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 analyze the genetic quality and to determine the strain of laboratory mice. Methods Ten strains of mice were analyzed by using AFLP method. Results Polymorphism was detected in 10 strains of mice by 17 single enzyme primers and 20 pairs of double enzyme primers amplification. A total of 251 bands were shown by single enzyme AFLP in agarose gel with the size of the bands ranging from 100 bp to 2 000 bp and 89 polymorphic loci were detected. A total of 1507 clear bands between 50 bp and 600 bp were shown by double enzyme AFLP and 378 polymorphic loci were detected. Through statistical analysis, we calculated the similar index and genetic distance index. Our results showed BALB/c and BALB/c nu had the closest relationship and KM had a closer relationship with TA2, BALB/c and BALB/c nu, while DBA/2 showed a distant relationship with T739, 615 and C57BL/6J, coinciding with the origins of breeds. Conclusion Each strain could be distinguished from others by using the AFLP polymorphic primers, which provides reference data for genetic quality analysis and strain determination of mice.

Objective To study the clinical profile of patients with central nervous system (CNS) involvement in relapsing polychondritis (RP).Methods Clinical data of five patients of RP with CNS lesions were collected,and compared with those from the literatures.T test and Fisher exact test were used for statistical analysis.Results Among 205 patients with RP in Peking Union Medical College Hospital,five cases (2.4％) had CNS damage.All 5 cases presented an active onset and occurred in the active phase of RP.All of them manifested as meningoencephalitis,complicating cranial neuropathies in 2 cases (2/5).Cerebrospinalfluid examination revealed non-specific meningeal inflammation,and magnetic resonance image (MRI) showed long T2 signals in brain lesions.Four patients (4/5) showed good response to high-dose glucocorticosteroid plus immunosuppressive agent combined therapy.The average age of our patients was younger than those in the literatures [(44± 14),(58± 11) years,respectively; t=2.547,P＜0.05],while other clinical features was not significantly different between the two groups.Conclusion CNS involvement is a rare condition in RP patients,and usually occurrs in the early course of active RP.Meningoencephalitis/ meningitis is the major clinical manifestations.MRI and cerebrospinal fluid examination may help to confirm the diagnosis.Treatment with corticosteroid and immunosuppressant can result in favorable response.

Objective To summarize the clinical characteristics of SLE patients complicated with Listeria monocytogenes meningitis (LMM).Methods The clinical manifestations,systemic involvements,laboratory tests,cerebrospinal fluid (CSF) examinations,treatment and prognosis of SLE inpatients combined with LMM in Peking Union Medical College Hospital during Janurary 2000 to December 2015 were retrospectively analyzed.Results Eight SLE patients with LMM accounted for 0.14％ of the 5 675 hospitalized SLE patients.Seven were female and 1 was male,with the mean age of (35±16) year.Headache (7/8) was the most common neurological symptom followed by positive meningeal irritation sign (5/8),unconsciousness (3/8) and nausea or vomiting (2/8).Increased intracranial pressure was found in 7 cases and 4 of them were even higher than 330 mmH2O.CSF protein elevated in 6 cases and CSF glucose decreased in 6 cases.LMM often occurred in inactive or mild active SLE with the average SLE disease activity index (SLEDAI) score of 4.7±1.5.Systemic manifestations included hematologic changes (6/8),rashes (4/8),renal involvement (4/8) and arthritis (3/8),etc.Treated by autibiotics such as penicillin and medications for SLE,7 patients achieved clinical improvements,1 patient died.Conclusion LMM is one of the opportunistic infections in SLE patients and tends to occur in inactive lupus cases.There are unspecific clinical and laboratory features and CSF changes which maybe overlap and mix up with systemic manifestations of SLE.That's why it is necessary to take continuous cultures of CSF.Early identification and appropriate treatment lead to prognostic improvement of these patients.

Objective To compare the clinical response with etoricoxib 60 mg once daily with diclofenac sodium tablet 75 mg two times daily in the treatment of osteoarthritis of the knee or hip joint.Methods A 4-week multicenter,randomized,double-blinded and active comparator-controlled clinical trial was performed during January 2005 and June 2005 in 6 medical centers in China.Eligible patients (≥40 years old Chinese patients with osteoarthritis of the knee and hip) were randomized (1:1 ratio) to receive etoricoxib 60 mg once daily (n=90),or diclofenac sodium 75 mg twice daily (n=90).Primary efficacy end point is the change of WOMAC (Western Ontario and McMaster Universities osteoarthritis index) pain subscale from baseline to 4 weeks; non-inferiority bounds were pre-defined [if the upper bound of 95％ confidence interval (CI) for the difference is less than 10 mm on a 100-mm VAS WOMAC pain subscale] for the comparison of the change between the two groups.The secondary efficacy endpoints include WOMAC physical function subscale,WOMAC stiffness subscale,patient's global assessment of response to therapy (PGART),investigator's global assessment of disease status (IGADS),discontinuation due to lack of efficacy and rescue paracetamol tablet count.Safety was assessed by physical examination,adverse experience reported,and laboratory safety data.Results C6mpared to baseline,the changes of WOMAC pain subscale after 4 weeks treatment were statistically significant (P＜0.01) in both groups (etoricoxib group:51±16 vs 21± 19; diclofenac sodium group:53±16 vs 22±19).There was no difference in the change of WOMAC pain subscale between the two groups.The change in WOMAC stiffness subscale,WOMAC physical function subscale,PGART and IGADS in both groups were statistically significant (P＜0.01),but there was no difference between treatment groups according to the pre-defined non-inferiority criteria.No drug related serious adverse events were observed during the study.The difference in drug-related adverse event incidence between the two groups was not statistically significant.Etoricoxib and diclofenac sodium were generally safe and well tolerated.Conclusion Etoricoxib 60 mg administered once daily is efficacious and shows clinical efficacy notinferior to that of diclofenac sodium 75 mg administered twice daily for the treatment of osteoarthritis.Etoricoxib 60 mg administered once daily for 4 weeks is generally safe and well tolerated.

ObjectiveTo investigate the clinical outcome of laparoscopic nephroureterectomy (LUNT) for native upper tract urothelial carcinoma (UC) in renal transplant (RT) recipients.Methods We conducted a retrospective analysis on 1130 RT recipients,and 9 patients (0.8％,9/1130) with native upper tract UC were identified. UC was confirmed pathologically in the 9 patients,including 3 cases of unilateral ureter tumor (2 on the right,and 1 on the left),4 cases of unilateral renal pelvis tumor (2 on the right,2 on the left),1 case of bilateral ureter tumor and 1 case of tumor in the right ureter and left kidney.Females predominated (8/9) in the 9 patients with upper tract UC.The patients with left upper urinary tract cancer underwent LUNT using a retroperitoneal approach with a technique of transurethral circumcision of the ureteral orifice.The patients with right upper urinary tract cancer were subjected to nephroureterctomy with ureterectomy and bladder cuff excision by complete laparascopy through a transperitoneal approach. Immunosuppressive protocol conversion from calcineurin inhibitors to sirolimus was performed on all cases. Results In the 9 patients,11 LUNTs were performed successfully without conversion to open surgery.The follow-up period was from 6 to 48 months. One patient died of lung metastasis at 8th month after tumor excision,and 1 patient displayed ductal cancer of the left breast at 7th month after LNUT.Another seven patients showed no evidence of disease during the follow-up period with normal renal function.ConclusionOur present clinical experience suggested that LNUT for the native upper tract cancer in renal transplant recipients is feasible,safe,and effective.

AIM: To assess the role of surface free radicals and electromotive voltage of fibrous mineral dusts in rabbit pulmonary alveolar macrophage injuries induced by fibrous mineral dusts. METHODS: Changes in cell death ratio, malandialdehyde (MDA) and cellur electrophoresis ratio, lactate dehydrogenate (LDH)and superoxide dismitase(SOD) activities were determined, the technique of cell culture and Scanning electron Microscopy were used to examine the change of membranous permeability, charge and cellular shape. RESULTS: Fibrous wollastonite and tabulate clinoptilolite, which had no OH-, had no cytotoxicity, while fibrous sepiolite, fibrous palygorskite, fibrous brucite and chrysolite asbestos damaged pulmonary alveolar macrophages in various degrees because of the different OH- levels. All the six fibrous mineral dusts changed the cellular electrophoresis ratio. CONCLUSION: The surface electromotive voltage of fibrous mineral dusts is not an important factor, and the cytotoxicity of them may be related to OH- levels on the mineral dust surface.

Objective To study the clinical, laboratory, radiological and pathological findings of patients with hypertrophic cranial pachymeningitis (HCP) in Wegner's granulomatosis (WG) to improve the recognition of the disease, even when it occurs in limited form. Methods Three patients were described and English literatures of biopsy-proven pachymeningitis in WG were reviewed. Results The features of WG-associated pachymeningitis included: ① Frequently occurred early in the course of active limited WG; ② Commonly presented with sever headache and cranial neuropathies in the absence of other meningeal irritative signs; ③ Variable cerebrospinal fluid findings with mild predominantly lymphocytic pleocytosis and elevated protein concentration were major laboratorg findings; ④Elevated ESR and positive serum anti-neutrophilic cytoplasmic antibody (ANCA) could be found in most patients; ⑤ Gadolinium-enhanced brain MRI is very senitive in the detection of pachymeningitis; ⑥A dural biopsy showed granulomatous necrotizing inflammation, giant cell, and evidence of vasculitis;⑦ A favorable response to standard treatment with corticosteroid, cyclophosphamide or other cytotoxic drugs could be observed. Conclusion HCP may be the initial or cardinal manifestation of the limited form of WG. Early diagnosis by ANCA, MRI and dural biopsy may facilitate diagnosis Corticosteroid and immunosupressant are the choices of treatment.

Objective To examine the cognitive function in healthy first-degree relatives (FDRs) of patients with bipolar Ⅰ disorder.Methods Cognitive function were studied in one hundred twenty healthy FDRs of patients with bipolar Ⅰ disorder and one hundred normal controls using digital symbol, digital span, visual reproduction, trail making test A (TMT-A) and trail making test B (TMT-B).Results Compared with normal controls, FDRs showed impairment in all indexes of the tests, including digital symbol, digital span (forward, reversed and forward + reversed), visual reproduction, TMT-A and TMT-B (t=-3.44、-4.23、-4.32、-4.98、-2.59、4.32、3.78, respectively, Ps≤0.01).By analysis of covariance (covariant: age and years of education), FDRs were still impaired in these indexes (P<0.05).Compared with sex-matched normal controls, male FDRs showed impairment in all indexes of the tests, but female FDRs only showed impairment in digital span (reversed, forward + reversed), TMT-A and TMT-B (P<0.05).Conclusions Attention, memory and executive function are impaired in healthy first-degree relatives of patients with bipolar Ⅰ disorder.

Objective To study the clinical profile of patients with multicentric reticulohistiocytosis (MRH).Methods The clinical manifestations,image and histologic characteristics of ten patients with MRH were collected,and the relevant literatures were reviewed.Results Seven cases were women.The median (quartile) age was 46.5 (44.5-55.3) years.Clinical symptoms included papulonodular lesions on the skin (ten cases),erosive polyarthritis (ten cases),myositis (four cases),lung nodules (three cases),larynx nodules (one case) and serositis (one case).Biopsy demonstrated infiltration of multinucleated giant cells with CD68 positive and glassy cytoplasm.All of the ten patients were treated with corticosteroids and disease-modifying antirheumatic drugs,skin lesions of seven patients were ameliorated and arthritis was improved in three patients.Conclusion MRH is a systemic disease that has characteristic clinical manifestations and unique histological findings.Deep understanding of this disease can avcid.isdiagnose.Further studies are needed to explore effective treatment.