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
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
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
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
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
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
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
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
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
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
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
基金Supported by Sanitation Research Project of Kunming Municipal Health Commission,No.2020-06-01-119.
文摘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.
文摘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.
基金Supported by the Joint Project of Chongqing Health Commission and Science and Technology Bureau,No.2022MSXM103.
文摘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.
基金the National Science and Technology Major Project of the Ministry of Science and Technology of China,No.2018ZX10715-003.
文摘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.
文摘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.
基金Supported by The Medical and Health Technology Project of Zhejiang Province,No.2018KY567and the Hangzhou Municipal Science and Technology Development Program,No.20142013A61.
文摘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.
文摘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
文摘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.
文摘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.
文摘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）.
文摘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.
