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Pulmonary alveolar proteinosis induced by X-linked agammaglobulinemia:A case report 被引量:1
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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
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Clinical approach for pulmonary alveolar proteinosis in children
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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
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Unexpected diffuse lung lesions in a patient with pulmonary alveolar proteinosis:A case report
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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
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Pulmonary alveolar proteinosis complicated with nocardiosis: A case report and review of the literature 被引量:2
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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
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Pulmonary alveolar proteinosis complicated with tuberculosis:A case report 被引量:2
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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
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Chronic myelomonocytic leukemia-associated pulmonary alveolar proteinosis:A case report and review of literature 被引量:1
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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
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CT MANIFESTATIONS IN PULMONARY ALVEOLAR PROTEINOSIS AND COMPARED WITH CHEST RADIOGRAPHY(REPORT OF SIX CASES)
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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
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A Case Report of Pulmonary Alveolar Proteinosis with Associated Opportunistic Infection of Pneumocystis jirovecii and Molluscum Contagiosum
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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
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Analysis of the GM-CSF and GM-CSF/IL-3/IL-5 receptor common beta chain in a patient with pulmonary alveolar proteinosis
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作者 王选锭 刘富光 Burkhard Bewig 《Chinese Medical Journal》 SCIE CAS CSCD 2002年第1期76-80,149,共5页
Objective To investigate the expression of the granulocyte-macrophage colony-stimulating factor (GM-CSF) and GM-CSF/IL-3/IL-5 receptor common beta chain (βc receptor) in an adult patient with idiopathic pulmonary al... Objective To investigate the expression of the granulocyte-macrophage colony-stimulating factor (GM-CSF) and GM-CSF/IL-3/IL-5 receptor common beta chain (βc receptor) in an adult patient with idiopathic pulmonary alveolar proteinosis (PAP), so as to demonstrate the possible association of the GM-CSF and βc receptor with the pathogenesis of human PAP.Methods The GM-CSF levels were measured with a commercial ELISA kit (sensitivity 5?pg/ml) and the βc receptor expression on the cell surface was detected by flow cytometry analysis. Reverse transcription-polymerase chain reaction (RT-PCR) analysis was employed to detect the expression of the GM-CSF mRNA and the βc receptor mRNA in peripheral blood mononuclear cells and alveolar macrophages. The entire coding regions of the GM-CSF cDNA and the βc receptor cDNA were sequenced by the Sanger dideoxy-mediated chain termination method to detect possible mutations.Results The patient with PAP failed to release the GM-CSF protein either from circulating mononuclear cells or from alveolar macrophages. The expression of the GM-CSF mRNA was normal after the stimulation of lipopolysaccharide, whereas a point mutation at position 382 of the GM-CSF cDNA from 'T' to 'C' was revealed by cDNA sequencing, which caused a change in amino acid 117 of the protein from isoleucine to threonine. The βc receptor expression on the cell surface was normal, and the βc receptor mRNA expression and the sequence of the entire coding region of the βc receptor were also normal.Conclusions The decreased GM-CSF production is associated with the pathogenesis of human PAP. A point mutation of the GM-CSF cDNA may contribute to the decreased GM-CSF production in our adult PAP patient. The mutation of the βc receptor in some of paediatric patients with PAP may not be a common problem in adult patients. 展开更多
关键词 pulmonary alveolar proteinosis · granulocyte-macrophage colony-stimulating factor (GM-CSF) · GM-CSF/IL-3/IL-5 receptor common beta chain · mutation
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Pulmonary alveolar proteinosis in an indium-processing worker 被引量:23
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作者 XIAO Yong-long CAI Hou-rong +2 位作者 WANG Yi-hua MENG Fan-qing ZHANG De-ping 《Chinese Medical Journal》 SCIE CAS CSCD 2010年第10期1347-1350,共4页
With the increasing number of workers engaged in liquid-crystal displays (LCD) manufacturer, lung diseases related to this occupational exposure are attracting more attention. Herein we report a case of interstitial... With the increasing number of workers engaged in liquid-crystal displays (LCD) manufacturer, lung diseases related to this occupational exposure are attracting more attention. Herein we report a case of interstitial lung disease in a LCD processing worker, which was pathologically confirmed as pulmonary alveolar proteinosis (PAP). 展开更多
关键词 CHOLESTEROL INDIUM lung lavage pulmonary alveolar proteinosis
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Hyperoxygenated solution for improved oxygen supply in patients undergoing lung lavage for pulmonary alveolar proteinosis 被引量:5
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作者 ZHOU Bin ZHOU Hai-yan +3 位作者 XU Pei-hua WANG Hong-mei LIN Xian-ming WANG Xuan-ding 《Chinese Medical Journal》 SCIE CAS CSCD 2009年第15期1780-1783,共4页
Background At present, the most effective treatment for pulmonary alveolar proteinosis (PAP) remains whole-lung lavage in spite of the usually accompanying severe hypoxemia, which is expected to be prevented by hype... Background At present, the most effective treatment for pulmonary alveolar proteinosis (PAP) remains whole-lung lavage in spite of the usually accompanying severe hypoxemia, which is expected to be prevented by hyperoxygenated solution improving oxygen supply during lavage. In this study, the efficacy and safety of the effect of hyperoxygenated solution were evaluated. Methods Five patients underwent whole-lung lavage over a 28-month period. Each lung was lavaged with hyperoxygenated (HO) and normal saline solution (plain lactated Ringer's solution, NO) randomly and alternatively until the reclaimed fluid was clear. Random number was generated by computer before every cycle of lavage. If the number was odd, the patient was assigned to receive a lavage cycle with hyperoxygenated solution (HO group, n=-109); if the number was even, normal saline solution was used (NO group, n=-115). Data of saturation of peripheral oxygen (SPO2), mean arterial pressure (MAP), central venous pressure (CVP), heart rate (HR) and end-tidal carbon dioxide tension (PETCO2) were taken down at 0, 30, 60, 90, 120, 150, 180, 210 and 240 seconds from the beginning of the instillation of solution, and frequency and volume of unilateral lung lavage were also recorded. Time interval between the leR and the right lung lavage was 1 week. Results No patient was withdrawn from the study due to low SPO2 or leakage. Oxygen pressure was (730.21±7.43) mmHg in the hyperoxygenated solution against (175.73±5.92) mmHg in the normal saline solution (P 〈0.01). Compared with baseline, 8PO2 increased significantly as the instillation of solution began (P〈0.01), leveled for about 30 seconds (P 〉0.05), and then decreased significantly to the lowest at the time of drainage (compared with 120 seconds or peak, P 〈0.01). SPO2 was higher in HO group than in NO group (P 〈0.01). There were no significant differences in MAP, HR, CVP and PETCO2 between HO group and NO group (P 〉0.05) and also among different time points (P 〉0.05). Conclusion During the lung lavage for pulmonary alveolar proteinosis, hyperoxygenated solution could significantly improve oxygen supply in comparison with normal saline solution without obvious side effects. 展开更多
关键词 pulmonary alveolar proteinosis oxygen bronchoalveolar lavage
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Pulmonary alveolar proteinosis treated with whole-lung lavage utilizing extracorporeal membrane oxygenation:a case report and review of literatures 被引量:3
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作者 蔡后荣 崔叔阳 +6 位作者 金陵 黄贻真 王喆研 曹彬 母国华 王尔东 周贤梅 《Chinese Medical Journal》 SCIE CAS CSCD 2004年第11期1746-1749,共4页
关键词 extracorporeal membrane oxygenation cardiopulmo nary bypass pulmonary alveolar proteinosis
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Pulmonary alveolar proteinosis associated with tuberculosis and aspergilloma formation 被引量:2
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作者 HUANG Xiao-ying YU Chang +4 位作者 XU Xiao-mei YANG Wen-tao ZHANG Xie LIU Pan-pan WANG Liang-xing 《Chinese Medical Journal》 SCIE CAS CSCD 2012年第17期3191-3192,共2页
Pulmonary alveolar proteinosis (PAP) is a rare diffuse lung disease, characterized by accumulation of surfactant-associated phosphor lipoproteinaceous material in the alvoli. Finding the periodic acid-Schiff (PAS)... Pulmonary alveolar proteinosis (PAP) is a rare diffuse lung disease, characterized by accumulation of surfactant-associated phosphor lipoproteinaceous material in the alvoli. Finding the periodic acid-Schiff (PAS) positive substance in the alveolar lavage can help to make the diagnosis. 展开更多
关键词 pulmonary alveolar proteinosis TUBERCULOSIS ASPERGILLOMA whole lung lavage
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Whole Lung Lavage Treatment of Chinese Patients with Autoimmune Pulmonary Alveolar Proteinosis: A Retrospective Long-term Follow-up Study 被引量:2
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作者 Yu-Yue Zhao Hui Huang Yong-Zhe Liu Xin-Yu Song Shan Li Zuo-Jun Xu 《Chinese Medical Journal》 SCIE CAS CSCD 2015年第20期2714-2719,共6页
Background: Pulmonary alveolar proteinosis (PAP) is a rare lung disease, the most common type of which is autoimmune PAP. The gold standard therapy for PAP is whole lung lavage (WLL). Few studies have reported th... Background: Pulmonary alveolar proteinosis (PAP) is a rare lung disease, the most common type of which is autoimmune PAP. The gold standard therapy for PAP is whole lung lavage (WLL). Few studies have reported the optimal technique with which to evaluate the response to WLL. In this study, we aimed to identify parameters with which to assess the need for repeat WLL during a long-term 8-year follow-up. Methods: We conducted a retrospective analysis of 120 patients with autoimmune PAP with 80 of whom underwent WLL. Physiologic, serologic, and radiologic features of the patients were analyzed during an 8-year follow-up after the first WLL treatment. Results: Of the 40 patients without any intervention, 39 patients either achieved remission or remained stable and only one died of pulmonary infection. Of the 56 patients who underwent WLL for 1 time, 55 remained free from a second WLL and 1 patient died of cancer. Twenty-four required additional treatments after their first WLL. The baseline PaO2, (P = 0.000), PA-aO2 (P = 0.000), shunt fraction rate (P = 0.001), percent of predicted normal diffusing capacity of the lung for carbon monoxide (DLCO%Pred) (P = 0.016), 6-rain walk test (P = 0.013), carcinoembryonic antigen (CEA) (P = 0.007), and neuron-specific enolase (NSE) (P = 0.003) showed significant differences among the three groups. The need for a second WLL was significantly associated with PaO2 (P = 0.000), CEA (P= 0.050), the 6-minute walk test (P= 0.026), and DLCO%Pred (P = 0.041 ). The DLCO%Pred on admission with a cut-off value of42.1% (P = 0.001) may help to distinguish whether patients with PAP require a second WLL. Conclusions: WLL is the optimal treatment method for PAP and provides remarkable improvements for affected patients. The DLCO%Pred on admission with a cut-offvalue of 42.1% may distinguish whether patients with PAP require a second WLL. 展开更多
关键词 Diffusing Capacity of the Lung fbr Carbon Monoxide PROGNOSIS Pulmonary Alveolar proteinosis Treatment:Whole Lung Lavage
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Secondary pulmonary alveolar proteinosis associated with myelodysplastic syndrome 被引量:1
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作者 XIE Li-xin ZHAO Tie-mei +5 位作者 WANG Qiao-yun CHEN Liang-an LI Ai-min WANG Dian-jun QI Fei LIU You-ning 《Chinese Medical Journal》 SCIE CAS CSCD 2007年第12期1114-1116,共3页
Pulmonary alveolar proteinosis (PAP) IS an uncommon disease first reported by Rosen et al^1 in 1958, and characterized by the accumulation of surtactant proteins and phospholipids within the alveolar spaces. Acquire... Pulmonary alveolar proteinosis (PAP) IS an uncommon disease first reported by Rosen et al^1 in 1958, and characterized by the accumulation of surtactant proteins and phospholipids within the alveolar spaces. Acquired PAP is divided into two forms based on clinical features: idiopathic PAP and secondary PAE Secondary PAP is reported to be associated with haematological malignancies, Pneumocystis carinii pneumonia and inhalation of silica or titanium, and the most frequent underlying disease of secondary PAP is haematological malignancy. The exact incidence of PAP in haematological malignancies is still obscure, since there have been only sporadic reports of secondary PAP.^ 2, 3 展开更多
关键词 myelodysplastic syndrome pulmonary proteinosis AUTOPSY leukaemic phase
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Isolated cerebral aspergillus abscess as a complication of pulmonary alveolar proteinosis in a child
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作者 Rachna Khera Venkateswara Rao +3 位作者 Mohan Krishna Pasam Ravindranath Tagore Sudha SMurthy Challa Sundaram 《Chinese Neurosurgical Journal》 CSCD 2019年第3期182-185,共4页
Pulmonary alveolar proteinosis (PAP) poses a risk of opportunistic infections with a variety of organisms with Nocardia being the most common pathogen followed by mycobacteria and fungi. Case presentation: A 7-year-ol... Pulmonary alveolar proteinosis (PAP) poses a risk of opportunistic infections with a variety of organisms with Nocardia being the most common pathogen followed by mycobacteria and fungi. Case presentation: A 7-year-old female child, presented with headache and multiple episodes of vomiting. There was no fever or altered sensorium. On examination, there were no focal deficits or cranial nerve palsies. An MRI brain showed a small T2 hyperintense lesion in the left superior parietal lobe suggestive of an abscess. She was diagnosed as PAP based on CT chest and bronchioloalveolar lavage 7 months earlier and treated with corticosteroids. A left parieto-occipital craniotomy was done with drainage of abscess and abscess wall excision. Histopathology revealed a suppurative lesion with slender septate acute angle branching hyphae which were positive on fungal stains. Culture done on the pus was positive for Aspergil us fumigatus. The patient was treated with voriconazole and stable at 1 year follow-up. Conclusion: Opportunistic infections are common in patients diagnosed with PAP. High index of clinical suspicion and early diagnosis are important for favorable outcome. 展开更多
关键词 Pulmonary ALVEOLAR proteinosis OPPORTUNISTIC infection ASPERGILLUS FUMIGATUS CEREBRAL ABSCESS
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Diagnostic investigation of the detection of granulocyte-macrophage colony stimulating factor antibody in serum for pulmonary alveolar proteinosis
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作者 李玉茜 《China Medical Abstracts(Internal Medicine)》 2013年第3期164-164,共1页
Objective To evaluate the diagnostic value of granulocyte-macrophage colony stimulating factor (GM-CSF) antibody in serum for pulmonary alveolar proteinosis (PAP) .Methods Twelve PAP patients visiting Peking Universit... Objective To evaluate the diagnostic value of granulocyte-macrophage colony stimulating factor (GM-CSF) antibody in serum for pulmonary alveolar proteinosis (PAP) .Methods Twelve PAP patients visiting Peking University People’s Hospital or Fujian Provincial 展开更多
关键词 proteinosis ALVEOLAR MACROPHAGE GRANULOCYTE stimulating COLONY Fujian PEKING visiting TWELVE
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The ultrastructural features of sputum deposition and its value in the diagnosis of pulmonary alveolar proteinosis
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作者 罗本芳 《China Medical Abstracts(Internal Medicine)》 2013年第1期44-44,共1页
Objective To investigate the ultrastructural features of sputum deposition(SD) and its value in the diagnosis of pulmonary alveolar proteinosis(PAP). Methods Seven patients with PAP diagnosed by lung biopsy and cytolo... Objective To investigate the ultrastructural features of sputum deposition(SD) and its value in the diagnosis of pulmonary alveolar proteinosis(PAP). Methods Seven patients with PAP diagnosed by lung biopsy and cytology were enrolled in this study. The patients consisted 展开更多
关键词 proteinosis ALVEOLAR CYTOLOGY enrolled biopsy Seven LAVAGE macrophages DEGENERATION LAMELLAR
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类脂质蛋白沉积症1例并文献复习 被引量:1
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作者 李云霞 马丽晶 +1 位作者 徐文 周兵 《中国耳鼻咽喉头颈外科》 CSCD 2016年第3期185-186,共2页
1临床资料 患者,女,32岁。因"自幼持续性声音嘶哑,加重1年"就诊于我院。患者出生时哭声弱,学语时出现声音嘶哑,发音费力,无构音障碍,无口腔溃疡,无癫痫发作,无脱发、无睫毛脱落,感冒时声音嘶哑加重,可出现失声。5岁出现双侧上眼睑淡... 1临床资料 患者,女,32岁。因"自幼持续性声音嘶哑,加重1年"就诊于我院。患者出生时哭声弱,学语时出现声音嘶哑,发音费力,无构音障碍,无口腔溃疡,无癫痫发作,无脱发、无睫毛脱落,感冒时声音嘶哑加重,可出现失声。5岁出现双侧上眼睑淡黄色串珠样丘疹,无舌体变硬,口唇变厚。 展开更多
关键词 Urbach-Wiethe类脂蛋白沉积症(Lipoid proteinosis of Urbach and Wiethe) 喉(Larynx) 皮肤(Skin)
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肺蛋白沉积症肺泡灌洗术的护理 被引量:2
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作者 宋晨 《罕少疾病杂志》 2015年第2期4-5,共2页
肺蛋白沉积症(Pulmonary alveolar proteinosis,PAP)是一种以肺泡和终末呼吸性细支气管腔富含的磷脂蛋白质物质沉积为特征的肺部少见病,本病主要影响肺部气体交换,发病多隐匿。临床表现差异较大,有的无临床症状,有的以进行性气促、低... 肺蛋白沉积症(Pulmonary alveolar proteinosis,PAP)是一种以肺泡和终末呼吸性细支气管腔富含的磷脂蛋白质物质沉积为特征的肺部少见病,本病主要影响肺部气体交换,发病多隐匿。临床表现差异较大,有的无临床症状,有的以进行性气促、低氧血症为主要表现,有的因呼吸衰竭最终导致死亡。本文就肺蛋白沉积症肺泡灌洗护理的进展做一综述。 展开更多
关键词 肺蛋白沉积症 proteinosis 灌洗术 肺泡灌洗 低氧血症 ALVEOLAR 呼吸性细支气管 气体交换 肺泡蛋白沉积症 呼吸衰竭
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