the antihacterial inorganic fillers nano-silica particle grafted with quaternary ammonium salt. All the products were analyzed and identified by infrared spectrum analysis. Then Streptococcus mutans were chosen as experimental object to analysis the antibacterial property of nanoantibacterial inorganic filler.

RESULTSQuaternary ammonium salt was grafted to the surface of nano-silica particles successfully by infrared spectrum analysis. Compared with the control group, the nano-silica particle grafted with quaternary ammonium salt had a strong bactericidal effect on Streptococcus mutons (P < 0.01).

CONCLUSIONThe nano-silica particle grafted with quaternary ammonium salt has a strong antibacterial property and could be used to improve dental composite resin antibacterial property.

A 78-year-old man was admitted to Peking Union Medical College Hospital with fever, weakness of lower extremities, less speech, loss of memory. Fever was relieved after antibiotic treatment, while cognitive impairment and disorder of consciousness progressed rapidly, followed by critical pulmonary infections, respiratory failure, and septic shock. Lab tests showed negative occult blood, normal serum CEA level and positive Anti-nuclear-antibody. PET-CT suggested that strong FDG uptake signals were seen at sigmoid, while bilateral frontal lobe, temporal lobe, parietal lobe, posterior cingulate gyrus showed lower metabolic activity. Colonoscopy biopsy revealed differentiated adenocarcinoma of sigmoid colon. Therefore, paraneoplastic syndrome of nervous system secondary to colon cancer was considered. Rapid and proper diagnosis and treatment were completed by multidisciplinary team including departments of neurology, gastroenterology, general surgery, ICU, rheumatology, clinical nutrition. The laparoscopic sigmoid colectomy was performed under general anesthesia. The patient finally presented with significant improvement of cognition and consciousness. Respiratory function was totally recovered.

Objective: This study is to repair nostril sill deformity in the cleft lip patients by reconstructing the first auxiliary muscle tension line group and to assess the therapeutic outcome. Methods: 437 cleft lip patients with nostril sill deformity underwent the surgery from January 1, 2012 to November 1, 2016.They were treated using the technique of first auxiliary muscle tension line group reconstruction to repair the deformity. Aesthetic correction evaluations were rated by the GAIS. Random digit was used to randomly select 24 patients during the follow-up for three-dimensional measurement and analysis. The preoperative and postoperative symmetry of the nostril sills were evaluated by paired t test. Results: Mucosal ischemia or wound infection occurred in 11 cases, left obvious scar on the nostril floor. Surgical incisions of the other patients were primary healing. After 6 months to 3 years follow up, GAIS questionnaires of 378 patients demonstrated that 84% patients reported great or moderate improvement of nostril sills. Three-dimensional measurements of 24 patients suggested that there were no significant differences of the nostril sill and the nasal ala between the cleft side and the normal side. Conclusions First auxiliary tension line group reconstruction, which is to restore biomechanical balance rather than merely increase muscle volume, is an effective method of nostril sill repair in the cleft lip patients. Post-operatively, the patients achieved a stable and natural nostril sill, a middle columella, as well as a narrowed nasal ala.

Objective:To analyze the clinical characteristics and risk factors of rheumatic diseases complicated with Pneumomediastinum (PnM).Methods:A retrospective analysis of 94 inpatients with rheumatic diseases associated PnM from Peking Union Medical College Hospital and Shanxi Bethune Hospital between January 1998 and October 2018 was carried out. Patients were divided into idiopathic inflammatory myopathies (IIM) and the non-IIM group. Clinical features, laboratory examinations and treatment were compared between the two groups. Cox proportional hazard model was used to investigate the risk factors for prognosis.Results:A total of 94 patients were included in the study, with an average age of (45±14) years. Forty-five (48%) of them were male. There were 62 patients in the IIM group and the other 32 were in the non-IIM group. Sixty-nine patients had predisposing factors before PnM. Severe cough and assisted mechanical ventilation were the most common causes. Compared with the non-IIM group, the incidence of digital vasculitis (29% vs 6%, χ2=6.540, P=0.008), arthritis (60% vs 28%, χ2=8.409, P=0.004), interstitial lung disease (ILD)(98% vs 78%, χ2=11.129, P=0.002) were higher in the IIM group, treatment with cyclophosphamide was higher in the IIM group ( χ2=4.458, P=0.035). There was non-significant difference in mortality between the two groups during hospitalization (50% vs 59%) and 6 months after PnM (64% vs72%) ( P>0.05). Pulmonary infection was the only risk factor for poor prognosis [ HR=3.131, 95% CI (1.025, 9.561), P=0.045], in which bacteria infection was the most common cause (65/75, 86.7%). Conclusion:PnM is a severe complication of rheumatic diseases. To get a good prognosis, rheumatol-ogists should balance the benefit and risk of infection of immunosuppressive therapy.

Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease with high prevalence and possible poor prognosis. Though the pathogenesis of pSS has not been fully elucidated, B cell hyperactivity is considered as one of the fundamental abnormalities in pSS patients. It has long been identified that Janus kinases-signal transducer and activator of transcription (JAK-STAT) signaling pathway contributes to rheumatoid arthritis and systemic lupus erythematosus. Recently, increasing numbers of studies have provided evidence that JAK-STAT pathway also has an important role in the pathogenesis of pSS via direct or indirect activation of B cells. Signal transducer and activator of transcription 1 (STAT1), STAT3, and STAT5 activated by various cytokines and ribonucleic acid contribute to pSS development, respectively or synergically. These results reveal the potential application of Janus kinase inhibitors for treatment of pSS, which may fundamentally improve the quality of life and prognosis of patients with pSS.

Objective:To investigate the clinical characteristics of patients with rheumatic diseases and abnormal liver function, as well as determine the proportion and severity of liver function abnormalities.Methods:Cross-sectional study. Data were collected from patients registered in the Chinese Rheumatism Date Center from 2011 to 2021. The rheumatic diseases analyzed in this study were rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), Sjogren syndrome (SS), ankylosing spondylitis (AS), and gout. Patient data, including demographic characteristics [ such as age, sex, body mass index,(BMI), and smoking history], liver function test results [including alanine aminotransferase (ALT), aspartate aminotransferase, alkaline phosphatase(ALP), and total bilirubin], and use of anti-rheumatic immune drugs and liver-protective drugs, were collected and compared between groups with normal and abnormal liver functions. In addition, the proportions of abnormal liver function were compared between sex and age groups.Results:A total of 116 308 patients were included in this study, including 49 659 with RA, 17 597 with SLE, 9 039 with SS, 11 321 with AS, and 28 692 with gout. The lowest proportion of liver function abnormalities was observed in patients with RA[11.02% (5 470/49 659)], followed by those with SS[17.97% (1 624/9 039)] and AS [18.22% (2 063/11 321) ], whereas patients with SLE [21.14% (3 720/17 597) ] and gout [28.73% (8 242/28 692)] exhibited the highest proportion of these abnormalities. Elevated ALT, mostly classified as grade 1, was the most commonly noted liver function abnormality, whereas elevated ALP was the least common. Some patients who took liver-protective drugs had normal liver function, with the lowest percentage observed in patients with gout [7.45% (36/483) ] and ranging from 21.7% to 30.34% in patients with RA, SLE, SS, and AS. The proportion of liver function abnormalities was higher in males than in females for all disease types [RA: 13.8%(1 368/9 906) vs. 10.3%(4 102/39 753); SLE: 33.6% (479/1 424) vs. 20.0% (3 241/16 173); SS: 25.4%(111/437) vs. 17.6%(1 513/8 602); AS: 20.1%(1 629/8 119) vs. 13.6% (434/3 202); and gout: 29.3% (8 033/27 394) vs. 16.1% (209/1 298)]. In RA, SLE, and AS, the proportions of liver function abnormalities were similar across all age groups. In SS, the proportion of liver function abnormalities increased with age [<40 years: 14.9%(294/1 979); 40-59 years: 18.1%(858/4 741); ≥60 years: 20.4%(472/2 319)], whereas a reversal of this trend was observed in gout [<40 years: 34.9%(4 294/12 320); 40-59 years: 25.5%(2 905/11 398);≥60 years: 21.0%(1 042/4 971)].Conclusions:The proportions of combined liver function abnormalities in patients with rheumatologic diseases were high, and the utilization rates of liver-protective drugs were low. It is necessary to pay more attention to monitoring patients′ liver function, timely administer liver-protective drugs, and optimize liver-protective regimens during the treatment of rheumatic diseases.