ABSTRACT
BACKGROUND@#The clinical features of enthesitis in patients with psoriatic arthritis (PsA) have been reported in some Western countries, but data in China are very limited. This study aimed to describe the characteristics of enthesitis in Chinese patients with PsA and compared them with those in other cohorts.@*METHODS@#Patients with PsA enrolled in the Chinese Registry of Psoriatic Arthritis (CREPAR) (December 2018 to June 2021) were included. Data including demographics, clinical characteristics, disease activity measures, and treatment were collected at enrollment. Enthesitis was assessed by the Spondyloarthritis Research Consortium of Canada (SPARCC), Maastricht ankylosing spondylitis enthesitis score (MASES), and Leeds enthesitis index (LEI) indices. A multivariable logistic model was used to identify factors related to enthesitis. We also compared our results with those of other cohorts.@*RESULTS@#In total, 1074 PsA patients were included, 308 (28.7%) of whom had enthesitis. The average number of enthesitis was 3.3 ± 2.8 (range: 1.0-18.0). More than half of the patients (165, 53.6%) had one or two tender entheseal sites. Patients with enthesitis had an earlier age of onset for both psoriasis and arthritis, reported a higher proportion of PsA duration over 5 years, and had a higher percentage of axial involvement and greater disease activity. Multivariable logistic regression showed that axial involvement (odds ratio [OR] 2.21, 95% confidence interval [CI], 1.59-3.08; P <0.001), psoriasis area and severity index (PASI) (OR: 1.03, 95% CI: 1.01-1.04; P = 0.002), and disease activity score 28-C reactive protein (DAS28-CRP) (OR: 1.25, 95% CI: 1.01-1.55; P = 0.037) were associated with enthesitis. Compared with the results of other studies, Chinese patients with enthesitis had a younger age, lower body mass index (BMI), a higher rate of positive human leukocyte antigen (HLA)-B27, more frequent dactylitis, and a higher proportion of conventional synthetic disease-modifying antirheumatic drugs' (csDMARDs) use.@*CONCLUSIONS@#Enthesitis is a common condition among Chinese patients with PsA. It is important to evaluate entheses in both peripheral and axial sites.
Subject(s)
Humans , Arthritis, Psoriatic/drug therapy , East Asian People , Enthesopathy/complications , Registries , Severity of Illness Index , Spondylarthritis/epidemiologyABSTRACT
Objective:To evaluate the safety of thymic surgery in patients aged 65 years and over.Methods:A total of 696 patients who underwent thymectomy/thymoma resection in the Department of Thoracic Surgery of Beijing Hospital from November 2011 to March 2022 were collected and divided into two groups according to the age of 65 years old.The preoperative course of disease, MG stage, dosage of pyridostigmine bromide, American College of Anesthesiologists(ASA)score, surgical method, intraoperative bleeding, postoperative drainage, postoperative complications, Clavien-dindo score(CDC), and myasthenic crisis were recorded and statistically analyzed.Results:A total of 696 patients were enrolled, including 364 males and 332 females, aged 15~86 years, with an average age of 49.1 years.There were 309 patients with thymoma, 565 patients with MG, and 178 patients with both.There were 124 cases in the elderly group(≥65 years old)and 572 cases in the non-elderly group(<65 years old). The incidence of thymoma was higher in the elderly group(54.8 % versus 42.1 %, χ2=6.664, P=0.010), while the incidence of MG was lower(67.7 % versus 84.1 %, χ2=17.827, P<0.001). The ASA score of the elderly group was higher than that of the non-elderly group( χ2=52.372, P=0.000), and the preoperative ventilation function FEV1 and FEV1/FVC were also significantly lower than those of the non-elderly group( z=8.187, 4.580, P=0.000 for all). The drainage volume in the first 3 days after operation and postoperative drainage tube time in the elderly group were significantly higher than those in the non-elderly group( P=0.018, P=0.003). The incidence of postoperative myasthenia crisis in the elderly group was higher than that in the non-elderly group( P=0.034). There was no significant difference in the incidence of postoperative complications between the two groups, but after Clavien-dindo classification, the score of the elderly group was higher than that of the non-elderly group( P=0.003). Although the ASA score and Clavien-dindo score of the elderly group were both higher than those of the non-elderly group, there was no correlation between the two. Conclusions:Although the preoperative ASA score and pulmonary function of elderly patients were poorer than those in the non-elderly group, while the incidence of postoperative myasthenia crisis was higher, and the incidence of postoperative complications was not higher, the Clavien-dindo classification, however, was higher in elderly patients than that of the non-elderly group.After careful preoperative evaluation and strengthening perioperative management, most elderly patients can receive thymus surgery safely with acceptable risks.
ABSTRACT
Objective:To proceed a comparative study of characteristics and safety of video-assisted thoracoscopic thymectomy between artificial pneumothorax combining subxiphoid-costal margin approach versus right thoracic cavity approach.Methods:From January 2018 to December 2021, 251 patients(including myasthenia gravis with thymic hyperplasia or thymoma, thymic cysts and anterior mediastinal occupying lesions)undergoing thoracoscopic extended thymectomy in our department were retrospectively studied, and their clinical data were collected.The surgical methods were divided into artificial pneumothorax combining xiphoid costal margin approach(artificial pneumothorax group, n=165)and via right thoracic approach(right thoracic group, n=86). Clinical data such as preoperative information, operative duration, blood loss, postoperative drainage, drainage duration, and surgical complication were compared between two groups.Results:There were no statistical differences in age, gender and pathology between two groups(all P>0.05). Compared with the right thoracic group, operative duration[(108.6±45.2)min vs.(127.6±42.1)min, t=-3.628, P=0.000], intraoperative blood loss[(37.9±131.7)ml vs.(107.4±284.8)ml, t=-8.215, P=0.000], postoperative drainage volume[(379.0±285.5)ml vs.(646.6±373.3 ml), t=-6.277, P=0.000]and drainage duration[(2.2±1.0)d vs.(3.1±1.0)d, t=-7.275, P=0.000]were statistically significantly decreased in the artificial pneumothorax group.No significant difference was found(all P>0.05)in phrenic nerve injury(1/165 vs.0/86, P=1.000), myasthenia crisis(3/89 vs.2/66, χ2=0.014, P=0.906), and the conversion to thoracotomy(3/165 vs.2/86, P=1.000). Conclusions:VATS thymectomy by artificial pneumothorax combining subxiphoid-costal margin approach is a safer method, having a less trauma, less bleeding and less incidence rate of complication.Especially, it is more suitable for elderly female patients who cannot be intubated in double lumen, cannot tolerate one-lung ventilation, and have pulmonary insufficiency.
ABSTRACT
Objective:To evaluate the clinical characteristics and the surgical safety in patients aged 65 years and over with thymic epithelial tumor, and analyze the prognosis of 3-10 years postoperative tumor and myasthenia gravy.Methods:A total of 228 patients diagnosed as thymic tumor and undergoing surgical surgery to remove the tumor in Beijing Hospital from Jan.2011 to Dec.2018 were retrospectively enrolled.Patients were divided into a young(≤ 65 yrs)and old(>65 yrs)groups.The operation time, intra-operative bleeding, drainage volume in the first 3 days after operation, days with drainage tube after operation, postoperative days of hospital stays, the diameter of the tumors, pathological classification, Tumor-Node-Metastasis(TNM)staging, Masaoka-Koga staging, whether or not complicated with myasthenia gravis and complications were compared between two groups.The patients were followed up by outpatient or telephone, and recurrence of thymoma, survival status and improvement of myasthenia gravis were tracked.Results:There were significant differences in pathological classification between the two groups( P=0.002). The postoperative days with drainage tube were longer in patients≥65 years old than in patients<65 years old[4(2-17)days and 3(1-9), Z=2.316, P=0.021]. Thymic atrophy was more common in patients ≥ 65 years old than in patients <65 years old(10.2% and 1.7%, χ2=5.937, P=0.015). Incidence of thymoma plus myasthenia gravis were higher in patients aged <65 years than those aged ≥65years(68.2% vs.40.8%, χ2=12.240, P<0.001), and incidence of thymic hyperplasia were higher in patients aged <65 years than those aged ≥65years(58.1% and 38.8%, χ2=2.316, P=0.016). The recurrence of thymoma was a poor prognostic factor affecting the survival of patients.Meanwhile, Masaoka-Koga stage Ⅲ and Ⅳ and TNM stage Ⅲ and Ⅳ were independent risk factors for postoperative recurrence of thymoma. Conclusions:Thymectomy is safe and effective in the patients aged 65 and over, and may have a better long-term prognosis.
ABSTRACT
Idiopathic Pulmonary fibrosis (IPF) is a chronic, progressive, fibrotic interstitial lung disease with unknown cause, which is closely related to lung cancer. A serious complication called Acute exacerbation of IPF (AE-IPF) is prone to occur after lung resection. It progresses rapidly without effective treatment and has a poor prognosis. A typical case of AE-IPF after lung cancer surgery was reported, and its clinical characteristics, imaging features, diagnosis and treatment were summarized. .
Subject(s)
Humans , Disease Progression , Idiopathic Pulmonary Fibrosis/surgery , Lung Neoplasms/surgery , Treatment OutcomeABSTRACT
Objective@#To summarize the clinical features, diagnosis and treatment of postoperative acute exacerbation of interstitial lung disease (ILD) after pneumonectomy.@*Methods@#The clinical data of 4 patients with postoperative acute exacerbation of ILD after pneumonectomy admitted in Beijing Hospital from October 2014 to November 2015 were retrospectively analyzed and related literatures were reviewed.@*Results@#Four patients were aged 60 to 74 years, including 3 males and 1 female, 2 of whom were idiopathic pulmonary fibrosis and 2 were connective tissue diseases related ILD. Chest high resolution computed tomography (HRCT) showed multiple lobular septal thickening, ground glass opacities, reticular shadow and strip shadow in both lungs. The patients developed acute dyspnea 2 to 3 days after pneumonectomy. In 2 cases, HRCT showed new patchy ground-glass opacities, reticular shadow and strip shadow in both lungs on the basis of preoperative pulmonary fibrosis. The sputum smear and culture showed no pulmonary infection in all 4 cases. Three patients were treated with glucocorticoids and 3 received mechanical ventilation with endotracheal intubation. One patient was successfully treated by early using adequate glucocorticoids, one patient improved after treatment but died after re-exacerbation, and the other two patients died after treatment failed.@*Conclusions@#Acute exacerbation of ILD after pneumonectomy often occurs in early postoperative period and it is characterized by progressive dyspnea. Chest CT and respiratory pathogen examination are helpful for early diagnosis. Postoperative acute exacerbation of ILD often lacks effective treatment, requires mechanical ventilation, and the prognosis is poor. Although glucocorticoids may be effective, re-exacerbation should be alert during treatment.
ABSTRACT
The 21-year-old male patient was admitted to the Department of Rheumatology and Immunology at Peking Union Medical College Hospital with chief complaints of "skin rash for 1 year and edema for 2 months". He was diagnosed with systemic lupus erythematosus (SLE) with renal, cardiac and hematological involvement. Remission was not achieved after glucocorticoid pulse treatment. The patient experienced oliguria, malignant hypertension, accompanied by thrombocytopenia and low serum complements, and elevated lactate dehydrogenase and serum creatinine. Schistocytes were seen in the peripheral blood smear. Thrombotic microangiopathy (TMA) secondary to SLE was diagnosed. Though plasma exchange was partially effective, TMA could not be controlled yet. The activity of serum von Willebrand factor -cleaving protease (ADAMTS 13) was 100%, and ADAMTS 13 inhibitor was negative. Finally, remission of the disease was achieved after second glucocorticoid pulse therapy and rituximab treatment. At the 3-month follow-up, the patient′s condition was stable with mild anemia and normal platelet count. Patients with TMA secondary to SLE are heterogenous, while normal ADAMT 13 activity indicates poor prognosis. Early and aggressive treatment is important for disease control, and plasma exchange is helpful as a supportive care.
ABSTRACT
The 21-year-old male patient was admitted to the Department of Rheumatology and Immunology at Peking Union Medical College Hospital with chief complaints of "skin rash for 1 year and edema for 2 months". He was diagnosed with systemic lupus erythematosus (SLE) with renal, cardiac and hematological involvement. Remission was not achieved after glucocorticoid pulse treatment. The patient experienced oliguria, malignant hypertension, accompanied by thrombocytopenia and low serum complements, and elevated lactate dehydrogenase and serum creatinine. Schistocytes were seen in the peripheral blood smear. Thrombotic microangiopathy (TMA) secondary to SLE was diagnosed. Though plasma exchange was partially effective, TMA could not be controlled yet. The activity of serum von Willebrand factor -cleaving protease (ADAMTS 13) was 100%, and ADAMTS 13 inhibitor was negative. Finally, remission of the disease was achieved after second glucocorticoid pulse therapy and rituximab treatment. At the 3-month follow-up, the patient′s condition was stable with mild anemia and normal platelet count. Patients with TMA secondary to SLE are heterogenous, while normal ADAMT 13 activity indicates poor prognosis. Early and aggressive treatment is important for disease control, and plasma exchange is helpful as a supportive care.
ABSTRACT
Objective:To summarize the clinical features, diagnosis and treatment of postoperative acute exacerbation of interstitial lung disease (ILD) after pneumonectomy.Methods:The clinical data of 4 patients with postoperative acute exacerbation of ILD after pneumonectomy admitted in Beijing Hospital from October 2014 to November 2015 were retrospectively analyzed and related literatures were reviewed.Results:Four patients were aged 60 to 74 years, including 3 males and 1 female, 2 of whom were idiopathic pulmonary fibrosis and 2 were connective tissue diseases related ILD. Chest high resolution computed tomography (HRCT) showed multiple lobular septal thickening, ground glass opacities, reticular shadow and strip shadow in both lungs. The patients developed acute dyspnea 2 to 3 days after pneumonectomy. In 2 cases, HRCT showed new patchy ground-glass opacities, reticular shadow and strip shadow in both lungs on the basis of preoperative pulmonary fibrosis. The sputum smear and culture showed no pulmonary infection in all 4 cases. Three patients were treated with glucocorticoids and 3 received mechanical ventilation with endotracheal intubation. One patient was successfully treated by early using adequate glucocorticoids, one patient improved after treatment but died after re-exacerbation, and the other two patients died after treatment failed.Conclusions:Acute exacerbation of ILD after pneumonectomy often occurs in early postoperative period and it is characterized by progressive dyspnea. Chest CT and respiratory pathogen examination are helpful for early diagnosis. Postoperative acute exacerbation of ILD often lacks effective treatment, requires mechanical ventilation, and the prognosis is poor. Although glucocorticoids may be effective, re-exacerbation should be alert during treatment.
ABSTRACT
Objective:To compare the surgical effect of single-utility port and three port video-assisted thoracoscopic surgery(VATS) for patients of thoracic esophageal cancer.Methods:Patients who underwent McKeown surgery with single-utility port and three port VATS in Department of Thoracic Surgery at Beijing Hospital from January 2013 to December 2018 were enrolled. During the thoracic procedure, patients were placed forwardly in left lateral decubitus position. The video port was made at the eighth mid-axillary intercostal space, and the single-utility port was at the fourth intercostal space between antero-axillary and mid-axillary line. For three port VATS, another incision was made at the eighth posto-axillary intercostal space. The posterior mediastinum was exposed by the "suture-drawing exposure" method, then the thoracic esophagus was carefully dissected and mediastinal lymphadenectomy was done. Information was collected including demographic, perioperative and pathological data. Patients were followed up by telephone or outpatient record.Results:A total of 62 patients of single-utility port VATS and 28 three port VATS were included. All cases were accomplished by VATS, and none of them were converted to thoracotomy or adding additional ports. Sex proportion and ages of the two groups were comparable( P>0.05). The surgery duration[(336.9±55.7) min vs.(319.7±66.3)min, P=0.205] and thoracic procedure duration[(112.5±36.7)min vs.(108.9±43.6)min, P=0.686] of two groups were also similar. There were no statistical differences in volume of drainage, postoperative stay, thoracic complications, number of lymph nodes dissected, and survival after surgery between two groups( P>0.05). Peripheral blood oxygen saturation in first 3 days after surgery of single-utility port VATS patients was significantly higher[ Day 1: (97.00±1.57)% vs.(96.10±1.23)%, P=0.009; Day 2: ( 96.60±1.84 )% vs.(95.70±1.19)%, P=0.020; Day 3: (97.40±1.56)% vs.(96.30±1.42)%, P=0.002] and respiratory rate was significantly lower[(19.70±1.48) times/min vs.(20.70±1.70) times/min, P=0.006]than three-port VATS patients. Conclusion:Single-utility port VATS for McKeown surgery is safe and feasible, which tends to reduce the surgical trauma and can be a routine surgical procedure for minimally invasive esophagectomy.
ABSTRACT
Objective:To discuss the clinical features and treatment of 19 patients with granulomatosis with polyangiitis (GPA) complicated with hypertrophic cranial pachymeningitis (HCP).Methods:The clinical features of 19 patients diagnosed with GPA complicated with HCP in Peking Union Medical College Hospital were retrospectively analyzed.Results:Among the 315 patients with GPA, 19 (12 males, 7 females, with) were diagnosed with HCP at Peking Union Medical College Hospital. The median age was 57 (19-64) years. In the neurological manifestations per se, all patients had headache, 16 patients had cerebral involvement, which included 8 cases at the frontal area, 8 cases at the temporal area, 8 cases at the skull base area (4 cases with parasellar involvements including 3 cases with cavernous sinus involvement and 2 cases with orbital involvement), 6 cases of tentorium involvement, 2 cases of cerebral palsy, 1 case of calvarium, 1 case of occipital, and 1 case with combined spinal pachymeningitis, respectively. In systemic manifestations, 10 patients had fever, 8 patients had weight lose, 4 patients had lung involvement, 3 patients had kidney involvement, 16 patients had nasosinusitis, 10 patients had tympanitis, and 16 patients had localized GPA. The laboratory tests showed that 15 patients had positive anti-neutrophil cytoplasmic antibodies (ANCA), including 8 cases with positive proteinase 3 (PR3)-ANCA and 6 cases with positive myeloperoxidase (MPO)-ANCA. Sixteen patients had lumbar puncture examination, 9 cases had elevated cerebrospinal pressure, 10 cases had elevated level of protein in cerebrospinal fluid. Nineteen patients were treated with glucocorticoids (12 patients accepted pulse therapy) and immunosuppressive agents. Twelve patients were treated with intrathecal injections of dexamethasone combined with or without Methotrexate (MTX). All 19 patients were improved.Conclusion:HCP, as a rare but serious manifestation of GPA, is not rare in active cases and should be intensively treated.
ABSTRACT
Objective:To compare the characteristics and therapeutic effects of video-assisted thoracoscopic(VATS)extended thymectomy by subxiphoid-costal margin approach versus right intercostal approach for the treatment of myasthenia gravis(MG).Methods:A retrospective analysis was conducted on 230 non-thymomatous MG patients undergone VATS extended thymectomy in our department from August 2015 to August 2019.According to the operation approach and method, patients were divided into two groups: the subxiphod-costal margin approach group(n=102)and the right intercostal approach group(n=128).Results:Intraoperative blood loss and the postoperative drainage were less in the subxiphod-costal margin approach group than in the right intercostal approach group[(30.3±25.2) ml vs. (45.1±30.6) ml, (178.6±90.5) ml vs.(205.4±87.6) ml, t=-3.935 and -2.27, P=0.003 and 0.024)]. Postoperative pain degree was lower in the subxiphod-costal margin approach group than in the right intercostal approach group [the Numerical Rating Scale(NSR)scores: 3.1±1.0 vs. 4.6±1.5, t=-8.677, P=0.001]. A difference in incidence rate of pore malunion also existed between the subxiphod-costal margin approach group and the right intercostal approach group, but it had no statistical significance(1/102 vs. 7/128, χ2=2.200, P=0.138). The complete remission rate and the overall effectiveness rate had no significant difference between the two groups(29.6% vs.32.4%, 85.7% vs. 84.7%, χ2=0.196 and 0.044, P=0.658 and 0.834). Conclusions:VATS extended thymectomy by subxiphoid-costal margin approach shows excellent safety and effectiveness in treating MG.It has advantages of low trauma and complications, and is particularly suited for elderly and female MG patients who are unable to receive double-lumen endotracheal intubation, are intolerant to one-lung ventilation or have pulmonary insufficiency.
ABSTRACT
BACKGROUND@#Interstitial lung disease (ILD) is a group of diffuse lung diseases that mainly involve the interstitial and alveolar cavities and result in loss of alveolar-capillary functional units, leading to restrictive ventilatory dysfunction and diffusion impairment. There was an increased incidence of lung cancer on the basis of ILD, and perioperative risk of patients with lung cancer combined with ILD (LC-ILD) was significantly increased. The aim of this study is to summarize the safety and experience of surgical treatment of LC-ILD.@*METHODS@#A retrospective analysis was performed on 23 patients with LC-ILD who underwent pneumonectomy in Beijing Hospital from January 2012 to December 2019, and their clinical manifestations, image feature, pathology, surgical safety, perioperative complications and treatment experience were summarized.@*RESULTS@#A total of 23 patients were included in this study, including 20 males (87.0%) with an average age of (69.1±7.8) years, and 19 cases (82.6%) were smokers. Of the ILD types, 14 cases (60.9%) were idiopathic pulmonary fibrosis, 7 cases (30.4%) were idiopathic nonspecific interstitial pneumonia, and 2 (8.7%) were interstitial lung disease associated with connective tissue diseases. The pathology of lung cancer included adenocarcinoma (30.4%, 7/23), small cell carcinoma (30.4%, 7/23), squamous cell carcinoma (26.1%, 6/23), small cell carcinoma mixed with squamous cell carcinoma (4.3%, 1/23) and large cell neuroendocrine carcinoma (8.7%, 2/23). Surgical approaches included video assisted thoracoscopy (69.6%, 16/23) and anterolateral thoracotomy (30.4%, 7/23), with lobectomy (52.2%, 12/23), double lobectomy (4.3%, 1/23), and sublobectomy (39.1%, 9/23). There were 11 cases (47.8%) of postoperative complications, including 8 cases (34.8%) of pulmonary complications, 4 cases (17.4%) of acute exacerbation of ILD (AE-ILD), 6 cases (26.1%) of atrial fibrillation, and 1 case (4.3%) of acute left ventricular dysfunction. The 90-day mortality is 8.7% (2/23) and the cause of death was acute exacerbation of ILD.@*CONCLUSIONS@#Most of LC-ILD were elderly patients with multiple comorbidities and decreased pulmonary function, leading to significantly increased surgical risk. The ILD should be fully evaluated and controlled before surgery, intraoperative trauma should be minimized, and special attention should be paid to pulmonary complications and AE-ILD after surgery. Postoperative AE-ILD has a poor prognosis and glucocorticoids may be effective. Early diagnosis and treatment is the key to treatment of AE-ILD.
ABSTRACT
A 28-year-old woman with multiple abscesses for 2 month and fever for 1 month was admitted in Peking Union Medical College Hospital.The skin abscesses gradually developed at skull,face,abdominal wall and pelvis.Laboratory examinations related to inflammatory reactions were strongly high including erythrocyte sedimentation rate 99 mm/1 h,C-reactive protein160.28 mg/L and ferritin 1 584 μg/L.Multiple nodules and cavities were detected in lungs.And vesico-cervical fistula was found during exploratory laparotomy.The pathological tests of abdominal tissues demonstrated necrosis and granuloma.Evidence of infectious diseases was not definite.Positive anti-proteinase 3 (PR3) antibody confirmed the diagnosis of granulomatosis with polyangiitis.After treated with glucocorticoid and immunosuppressants,the patient's symptoms improved remarkably.This case suggested that systemic vasculitis should be considered as a differential diagnosis of multiple abscesses which are not explained by infections.
ABSTRACT
BACKGROUND@#DNA polymerase β is one of the key enzymes for DNA repair and it was reported that about 30 percent of different types of cancers carried mutations in its coding gene Polb. However, it is still controversial whether it is true or false because of the small sample size in these studies. In current study, we performed genetic screening of promoter and coding regions of Polb gene in 69 Chinese lung cancer patients using Sanger sequencing method, so as to elucidate real mutation frequency of Polb mutations in Chinese Han population.@*METHODS@#Salting out extraction method was used to get the genome DNAs from tumor and normal matched tissues of 69 lung cancer patients. The promoter and 14 coding regions of Polb gene were then amplified using these DNAs as the template. After purification, amplicons were sequenced and aligned to the wild type Polb gene in NCBI database, in order to find out the mutated sites of Polb gene in Chinese lung cancer patients.@*RESULTS@#In this study, we totally found only 5 mutated sites in Polb gene. In detail, 3 mutations (-196G>T, -188_-187insCGCCC, -168C>A) were located in the promoter region; 2 mutations (587C>G, 612A>T) were found in coding regions. Specially, mutations of -188_-187insCGCCC and 587C>G (resulting to the amino acid substitution of Thr to Ser at position 196) had never been reported by other groups before. However, all these 5 mutated sites could be detected in both tumor and matched normal tissues, which inferred that they are not lung tumor specific mutations.@*CONCLUSIONS@#No lung tumor specific mutations of Polb gene could be found in Chinese lung cancer patients and Polb gene mutation might not be a molecular marker for Chinese lung cancer patients.
ABSTRACT
Fetal adenocarcinoma of the lung (FLAC) is an extremely rare subtype of lung cancer, accounting for only 0.1% to 0.5% of primary pulmonary malignancy. In 2011, international multidisciplinary classification of lung adenocarcinoma developed by the International Association for the Study of Lung Cancer (IALSC), the American Thoracic Society (ATS) and the European Respiratory Society (ERS) classified FLAC as a variant of invasive adenocarcinoma. FLAC has been further divided into low-grade fetal adenocarcinoma (L-FLAC) and high-grade fetal adenocarcinoma (H-FLAC) as these two categories exhibit different clinicopathological features and biological behaviors. Here we report a case of high-grade fetal adenocarcinoma and summarize clinicopathologic features of fetal lung adenocarcinoma. .
Subject(s)
Humans , Male , Middle Aged , Adenocarcinoma of Lung , Diagnostic Imaging , Pathology , General Surgery , Fetus , Neoplasm Grading , Prognosis , Tomography, X-Ray ComputedABSTRACT
Objective To investigate the value of antineutrophil cytoplasmic antibody (ANCA) in clinical phenotype of eosinophilic granulomatosis with polyangiitis (EGPA).Methods The clinical data of 64 patients with EGPA from Peking Union Medical College Hospital between 2007 to 2016 were retrospectively analyzed,and the patients were followed up.Characteristics of patients with ANCA positive and ANCA negative were compared by independent-samples t test,Mann-Whitney U test and Chi-square test.Results Among 64 patients with EGPA,12(19%) were serum ANCA positive and 52(81%) were negative.The incidence of fever (77% vs 35%,x2=9.403,P=0.002) and renal involvement,including proteinuria (67% vs 25%,x2=7.678,P=0.006),hematuria (58% vs 8%,x2=17.57,P<0.01),renal inadequacy (33% vs 4%,x2=9.978,P=0.002),and the BVAS score higher than 15 (92% vs 60%,x2=4.440,P=0.035) in ANCA positive group were higher than ANCA negative group,while the presence of allergic rhinitis (17% vs 56%,x2=5.969,P=0.015),mucocutaneous lesion (33% vs 65%,x2=4.152,P=0.042) and cardiac involvement (8% vs 44%,x2=3.361,P=0.021) in the ANCA-positive group was lower when compared with ANCA-negative patients.The positive ratio of rheumatoid factor (RF) (100% vs 42%,x2=7.723,P=0.006),and the level of erythrocyte sedimentation rate (ESR) (50 vs 35.5 mm/1 h,P=0.034) in ANCA-positive group were higher than in ANCA negative group.There was no significant difference in pathological characteristics between the two groups.According to the treatment and prognosis,there were no significant differences between the two groups in the usage and dosage of steroids and immunosuppressant,the remission rate and recurrence rate of the disease,and the death rate due to the primary disease.Conclusion The clinical manifestations of EGPA are complicate.Whether ANCA is positive or not may be related to the clinical phenotypes.More attention should be paid to renal involvement in ANCA positive patients while cardiac involvement in ANCA negative patients.
ABSTRACT
Objective The aim of this study was to evaluate the long-term efficacy and safety of tocilizumab (TCZ) in Chinese patients with Takayasu's arteritis (TA).Methods We retrospectively studied the active TA patients treated with TCZ from January 2014 to April 2016 in our hospital.The demographic and clinical data,laboratory tests and vascular images were collected.Doppler ultrasonography was used to track vascular changes during TCZ treatment.The data were analyzed by t test and rank sum test.The data were analyzed by Fisher exact test or Chi square test.Results Eighteen patients with a median age of 25.5 (range 18,47) were analyzed.Seventeen patients had taken a median of 3 (range 1,5) immune suppressants before TCZ therapy.Three patients withdrew TCZ after receiving 1 infusion each due to unbearable neck pain.The other 15 patients were treated with TCZ for a median of 13 (range 6,26) months.After TCZ treatment,the median ESR level,hsCRP level,mural thickness of common carotid artery and subclavical artery decreased from 45.5 (range 7-101) mm/1 h (Z=6.731,P<0.01),37.40 (range 7.6-163.12) mg/L,0.25 (range 0.06-0.59) cm,0.18 (range 0.07-0.47) cm to 7 (range 1-44) mm/l h (Z=6.731,P<0.01),0.94 (range 0.08-93.21) mg/L (Z=3.212,P=0.016),0.18 (range 0.04-0.53) cm (Z=4.873,P=0.001),and 0.12 (range 0.07-0.18) cm (Z=5.568,P< 0.01) respectively,with median glucocorticoid dosage being successfully tapered.One patient discontinued TCZ for unsatisfactory response after 6 TCZ infusions.One episode of urinary infection was recorded and relieved after antibiotic therapy.Neither neutropenia nor abnormal liver enzyme was observed.Conclusion Our study suggests that tocilizumab is a safe and effective agent for long term use among Chinese TA patients,especially for those refractory cases.
ABSTRACT
A 56-year-old female was admitted to the Department of Rheumatology,Peking Union Medical College Hospital with complaint of recurrent fever and acute lumbar pain.Fever was complicated with malaise,cough and occasional blood-streaked sputum.Lab tests showed elevated white blood cell count,increased serum creatinine,erythrocyte sedimentation rate and C-reactive protein.Other lab findings included severe anemia,hematuria,and proteinuria.Immunological examinations were positive for antinuclear antibodies,antineutrophil cytoplasmic antibodies and antiglomerular basement membrane antibody.Ultrasonography and CT scan detected a huge spontaneous perirenal hematoma at right side.Angiography revealed multiple microaneurysms on bilateral renal arteries and branches.A diagnosis of systemic vasculitis was suggested.Under the combination therapy of corticosteroids and cyclophosphamide,the patient presented sustained remission for one year.This case indicates that prompt and sufficient treatment of primary disease is essential to a promising outcome.
ABSTRACT
Objective To improve the recognition of Pancreatitis, Panniculitis, and Polyarthritis syndrome (PPP syndrome). Methods We described the diagnosis and treatment of a patient with PPP syndrome, including the clinical, radiological, and pathological features. Results Here we report a 67-year-old man with chronic calcified pancreatitis with multiple subcutaneous nodules and polyarthritis, but without any abdominal pain or other abdominal symptoms. His serum pancreatic amylase and lipase were markedly elevated. Abdomen CT scan showed features of chronic calcified pancreatitis. MR imaging of ankle revealed intramedullary fat necrosis. Biopsy from subcutaneous swellings revealed fat necrosis without vasculitis and typical "ghost-like cells". He failed to response to corticosteroids therapy. When the diagnosis of pancreatitis was confirmed, he was managed conservatively with supportive measures, and recovered. Conclusion The diagnosis of PPP syndrome is often difficult when abdominal symptoms are absent, be aware the association between panniculitis and polyarthritis with pancreatic disease may lead to a prompt diagnosis and management.