Diagnosing relapsing polychondritis remains a common challenge: experience from a Chinese retrospective cohort.

OBJECTIVE: The diagnosis of relapsing polychondritis (RP) is often mistaken or delayed. In this retrospective cohort, we aimed to unveil the causes responsible for such phenomenon, to determine the associated factors, and to compare diagnosis in clinical settings with the current diagnostic criteria. METHOD: Eighty-seven RP patients followed-up by rheumatologists from January 1, 2008, to October 31, 2018, were retrospectively analyzed. RESULTS: A total of 50 male and 37 female patients were included with a mean age of 45.9 ± 14.5 years. Ninety-three percent were initially admitted by non-rheumatologic specialists .Twenty-eight percent were correctly diagnosed, while 72% were misdiagnosed at the first visits, all by non-rheumatologic specialists. Patients admitted by non-rheumatologic specialists had increased odds of misdiagnosis (odds ratio [OR] = 1.3, 95% confidence interval [95% CI] 1.1-1.7, P = 0.000). Fifty-seven (65.5%) patients did not meet with Michet or Damiani criteria, with 16 (18.4%) patients diagnosed as partial RP and 41( 47.1%) patients diagnosed as limited RP. CONCLUSIONS: Incorrect and delayed diagnosis of RP is common in our cohort, and insufficient awareness of the disease in non-rheumatologic specialists at least partially contributes to this. It is imperative to revise the current criteria for early diagnosis.Key Points• Diagnosing relapsing polychondritis (RP) in early stage remains challenging after all these years, especially among non-rheumatologic specialists, indicating the importance of teaching non-rheumatologic specialists to improve their understanding of this rare disease.• Many RP patients did not fully meet with the current criteria, suggesting that revision of the current criteria is imperative for early diagnosis of this rare disease.

Reliability and validity of the Chinese version of the Scale for Assessment and Rating of Ataxia.

BACKGROUND: The Scale for the Assessment and Rating of Ataxia (SARA) was shown to be a reliable and valid measurement for patients with spinocerebellar ataxia (SCA). The Brazilian version and the Japanese version of SARA were favorable for good reliability and validity. This study aimed to translate SARA into Chinese and test its reliability and validity in measurement of cerebellar ataxia. METHODS: SARA was translated into Chinese. A total 39 patients with degeneration cerebellar ataxia were evaluated independently by two neurologists with the Chinese version of SARA. Then the patients were evaluated by one of above neurologists with International Cooperative Ataxia Rating Scale (ICARS). The statistical analyses were performed using SPSS 17.0 for Windows. RESULTS: The Cronbach's alpha coefficient of the Chinese version of SARA was 0.78, which represents a good internal consistence. The correlation coefficient of the Chinese version of SARA scores between the two evaluators was 0.86, illustrating that the inter-rater reliability of Chinese version of SARA was good. The correlation coefficient between the Chinese version of SARA and ICARS was 0.91, illustrating that the criterion validity of Chinese version of SARA was not bad. CONCLUSIONS: The Chinese version of SARA is reliable and effective for the assessment of degeneration cerebellar ataxia. Compared with ICARS, the evaluation of Chinese version of SARA is more objective, the assessment time is shortened, and the maneuverability is better.

[Correlation of H-type hypertension and prognosis of ischemic stroke].

OBJECTIVE: To examine the correlation between hypertension with hyperhomocysteinemia and prognosis of ischemic stroke. METHODS: A total of 634 patients with acute ischemic stroke confirmed by computed tomography or magnetic resonance imaging were recruited at Department of Neurology, First Affiliated Hospital, Zhengzhou University from January 1, 2007 to May 30, 2010. Their NIHSS (National Institute of Health Stroke Scale) scores were evaluated on admission. And their baseline profiles and probable prognostic factors were recorded. Recovery was assessed by modified Rankin score (MRS) during a 6-month follow-up. Multivariate Logistic regression was performed for statistical analysis. RESULTS: Among them, 32 became lost to follow-up. There were 197 females (32.7%) and 405 males (67.3%) with an average age of (59 ± 13) years (range: 19 - 92). The average level of homocysteine was (19 ± 11) µmol/L. There were 343 (57%) patients with hypertension and MRS ≥ 3 was in 145 (31%) patients. Logistic regression analysis showed that significant correlations existed between H-type hypertension and 6-month MRS (P = 0.012, OR = 2.566, 95%CI: 1.299 - 5.357) when homocysteine ≥ 15 µmol/L was defined for hyperhomocysteinemia. And there was a total sum of interaction between hypertension and hyperhomocysteinemia. The parameters of relative excess risk of interaction (RERI), attributable proportion due to interaction (AP) and synergy index (S) were 0.683, 26.61% and 1.59 respectively. Other parameters with significant prognostic correlations included age, history of stroke, NIHSS score on admission and diastolic blood pressure on admission. CONCLUSION: At the homocysteine level of ≥ 15 µmol/L, H-type hypertension and 6-month MRS have significant correlations. And H-type hypertension is a risk factor for the prognosis of ischemic stroke. When hypertension and hyperhomocysteinemia coexist, there is a total sum of interaction.

[Construction of the HSV-1 strain HF amplicon and study on its unversal function between different HSV serotypes].

The study aimed to construct the amplicon vector of HSV-1 strain HF and explore its universal package function between different serotypes of HSV. OriS and pac elements were obtained by enzyme digestion from the Plasmid BAC-HSV-1 strain HF and sequenced. With red fluorescence (DsRed) as a reporter gene, the amplicon vector of HSV-1 strain HF was constructed based on pSilencer2.0-U6. The amplicon vector was transfected into Vero cells by lipofectamine 2000, then packaged by HSV-1 strain HF and HSV-2 strain HG52 as helper virus separately. The supernatant was collected after cytopathic effect. Red fluorescence was observed in Vero cells reinfected by the supernatant. In this study,the amplicon vector of HSV-1 strain HF was successfully constructed and it could be packaged by HSV-1 strain HF and HSV-2 strainHG52.

[Construction of a recombinant BAC-HSV-1 strain HF with a GFP reporter gene and characterization of its infectious progeny virus].

To construct the plasmid of BAC-HSV-1 with GFP reporter gene and research the biological property of its infectious progeny virus. We constructed the plasmid C223-UL43-left-arms-UL47-right-arms which carried the homologous sequences of HSV-1. Liposome embedding method was used to transfect HSV-1 genome and the plasmid C223-UL43-left-arms-UL47-right-arms linearized by Mlu I digestion into Vero cells. After the successful homologous recombination in the eukaryotic cells, the recombinant BAC-HSV-1 with GFP reporter gene was generated. Then, the positive CPE were taken by plaque purification and by hirt extraction during the moment of the circularization of HSV-1 DNA, and the plasmid of BAC -HSV-1 was acquired. Electroporation was used to transfect the BAC -HSV-1 into DH10B, and then the single colonies of interest were confirmed both by MluI digestion and PCR. Experimental group and the control group cells were given BAC-HSV-1 plasmid and HSV-1 genomic DNA respectively to produce the BAC-HSV-1 and HSV-1 progeny virions. Vero cells were inoculated with the progeny virions at MOI = 0.1 and then a TCID50 assay was performed to determine the titers of virons in the two groups at 48 hours post inoculation. The plasmid BAC-HSV-1 was successfully constructed by the restriction enzyme analysis and the PCR. The titers of progeny virions were calculated by the TCID50 assay. No significant difference in the titers of virions between two groups was observed (P > 0.05). The infectious BAC-HSV-1 shuttle virus/plasmid between eukaryotic and prokaryotic cells was successfully constructed.