PULMONARY ALVEOLAR PROTEINOSIS IN THREE INFANTS

Chronic lung disease in children may present a perplexing diagnostic problem. While open lung biopsy usually will provide a diagnosis without excessive morbidity, we believe that pulmonary alveolar proteinosis can be diagnosed radiographically, and we wish to point out the early age at which this disease can occur. The clinical course of three infants is described together with the necropsy findings. Two 3-month-old and one 23-month-old infants are reported. The radiographic appearance of diffuse alveolar consolidation and the progressive respiratory involvement should alert physicians to the true nature of this disease. Recovery is reported in adults, but results of treatment in infants is disappointing.

Download Full-text

A case of uncomplicated pulmonary alveolar proteinosis evolving to pulmonary fibrosis

Monaldi Archives for Chest Disease ◽

10.4081/monaldi.2007.480 ◽

2016 ◽

Vol 67 (4) ◽

Cited By ~ 1

Author(s):

A. Chroneou ◽

N. Zias ◽

B.S. Tronic ◽

A.V. Gonzalez ◽

J.F. Beamis Jr.

Keyword(s):

Pulmonary Fibrosis ◽

Pulmonary Alveolar Proteinosis ◽

Clinical Course ◽

Case Reports ◽

Disease Onset ◽

Open Lung Biopsy ◽

Open Lung ◽

Cellular Debris ◽

Surfactant Production ◽

Alveolar Proteinosis

Pulmonary alveolar proteinosis (PAP) is a rare syndrome characterised by intra-alveolar accumulation of surfactant components and cellular debris, with minimal interstitial inflammation or fibrosis. Since surfactant accumulates abnormally, a disturbance in the normal pathway of surfactant production, metabolism, recycling or degradation has been postulated. This disease has a variable clinical course: from spontaneous resolution to respiratory failure and death due to disease progression or superimposed infections. PAP leading to pulmonary fibrosis is rarely seen, and few case reports describe this association. Here, we describe the case of a patient with a diagnosis of PAP confirmed by open lung biopsy, who developed interstitial pulmonary fibrosis years after disease onset.

Download Full-text

Exogenous Lipid Pneumonia Related to Smoking Weed Oil Following Cadaveric Renal Transplantation

Canadian Respiratory Journal ◽

10.1155/2000/248915 ◽

2000 ◽

Vol 7 (4) ◽

pp. 338-342 ◽

Cited By ~ 22

Author(s):

Dilini Vethanayagam ◽

Stewart Pugsley ◽

EJ Dunn ◽

David Russell ◽

J Michael Kay ◽

...

Keyword(s):

Renal Transplantation ◽

Pulmonary Alveolar Proteinosis ◽

Open Lung Biopsy ◽

Cadaveric Renal Transplantation ◽

Open Lung ◽

Exogenous Lipid ◽

Progressive Respiratory Failure ◽

Initial Improvement ◽

Alveolar Proteinosis ◽

Lipid Pneumonia

A 30-year-old female presented shortly after cadaveric renal transplantation with respiratory distress typical of a bacterial infection. Following initial improvement, she developed progressive respiratory failure, initially felt to be secondary to cytomegalovirus infection. Two bronchoalveolar lavages were nondiagnostic, and an open lung biopsy was performed, which revealed a pulmonary alveolar proteinosis (PAP) reaction and exogenous lipid pneumonia (ELP). The ELP was considered to be secondary to the use of marijuana, in the form of weed oil, that was smoked daily for over 10 years and stopped just before renal transplantation. This is the first description of both PAP and ELP following renal transplantation, and the first description of ELP related to smoking weed oil. Physicians should be aware of the different forms of marijuana available and of their potential medical complications.

Download Full-text

Primary pulmonary alveolar proteinosis

Vojnosanitetski pregled ◽

10.2298/vsp1211005s ◽

2012 ◽

Vol 69 (11) ◽

pp. 1005-1008 ◽

Cited By ~ 1

Author(s):

Sanja Sarac ◽

Rade Milic ◽

Lidija Zolotarevski ◽

Slobodan Acimovic ◽

Ilija Tomic ◽

...

Keyword(s):

Lung Biopsy ◽

Pulmonary Alveolar Proteinosis ◽

Lung Lavage ◽

Open Lung Biopsy ◽

Adequate Treatment ◽

Open Lung ◽

Uncommon Disease ◽

Colonystimulating Factor ◽

Alveolar Proteinosis ◽

Bilateral Lung

Introduction. Pulmonary alveolar proteinosis is an uncommon disease characterized by the accumulation of surfactant proteins and phospholipids within the alveolar spaces. Acquired disease can be idiopathic (primary) and secondary. The prevalence of acquired pulmonary alveolar proteinosis is about 0.37 per 100,000 persons. Common symptoms are dyspnea and cough. Chest X-ray shows bilateral perihilar infiltrates. Open-lung biopsy is the gold standard for the diagnosis. Treatment includes whole-lung lavage, application of granulocyte-macrophage colonystimulating factor and lung transplantation. Case report. We reported a 51 year-old man with primary form of the disease. It was the second case of this extremely rare disease in the past 30 years in our clinic. The symptoms were longlasting dry cough, fever and physical deterioration. Chest Xray revealed bilateral pulmonary infiltrates; computed tomography showed patchy ground-glass opacification with interlobular thickening. The diagnosis was established by open lung biopsy. Additional tests were performed to exclude secondary form of the disease. Conclusion. We presented a rare clinical entity with typical clinical features and clinical and radiological course of the disease, in order to improve differential diagnostic approach to patients with bilateral lung infiltrations. In patients with pulmonary alveolar proteinosis timely diagnosis and adequate treatment can improve a prognosis.

Download Full-text

Cytopathological examination of bronchoalveolar lavage fluid in diagnosis of pulmonary alveolar proteinosis

Journal of Laboratory Physicians ◽

10.4103/jlp.jlp_109_17 ◽

2018 ◽

Vol 10 (01) ◽

pp. 109-112 ◽

Cited By ~ 1

Author(s):

Manjari Kishore ◽

Manju Kaushal ◽

Desh Deepak ◽

Manju Kumari

Keyword(s):

Bronchoalveolar Lavage ◽

Bronchoalveolar Lavage Fluid ◽

Pulmonary Alveolar Proteinosis ◽

Diagnostic Yield ◽

Lavage Fluid ◽

Open Lung Biopsy ◽

Open Lung ◽

Elderly Female ◽

Alveolar Proteinosis ◽

Fluid Cytology

AbstractPulmonary alveolar proteinosis (PAP) is a rare disease characterized by the deposition of extracellular lipoproteinaceous material within the air spaces. Although the diagnosis is mainly based on histopathological findings, sometimes, the diagnostic yield of transbronchial and even open lung biopsy can be unsatisfactory. The advantage with bronchoalveolar lavage (BAL) cytology is that apart from being safer for the patient, it can sample a much wider area and help in giving an early diagnosis and treatment to the patient. Herein, we present a case of PAP diagnosed on BAL fluid cytology in an elderly female.

Download Full-text

Pulmonary Alveolar Proteinosis in an AIDS Patient without Concurrent Pulmonary Infection

Canadian Respiratory Journal ◽

10.1155/1995/958415 ◽

1995 ◽

Vol 2 (3) ◽

pp. 183-186

Author(s):

Allen T Liu ◽

Lil J Miedzinski ◽

Eric Vallieres ◽

David C Rayner ◽

Dale C Lien

Keyword(s):

Pulmonary Infection ◽

Pulmonary Alveolar Proteinosis ◽

Open Lung Biopsy ◽

Open Lung ◽

Increased Risk ◽

Acquired Immunodeficiency ◽

Lung Infections ◽

Alveolar Proteinosis ◽

Immunodeficiency Syndrome ◽

Secondary Pulmonary Alveolar Proteinosis

Patients with acquired immunodeficiency syndrome (AIDS) are potentially at increased risk for developing secondary pulmonary alveolar proteinosis because of underlying immunosuppression and frequent opportunistic lung infections. This condition. however, has been diagnosed uncommonly in these patients and, with the exception of one previously reported case. only in the presence of concurrent pulmonary infection. The case of a 35-year-old male with AIDS who was found on open lung biopsy to have pulmonary alveolar proteinosis without evidence of associated lung infection is presented.

Download Full-text

The Clinical Clues of Pulmonary Alveolar Proteinosis: A Report of 11 Cases and Literature Review

Canadian Respiratory Journal ◽

10.1155/2016/4021928 ◽

2016 ◽

Vol 2016 ◽

pp. 1-5 ◽

Cited By ~ 1

Author(s):

Qiongya Mo ◽

Bingbin Wang ◽

Nian Dong ◽

Lianmin Bao ◽

Xiaoqiong Su ◽

...

Keyword(s):

Bronchoalveolar Lavage Fluid ◽

Clinical Symptoms ◽

Pulmonary Alveolar Proteinosis ◽

Relevant Literature ◽

Neuron Specific Enolase ◽

Lavage Fluid ◽

Lung Lavage ◽

Serum Markers ◽

Open Lung Biopsy ◽

Alveolar Proteinosis

Pulmonary alveolar proteinosis (PAP) is a rare interstitial lung disease characterized by the abnormal alveolar accumulation of surfactant components. The diagnosis of PAP can be easily missed since it is rare and lacks specific clinical symptoms. It is of great importance to have a better understanding of the crucial clue to clinically diagnose PAP and take PAP into consideration in the differential diagnosis of interstitial pulmonary diseases or other diseases with similar manifestations. Here, we analyze the clinical characteristics of 11 cases of PAP patients in local hospital and review the relevant literature in order to provide more information in diagnosis and management of PAP. In our observation, cyfra21-1 and neuron-specific enolase (NSE) known as tumor markers probably can be useful serum markers for diagnosis of PAP. As for the method of pathologic diagnosis, open-lung biopsy was the gold standard but now it is less required because findings on examination of bronchoalveolar lavage fluid (BALF) can help to make the diagnosis. We also have deep experience about when and how to carry out lung lavage.

Download Full-text