期刊文献+
共找到995篇文章
< 1 2 50 >
每页显示 20 50 100
Pulmonary alveolar proteinosis induced by X-linked agammaglobulinemia:A case report 被引量:1
1
作者 Ting Zhang Ming Li +1 位作者 Li Tan Xin Li 《World Journal of Clinical Cases》 SCIE 2024年第9期1644-1648,共5页
BACKGROUND Pulmonary alveolar proteinosis(PAP)and X-linked agammaglobulinemia(XLA)are rare diseases in children.Many theories infer that immunodeficiency can induce PAP,but these reports are almost all review articles... BACKGROUND Pulmonary alveolar proteinosis(PAP)and X-linked agammaglobulinemia(XLA)are rare diseases in children.Many theories infer that immunodeficiency can induce PAP,but these reports are almost all review articles,and there is little clinical evidence.We report the case of a child with both PAP and XLA.CASE SUMMARY A 4-month-old boy sought medical treatment due to coughing and difficulty in breathing for>2 wk.He had been hospitalized multiple times due to respiratory infections and diarrhea.Chest computed tomography and alveolar lavage fluid showed typical PAP-related manifestations.Genetic testing confirmed that the boy also had XLA.Following total lung alveolar lavage and intravenous immunoglobulin replacement therapy,the boy recovered and was discharged.During the follow-up period,the number of respiratory infections was significantly reduced,and PAP did not recur.CONCLUSION XLA can induce PAP and improving immune function contributes to the prognosis of children with this type of PAP. 展开更多
关键词 pulmonary alveolar proteinosis X-linked agammaglobulinemia CHILDREN IMMUNODEFICIENCY alveolar lavage Case report
下载PDF
Clinical approach for pulmonary alveolar proteinosis in children
2
作者 Anuvat Klubdaeng Prakarn Tovichien 《World Journal of Clinical Cases》 SCIE 2024年第30期6339-6345,共7页
In this editorial,we discuss the clinical implications of the article by Zhang et al.Pulmonary alveolar proteinosis(PAP)is a rare lung disease characterized by excessive surfactant accumulation in the alveoli.It is cl... In this editorial,we discuss the clinical implications of the article by Zhang et al.Pulmonary alveolar proteinosis(PAP)is a rare lung disease characterized by excessive surfactant accumulation in the alveoli.It is classified into four categories:Primary,secondary,congenital,and unclassified forms.Primary PAP is caused by the disruption of granulocyte-macrophage colony-stimulating factor(GM-CSF)receptor signaling,which is necessary for the clearance of surfactant by alveolar macrophages.It is further divided into autoimmune PAP,caused by anti-GM-CSF antibodies blocking alveolar macrophage activation,and hereditary PAP,resulting from mutations in genes encoding GM-CSF receptors.Secondary PAP develops due to conditions affecting the number or function of alveolar macrophages,such as infections,immunodeficiency,hematological disorders,or exposure to inhaled toxins.Congenital PAP is linked to mutations in genes involved in surfactant protein production.Notably,the causes of PAP differ between children and adults.Diagnostic features include a characteristic"crazypaving"pattern on high-resolution computed tomography,accompanied by diffuse ground-glass opacities and interlobular septal thickening.The presence of PAP can be identified by the milky appearance of bronchoalveolar lavage fluid and histological evaluation.However,these methods cannot definitively determine the cause of PAP.Whole lung lavage remains the standard treatment,often combined with specific therapies based on the underlying cause. 展开更多
关键词 alveolar lavage CHILDREN IMMUNODEFICIENCY pulmonary alveolar proteinosis X-linked agammaglobulinemia
下载PDF
Unexpected diffuse lung lesions in a patient with pulmonary alveolar proteinosis:A case report
3
作者 Li Jian Qi-Quan Zhao 《World Journal of Clinical Cases》 SCIE 2023年第20期4932-4936,共5页
BACKGROUND Pulmonary alveolar proteinosis(PAP)often presents nonspecifically and can be easily confused with:(1)Idiopathic interstitial lung fibrosis;(2)alveolar carcinoma;(3)pulmonary tuberculosis;and(4)other lung di... BACKGROUND Pulmonary alveolar proteinosis(PAP)often presents nonspecifically and can be easily confused with:(1)Idiopathic interstitial lung fibrosis;(2)alveolar carcinoma;(3)pulmonary tuberculosis;and(4)other lung diseases such as viral pneumonia,mycoplasma pneumonia,and chlamydial pneumonia.CASE SUMMARY Diagnosis:In this case,a patient was diagnosed with PAP through transbronchial cryobiopsy(TBCB)and quantitative metagenomic next-generation sequencing,which confirmed the impairment of surfactant turnover as the underlying cause of PAP.Interventions:High-volume total lung lavage was performed for this patient.Outcomes:The patient's clinical condition had improved significantly by the 6-month follow-up,with a 92%finger oxygen saturation.A repeat chest computed tomography scan revealed scattered patchy ground-glass shadows in both lungs,which was consistent with alveolar protein deposition but with a lower density than in the radiograph from October 23,2022.CONCLUSION TBCB has unique advantages in diagnosing atypical alveolar protein deposition,particularly for enabling the early detection of PAP.This information can help patients take preventive measures to prevent or halt PAP development by avoiding dusty environments and seeking treatment with total lung lavage and inhaled granulocyte macrophage colony-stimulating factor. 展开更多
关键词 Diffuse lung lesions pulmonary alveolar proteinosis Quantitative metagenomic next-generation sequencing Transbronchial cryobiopsy High-volume double lung
下载PDF
Pulmonary alveolar proteinosis complicated with tuberculosis:A case report 被引量:2
4
作者 Hao Bai Zi-Rui Meng +1 位作者 Bin-Wu Ying Xue-Rong Chen 《World Journal of Clinical Cases》 SCIE 2021年第17期4400-4407,共8页
BACKGROUND Pulmonary alveolar proteinosis(PAP)is a rare lung disease characterized by the accumulation of phospholipoproteinaceous material in the alveoli.Cases of PAP complicated with tuberculosis are much more compl... BACKGROUND Pulmonary alveolar proteinosis(PAP)is a rare lung disease characterized by the accumulation of phospholipoproteinaceous material in the alveoli.Cases of PAP complicated with tuberculosis are much more complex and have rarely been well recorded.CASE SUMMARY We describe a 21-year-old Han Chinese patient with suspicious lung infection associated with mild restrictive ventilatory dysfunction and diffusion reduction.High resolution computed tomography revealed a“crazy-paving”appearance and multiple pulmonary miliary nodules around the bronchi.Bronchoalveolar lavage demonstrated a small amount of periodic acid-Schiff positive proteinaceous materials.A serological test for the presence of a Mycobacterium tuberculosis antibody and an interferon-gamma release assay were both positive.The patient received a standard course of first-line anti-tuberculosis treatment after diagnostic bronchoalveolar lavage.To date,clinical remission has been achieved and maintained for five years.CONCLUSION In summary,the diagnosis of PAP complicated with tuberculosis was supported by a combination of clinical manifestations,imaging,pulmonary function,laboratory examinations,bronchoalveolar lavage,etc.This case highlighted that diagnostic bronchoalveolar lavage in combination with anti-tuberculosis treatment is a safe and effective option for mild PAP patients with tuberculosis. 展开更多
关键词 pulmonary alveolar proteinosis pulmonary tuberculosis Bronchoalveolar lavage Case report
下载PDF
Pulmonary alveolar proteinosis complicated with nocardiosis: A case report and review of the literature 被引量:2
5
作者 Xiao-Kang Wu Quan Lin 《World Journal of Clinical Cases》 SCIE 2021年第12期2874-2883,共10页
BACKGROUND Pulmonary alveolar proteinosis(PAP)is a pulmonary syndrome wherein large volumes of phospholipid and protein-rich surfactants accumulate within the alveoli.PAP forms include primary(auto-immune PAP),seconda... BACKGROUND Pulmonary alveolar proteinosis(PAP)is a pulmonary syndrome wherein large volumes of phospholipid and protein-rich surfactants accumulate within the alveoli.PAP forms include primary(auto-immune PAP),secondary,and congenital.Nocardiosis is a form of suppurative disease induced upon infection with bacteria of the Nocardia genus.Clinically,cases of PAP complicated with Nocardia infections are rare,regardless of form.Unfortunately,as such,they are easily overlooked or misdiagnosed.We describe,here,the case of a patient suffering from simultaneous primary PAP and nocardiosis.CASE SUMMARY A 45-year-old Chinese man,without history of relevant disease,was admitted to our hospital on August 8,2018 to address complaints of activity-related respiratory exertion and cough lasting over 6 mo.Lung computed tomography(CT)revealed diffuse bilateral lung infiltration with local consolidation in the middle right lung lobe.Subsequent transbronchial lung biopsy and CT-guided lung biopsy led to a diagnosis of primary PAP(granulocyte-macrophage colonystimulating factor antibody-positive)complicated with nocardiosis(periodic acid-Schiff-positive).After a 6 mo course of anti-infective treatment(sulfamethoxazole),the lesion was completely absorbed,such that only fibrous foci remained,and the patient exhibited significant symptom improvement.Followup also showed improvement in pulmonary function and the CT imaging findings of PAP.No whole-lung lavage has been conducted to date.This case highlights that active anti-nocardia treatment may effectively improve the symptoms and alleviate PAP in patients with PAP and nocardia,possibly reducing the need for whole-lung lavage.CONCLUSION When evaluating patients presenting with PAP and pulmonary infections, thepotential for nocardiosis should be considered. 展开更多
关键词 pulmonary alveolar proteinosis NOCARDIOSIS Vitek mass spectroscopy Whole-lung lavage Case report
下载PDF
Chronic myelomonocytic leukemia-associated pulmonary alveolar proteinosis:A case report and review of literature 被引量:1
6
作者 Can Chen Xi-Lian Huang +2 位作者 Da-Quan Gao Yi-Wei Li Shen-Xian Qian 《World Journal of Clinical Cases》 SCIE 2021年第5期1156-1167,共12页
BACKGROUND Pulmonary alveolar proteinosis(PAP)is a rare condition that can cause progressive symptoms including dyspnea,cough and respiratory insufficiency.Secondary PAP is generally associated with hematological mali... BACKGROUND Pulmonary alveolar proteinosis(PAP)is a rare condition that can cause progressive symptoms including dyspnea,cough and respiratory insufficiency.Secondary PAP is generally associated with hematological malignancies including chronic myelomonocytic leukemia(CMML).To the best of our knowledge,this is the first reported case of PAP occurring secondary to CMML.CASE SUMMARY We report the case of a 63-year-old male who presented with a recurrent cough and gradually progressive dyspnea in the absence of fever.Based upon clinical symptoms,computed tomography findings,bone marrow aspiration,flow cytometry studies and cytogenetic analyses,the patient was diagnosed with PAP secondary to CMML.He underwent whole lung lavage in March 2016 to alleviate his dyspnea,after which he began combined chemotherapeutic treatment with decitabine and cytarabine.The patient died in January 2020 as a consequence of severe pulmonary infection.CONCLUSION This case offers insight regarding the mechanistic basis for PAP secondary to CMML and highlights potential risk factors. 展开更多
关键词 pulmonary alveolar proteinosis Chronic myelomonocytic leukemia DIAGNOSIS TREATMENT PROGNOSIS Case report
下载PDF
CT MANIFESTATIONS IN PULMONARY ALVEOLAR PROTEINOSIS AND COMPARED WITH CHEST RADIOGRAPHY(REPORT OF SIX CASES)
7
作者 俞肖一 严洪珍 +3 位作者 潘杰 张涛 刘维 杨宏 《Chinese Medical Sciences Journal》 CAS CSCD 1998年第4期238-241,共4页
The CT including HRCT appearances of six patients with histopathologically confirmed pulmonary alveolar proteinosis(PAP) were evaluated and compared with those of chest radiographs In all pati... The CT including HRCT appearances of six patients with histopathologically confirmed pulmonary alveolar proteinosis(PAP) were evaluated and compared with those of chest radiographs In all patients the CT manifestations were quite similar: bilateral and diffuse airspace consolidation was usually patchy or confluent with sharply defined margins, intermingled with normal lung tissue The configuration of lung lesions was “geographical” in outline with angulate, strait and curved margins There were white branching linear opacities within the ground glass background Although various pulmonary diseases may mimic PAP in some way, a full awareness of the characteristic CT appearances of PAP is helpful in achieving a correct diagnosis CT may provide more accurate evidence than chest radiograph for the evaluation of the extent and delineation of PAP 展开更多
关键词 pulmonary alveolar proteinosis CT
下载PDF
A Case Report of Pulmonary Alveolar Proteinosis with Associated Opportunistic Infection of Pneumocystis jirovecii and Molluscum Contagiosum
8
作者 Sapna Patel Deepika Gurumurthy +1 位作者 Smitha Rani Sheela Devi 《Journal of Forensic Science and Medicine》 2024年第1期68-71,共4页
Pulmonary alveolar proteinosis(PAP)is an idiopathic rare diffuse pulmonary disease,first described in 1958 by Rosen et al.Its estimated prevalence is about 1 in 3.7-6.9×10^(6) with a male:female ratio of 1:1-2:1.... Pulmonary alveolar proteinosis(PAP)is an idiopathic rare diffuse pulmonary disease,first described in 1958 by Rosen et al.Its estimated prevalence is about 1 in 3.7-6.9×10^(6) with a male:female ratio of 1:1-2:1.Majority of the patient’s age ranges between 20 and 50 years.PAP on microscopy is characterized by the presence of massive insoluble,amorphous,phospholipid-rich protein deposits in the bronchial and alveolar cavities.Most patients with acquired PAP present with cough and exertional dyspnea.It has been studied that there is increased risk of superinfection in PAP with opportunistic organisms like pneumocystis and vice versa.Definitive diagnosis of Pneumocystis jirovecii pneumonia rests on the demonstration of the organism within the alveoli by special stains like Grocott Methenamine Silver stain.Molluscum contagiosum(MC)is a common superficial skin infection caused by the poxvirus.MC is characterized by painless papules commonly seen in children and immunocompromised individuals.Here,we present a 34-year-old female who had complaints of severe difficulty in breathing and was brought dead to our hospital.On external examination,she had multiple warts over chest,abdomen,and over genitalia.Internal examination was unremarkable.Specimens of kidney,lung,and skin biopsy of genital warts sent for histopathological examination revealed acute tubular necrosis,P.jirovecii with PAP,and MC respectively. 展开更多
关键词 Bronchoalveolar lavage molluscum contagiosum Pneumocystis jirovecii pulmonary alveolar proteinosis
原文传递
TNF-α Up-regulates Matrix Metalloproteinase-9 Expression and Activity in Alveolar Macrophages from Patients with Chronic Obstructive Pulmonary Disease 被引量:3
9
作者 李亚清 张珍祥 +3 位作者 徐永健 熊盛道 倪望 陈仕新 《Journal of Huazhong University of Science and Technology(Medical Sciences)》 SCIE CAS 2006年第6期647-650,共4页
To study the effects of tumor necrosis factor (TNF)-α on matrix metalloproteinase (MMP)-9 expression and activity in alveolar macrophages (AM) and to investigate the role of NF-κB in the induction, AM were col... To study the effects of tumor necrosis factor (TNF)-α on matrix metalloproteinase (MMP)-9 expression and activity in alveolar macrophages (AM) and to investigate the role of NF-κB in the induction, AM were collected from bronchoalveolar lavage fluid (BALF) of healthy subjects and patients with chronic obstructive pulmonary disease (COPD). MMP-9 expression and activity were detected by semi-quantitative reverse transcription-polymerase chain reaction (RT-PCR), Western blotting and zymography. NF-κB activity was detected by electrophoretic mobility shift assay (EMSA). MMP-9 expression and activity induced by TNF-α in AM from healthy subjects or patients with COPD were significantly increased in a dose-dependent manner (P〈0.05). NF-κB activity induced by TNF-α was significantly increased in AM from patients with COPD, and pyrrolidine dithiocarbamate (PDTC) and N-acetyl-L-cysteine (NAC) significantly inhibited the activation of NF-κB induced by TNF-α (P〈0.05). The presents study suggested that the expression and activity of MMP-9 from AM can be induced by TNF-α, and TNF-α/NF-κB signal pathway may play an important role in the induction. 展开更多
关键词 chronic obstructive pulmonary disease alveolar macrophage matrix metalloproteinase NF-kappa B
下载PDF
Massive pulmonary haemorrhage due to severe trauma treated with repeated alveolar lavage combined with extracorporeal membrane oxygenation:A case report 被引量:2
10
作者 Bei-Yuan Zhang Xian-Cheng Chen +2 位作者 Yong You Ming Chen Wen-Kui Yu 《World Journal of Clinical Cases》 SCIE 2020年第18期4245-4251,共7页
BACKGROUND Massive pulmonary haemorrhage can spoil the entire lung and block the airway in a short period of time due to severe bleeding,which quickly leads to death.Alveolar lavage is an effective method for haemosta... BACKGROUND Massive pulmonary haemorrhage can spoil the entire lung and block the airway in a short period of time due to severe bleeding,which quickly leads to death.Alveolar lavage is an effective method for haemostasis and airway maintenance.However,patients often cannot tolerate alveolar lavage due to severe hypoxia.We used extracorporeal membrane oxygenation(ECMO)to overcome this limitation in a patient with massive pulmonary haemorrhage due to severe trauma and succeeded in saving the life by repeated alveolar lavage.CASE SUMMARY A 22-year-old man sustained multiple injuries in a motor vehicle accident and was transferred to our emergency department.On admission,he had a slight cough and a small amount of bloody sputum;computed tomography revealed multiple fractures and mild pulmonary contusion.At 37 h after admission,he developed severe chest tightness,chest pain,dizziness and haemoptysis.His oxygen saturation was 68%.Emergency endotracheal intubation was performed,and a large amount of bloody sputum was suctioned.After transfer to the intensive care unit,he developed refractory hypoxemia and heparin-free venovenous ECMO was initiated.Fibreoptic bronchoscopy revealed diffuse and profuse blood in all bronchopulmonary segment.Bleeding was observed in the trachea and right bronchus,and repeated alveolar lavage was performed.On day 3,the patient’s haemoptysis ceased,and ECMO support was terminated 10 d later.Tracheostomy was performed on day 15,and the patient was weaned from the ventilator on day 21.CONCLUSION Alveolar lavage combined with ECMO can control bleeding in trauma-induced massive pulmonary haemorrhage,is safe and can be performed bedside. 展开更多
关键词 pulmonary contusion Massive pulmonary haemorrhage alveolar lavage Extracorporeal membrane oxygenation Case report
下载PDF
An In Vitro Investigation of Pulmonary Alveolar Macrophage Cytotoxicity Introduced by Fibrous and Grainy Mineral Dusts
11
作者 DONG Faqin DENG Jianjun +4 位作者 WU Fengchun PU Xiaoyong John HUANG FENG Qiming HE Xiaochun 《Acta Geologica Sinica(English Edition)》 SCIE CAS CSCD 2006年第4期589-596,共8页
In order to study the damage mechanism of mineral dusts on the pulmonary alveolar macrophage (AM), the changes in their death ratio, malandialdthyde (MDA) content and activities of lactate dehydrogenase (LDH) an... In order to study the damage mechanism of mineral dusts on the pulmonary alveolar macrophage (AM), the changes in their death ratio, malandialdthyde (MDA) content and activities of lactate dehydrogenase (LDH) and superoxide dismutase (SOD) were measured, and the technique of cell culture in vitro was used to investigate the cytotoxicity of six mineral dusts (twelve crystal habits) from twelve mineral deposits. The results show that woUastonite and clinoptilolite have no AM cytotoxicity, while other fibrous and grainy mineral dusts damage pulmonary AM in various degrees. The cytotoxicity of fibrous mineral dusts was greater than that of the grainy ones, and the cytotoxicity of dusts was positively correlated with the active OH- content in dusts, but not necessarily so with its SiO2 content. The high pH values produced by dust was unfavorable for the survival of cells and the dusts with low bio-resistance were safe for cells. The content of variable valence elements in dusts might influence their cytotoxicity and the surface charge of dusts was not a stable factor for their toxicity. It is demonstrated that the shape of mineral dusts was one of the factors affecting cytotoxicity, and that the cytotoxicity of mineral dusts depends mainly on their properties. 展开更多
关键词 grainy mineral fibrous mineral DUST pulmonary alveolar macrophage CYTOTOXICITY
下载PDF
Single-lung transplantation for pulmonary alveolar microlithiasis: A case report
12
作者 Xing-Yu Ren Xiang-Ming Fang +6 位作者 Jing-Yu Chen Hao Ding Yan Wang Qiu Lu Jia-Lei Ming Li-Juan Zhou Hong-Wei Chen 《World Journal of Clinical Cases》 SCIE 2019年第22期3851-3858,共8页
BACKGROUND Pulmonary alveolar microlithiasis(PAM)is a rare idiopathic lung disease characterized by the accumulation of innumerable microliths.Currently,effective therapeutics for PAM are not available,and the only tr... BACKGROUND Pulmonary alveolar microlithiasis(PAM)is a rare idiopathic lung disease characterized by the accumulation of innumerable microliths.Currently,effective therapeutics for PAM are not available,and the only treatment for end-stage lung disease is lung transplantation(LuTx).Further,there are few reports that focus on LuTx for the treatment of PAM,and the follow-up reports of postoperative imaging are even rarer.CASE SUMMARY A 52-year-old man presented to Shanghai Pulmonary Hospital in 2017 after experiencing shortness of breath and exacerbation.The patient was diagnosed with PAM and referred for single-LuTx(SLuTx)on March 14,2018.Preoperative imaging results from a chest X-ray demonstrated bilateral,diffuse,symmetrical,sandstorm-like radiopaque micronodules,and pneumothorax and a computed tomography scan revealed minute,calcified military nodules in both lungs.We performed a left SLuTx,and intraoperative pathology was consistent with PAM.One week after surgery,a chest X-ray revealed slight exudation of the left lung,and one month later,the left transplanted lung exhibited good dilation,mild pulmonary perfusion injury with local infection,and left pleural effusion.Fiberoptic bronchoscopy revealed left hyperplastic granulation at the left bronchial anastomosis.Multiple sputum cultures suggested the presence of Klebsiella pneumoniae and Acinetobacter baumannii.The last follow-up was conducted in April 2019;the patient recovered well.CONCLUSION This case presents the imaging findings of a patient with PAM before and after LuTx and confirms the effectiveness of LuTx for the treatment of this disease. 展开更多
关键词 pulmonary alveolar MICROLITHIASIS Lung TRANSPLANTATION COMPLICATIONS CHEST X-ray COMPUTED tomography Case report
下载PDF
Update on diagnosis and treatment of pulmonary alveolar microlithiasis
13
作者 Hui-Ying Wang Ni-Ya Zhou Xu-Yan Yang 《World Journal of Respirology》 2014年第3期26-30,共5页
Pulmonary alveolar microlithiasis(PAM)(MIM265100)is a rare disease characterized by the diffuse deposit of microlithiasis in alveolar spaces.PAM could occur worldwide with high prevalence in Asia and Europe.Familial o... Pulmonary alveolar microlithiasis(PAM)(MIM265100)is a rare disease characterized by the diffuse deposit of microlithiasis in alveolar spaces.PAM could occur worldwide with high prevalence in Asia and Europe.Familial occurrence indicates its autosomal recessive trait and the SLC34A2 gene was identified as the responsible gene for the disease.In spite of the versatile mutation sites in patients from other countries,exon 7and exon 8 might be the most liable gene in Chinese and Japanese patients.Most mutations caused the premature termination of proteins and produced truncated proteins,leading to the blocking of the recycling and degrading of outdated surfactant which is full of phospholipids.The most outstanding clinical feature of PAM is the discrepancy between the paucity of symptoms and the degree of pulmonary involvement.Diagnosis is easy to establish based on typical chest radiograph image and nuclear medicine improves its early diagnosis and active evaluation.Pathology of the unique intra-alveolar lamellar microliths gives strong support for diagnosis.No effective treatment is considered valid currently.However,lung transplantation is effective for advanced-stage patients,and long term treatment of disodium etidronate seems promising. 展开更多
关键词 pulmonary alveolar MICROLITHIASIS SLC34A2 MUTATION CHEST COMPUTED tomography Treatment
下载PDF
Diffuse alveolar hemorrhage with histopathologic manifestations of pulmonary capillaritis:Three case reports
14
作者 Jun Xie Ying-Yue Zhao +1 位作者 Jing Liu Guang-Min Nong 《World Journal of Clinical Cases》 SCIE 2020年第12期2662-2666,共5页
BACKGROUND Diffuse alveolar hemorrhage(DAH)is a multicause pulmonary capillary hemorrhage or pulmonary vascular small vessel injury(mainly capillaries,including arteries and veins),causing pulmonary microcirculation b... BACKGROUND Diffuse alveolar hemorrhage(DAH)is a multicause pulmonary capillary hemorrhage or pulmonary vascular small vessel injury(mainly capillaries,including arteries and veins),causing pulmonary microcirculation blood to accumulate in the alveolar space.DAH is classified by the histological absence or presence of pulmonary capillaritis(PC)and is rarely reported in the literature.CASE SUMMARY This is a report of three girls aged 6-11 years with DAH and PC.Two patients had decreased hemoglobin and one had increased erythrocyte sedimentation rate.High-resolution computed tomography showed bilateral diffuse pulmonary infiltrate,and diagnosis of PC was confirmed by lung biopsy.Immunofluorescence test in one case showed granular IgG and a small amount of granular IgA deposit on the alveolar walls,and was negative in the other two cases,describing isolated pauci-immune PC.Treatment was with glucocorticoid alone or combination with immunosuppressants,and the symptoms resolved in all patients.CONCLUSION PC is classified as isolated and immune-mediated PC associated with systemic disease.It can be controlled in most children with glucocorticoid alone or combined with immunosuppressants. 展开更多
关键词 Diffuse alveolar hemorrhage pulmonary capillaritis GLUCOCORTICOID IMMUNOSUPPRESSANT Lung biopsy Case report
下载PDF
A Novel Mutation in FOXF1 Gene Associated with a Delayed Presentation of Alveolar Capillary Dysplasia with Misalignment of Pulmonary Veins
15
作者 Maxine S. Eikani Vladimir V. Kalinichenko +1 位作者 Arun Pradhan Julie Noe 《Case Reports in Clinical Medicine》 2015年第3期97-101,共5页
Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is a rare, fatal developmental lung disorder of neonates and infants, associated with severe persistent pulmonary hypertension unresponsive t... Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is a rare, fatal developmental lung disorder of neonates and infants, associated with severe persistent pulmonary hypertension unresponsive to treatment. We reported the case of a term newborn with delayed presentation of ACD/MPV and a novel mutation of FOXF1 gene that received supportive cardiopulmonary treatments, inhaled nitric oxide, oral sildenafil and nebulized iloprost with no clinical improvement. DNA sequence analysis of FOXF1 gene identified a novel heterozygous variant c.257G > C;p.R86P, in exon 1. At autopsy, lung histology showed the characteristic features of ACD/MPV. FOXF1 has been identified as one of the genes responsible for ACD/MPV associated with multiple congenital malformations. This is a report of a novel heterozygous variant c.257G > C;p.R86P, in the first exon of FOXF1, in a patient with delayed presentation of ACD/MPV. 展开更多
关键词 alveolar CAPILLARY DYSPLASIA with MISALIGNMENT of pulmonary VEINS Persistent pulmonary Hypertension FOXF1
下载PDF
脂氧素A4对Ⅱ型肺泡上皮细胞-间质转化的抑制作用及其机制 被引量:1
16
作者 王贵佐 杨淑梅 刘璐 《山西医科大学学报》 CAS 2024年第2期157-163,共7页
目的 探讨脂氧素A4(lipoxin A4, LXA4)对Ⅱ型肺泡上皮细胞(alveolar epithelial typeⅡcell, ATⅡ)间质转化(epithelial-mesenchymal transition, EMT)的影响及可能机制。方法 将A549细胞分为对照组、转化生长因子β1(transforming grow... 目的 探讨脂氧素A4(lipoxin A4, LXA4)对Ⅱ型肺泡上皮细胞(alveolar epithelial typeⅡcell, ATⅡ)间质转化(epithelial-mesenchymal transition, EMT)的影响及可能机制。方法 将A549细胞分为对照组、转化生长因子β1(transforming growth factor, TGF-β1)组、空质粒+TGF-β1组、pcDNA3.1-Nrf2+TGF-β1组及LXA4+TGF-β1组。TGF-β1组以5 ng/mL TGF-β1干预A549细胞48 h;空质粒+TGF-β1组和pcDNA3.1-Nrf2+TGF-β1组A549细胞先分别转染空质粒或Nrf2过表达质粒,再以5 ng/mL TGF-β1干预48 h;LXA4+TGF-β1组A549细胞先用100 nmol/L LXA4预处理6 h再给予5 ng/mL TGF-β1干预48 h。光镜观察细胞形态变化,免疫印迹及免疫荧光法检测上皮标志物E钙黏蛋白(E-cadherin)、间质标志物α-平滑肌肌动蛋白(α-smooth muscle actin, α-SMA)表达;免疫印迹法检测核因子红细胞2相关因子2(nuclear factor erythroid 2-related factor 2, Nrf2)蛋白水平。结果 与对照组相比,TGF-β1组光镜下可见部分A549细胞形态从鹅卵石状变成细长不规则状,呈间质性改变,E-cadherin表达减少(P<0.05)、α-SMA表达增多(P<0.01),Nrf2蛋白水平下调(P<0.05)。与TGF-β1组相比,pcDNA3.1-Nrf2+TGF-β1组A549细胞E-cadherin表达增多(P<0.05)、α-SMA表达减少(P<0.01);LXA4+TGF-β1组A549细胞E-cadherin上调、α-SMA下调以及Nrf2蛋白水平增多(P<0.05)。结论 LXA4可抑制A549细胞发生EMT,其机制与上调Nrf2表达相关。 展开更多
关键词 肺纤维化 Ⅱ型肺泡上皮细胞 上皮细胞-间质转化 脂氧素A4 NRF2
下载PDF
单核细胞减少与分枝杆菌感染综合征患儿的护理
17
作者 徐小燕 吕张红 孙赛君 《中华急危重症护理杂志》 CSCD 2024年第6期548-551,共4页
总结1例单核细胞减少与分枝杆菌感染综合征患儿的护理体会。针对患儿疾病罕见、移植高风险、预后未知等特点,采取降低有创诊疗风险等级,协助诊断、明确移植需求;居家肺部治疗联合远程康复,寻找最佳移植时机;双学科协同长程随访,优化患... 总结1例单核细胞减少与分枝杆菌感染综合征患儿的护理体会。针对患儿疾病罕见、移植高风险、预后未知等特点,采取降低有创诊疗风险等级,协助诊断、明确移植需求;居家肺部治疗联合远程康复,寻找最佳移植时机;双学科协同长程随访,优化患者预后等措施。患儿多次住院经反复穿刺活检及病理会诊,提示鸟分枝杆菌感染,PAS(+),DPAS(+),GATA2基因2号外显子插入突变,确诊为单核细胞减少与分枝杆菌感染综合征合并肺泡蛋白沉积症。于2022年10月行造血干细胞移植术,术后继续抗感染治疗。随访至今,间质性病变较移植前明显改善,继续抗排异治疗,现居家学习中。 展开更多
关键词 MonoMAC综合征 MAC肺病 肺泡蛋白沉积症 儿童 危重病护理
下载PDF
肺泡蛋白沉积症患者全肺灌洗术中的风险管理
18
作者 徐小燕 吕张红 孙赛君 《中华急危重症护理杂志》 CSCD 2024年第1期64-67,共4页
总结12例肺泡蛋白沉积症患者行全肺灌洗术中应用霍尔三维模型进行风险管理的护理经验。针对疾病特殊性及全肺灌洗术的多重风险,对患者风险分级管控,提前预警;引入霍尔三维模型,以全肺灌洗术围手术期为线轴,以专科知识及思辨逻辑能力系... 总结12例肺泡蛋白沉积症患者行全肺灌洗术中应用霍尔三维模型进行风险管理的护理经验。针对疾病特殊性及全肺灌洗术的多重风险,对患者风险分级管控,提前预警;引入霍尔三维模型,以全肺灌洗术围手术期为线轴,以专科知识及思辨逻辑能力系统排查及处理风险隐患,提升安全护理效能。该组患者住院治疗护理4~29 d,症状好转出院。 展开更多
关键词 霍尔三维模型 肺泡蛋白沉积症 支气管肺泡灌洗术 风险管理 危重病护理
下载PDF
注射用牛肺表面活性剂不同给药方式在新生儿胎粪吸入综合征中的应用
19
作者 张莉 黄玉焕 周曼丽 《海南医学》 CAS 2024年第6期823-828,共6页
目的探讨注射用牛肺表面活性剂不同给药方式在新生儿胎粪吸入综合征(MAS)中的应用效果。方法回顾性分析2021年3月至2023年3月南阳市第一人民医院收治的120例MAS患儿的临床资料,根据给药方式不同分为A组和B组各60例。A组患儿采用肺泡灌洗... 目的探讨注射用牛肺表面活性剂不同给药方式在新生儿胎粪吸入综合征(MAS)中的应用效果。方法回顾性分析2021年3月至2023年3月南阳市第一人民医院收治的120例MAS患儿的临床资料,根据给药方式不同分为A组和B组各60例。A组患儿采用肺泡灌洗+气管内滴入给药,B组患儿采用气管内滴入给药,连续治疗48 h。比较两组患儿的临床疗效,以及治疗前后的血气指标[氧分压(PaO_(2))、二氧化碳分压(PaCO_(2))、氧指数(OI)]、肺动脉收缩压(SPAP)、凝血纤溶指标[D-二聚体(D-D)、纤溶酶原激活抑制剂-1(PAI-1)/组织型纤溶酶原激活物(t-PA)、血小板活化因子(PAF)]、炎症因子[肿瘤坏死因子-α(TNF-α)、降钙素原(PCT)、白细胞介素-5(IL-5)、白细胞介素-13(IL-13)]水平,同时比较两组患者的康复相关指标和并发症发生情况。结果A组患儿的治疗总有效率为91.67%,明显高于B组的76.67%,差异有统计学意义(P<0.05);治疗后,A组患儿的PaO_(2)为(60.65±6.33)mmHg,明显高于B组的(56.12±5.93)mmHg,PaCO_(2)、OI、SPAP分别为(42.36±4.02)mmHg、13.10±1.12、(26.14±2.67)mmHg,明显低于B组的(45.66±4.33)mmHg、(15.66±1.53)、(29.46±3.11)mmHg,差异均有统计学意义(P<0.05);治疗后,A组患儿的血浆D-D、PAI-1/t-PA、PAF含量分别为(1.35±0.38)mg/L、3.52±0.78、(404.55±78.78)×10^(9)/L,明显高于B组的(1.00±0.31)mg/L、2.64±0.71、(340.59±65.33)×10^(9)/L,差异均有统计学意义(P<0.05);治疗后,A组患儿的血清TNF-α、PCT、IL-5、IL-13含量分别为(11.11±1.01)ng/L、(0.78±0.23)ng/mL、(0.90±0.34)pg/mL、(1.15±0.66)pg/mL,明显低于B组的(13.75±1.63)ng/L、(1.46±0.34)ng/mL、(1.50±0.40)pg/mL、(1.63±0.94)pg/mL,差异均有统计学意义(P<0.05);A组患儿的发绀、吸气性三凹征消失时间及氧疗时间、住院时间、机械通气时间明显短于B组,差异均有统计学意义(P<0.05);A组患者的并发症总发生率为1.67%,明显低于B组的15.00%,差异有统计学意义(P<0.05)。结论相较于气管内滴入给药,注射用牛肺表面活性剂肺泡灌洗+气管内滴入给药治疗MAS患儿的治疗效果更优,其可促进血气指标、凝血纤溶指标恢复,降低肺动脉高压,减轻机体炎性损伤,降低并发症发生率。 展开更多
关键词 胎粪吸入综合征 牛肺表面活性剂 肺泡灌洗 气管内滴入 炎症因子 疗效
下载PDF
肺泡灌洗术联合肺表面活性物质治疗1例儿童外源性脂质性肺炎并文献复习
20
作者 韩洁 叶泽慧 杨洋 《儿科药学杂志》 CAS 2024年第6期41-44,共4页
目的:探讨经支气管镜肺泡灌洗术联合肺表面活性物质治疗1例儿童外源性脂质性肺炎的应用价值。方法:收集我院收治的1例外源性脂质性肺炎患儿的临床诊疗资料,并进行文献分析。结果:患儿意外吸入油性稀释液体后出现咳嗽、气促进行性加重,... 目的:探讨经支气管镜肺泡灌洗术联合肺表面活性物质治疗1例儿童外源性脂质性肺炎的应用价值。方法:收集我院收治的1例外源性脂质性肺炎患儿的临床诊疗资料,并进行文献分析。结果:患儿意外吸入油性稀释液体后出现咳嗽、气促进行性加重,血氧饱和度降低,血炎症指标增高,胸部计算机断层扫描(CT)见双肺多发性病变,确诊外源性脂质性肺炎后及时予以经支气管镜肺泡灌洗术,并首次联合应用肺表面活性物质经支气管镜注入治疗,同时予以无创正压机械通气、抗感染、糖皮质激素雾化等治疗。治疗后,患儿咳嗽、气促、低氧血症等临床表现及胸部影像学短期内明显好转。结论:儿童外源性脂质性肺炎早期应用经支气管镜肺泡灌洗术联合肺表面活性物质治疗临床疗效好,有较大临床推广价值。 展开更多
关键词 儿童 外源性脂质性肺炎 胸部计算机断层扫描 经支气管镜肺泡灌洗术 肺表面活性物质
下载PDF
上一页 1 2 50 下一页 到第
使用帮助 返回顶部