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1.
Diagnostics (Basel) ; 13(11)2023 Jun 05.
Artigo em Inglês | MEDLINE | ID: mdl-37296822

RESUMO

Mycobacterium kansasii infections predominantly manifest in immunocompromised people and are primarily responsible for lung disease and systemic disseminated infection. Osteopathy is a rare consequence of M. kansasii infection. Here, we present imaging data from a 44-year-old immunocompetent Chinese woman diagnosed with multiple bone destruction, particularly of the spine, secondary to M. kansasii pulmonary disease, which is easily misdiagnosed. The patient underwent an emergency operation after experiencing unexpected incomplete paraplegia during hospitalization, indicating an aggravation of bone destruction. Preoperative sputum testing and next-generation sequencing of DNA and RNA of intraoperative samples confirmed the diagnosis of M. kansasii infection. Treatment with anti-tuberculosis therapy and the subsequent patient response supported our diagnosis. Given the rarity of osteopathy secondary to M. kansasii infection in immunocompetent individuals, our case offers some insight into this diagnosis.

3.
Entropy (Basel) ; 24(8)2022 Jul 31.
Artigo em Inglês | MEDLINE | ID: mdl-36010725

RESUMO

Polar cruise ships are exposed to extreme external conditions during voyages, resulting in cockpit windscreens that are prone to fogging and frosting, seriously affecting the driver's vision and even threatening navigation safety. However, the current research mainly focuses on cabin comfort, ignoring the coupling of defogging and comfort. Accordingly, this paper combines cockpit-windshield-defogging design and cockpit comfort considerations, and proposes 108 orthogonal-ventilation design parameters based on the four basic ventilation methods. The effects of different air supply parameters on comfort and anti-fog characteristics are investigated by using fluid dynamics simulation methods. The entropy weight-TOPSIS algorithm is employed to find the optimal ventilation parameters. The results show that the "Down-supply up-return type vertical jet" air supply method corresponding to an air supply velocity of 1 m/s, an air supply temperature of 297 K, and an air supply relative humidity of 30% has the smallest Euclidean distance di+ from the positive ideal solution, and the largest Euclidean distance di- from the negative ideal solution; thus, it obtains a higher ci and the highest priority. This air supply method provides the best thermal comfort for the drivers, as well as the best anti-fogging and defogging effect. The results can be useful to provide suggestions for the future design of the air-conditioning systems in polar cruise ships.

4.
Biomaterials ; 285: 121519, 2022 06.
Artigo em Inglês | MEDLINE | ID: mdl-35552116

RESUMO

Volumetric muscle loss (VML) healing is often complicated by fibrosis, which impairs muscle regeneration and function. Adjusting mechanical stress in the repair environment may modulate immunity and reduce fibrosis. In this study, we aimed to create a biomaterial with suitable tension conditions and bidirectional tissue-inducing abilities to prevent fibrosis thus promote muscle regeneration and induce aponeurosis-like structures to restore muscle force transmission. A protocol was developed to manufacture decellularized muscle aponeurosis (D-MA) patches with an intact extracellular matrix (ECM) and low cytotoxicity. D-MA optimized the mechanical stress distribution in muscle injury sites and decreased the number of proinflammatory macrophages and myofibroblasts, thereby attenuating muscle fibrosis. Muscle and aponeurosis ECM environments had different microstructures and mechanical properties, which specifically enhanced stem cell differentiation into muscle-like cells on muscle ECM and tenocyte-like cells on aponeurosis ECM in vitro. Four weeks after orthotopic implantation, the biphasic muscle-aponeurosis-like tissue was successfully regenerated by the D-MA scaffold. The regenerated muscle fibers in D-MA were more abundant than those in the fibrotic decellularized muscle (D-M) scaffold. D-MA can be used to repair abdominal defects, which significantly improves the repair outcomes. Our results suggest D-MA as a promising material for VML repair.


Assuntos
Parede Abdominal , Doenças Musculares , Matriz Extracelular , Fibrose , Humanos , Músculo Esquelético/fisiologia , Doenças Musculares/patologia , Regeneração , Estresse Mecânico , Alicerces Teciduais/química
5.
Ann N Y Acad Sci ; 1503(1): 88-101, 2021 11.
Artigo em Inglês | MEDLINE | ID: mdl-34480353

RESUMO

Circular RNA (circRNA) is implicated in many types of cancer; however, the expression and role of circRNAs in colorectal cancer (CRC) remains poorly understood. In this study, a circRNA microarray assay was performed to detect abnormally expressed circRNAs in CRC, and tissue arrays were used to determine the prognosis for CRC patients. Cell counting kit-8, clone formation, wound healing, and transwell assays were used to evaluate cell functions in vitro, and a mouse subcutaneous tumor model was designed for in vivo analysis. Autophagy was observed using confocal laser scanning and transmission electron microscopy. The expression of circRNA, miRNA, and mRNA was detected using qPCR; western blot, RNA pull-down assay, RNA immunoprecipitation, and dual luciferase assessment were applied for mechanistic studies. We found that circRNA_103948 expression is upregulated in CRC tissues, compared with adjacent normal tissues, and associated with poor prognosis. Knockdown of circRNA_103948 suppressed CRC both in vitro and in vivo. Mechanistically, circRNA_103948 could directly bind to miR-1236-3p and relieve suppression of the target TPT1. Furthermore, circRNA_103948 inhibited autophagy of CRC cells. Taken together, circRNA_103948 knockdown inhibited CRC cell growth by targeting miR-1236-3p/TPT1 axis-mediated autophagy. Thus, the circRNA_103948/miR-1236-3p/TPT1 axis affects CRC progression via modulation of autophagy.


Assuntos
Autofagia/genética , Neoplasias Colorretais/genética , Regulação Neoplásica da Expressão Gênica , RNA Circular , Apoptose/genética , Biomarcadores Tumorais , Linhagem Celular Tumoral , Movimento Celular/genética , Neoplasias Colorretais/metabolismo , Neoplasias Colorretais/patologia , Genes Reporter , Humanos , MicroRNAs/genética , Interferência de RNA , RNA Mensageiro/genética , Regulação para Cima
6.
Environ Sci Pollut Res Int ; 28(41): 57695-57705, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-34091839

RESUMO

Sewage sludge (SS) dewatering is a key step in sludge disposal, which plays an important role in reducing sludge volume, facilitating transportation and subsequent treatment. In this paper, a facile hydrothermal-alkaline treatment for SS was proposed, which can be used for sludge dewatering and humic acid (HA) recycling at the same time. Response surface methodology (RSM) was used to determine the optimal conditions, and a mathematical model was established to accurately predict the changes of sludge water content and the extraction rate of HA. Under the optimal conditions of 170 °C/42 min/0.05 (for hydrothermal temperature, hydrothermal time, and mass ratio of KOH to wet sludge, respectively), the water content decreased to 46.7% and the extraction rate of HA (with a purity of 96.2%) was 89.1%. The improvement of the dewatering performance effectively facilitates the subsequent disposal of the sludge. The hydrothermal-alkaline method not only realizes the efficient dehydration of the sludge, but also obtains HA from the sludge extract. The obtained HA has potential economic value in the fields of agriculture, biological medicine, environment, and the like.


Assuntos
Esgotos , Eliminação de Resíduos Líquidos , Substâncias Húmicas , Temperatura , Água
7.
Circ Rep ; 3(2): 86-94, 2021 Jan 16.
Artigo em Inglês | MEDLINE | ID: mdl-33693294

RESUMO

Background: Atherosclerosis is an inflammatory disease involving activation of adaptive and innate immune responses to antigens, including oxidized low-density lipoprotein (oxLDL) and phosphorylcholine (PC). Dendritic cells (DCs), which are antigen-presenting cells that activate T cells, are present in atherosclerotic lesions and are activated in immune organs. However, the mechanism by which PC promotes atherosclerosis is unclear. Methods and Results: To evaluate whether PC promotes atherosclerosis via DCs, 2×105 DCs activated by PC-keyhole limpet hemocyanin (DCs+PC-KLH) were injected into ApoE-/- mice and the features of the plaques and the effects of the DCs on cellular and humoral immunity against PC-KLH were determined. Mice injected with DCs+PC-KLH had significantly larger atherosclerotic lesions than controls, with increased inflammation in the lesions and plaque instability. Furthermore, DCs+PC-KLH were characterized using flow cytometry after coculture of bone marrow-derived DCs and naïve T cells. DCs+PC-KLH showed an inflammatory phenotype, with increased CD86, CD40, and major histocompatibility complex Class II molecules (MHC-II), which promoted PC-specific T helper (Th) 1 and Th17 cell differentiation in vivo and in vitro. Moreover, 2 weeks after the administration of DCs+PC-KLH to mice, these mice produced PC- and oxLDL-specific IgG2a, compared with no production in the controls. Conclusions: These findings suggest that DCs presenting PC promote specific immunity to PC, increase lesion inflammation, and accelerate atherosclerosis, which may explain how PC promotes atherosclerosis.

8.
Andrologia ; 53(4): e13923, 2021 May.
Artigo em Inglês | MEDLINE | ID: mdl-33583046

RESUMO

This study established an oligoasthenospermic rat model using tripterygium glycosides (TGs) and investigated the mechanism by which Qilin pills (QLPs) ameliorate reproductive hypofunction. Thirty-two male Sprague Dawley rats were allocated to four equal-sized groups: (1) the control group received continuous physiological levels of saline; (2) the oligoasthenospermia model group was induced with TGs by daily intragastric administration for 28 days; (3 and 4) oligoasthenospermic rats were treated intragastrically with low dose (1.62 g kg-1  d-1 ) and high dose (3.24 g kg-1  d-1 ) of QLPs once daily for 60 days. The QLP-treated rats showed a marked increase (p < .05) in testicular mass, testicular index and semen parameters compared with the untreated rats. Histopathologically, the QLP-treated groups exhibited restored seminiferous tubules in contrast to the model group. Reactive oxygen species and malondialdehyde levels were dramatically decreased (p < .05) in the testes of the QLP-treated rats. QLP treatment partly reverted (p < .05) the circulatory levels of reproductive hormones (FSH, LH, testosterone, prolactin and SHBG) and hepatic and renal function (AST, Cr and urea). Our results showed that oral QLP treatment had a curative effect on the testicular mass, sperm quality, testicular pathomorphology, antioxidants, plasmatic hormones, and liver and renal function of rats.


Assuntos
Medicamentos de Ervas Chinesas , Oligospermia , Animais , Glicosídeos/farmacologia , Humanos , Masculino , Oligospermia/induzido quimicamente , Oligospermia/tratamento farmacológico , Ratos , Ratos Sprague-Dawley , Contagem de Espermatozoides , Espermatozoides , Testículo , Testosterona , Tripterygium
9.
Chin Med Sci J ; 36(4): 265-278, 2021 Dec 31.
Artigo em Inglês | MEDLINE | ID: mdl-34986963

RESUMO

Objective BAG3-related myopathy is a rare condition so far reported in twenty patients worldwide. The purpose of this study was to draw attention to this rare disease and to the fact that BAG3-related myopathy should be considered as a rare differential diagnosis of hypercapnia. Methods We report a sporadic case of a 14-year-old Chinese girl with a de novo p.Pro209Leu mutation in BAG3 and reviewed the literatures for reported cases related to this mutation. Results We described a 14-year-old Chinese girl who presented with gradually appearing symptoms of hypercapnia that required assisted ventilation. The muscle biopsy and the blood whole-exome sequencing results confirmed the diagnosis of myofibrillar myopathy with a de novo p.Pro209Leu mutation in BAG3. Totally twenty-one patients from twenty families with a confirmed diagnosis of BAG3-related myopathy were reported to date, including this patient and literature review. The male to female ratio was 11:10 and most showed initial symptoms in the first decade of life. Most patients presented toe/clumsy walking or running as the onset symptom, followed by muscle weakness or atrophy. Creatine kinase levels were elevated in fourteen patients and were normal in three. Eighteen patients developed respiratory insufficiency during the disease course and thirteen (one could not tolerate non-invasive assisted ventilation) required non-invasive assisted ventilation for treatment. Except for one not reported, heart involvement was found in seventeen patients during the disease course and seven underwent heart transplantation. Z-disk streaming and aggregation could be observed in most of the patients' muscle histology. In the long-term follow-up, five patients died of cardiac or respiratory failure. Conclusion BAG3-associated myopathy is a rare type of myofibrillar myopathy. It should be considered as a rare differential diagnosis of hypercapnia.


Assuntos
Hipercapnia , Miopatias Congênitas Estruturais , Proteínas Adaptadoras de Transdução de Sinal/genética , Proteínas Adaptadoras de Transdução de Sinal/metabolismo , Adolescente , Proteínas Reguladoras de Apoptose/genética , Proteínas Reguladoras de Apoptose/metabolismo , Feminino , Humanos , Masculino , Mutação , Miopatias Congênitas Estruturais/diagnóstico , Miopatias Congênitas Estruturais/genética
10.
Clin Ther ; 41(5): 972-980.e8, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-31030996

RESUMO

PURPOSE: Chemotherapy-induced alopecia (CIA) is a common and often stressful adverse effect associated with chemotherapy. CIA can cause more psychosocial pressure in patients, including effects on sexuality, self-esteem, and social relationships. We analyzed publicly available data to identify drugs formulated for topical use targeting the relevant CIA molecular pathways by using computational tools. METHODS: The genes associated with CIA were determined by text mining, and the gene ontology of the gene set was studied using the Functional Enrichment analysis tool. Protein-protein interaction network analysis was performed using the String database. Enriched gene sets belonging to the identified pathways were queried against the Drug-Gene Interaction database to find drug candidates for topical use in CIA. FINDINGS: Our analysis identified 427 genes common to CIA text-mining concepts. Gene enrichment analysis and protein-protein interaction analysis yielded 19 genes potentially targetable by a total of 29 drugs that could possibly be formulated for topical application. IMPLICATIONS: The findings from the present analysis would give a new thought to help discover more effective agents, and present tremendous opportunities to study novel target pharmacology and facilitate drug repositioning efforts in the pharmaceutical industry.


Assuntos
Alopecia/tratamento farmacológico , Mineração de Dados , Descoberta de Drogas , Alopecia/induzido quimicamente , Antineoplásicos/efeitos adversos , Antineoplásicos/uso terapêutico , Biologia Computacional , Bases de Dados Factuais , Reposicionamento de Medicamentos , Humanos
11.
Clin Respir J ; 12(3): 1134-1140, 2018 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-28419740

RESUMO

INTRODUCTION: Both fibrosing mediastinitis (FM) and bronchial anthracofibrosis (BAF) are unique diseases. The combined appearance of FM and BAF is extremely rare. OBJECTIVES: The aim of this study was to investigate the clinical features of patients with coexisting FM and BAF. METHOD: Between January 2003 and December 2015, a total of eight patients were diagnosed at the Peking Union Medical College Hospital as having combined FM and BAF. The clinical presentations, radiographic features and bronchoscopic findings of the eight patients were reviewed. RESULTS: The patients were five women and three men with a median age of 64 years (range 56-86 years). Symptoms included dyspnea (eight patients), cough (seven patients), chest pain (two patients), hemoptysis (two patients) and so on. Chest CT of all eight patients showed mediastinal soft-tissue lesions, with multiple narrowed or obliterated lobar or segmental bronchi and arteries. Bronchoscopy showed that all of the patients had multiple stenoses of lobar or segmental bronchi with anthracotic pigmentation on the mucosa. Echocardiography showed that all of the patients had elevated pulmonary arterial systolic pressure (median 81 mm Hg, range 51-107 mm Hg). Each of the eight patients had a history of exposure to, or infection with, tuberculosis, although there was no evidence of active disease. All of the eight patients had long-term exposure to indoor coal or biomass fuel smoke. CONCLUSIONS: FM can coexist with BAF, characterized by prominent pulmonary hypertension. The possible etiological factors are tuberculosis and coal or biomass fuel exposure.


Assuntos
Antracose/complicações , Broncopatias/complicações , Hipertensão Pulmonar/etiologia , Mediastinite/complicações , Mediastino/diagnóstico por imagem , Esclerose/complicações , Idoso , Idoso de 80 Anos ou mais , Angiografia , Antracose/diagnóstico , Antracose/epidemiologia , Brônquios/diagnóstico por imagem , Broncopatias/diagnóstico , Broncopatias/epidemiologia , Broncoscopia , China/epidemiologia , Feminino , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/epidemiologia , Incidência , Masculino , Mediastinite/diagnóstico , Mediastinite/epidemiologia , Pessoa de Meia-Idade , Prognóstico , Artéria Pulmonar/diagnóstico por imagem , Pressão Propulsora Pulmonar/fisiologia , Doenças Raras , Estudos Retrospectivos , Esclerose/diagnóstico , Esclerose/epidemiologia , Tomografia Computadorizada por Raios X
12.
Sci Rep ; 7(1): 16912, 2017 12 05.
Artigo em Inglês | MEDLINE | ID: mdl-29208971

RESUMO

Nonspecific interstitial pneumonia (NSIP) is characterised by interstitial infiltration of lymphocytes and varying amounts of interstitial fibrosis. B cells have been suggested to contribute to the pathogenesis of NSIP. However, the relationship between B-lymphocyte and the clinical outcomes of NSIP was unclear. In this study, 50 patients with histopathologically confirmed NSIP from Peking Union Medical College Hospital between April 2003 to December 2012 were retrospectively analyzed. Using immunohistochemical analyses, CD20+ B cells were counted in the lymphoid follicles, perivascular, interstitial, and peribronchiolar regions of lung tissure. The CD20+ lymphocytes were mainly present in the lymphoid follicles. The number of follicular CD20+ lymphocytes was higher in the fibrosing than cellular NSIP pattern [255.08 (132.92-449.71) vs. 121.33 (63.54-282.88)/0.1 mm2, p = 0.017]. After 1 year of therapy, the follicular CD20+ lymphocytes were significantly higher in patients whose forced vital capacity (FVC) worsened as compared to those who improved (p = 0.014). Additionally, follicular CD20+ lymphocytes were negatively correlated with the post-treatment percentage change in FVC (rho = -0.397, p = 0.004). However, follicular CD20+ lymphocytes were not correlated with survival. These results suggested that pulmonary follicular CD20+ lymphocytes were correlated with the fibrosing pattern of NSIP and predicted less clinical improvement after treatment.


Assuntos
Antígenos CD20/metabolismo , Linfócitos B/patologia , Pneumonias Intersticiais Idiopáticas/patologia , Adulto , Idoso , Linfócitos B/metabolismo , Feminino , Humanos , Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Pneumonias Intersticiais Idiopáticas/mortalidade , Pneumonias Intersticiais Idiopáticas/fisiopatologia , Receptores de Lipopolissacarídeos/metabolismo , Pulmão/patologia , Tecido Linfoide/patologia , Masculino , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios X , Capacidade Vital
13.
Artigo em Inglês | MEDLINE | ID: mdl-28405161

RESUMO

Airway mucus hypersecretion is a frequent symptom associated with acute and chronic airway disease. Inhibition of mucus production or promotion of mucolysis not only relieved symptoms but also improved disease outcomes. There are numerous available mucoactive medicines for prescription, and how to select them properly for different diseases is important for clinical practice. So far, there is no one consensus or guideline reported. A group of Chinese pulmonary physicians worked together to complete this consensus based on literature review, summarized mechanism and usage of each classical mucoactive medicine. In general, antioxidant mucoactive medicines play an important role in chronic airway disease, including but not limited to airway mucus clearance, reduced acute exacerbation and improved pulmonary function.


Assuntos
Antioxidantes/uso terapêutico , Expectorantes/uso terapêutico , Pulmão/efeitos dos fármacos , Depuração Mucociliar/efeitos dos fármacos , Muco/metabolismo , Doença Pulmonar Obstrutiva Crônica/tratamento farmacológico , Pneumologistas , Antioxidantes/efeitos adversos , China , Consenso , Progressão da Doença , Expectorantes/efeitos adversos , Humanos , Pulmão/metabolismo , Pulmão/fisiopatologia , Doença Pulmonar Obstrutiva Crônica/diagnóstico , Doença Pulmonar Obstrutiva Crônica/fisiopatologia , Qualidade de Vida , Recuperação de Função Fisiológica , Resultado do Tratamento
14.
Hum Genome Var ; 3: 15063, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27081564

RESUMO

Cystic fibrosis (CF), the most common life-threatening autosomal recessive disorder in Caucasians, is caused by mutations in CF transmembrane conductance regulator gene (CFTR). We and others previously identified CFTR mutations in 20 Chinese patients with CF. In this study, eight Chinese patients with a clinical diagnosis of suspected CF were newly collected and screened for CFTR mutations using a combination of conventional Sanger sequencing and multiplex ligation-dependent probe amplification (MLPA) analysis. The CFTR mutations observed in Chinese CF patients, both reported previously and identified in the present study, were also summarized. In the newly collected patients, we identified 10 different CFTR mutations, including p.F508del, the most common CF-causing mutation in Caucasians, and three novel mutations (p.V1212Afs*15; p.L666* and p.A969A). Most notably, the previously reported p.G970D mutation was found in six patients, making it the most frequent CFTR mutation identified in Chinese CF patients thus far. In conclusion, we detected p.F508del for the first time, identified additional novel CFTR mutations and recorded the most frequent CF-causing mutation in Chinese CF patients.

15.
Oncotarget ; 7(11): 12121-36, 2016 Mar 15.
Artigo em Inglês | MEDLINE | ID: mdl-26933821

RESUMO

Intervertebral disc degeneration is associated with back pain and disc herniation. This study established a modified protocol for intervertebral disc (IVD) decellularization and prepared its extracellular matrix (ECM). By culturing mesenchymal stem cells (MSCs)(3, 7, 14 and 21 days) and human degenerative IVD cells (7 days) in the ECM, implanting it subcutaneously in rabbit and injecting ECM microparticles into degenerative disc, the biological safety and efficacy of decellularized IVD was evaluated both in vitro and in vivo. Here, we demonstrated that cellular components can be removed completely after decellularization and maximally retain the structure and biomechanics of native IVD. We revealed that allogeneic ECM did not evoke any apparent inflammatory reaction in vivo and no cytotoxicity was found in vitro. Moreover, IVD ECM can induce differentiation of MSCs into IVD-like cells in vitro. Furthermore, allogeneic ECM microparticles are effective on the treatment of rabbit disc degeneration in vivo. In conclusion, our study developed an optimized method for IVD decellularization and we proved decellularized IVD is safe and effective for the treatment of degenerated disc diseases.


Assuntos
Materiais Biocompatíveis/farmacologia , Matriz Extracelular/transplante , Degeneração do Disco Intervertebral/terapia , Disco Intervertebral/patologia , Transplante de Células-Tronco Mesenquimais/métodos , Células-Tronco Mesenquimais/citologia , Animais , Humanos , Degeneração do Disco Intervertebral/patologia , Coelhos
16.
Lab Invest ; 96(5): 561-9, 2016 05.
Artigo em Inglês | MEDLINE | ID: mdl-26901836

RESUMO

Intervertebral disc degeneration is a major cause of low back pain. The nucleus pulposus (NP) is an important intervertebral disc component. Recent studies have shown that carbonic anhydrase 12 (CA12) is a novel NP marker. However, the mechanism by which CA12 is regulated and its physiological function are unclear. In our study, CA12, hypoxia-inducible factor 1α (HIF-1α) and HIF-2α expression levels were examined in 81 human degenerated NP samples using real-time RT-PCR, immunohistochemistry and western blot. Rat NP cells were cultured in a hypoxic environment, and hypoxia-induced CA12 expression was examined. Rat NP cells were treated with HIF-1α siRNA or the prolyl hydroxylase (PHD) inhibitor dimethyloxalylglycine (DMOG) to evaluate the role of PHD/HIF-1 in regulating CA12 expression. Rat NP cells were treated with CA12 siRNA to determine the function of CA12. A rat ex vivo model was established to confirm that PHD, HIF-1, and CA12 have important roles in disc degeneration. We found that CA12 was significantly downregulated in degenerated human NP samples at the mRNA and protein levels. CA12 expression sharply increased by ~30-fold in response to hypoxia. The expression of HIF-1α, but not HIF-2α, also decreased in degenerated human NP samples and was positively correlated with CA12 expression. HIF-1α knockdown under hypoxia reduced the CA12 mRNA and protein expression levels. DMOG treatment increased HIF-1α and CA12 expression. CA12 knockdown significantly inhibited anabolic protein expression, whereas catabolic enzymes remained unchanged. The ex vivo experiments supported our in vitro studies of the role of PHD/HIF-1/CA12. In conclusion, CA12 is downregulated in degenerated NPs, and its expression may be regulated by the PHD/HIF-1 axis. Decreased CA12 expression may lead to decreased extracellular matrix synthesis, which contributes to degenerative disc disease progression.


Assuntos
Anidrases Carbônicas/metabolismo , Subunidade alfa do Fator 1 Induzível por Hipóxia/metabolismo , Degeneração do Disco Intervertebral/metabolismo , Degeneração do Disco Intervertebral/prevenção & controle , Prolil Hidroxilases/metabolismo , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Animais , Fatores de Transcrição Hélice-Alça-Hélice Básicos/metabolismo , Anidrases Carbônicas/genética , Hipóxia Celular/genética , Hipóxia Celular/fisiologia , Células Cultivadas , Feminino , Técnicas de Silenciamento de Genes , Humanos , Subunidade alfa do Fator 1 Induzível por Hipóxia/antagonistas & inibidores , Subunidade alfa do Fator 1 Induzível por Hipóxia/genética , Degeneração do Disco Intervertebral/genética , Masculino , Pessoa de Meia-Idade , Núcleo Pulposo/citologia , Núcleo Pulposo/metabolismo , RNA Mensageiro/genética , RNA Mensageiro/metabolismo , Ratos , Ratos Sprague-Dawley , Regulação para Cima , Adulto Jovem
17.
BMC Pulm Med ; 16: 20, 2016 Jan 25.
Artigo em Inglês | MEDLINE | ID: mdl-26809651

RESUMO

BACKGROUND: Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory disorder that may involve single or multiple organs. Biopsy-proven lung involvement of this disease is occasionally reported, but not well understood. METHODS: Patients with the diagnosis of biopsy-proven IgG4-related lung disease (IgG4-RLD) from Peking Union Medical College Hospital between January 2011 and July 2015 were retrospectively analyzed. Age, sex, clinical symptoms, laboratory findings, pulmonary function test results, chest CT tests, positron emission tomography (PET) examinations, treatments and prognoses were retrieved from medical records and analyzed. RESULTS: Seventeen patients were included in this study (mean age: 44.8 ± 15.0 years). Ten patients were diagnosed via surgery, and 7 patients were diagnosed via percutaneous transthoracic core-needle lung biopsy. Extrapulmonary involvement was observed in only one patient. The clinical symptoms included cough, fever, dyspnea, chest pain and hemoptysis. The serum IgG4 concentration was elevated in 7/13 patients (mean: 1955 ± 1968 mg/L). The chest CT findings included mainly nodules and masses with spiculated borders, alveolar consolidations with air bronchograms, and ground glass opacities with or without reticular opacities. PET scans indicated increased standardized uptake values, and 7/8 patients were correctly diagnosed with benign inflammation. Corticosteroids and immunosuppressants were administered to 14/17 patients and effectively alleviated the disease. CONCLUSIONS: In biopsy-proven IgG4-RLD, a normal serum IgG4 concentration is commonly seen, while extrapulmonary involvement is infrequent. Alveolar consolidation with air bronchograms is an important imaging finding of IgG4-RLD, which has not been emphasized before.


Assuntos
Doenças Autoimunes/imunologia , Imunoglobulina G/imunologia , Pneumopatias/imunologia , Pulmão/patologia , Adolescente , Adulto , Idoso , Doenças Autoimunes/complicações , Doenças Autoimunes/diagnóstico , Biópsia , Dor no Peito/etiologia , Tosse/etiologia , Dispneia/etiologia , Feminino , Febre/etiologia , Hemoptise/etiologia , Humanos , Pulmão/diagnóstico por imagem , Pneumopatias/complicações , Pneumopatias/diagnóstico , Masculino , Pessoa de Meia-Idade , Nódulos Pulmonares Múltiplos , Tomografia por Emissão de Pósitrons , Estudos Retrospectivos , Tomografia Computadorizada por Raios X , Adulto Jovem
18.
Medicine (Baltimore) ; 94(50): e2150, 2015 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-26683924

RESUMO

To investigate clinical and radiological features of IgG4-related disease (IgG4-RD) patients with intrathoracic involvement. A prospective cohort study was performed and IgG4-RD patients were enrolled from January 2011 to March 2015 in Peking Union Medical College Hospital, in which the clinical and radiological characteristics of IgG4-RD patients with intrathoracic involvement were summarized. Out of total 248 cases with IgG4-RD, 87 cases had intrathoracic lesions, including 58 male cases and 29 female cases, with average age of 54.19 ±â€Š13.80 years. Hilar and mediastinal lymphadenopathy were the most common manifestations of IgG4-related intrathoracic disease, accounting for 52.9% (46/87). Other imaging findings of pulmonary disease included: solid nodular (25.3%), round-shaped ground-glass opacities (9.2%), alveolar-interstitial type (20.7%), bronchovascular type (23.0%), pleural effusion (4.6%), and pleural nodules or thickening (16.1%). Only 27 patients presented with respiratory symptoms, including cough, breathless, chest pain, and asthma. Compared with patients without intrathoracic disease, IgG4-related intrathoracic disease had higher IgG4 and C-reactive protein level, and higher incidence of allergy, fever, and multi-organ involvement. Most of lung interstitial disease, mediastinal mass, and bronchial thickening were sensitive to corticosteroid and immunosuppressant therapy, while 36.3% (8/22) of solitary nodular lesions were unresponsive to treatment. Eight patients were on no treatment, with 5 cases remained stable, 2 patients improved spontaneously, and 1 patient was lost follow-up. Intrathoracic lesions are not rare in patients with IgG4-RD, involving bronchial thickening, nodules, ground glass opacity, pleural thickening/effusion, lymphadenopathy, etc. Efficacy of corticosteroid and immunosuppressant therapy were noted in most of patients with lung interstitial disease, mediastinal mass, and bronchial thickening.


Assuntos
Imunoglobulina G/imunologia , Pneumopatias/imunologia , Tórax/imunologia , Adulto , Idoso , Proteína C-Reativa , China , Feminino , Humanos , Imunossupressores/uso terapêutico , Pneumopatias/diagnóstico por imagem , Pneumopatias/tratamento farmacológico , Doenças Linfáticas/diagnóstico por imagem , Doenças Linfáticas/tratamento farmacológico , Doenças Linfáticas/imunologia , Masculino , Pessoa de Meia-Idade , Tomografia por Emissão de Pósitrons , Estudos Prospectivos , Tomografia Computadorizada por Raios X
19.
Respirology ; 20(2): 312-8, 2015 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-25580864

RESUMO

BACKGROUND AND OBJECTIVE: Cystic fibrosis (CF) is a relatively common autosomal recessive disorder in Caucasians. CF is considered a very rare disease in Asians, and fewer than 30 Chinese CF patients are reported in the literature. We enrolled seven patients of Chinese Han origin diagnosed with CF at the Peking Union Medical College Hospital, to characterize gene mutations and phenotypes of CF in Chinese patients. METHODS: We analysed the clinical presentation and screened the coding region of the CFTR gene for each patient. RESULTS: Patients were 0-6 years old at onset of symptoms and were 10-28 years old at the time of diagnosis with CF. None of the seven patients had a family history of CF, and only one patient had parents who were consanguineous. Two patients had gastrointestinal symptoms but stool Sudan III results were normal. Four of the seven CF patients also had allergic bronchopulmonary aspergillosis. The concentration of chloride in patients' sweat ranged from 66 mmol/l to 154 mmol/l. In total, we identified 11 different mutations in seven CF patients, including one novel mutation (△E7-E11). Only one of these mutations (R553X) is present in the Caucasian CFTR common mutation-screening panel; and none of the 11 mutations are common in Caucasian CF patients. CONCLUSIONS: CF in China is difficult to diagnose because of a combination of low awareness, atypical clinical symptoms, and a lack of sweat and genetic testing facilities in most hospitals. The mutations identified in Chinese CF patients are different from the common Caucasian gene mutations.


Assuntos
Povo Asiático/genética , Regulador de Condutância Transmembrana em Fibrose Cística/genética , Fibrose Cística/genética , Mutação , Adolescente , Adulto , Idade de Início , Compostos Azo/análise , Criança , Pré-Escolar , China , Cloretos/análise , Fibrose Cística/diagnóstico , Fezes/química , Feminino , Testes Genéticos , Humanos , Lactente , Recém-Nascido , Masculino , Fenótipo , Suor/química , Adulto Jovem
20.
J Clin Rheumatol ; 20(7): 369-72, 2014 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-25275763

RESUMO

OBJECTIVES: The aims of the study were to evaluate the effectiveness of bronchoalveolar lavage (BAL) for diagnosing pulmonary infection in patients with rheumatic autoimmune diseases and lung infiltrates and to evaluate factors that affect the diagnostic yield. METHODS: A retrospective study was performed in patients with rheumatic autoimmune diseases and lung infiltrates whose BALs were sent for microbial assays at Peking Union Medical College Hospital from January 2009 to June 2013. Patient characteristics, clinical symptoms, medication history, laboratory parameters, radiographic findings, lung lobe lavaged, and diagnostic yield were retrieved. RESULTS: Seventy BALs were performed in 69 patients. The overall diagnostic yield of BAL for pulmonary infection was 17.1% (12/70), sensitivity was 35.5%, and specificity was 97.4%. Twelve microorganisms were isolated from 12 different BALs conforming to diagnostic criteria, including 4 Aspergillus, 3 Pneumocystis jirovecii, 3 gram-negative bacilli, 1 gram-positive coccus, and 1 mycobacterium. Patients with clinical symptoms of fever, cough, or expectoration had a higher diagnostic yield than patients without either symptom (25.6% vs 3.7%, P = 0.042). Patients with ground-glass opacity, mass, or consolidation radiographically had a higher yield than did patients with reticular or nodular infiltrates (20.3% vs 0, P < 0.001). CONCLUSIONS: Bronchoalveolar lavage is a useful tool for patients with rheumatic autoimmune diseases and lung infiltrates, especially in cases where initial antimicrobial therapy is ineffective. Opportunistic pathogens are important in patients with rheumatic autoimmune diseases and lung infiltrates and should be considered when antibacterial treatment is ineffective.


Assuntos
Doenças Autoimunes/complicações , Lavagem Broncoalveolar , Pneumopatias/microbiologia , Infecções Respiratórias/diagnóstico , Infecções Respiratórias/microbiologia , Doenças Reumáticas/complicações , Adolescente , Adulto , Aspergillus/isolamento & purificação , Doenças Autoimunes/microbiologia , Criança , Corynebacterium/isolamento & purificação , Feminino , Humanos , Klebsiella pneumoniae/isolamento & purificação , Pneumopatias/diagnóstico , Masculino , Staphylococcus aureus Resistente à Meticilina/isolamento & purificação , Pessoa de Meia-Idade , Mycobacterium/isolamento & purificação , Pneumocystis carinii/isolamento & purificação , Valor Preditivo dos Testes , Estudos Retrospectivos , Doenças Reumáticas/microbiologia , Stenotrophomonas maltophilia/isolamento & purificação , Adulto Jovem
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