【摘要】 目的 探讨脑后部可逆性脑病综合征（posterior reversible encephalopathy syndrome，PRES）的临床及MRI表现，提高对PRES的认识。 方法 回顾性分析2007年1月-2010年12月期间5例PRES患者的MRI资料并进行追踪随访。5例PRES患者中男1例，女4例，肾性高血压及产后子痫各1例、妊娠高血压3例。5例均行MRI多序列平扫检查及弥散加权成像（diffusion weighted imaging，DWI），其中4例同时行磁共振血管造影（magnetic resonance angiography，MRA），2例行MRI增强检查。 结果 5例发病时均有高血压，以突发头痛、子痫或癫痫发作、意识障碍及视觉障碍为临床特点，及时正确治疗后症状于3~5 d左右消失，1例遗留肢体功能障碍。MRI显示5例多发病灶主要位于双侧顶枕叶皮质下白质内，额叶及颞叶后部各2例，两侧小脑及脑干1例，皮质受累2例，病变呈长T1、长T2信号、液体衰减反转恢复序列呈高信号，5例患者病灶DWI图呈高或等信号，表观弥散系数（apparent diffusion coefficient，ADC）图呈略高信号；1例顶叶及1例基底节区部分病灶DWI图呈高信号，ADC图呈低信号，提示弥散受限。2例增强无强化，脑膜呈线状强化。随访MRI显示3例病灶完全消失。1例左侧基底节区部分病灶发展为脑梗死。 结论 PRES是一种临床-影像综合征，正确认识这一综合征对其早期诊断和治疗具有非常重要的意义。MRI能够提供较为可靠明确的诊断，其扩散成像对判断PRES预后亦很有价值。【Abstract】 Objective To investigate the clinical features and MRI manifestations of patients with posterior reversible encephalopathy syndrome (PRES), in order to improve its recognition. Methods From January 2007 to December 2010, MRI data of 5 patients with PRES were retrospectively analyzed and the patients were followed up. Among them, one was male and four were female. There were one case of postpartum eclampsia, one of renal hypertention, and 3 of pregnancy-induced hypertension. All the five patients were examined by MRI diffusion weighted imaging (DWI), among whom four underwent additional magnetic resonance angiography (MRA) and one underwent enhanced MRI. Results All the five patients with PRES had hypertension and their clinical symptoms were characterized by sudden occurrence of headache, eclampsia or seizure of epilepsy, altered mental status, and visual disturbances. Clinical symptoms vanished about 3-5 days after prompt and appropriate treatment except one case of residual body function failure. MRI scanning demonstrated multifocal subcortical white lesions in bilateral parieto-occipital lobes in five patients, in bilateral frontal lobes in two patients, in bilateral post temporal lobes in two patients, in bilateral cerebellum in one patient, and in bilateral brainstem in one patient, and cortical involvement occurred in one patient. Lesions appeared as hyperintense signals on fluid attenuated inversion recovery (FLAIR) and T2-weighted images, hypointense signals on T1-weighted images, normal or increased intensity on DWI, and mild hypointensity on apparent diffusion coefficient (ADC) maps. Some lesions in parietal lobes or basal ganglia showed hyperintensity on DWI and isointensity on ADC maps. The lesions in two patients appeared unenhanced with gadolinium enhancement and the meninges assumed the linear strengthening. Follow-up MRI showed that foci in three patients vanished completely, and the focus in the left basal ganglia in one patient developed into cerebral infarction. Conclusions PRES is a clinical-neuroradiological transient condition. Correctly understanding this syndrome is meaningful for its early diagnosis. MRI can provide a more reliable diagnosis, and its proliferation image formation is also valuable in predicting the PERS prognosis.
Objective To explore the correlation of Gli1 expression with clinical characteristics and prognosis of liver cancer. Methods Such Databases as PubMed, Embase, CNKI, Wanfang Database and Chinese Biomedical Literature Database were searched to collect cohort studies, which has published on the correlation between the expression of Gli1 and the clinical pathologic features of liver cancer and its prognostic value from the establishment of the databases to December 2016. Results Twelve studies with a total of 831 patients were included in this study. The high expression of Gli1 was associated with the tumor diameter >5 cm [relative risk (RR)=1.39, 95% confidence interval (CI) (1.08, 1.79)], clinical stage [RR=1.26, 95%CI (1.05, 1.51)], intrahepatic metastasis [RR=1.39, 95%CI (1.06, 1.83)] and venous invasion [RR=1.44, 95%CI (1.01, 2.04)], but the correlation of Gli1 expression was not significant with gender, histological differentiation, tumor diameter >3 cm, hepatitis B virus, cirrhosis and alpha fetoprotein. Meanwhile, the results of Meta-analysis showed that the higher Gli1 expression in liver cancer patients had a worse 3-/5-year overall survival rate and overall survival rate than those of the lower Gli1 expression group [3-year, RR=3.38, 95%CI (2.01, 5.67); 5-year, RR=1.51, 95%CI (1.19, 1.91); overall survival, RR=1.60, 95%CI (1.11, 2.30)]. And the higher Gli1 expression in liver cancer patients had a worse disease free survival rate than that in the lower Gli1 expression group [RR=1.89, 95%CI (1.35, 2.66)]. Conclusion The high expression of Gli1 is associated with poor prognostic outcome in liver cancer, and may be used as an important prognostic marker for patients with liver cancer.
Objective To propose the terminology of acoustic hypersensitivity, and investigate its clinical features and relationship with tinnitus. Methods A total of 214 patients with acoustic hypersensitivity or tinnitus as their first chief complaint were recruited and studied between January 2014 to January 2016. Detailed information of clinical manifestations, accompanying symptoms and related medical history were collected in the patients with acoustic hypersensitivity. Patients were instructed to complete the Hyperacusis Questionnaire and the Self-rating Anxiety Scale. The Tinnitus Evaluation Questionnaire was used to evaluate tinnitus severity in patients with tinnitus. Results Among the patients with acoustic hypersensitivity as their first chief complaint, 93.3% had tinnitus; 47.3% of the patients with tinnitus as their first chief complaint had acoustic hypersensitivity and the prevalence of acoustic hypersensitivity increased as the tinnitus severity increased. In terms of onset of the two symptoms, simultaneous acoustic hypersensitivity and tinnitus occurred in 55.1% of the patients, acoustic hypersensitivity occurred after tinnitus in 34.7% of the patients, and acoustic hypersensitivity occurred before tinnitus in 10.2% of the patients. Most patients with acoustic hypersensitivity as the first chief complaint felt uncomfortable to any sounds that are louder than usual. The main manifestations included feeling disturbed, echoing in the ear or head, ear fullness or pain. Discomfort in 68.6% of the patients disappeared when there was no environmental sound. It was found that hearing loss, ear fullness, vertigo, and anxiety were usually present in patients with acoustic hypersensitivity, and 28.6% of the patients with acoustic hypersensitivity had a history of noise exposure. Conclusions Acoustic hypersensitivity occurs together with tinnitus for the majority of time, which shows a close relationship between these two symptoms. However, acoustic hypersensitivity shows different clinical manifestations from tinnitus. It is important to unify the terminology and standardize the concept of acoustic hypersensitivity among clinicians. It is also critical to conduct more clinical research in terms of diagnosing and evaluating acoustic hypersensitivity.
ObjectiveTo analyze the clinical characteristics and geographical distribution of Keshan disease in Chongqing city for prevention and disease control. MethodsWe collected the clinical data of patients with Keshan disease from 2008 to 2012 in Liangping, Shizhu, Fengdu and Dianjiang counties as well as Wanzhou district of Chongqing city including the medical history, physical examination, results of laboratory tests to analyze the clinical characteristics and geographical distribution. ResultsFifty-eight patients were included from Liangping (n=21), Shizhu (n=25), Fengdu (n=11) and Dianjiang (n=1). The number of patients with potential and chronic Keshan disease was 16 and 42, respectively. The average age of patients was 54.91±15.53 years. The proportion above age 60 was 32.76% and below age 10 was 3.45%. The patients had main clinical signs as heart enlargement (36.76%), low-weak first heart sound (22.41%), systolic murmur (10.34%), arrhythmia (8.62%), etc. Abnormal ECG detection rate was 98.28%, with common types followed by sinus rhythm (37.93%), complete right bundle branch block (25.86%), ST-T changes (24.14%), left ventricular hypertrophy (15.52%), atrial fibrillation (13.79%), occasional ventricular premature (10.34%), T changes (10.34%), sinus bradycardia (8.62%), and incomplete right bundle branch block (6.90%). X-ray results showed that heart enlargement accounted for 82.76%. The ratios of mild, moderate and significant expansion of the heart were 46.55%, 27.59%, and 8.62%, respectively. ConclusionIn recent years, most patients with Keshan disease in Chongqing are chronic type at older age. The main clinical symptom is heart enlargement with high abnormal ECG detection rate.
目的 总结多发性骨髓瘤（multiple myeloma, MM）的发病特征及临床特征，分析比较不同治疗方案对MM的疗效及不同类型与不同临床特征的MM治疗效果。 方法 回顾性分析2003年1月-2008年1月128例MM患者的发病和临床特征，以及与治疗效果的关系，并对不同治疗方案、不同类型间的疗效进行比较。 结果 MM患者发病的中位年龄为59岁，临床上以不明原因的骨痛、贫血、感染和球蛋白增高为主要表现。128例患者中行方案一（马法兰、强的松/地塞米松、反应停）的总有效率为53.3%（32/60）；方案二（环磷酰胺、长春新碱、马法兰、强的松、卡氮芥、阿霉素）为44.4%（8/18）；方案三（长春新碱、阿霉素/表阿霉素/脂质体阿霉素、地塞米松）为68.5%（24/35），方案四（硼替佐米、地塞米松/反应停）的总有效率为86.6%（13/15）。方案一和方案二间、方案三和方案四间疗效差异无统计学意义（Pgt;0.05），方案三、方案四的疗效均优于方案一和方案二（Plt;0.05）。IgG型总有效率为63.2%（48/76），IgA型为60.9%（14/23），kappa轻链型为42.8%（6/14），lammda轻链型为46.2%（6/13）。IgG型和IgA型间的疗效差异无统计学意义（Pgt;0.05），但IgG型、IgA型的疗效均优于kappa轻链型和lammda型（Plt;0.05）。不同类型及使用不同方案的患者，其中位生存期及3年和5年的生存率差异无统计学意义（Pgt;0.05）。 结论 MM患者发病高峰年龄介于40～70岁，骨痛和贫血是最常见的首发症状。长春新碱、阿霉素/表阿霉素/脂质体阿霉素、地塞米松以及硼替佐米、地塞米松/反应停方案总体疗效相当，但后者完全缓解率高于前者。
ObjectiveTo explore the epidemiological characteristics, clinical manifestations, laboratory examination and imaging findings of patients with coronavirus disease 2019 (COVID-19) outside Hubei Province in 2019.MethodsFrom January 21, 2020 to February 18, 2020, 17 patients with COVID-19 diagnosed by SARS-CoV-2 nucleic acid test in Xianyang Central Hospital of Shaanxi Province and 23 patients in Liaocheng Infectious Disease Hospital of Shandong Province were collected. The information of epidemiology, age, time of onset, first symptom, white blood cell, lymphocyte count, procalcitonin (PCT), serum amyloid protein A (SAA), C-reactive protein (CRP) and chest CT were collected and analyzed.Results40.0% of the 40 patients had a clear history of contact with Wuhan or other areas of Hubei, 60.0% had no clear exposure history, 60.0% were aggregative diseases, 40.0% were imported patients; the average visit time was (4.9±3.5) d, 35% of the patients had underlying diseases; 75.0% of the patients had fever, 7.5% of the initial symptoms were cough, 5.0% pharyngitis, 2.5% headache, and 10.0% were asymptomatic. In the laboratory examination, 77.5% of the patients' white blood cells were normal, 45.0% of the patients' lymphocyte count could be decreased, 72.5% and 55.0% of the patients' SAA and CRP increased respectively, 92.5% of the patients' PCT was normal, 92.5% of the patients were positive for the first nucleic acid test, and 72.5% of the patients' chest CT showed multiple ground glass lesions in one or both lungs, and the positive coincidence rate between the initial nucleic acid test and the chest CT test was 92.5%. 90% of the patients in the group were common type, and all patients were treated with aerosol inhalation of α-interferon combined with lopinavir/ritonavir tablets, 62.5% were treated with antibiotics and 15.0% were treated with glucocorticoid. All patients were improved and 14 patients were cured and discharged from hospital.ConclusionsSARS-COV-2 is highly contagious, family aggregation infection and asymptomatic infection may be the main mode of transmission outside Hubei Province, mild and common types are common, there are no specific changes in laboratory examination, and the condition is stable after treatment.
Objective To describe the disease characteristics of osteonecrosis of the femoral head (ONFH) in patients with systemic lupus erythematosus (SLE) who experiencing prolonged glucocorticoid (GC) exposure. Methods Between January 2016 and June 2019, 449 SLE patients meeting the criteria were recruited from multiple centers. Hip MRI examinations were performed during screening and regular follow-up to determine the occurrence of ONFH. The cohort was divided into ONFH and non-ONFH groups, and the differences in demographic baseline characteristics, general clinical characteristics, GC medication information, combined medication, and hip clinical features were compared and comprehensively described. ResultsThe age at SLE diagnosis was 29.8 (23.2, 40.9) years, with 93.1% (418 cases) being female. The duration of GC exposure was 5.3 (2.0, 10.5) years, and the cumulative incidence of SLE-ONFH was 9.1%. Significant differences (P<0.05) between ONFH and non-ONFH groups were observed in the following clinical characteristics: ① Demographic baseline characteristics: ONFH group had a higher proportion of patients with body mass index (BMI)<20 kg/m2 compared to non-ONFH group. ② General clinical characteristics: ONFH group showed a higher proportion of patients with cutaneous and renal manifestations, positive antiphospholipid antibodies (aPLs) and anticardiolipin antibodies, severe SLE patients [baseline SLE Disease Activity Index 2000 (SLEDAI-2K) score ≥15], and secondary hypertension. Fasting blood glucose in ONFH group was also higher. ③ GC medication information: ONFH group had higher initial intravenous GC exposure rates, duration, cumulative doses, higher cumulative GC doses in the first month and the first 3 months, higher average daily doses in the first 3 months, and higher proportions of average daily doses ≥15.0 mg/d and ≥30.0 mg/d, as well as higher full-course average daily doses and proportion of full-course daily doses ≥30.0 mg/d compared to non-ONFH group. ④ Combined medications: ONFH group had a significantly higher rate of antiplatelet drug use than non-ONFH group. ⑤ Hip clinical features: ONFH group had a higher proportion of hip discomfort or pain and a higher incidence of hip joint effusion before MRI screening than non-ONFH group. Conclusion The incidence of ONFH after GC exposure in China’s SLE population remains high (9.1%), with short-term (first 3 months), medium-to-high dose (average daily dose ≥15 mg/d) GC being closely associated with ONFH. Severe SLE, low BMI, certain clinical phenotypes, positive aPLs, and secondary hypertension may also be related to ONFH.
ObjectiveTo analyze the microbiological characteristics of airway bacteria in adult patients with bronchiectasis and to analyze their correlation with the clinical features. MethodsPatients diagnosed with bronchiectasis in the Department of Respiratory and Critical Care Medicine of West China Hospital of Sichuan University from October 2017 to April 2018 were classified into the bronchiectasis group, while the control group was those who were found to have pulmonary nodules (diameter less than 10 mm) requiring bronchoscopy by physical examination. All subjects in both groups had not used antibiotics or hormones within 4 weeks and had no other respiratory diseases. Bronchoalveolar lavage fluid (BALF) from the lesion site of the branchial expansion group was collected, and BALF from the basal segment of the contralateral inferior bronchial lobe of the pulmonary nodule was collected in the control group. Bacterial culture and 16S rRNA gene sequencing were performed in both groups. ResultsSeventeen cases and six controls were enrolled in this study and the BALF specimens were collected. Eight cases were in stable period and nine cases were in acute period. The case group was divided into the bacteria-positive group and negative group based on bacterial culture of BALF. Shannon index in the bacteria-positive group was significantly lower than the bacteria-negative group and the control group. And Shannon index showed a negative correlation with positive bacterial culture in BALF. When Shannon index ≤4.5 was used to predict positive bacterial culture, the sensitivity and specificity were 83.3% and 90.9% respectively. The average relative abundance of bacteria was higher and the average sample distribution uniformity was lower in patients with acute period, compared with those in patients with stable period. Shannon index was negatively correlated with the acute exacerbation in patients. When Shannon index <5.0 was used to predict acute exacerbation, the sensitivity and specificity were 77.8% and 100.0%, respectively. ConclusionsShannon index in 16S rRNA gene sequencing results has certain predictive value for acute exacerbation stage. 16S rRNA gene sequencing combined with bacterial culture results can help guide clinicians to provide more precise treatment plans.
Objective To summarize the clinical characteristics of interstitial pneumonia with autoimmune features (IPAF). Methods The interstitial lung disease (ILD) patients diagnosed in our department between January 2010 and August 2013 were retrospectively analyzed to screen out the patients with IPAF.The clinical manifestations, laboratory examination, imaging, pulmonary function and treatment were summarized. Results In 254 ILD patients, 25 patients met the diagnosis criteria of IPAF, and 26 patients were diagnosed with definite connective tissue diseases associated ILD (DCTD-ILD). There were differences in arthralgia, sicca symptoms, mechanic’s hand, positive antinuclear antibodies, anti-CCP antibodies and residual lung volume between the IPAF patients and the DCTD-ILD patients (all P < 0. 05). Five IPAF patients were revealed hug or “pancake” the diaphragm in their chest high resolution CT radiographs. The microscopic performance showed that diffuse thickened with collagen fiber, alveolar wall thickening with marked interstitial lymphocyte inflammatory cells infiltration, and granulation tissue that filled bronchiolar lumina. The patients were pathologically diagnosed with nonspecific interstitial pneumonia (NSIP) overlap organized pneumonia (OP). During following-up, the progression-free survival time of the IPAF patients was significant longer and that of the DCTD-ILD patients [(14.32±5.74)months vs. (10. 31± 3. 70) months, P < 0. 05]. Conclusions If an ILD patient has mechanic’s hand, positive antinuclear antibodies or NISP overlap OP in image, the diagnosis of IPAF should be considered. IPAF have slower disease progression and better prognosis than DCTD-ILD.