DOCSLIB.ORG

Successful Treatment of a Case with Rapidly Progressive Bronchiolitis

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Successful Treatment of a Case with Rapidly Progressive Bronchiolitis CASE REPORT Successful Treatment of a Case with Rapidly Progressive Bronchiolitis Obliterans Organizing Pneumonia (BOOP) using Cyclosporin A and Corticosteroid Daizo Koinuma, Makoto Miki, Masahito Ebina, Minoru Tahara, Koichi Hagiwara, Takashi Kondo*, Yoshio Taguchi** and Toshihiro Nukiwa Abstract therapy, which results in a better prognosis comparedwith id- iopathic pulmonary fibrosis (9, 10). There are reports, how- A60-year-old womanwas suffering from acute onset and ever, of patients with steroid-resistant idiopathic BOOPhav- progressive respiratory distress. Her radiographic findings ing an unfavorable outcome (1 1-15). showed bilateral volume loss in her lower lobes and con- In this report we present a steroid-resistant patient with an solidation predominantly distributed in peribronchovas- acute onset of idiopathic BOOP,showing consolidation dis- cular areas. The biopsied specimens performed by video- tributed in the peribronchovascular regions by chest CT, who assisted thoracoscopic surgery revealed prominent fibro- responded well to the simultaneous administration of myxoidconnective tissue within the terminal respiratory cyclosporin Aand corticosteroid. bronchioles and the alveolar spaces along the airways with- out marked interstitial fibrosis. No relevant cause was deter- Case Report mined, and she was diagnosed as having idiopathic BOOR Although her clinical course was fulminant with a poor A 60-year-old womanwas admitted to our hospital with reaction to steroid therapy, simultaneous administration of dyspneaand bilateral hilar consolidation shownon a chest roent- cyclosporin Aand corticosteroid elicited a rapid improve- genogram. She was a green grocer whohad never smoked. She ment. This case report presents the effectiveness of had been in good health until two months prior to admission, cyclosporin A in the treatment of progressive BOOR whenshe developed dyspnea with effort and a productive cough (Internal Medicine 41 : 26-29, 2002) after cleaning a dusty room that had not been used for years. She consulted a physician whoreferred her to us based on the Key words: cryptogenic organizing pneumonia,therapy roentogenographic findings. On admission, she presented as an obese woman(BH, 156.2 cm; BW, 72.2 kg) without fever or tachypnea (RR, 12/min; BT, 36.8°C). No cyanosis, eruption, or clubbing was noted. Introduction Chest auscultation revealed minimal coarse crackles at the base of each lung posteriorly. Chest roentgenogram and chest CT Bronchiolitis obliterans organizing pneumonia (BOOP)is a showed loss of volume bilaterally in the lower lobes and clinicopathological diagnosis with characteristic pathologic peribronchovascular consolidation in both lungs (Fig. 1A, and features reported first by Epler et al (1). Because organizing B). Her WBCcount was 6.4x109// without eosinophilia. The pneumonia is a non-specific inflammatory process it can be ESR was augmented (58 mrn/h), but the serum CRP level was caused by a number of pathogens (2, 3), and heterogeneity in within normal range (0.22 mg/dl). Neither rheumatoid factor radiographic features and clinical outcome has been reported nor anti-nuclear antibody was detected. Serum KL-6 and SP- (4-6) even in cases with idiopathic BOOPor cryptogenic or- Dlevels, markers for lung epithelial injury, were elevated to ganizing pneumonia (COP) (7). Lee et al characterized CT find- 1,390 U/ml and 341 ng/ml, respectively. ings in 43 patients with COPand categorized the distribution Arterial blood gas analysis under room air showedslight patterns of consolidation (8). As for the clinical course of pa- hypoxemia (PaCO2 of 42.3 Torr, PaO2 of 71.4 Torr). A pulmo- tients with BOOP,they usually respond well to corticosteroid nary function test revealed mild restrictive impairment (1.86 / From the Department of Respiratory Oncology and Molecular Medicine, *the Department of Thoracic Surgery, Institute of Development, Aging and Cancer, Tohoku University, Sendai and **Tenri Yorozu Sodansho Hospital, Tend Received for publication February 15, 2001 ; Accepted for publication September 24, 2001 Reprint requests should be addressed to Dr. Masahito Ebina, the Department of Respiratory Oncology and Molecular Medicine, Institute of Development, Aging and Cancer, Tohoku University, 4-1 Seiryo-machi, Sendai, Miyagi 980-8575 26 Internal Medicine Vol. 41, No. 1 (January 2002) Fulminant BOOPResponsive to Cyclosporin A Figure 1. A) Chest roentgenogram on admission. Bihilar consolidation was accompanied by depletion of lung volume. B) Chest CTon admission. Consolidation shows a peribronchovascular distribution. of VC(75.8%)) and deteriorated diffusion capacity (68.5% of %DLC0). Bronchoalveolar lavage fluid (BALF) from the right B5b showed a moderately increased cell count of 22.5x104/ml and minimal granulocytosis without eosinophilia. Immunocyto- metry of BALFcells showed a decreased CD4+/CD8+ratio of 0.14. No pathogen was cultured. A pathologic diagnosis of BOOP,depended on video-assisted lung-biopsy specimens from apical (SI), inferior lingular (S5) and anterior-basal (S8) seg- ments of her right lung, which showed patchy intraluminal or- ganization and proteinaceous exudates within the terminal and respiratory bronchioles. Granulation tissue was distributed in the alveolar spaces around the bronchioles without remarkable interstitial fibrosis (Fig. 2). Although she was first treated with 1 g/day of intravenous methylprednisolone for three days, followed by 30 mg/day (0.42 mg/kg) of oral prednisolone, her dyspnea was exacerbated and her arterial PaO2 declined to 58 mniHg. Adjunctive cyclosporin A therapy was initiated at 75 mg/day, which was later increased Figure 2. Elastica-Masson staining of a lung biopsied specimen to 150 mg/day. The serum trough levels of cyclosporin Awere obtained by video-assisted thoracoscopic surgery (original mag- 47 ng/ml at 75 mg/day and 160 ng/ml at 150 mg/day. As a nification at x40). Intraluminal organization within a terminal bronchiole (an arrow head) and a respiratory bronchiole (an ar- result, her dyspnea diminished and her laboratory data improved row), as well as within alveolar spaces along the bronchioles was to 82.1 TorrofPaO2, 2.25 /of VC (91.8%), and 85.8% of%DLC0. revealed without remarkable interstitial fibrosis. Serum markers also decreased to 646 U/ml of KL-6, and 55.4 ng/ml of SP-D. Peribronchovascular consolidation on chest radiography resolved steadily. After prednisolone was tapered to 25 mg/day, she was discharged on her 91st hospital day. Discussion Cyclosporin A was discontinued after four months of treat- ment without serious side effects. The patient had no signs of BOOPis a heterogeneous disease with a clinicopathologi- recurrence on 12.5 mg/day of prednisolone (213 U/ml of KL- cal definition, caused by various pathogens (2, 3). The typical 6) for eight months following discharge. Linear scars accom- presenting clinical symptomof BOOPstarts with a subacute panied by volumedepletion as seen on chest roentgenogram onset with patchy consolidation on the chest X-ray (2, 3, 16). have persisted (Fig. 3). A definitive diagnosis of BOOPrequires characteristic patho- Internal Medicine Vol. 41, No. 1 (January 2002) 27 Koinumaet al uted predominantly in both of the lower lobes is also an atypi- cal finding for BOOR Corticosteroid-resistance in patients with BOOPis reported to be rare (1 1-15, 21). Cyclophosphamide or azathioprine has been administered simultaneously with a corticosteroid as an alternative therapy with (21-23) or without (13, 15) underly- ing diseases, though their utility has not been established. To et al reported a 27-year-old man with BOOPwho recurred de- spite corticosteroid therapy but whoresponded to cyclosporin A and Pirfenidone, without corticosteroids (24). Wehave suc- cessfully treated a patient with fulminant BOOPusing cyclosporin A combined with corticosteroid. This case indi- cates that cyclosporin A can be useful for corticosteroid-resis- tant BOOP.Further study is warranted to define the utility of this treatment. References Figure 3. Chest CTsix months post treatment. Resolution of 1) Epler GR, Colby TV, McLoud TC, Carrington CB, Gaensler EA. Bron- consolidation was seen, but the bilateral volume loss in the lower chiolitis obliterans organizing pneumonia. N Engl J Med 312: 152-158, lobes persisted. 1985. 2) Epler GR. Bronchiolitis obliterans organizing pneumonia: definition and clinical features. Chest 102: 2s-6s, 1992. 3) Cordier JF. Organizing pneumonia. Thorax 55: 318-328, 2000. logic findings that demonstrate granulation tissue distributed 4) Cordier JF, Loire R, Brune J. Idiopathic bronchiolitis obliterans organiz- within the bronchioles into the distal air space, progressing from ing pneumonia, definition of characteristic clinical profiles in a series of 16 patients. Chest 96: 999-1004, 1989. fibrin exudates to loose collagen-containing fibroblasts ( 17, 1 8). 5) Yamamoto M, Ina Y, Kitaichi M, Harasawa M, Tamura M. Bronchiolitis The patient presented in this report started with acute onset obliterans organizing pneumonia (BOOP) in Japan. Nihon Kyobu Shikkan and progressive respiratory distress. It is plausible that her on- Gakkai Zasshi 28: 1164-1173, 1990 (in Japanese, Abstract in English). set was due to lung injury caused by the substances she in- 6) Nishimura K, Itoh H. High-resolution computed tomographic features of haled when cleaning the dusty roomone month before the bronchiolitis obliterans organizing pneumonia. Chest 102: 26s-3 1 s, 1992. 7) Davison AG, Heard BE, McAllister WA, Turner-Warwick
Load more

Recommended publications
  • Clarifying the Diagnosis of Post-Inflammatory Pulmonary Fibrosis: a Population-Based Study
    AGORA | RESEARCH LETTER Clarifying the diagnosis of post- inflammatory pulmonary fibrosis: a population-based study To the Editor: Epidemiological studies are important in defining the distribution and burden of diseases in a population. A common method of studying interstitial lung disease (ILD) epidemiology has been the analysis of insurance and billing claims databases, such as the Commercial Claims and Encounters Database and the Medicare Supplemental and Coordination of Benefits Database. These studies rely on the accuracy of International Statistical Classification of Diseases (ICD) codes to identify a patient population of interest. Several studies have described the incidence and prevalence of ILD by methodically searching ICD codes related to ILD or by using code-based algorithms [1–6]. Post-inflammatory pulmonary fibrosis (PPF) (ICD-9-CM 515) has been categorised as a general diagnostic code used by providers for IPF, an ILD characterised by progressive parenchymal fibrosis [1, 7]. Cases of PPF have been variably included in studies of IPF epidemiology. The prevalence of PPF may be comparable or higher to that of IPF. For example, COULTAS et al. [2] reported PPF to represent 16.7% of prevalent cases of ILDs while IPF comprised 22.5% in a population-based registry. RAGHU et al.[8] analysed a large healthcare claims database spanning the period 1996–2000 and found the prevalence of PFF to be nearly 11-fold higher than that of IPF identified by “broad case definition”. To our knowledge, however, cases designated as PPF have never been fully characterised. In particular, it is unknown to what extent PPF (ICD-9-CM 515) overlaps with the diagnosis of IPF.
    [Show full text]
  • Allergic Bronchopulmonary Aspergillosis
    Allergic Bronchopulmonary Aspergillosis Karen Patterson1 and Mary E. Strek1 1Department of Medicine, Section of Pulmonary and Critical Care Medicine, The University of Chicago, Chicago, Illinois Allergic bronchopulmonary aspergillosis (ABPA) is a complex clinical type of pulmonary disease that may develop in response to entity that results from an allergic immune response to Aspergillus aspergillus exposure (6) (Table 1). ABPA, one of the many fumigatus, most often occurring in a patient with asthma or cystic forms of aspergillus disease, results from a hyperreactive im- fibrosis. Sensitization to aspergillus in the allergic host leads to mune response to A. fumigatus without tissue invasion. activation of T helper 2 lymphocytes, which play a key role in ABPA occurs almost exclusively in patients with asthma or recruiting eosinophils and other inflammatory mediators. ABPA is CF who have concomitant atopy. The precise incidence of defined by a constellation of clinical, laboratory, and radiographic ABPA in patients with asthma and CF is not known but it is criteria that include active asthma, serum eosinophilia, an elevated not high. Approximately 2% of patients with asthma and 1 to total IgE level, fleeting pulmonary parenchymal opacities, bronchi- 15% of patients with CF develop ABPA (2, 4). Although the ectasis, and evidence for sensitization to Aspergillus fumigatus by incidence of ABPA has been shown to increase in some areas of skin testing. Specific diagnostic criteria exist and have evolved over the world during months when total mold counts are high, the past several decades. Staging can be helpful to distinguish active disease from remission or end-stage bronchiectasis with ABPA occurs year round, and the incidence has not been progressive destruction of lung parenchyma and loss of lung definitively shown to correlate with total ambient aspergillus function.
    [Show full text]
  • Cryptogenic Organizing Pneumonia: Evolution of Morphological Patterns Assessed by HRCT
    diagnostics Article Cryptogenic Organizing Pneumonia: Evolution of Morphological Patterns Assessed by HRCT Francesco Tiralongo 1,* , Monica Palermo 1, Giulio Distefano 1 , Ada Vancheri 2, Gianluca Sambataro 2,3 , Sebastiano Emanuele Torrisi 2, Federica Galioto 1, Agata Ferlito 1, Giulia Fazio 1, Pietro Valerio Foti 1, Letizia Antonella Mauro 1, Carlo Vancheri 2, Stefano Palmucci 1 and Antonio Basile 1 1 Radiology Unit 1, Department of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”—University Hospital “Policlinico-Vittorio Emanuele”, University of Catania, 95123 Catania, Italy; [email protected] (M.P.); [email protected] (G.D.); [email protected] (F.G.); [email protected] (A.F.); [email protected] (G.F.); [email protected] (P.V.F.); [email protected] (L.A.M.); [email protected] (S.P.); [email protected] (A.B.) 2 Regional Referral Centre for Rare Lung Diseases, A. O. U. “Policlinico-Vittorio Emanuele” Dept. of Clinical and Experimental Medicine, University of Catania, 95123 Catania, Italy; [email protected] (A.V.); [email protected] (G.S.); [email protected] (S.E.T.); [email protected] (C.V.) 3 Artroreuma S.R.L., Outpatient of Rheumatology associated with the National Health System, Corso S. Vito 53, 95030 Mascalucia (Catania), Italy * Correspondence: [email protected] Received: 14 April 2020; Accepted: 28 April 2020; Published: 29 April 2020 Abstract: To evaluate the radiological findings in patients with cryptogenic organizing pneumonia (COP) before steroid treatment and their behavior after therapy, we retrospectively evaluated a total of 22 patients with a diagnosis of COP made by bronchoalveolar lavage (BAL), biopsy or clinical/radiological features, and the patients were followed between 2014 and 2018 at the hospital; the demographic data, symptoms, radiologic findings, diagnostic methods and treatment plans of patients were collected from patients’ hospital records.
    [Show full text]
  • Cryptogenic Organizing Pneumonia
    462 Cryptogenic Organizing Pneumonia Vincent Cottin, M.D., Ph.D. 1 Jean-François Cordier, M.D. 1 1 Hospices Civils de Lyon, Louis Pradel Hospital, National Reference Address for correspondence and reprint requests Vincent Cottin, Centre for Rare Pulmonary Diseases, Competence Centre for M.D., Ph.D., Hôpital Louis Pradel, 28 avenue Doyen Lépine, F-69677 Pulmonary Hypertension, Department of Respiratory Medicine, Lyon Cedex, France (e-mail: [email protected]). University Claude Bernard Lyon I, University of Lyon, Lyon, France Semin Respir Crit Care Med 2012;33:462–475. Abstract Organizing pneumonia (OP) is a pathological pattern defined by the characteristic presence of buds of granulation tissue within the lumen of distal pulmonary airspaces consisting of fibroblasts and myofibroblasts intermixed with loose connective matrix. This pattern is the hallmark of a clinical pathological entity, namely cryptogenic organizing pneumonia (COP) when no cause or etiologic context is found. The process of intraalveolar organization results from a sequence of alveolar injury, alveolar deposition of fibrin, and colonization of fibrin with proliferating fibroblasts. A tremen- dous challenge for research is represented by the analysis of features that differentiate the reversible process of OP from that of fibroblastic foci driving irreversible fibrosis in usual interstitial pneumonia because they may determine the different outcomes of COP and idiopathic pulmonary fibrosis (IPF), respectively. Three main imaging patterns of COP have been described: (1) multiple patchy alveolar opacities (typical pattern), (2) solitary focal nodule or mass (focal pattern), and (3) diffuse infiltrative opacities, although several other uncommon patterns have been reported, especially the reversed halo sign (atoll sign).
    [Show full text]
  • Bronchiectasis
    Bronchiectasis By L. D. Eerland and N. G. M. Orie With 58 Figures A. Introduction~ Definition and Historical Survey 1. Introduction One of the most intricate fields of pulmonary pathology is that of the aetiology and pathogenesis of bronchiectasis. Much has remained unexplained, in spite of the extensive literature that has appeared since LAENNEC first described the affection in 1817. There are still many questions unanswered as regards the indications for operative treatment and the results obtained by surgery. At present there is no Ionger any difficulty in the diagnosis of bronchiectasis, espe­ cially owing to the development of bronchography, eventhough it should be remarked that technically perfect bronchograms are required to arrive at a therapeutically justi­ fied plan of campaign. l<'ig. I. Dorsoventral bronchogram of 4-year-old boy, who had a so.called left pneumonia four months previously. There is a considerable displacement of the mediastinum. The photo shows ampullary bronchi­ ectasis in the markedly shrivelled left lower lobe. Bronchoscopy reveals very little pus. Treatment was con- servative. This was a case of reversible bronchiectasis, as shown by Fig. 2 It must be admitted that the sulphonamides and modern antibiotics have been of inestimable value during the pre- and after-treatment of patients in whom resection of the diseased parts of the lung has been carried out. It is however doubtful whether parenteral or intratracheal administration of these agents alone yields lasting results. An operation is therefore often necessary, but, unfortunately, complete success is not always obtained with pulmonary resection, the only operation that comes into consideration.
    [Show full text]
  • Idiopathic Pulmonary Fibrosis
    IDIOPATHIC PULMONARY FIBROSIS Guidelines for Diagnosis UPDATE 2019 and Management An ATS Pocket Publication ATS Pocket Guide _v11_051319 copy.indd 1 5/13/19 10:51 AM GUIDELINES FOR THE DIAGNOSIS AND MANAGEMENT OF IDIOPATHIC PULMONARY FIBROSIS: UPDATE 2019 AN AMERICAN THORACIC SOCIETY POCKET PUBLICATION This pocket guide is a condensed version of the 2011, 2015 and 2018 American Thoracic Society (ATS), European Respiratory Society (ERS), Japanese Respiratory Society (JRS), and Latin American Thoracic Association (ALAT) Evidence-Based Guidelines for Diagnosis and Management of Idiopathic Pulmonary Fibrosis (IPF). This pocket guide was complied by Ganesh Raghu, MD and Bridget Collins, MD, University of Washington, Seattle from excerpts taken from the published official documents of the ATS. Readers are encouraged to consult the full versions as well as the online supplements, which are available at http://ajrccm.atsjournals.org/content/183/6/788.long. All information in this pocket guide is derived from the 2011, 2015 and 2018 IPF guidelines unless otherwise noted. Some tables and figures are reprinted with the permission from the journals referenced. Produced in Collaboration with Boehringer Ingelheim Pharmaceuticals, Inc. 2 Guidelines for the Diagnosis and Management of Idiopathic Pulmonary Fibrosis ATS Pocket Guide _v11_051319 copy.indd 2 5/13/19 10:51 AM CONTENTS List of Figures and Tables ..................................................................................................................4 List of Abbreviations and Acronyms
    [Show full text]
  • A Case of Cryptogenic Organizing Pneumonia in a Patient with Idiopathic Thrombocytopenic Purpura
    J Case Rep Images Med 2017;3:39–41. De Giorgi et al. 39 www.edoriumjournals.com/case-reports/jcrm CASE REPORT PEER REVIEWED OPEN| OPEN ACCESS ACCESS A case of cryptogenic organizing pneumonia in a patient with idiopathic thrombocytopenic purpura Alfredo De Giorgi, Marco Fiore, Federico Moro, Michele Domenico Spampinato, Fabio Fabbian ABSTRACT with corticosteroids obtaining progressive improvement of thrombocytopenia and Introduction: Cryptogenic organized pulmonary distress. Conclusion: Association pneumonia (COP) or bronchiolitis obliterans- between ITP and COP or BOOP could be ascribed organizing pneumonia (BOOP) is clinical to autoimmune derangement. Respiratory condition characterized by interstitial lung symptoms and imaging in patients with ITP disease with loss of functioning parenchyma could suggest association with COP or BOOP. due to inflammatory damage and pulmonary However, both conditions might ameliorate fibrosis. We report a case of COP related with corticosteroid treatment. to autoimmune condition in patients with idiopathic thrombocytopenic purpura (ITP) Keywords: Atoll sign, Bronchiolitis obliterans-or- and diabetes mellitus type 1. Case Report: A ganizing pneumonia, Cryptogenic organized pneu- 46-year-old deaf and mute male was admitted monia, Idiopathic thrombocytopenic purpura to our hospital for general sickness, severe dyspnea. He had a history of ITP started 20 How to cite this article years before, previous splenectomy, smoking, systemic hypertension, diabetes mellitus De Giorgi A, Fiore M, Moro F, Spampinato MD, type 1, glaucoma, previous admission for Fabbian F. A case of cryptogenic organizing pulmonary thromboembolism. High resolution pneumonia in a patient with idiopathic computed tomography (HRCT) found diffuse thrombocytopenic purpura. J Case Rep Images Med interstitial thickening with a bilateral ground- 2017;3:39–41.
    [Show full text]
  • Surgical Pathology of Non-Neoplastic Lung Disease Thomas V
    SHORT COURSE Surgical Pathology of Non-Neoplastic Lung Disease Thomas V. Colby, M.D. Department of Laboratory Medicine and Pathology, Mayo Clinic Scottsdale, Scottsdale, Arizona At inception, this course included some neoplastic Case 1: Acute Exacerbation of Idiopathic lesions, but each year, the case mix was changed in Pulmonary Fibrosis response to the comments (and criticism) of the A 51-year-old man had experienced 3 to 4 years attendees. As cases were added and deleted, in the of progressive dyspnea and interstitial infiltrates as end only examples of non-neoplastic lung disease observed on chest radiographs. One week before were discussed. Of the 19 cases used during the death, there was acute worsening of his dyspnea, course of 5 years, the following 8 were selected for with some associated chills and a sore throat. At this presentation. admission, he was hypoxemic, tachypneic, and afe- brile. The clinical impression was an acute (possibly CASES 1, 2, AND 3: IDIOPATHIC INTERSTITIAL infectious) process superimposed on idiopathic PNEUMONIAS AND RELATED LESIONS pulmonary fibrosis (IPF). An open-lung biopsy was performed. The patient died 1 week later. Cultures To the clinician, there are well over 100 causes of and special stains were negative. interstitial pneumonia, but among these, there is a select subgroup that has been called the idiopathic Histologic findings interstitial pneumonias. This group has evolved The open biopsy shows subpleural scarring and from Liebow and Carrington’s (1) classic classifica- microscopic honeycombing, with associated tion of chronic interstitial pneumonias: smooth muscle metaplasia and fibroblastic foci Usual interstitial pneumonia (UIP) (Figs. 1 and 2).
    [Show full text]
  • Sildenafil Acutely Reverses the Hypoxic Pulmonary
    0031-3998/08/6403-0251 Vol. 64, No. 3, 2008 PEDIATRIC RESEARCH Printed in U.S.A. Copyright © 2008 International Pediatric Research Foundation, Inc. Sildenafil Acutely Reverses the Hypoxic Pulmonary Vasoconstriction Response of the Newborn Pig ROGERIO TESSLER, SHENGPING WU, RENATO FIORI, CHRISTOPHER K. MACGOWAN, AND JAQUES BELIK Departments of Pediatrics [J.B.], Medical Biophysics and Medical Imaging [S.W., C.K.M.], University of Toronto, Toronto, Ontario, Canada M5G 1X8; Department of Pediatrics [R.T.], Pontifı´cia Universidade Cato´lica do Rio Grande do Sul, Rio Grande do Sul, Brazil, 90680000 ABSTRACT: Sildenafil is a pulmonary vasodilator shown to be nary hypertension (9) and worsening of arterial oxygenation has effective in neonates, but conflicting data exist regarding its effect on been reported in animal models of this disease (10–12). As such, arterial oxygenation. To address this issue, we tested the sildenafil it has been suggested that sildenafil inhibits the hypoxic pulmo- effect on the piglet’s hypoxic pulmonary vasoconstriction (HPV) nary vasoconstrictor (HPV) response leading to increased blood response. A segmental lung atelectasis was created by obstructing the flow to nonventilated lung units (10). This speculative mecha- corresponding bronchus. Total pulmonary and specific flows to the nism has never been properly evaluated. atelectatic and contra-lateral lobes were measured by magnetic res- onance (MR) before and 30-min post sildenafil (0.2 and 1 mg/kg i.v.) Little is known about the HPV response in the newborn. When or saline administration. Flow was reduced (p Ͻ 0.01) in the atelec- compared with the adult animal data, the HPV response is tatic and increased in the contra-lateral lobe indicating an effective reduced early in life in sheep and rabbits (13–15), but after HPV response.
    [Show full text]
  • Management of Allergic Bronchopulmonary Aspergillosis: a Review and Update Mahboobeh Mahdavinia and Leslie C
    Therapeutic Advances in Respiratory Disease http://tar.sagepub.com/ Management of allergic bronchopulmonary aspergillosis: a review and update Mahboobeh Mahdavinia and Leslie C. Grammer Ther Adv Respir Dis 2012 6: 173 originally published online 30 April 2012 DOI: 10.1177/1753465812443094 The online version of this article can be found at: http://tar.sagepub.com/content/6/3/173 Published by: http://www.sagepublications.com Additional services and information for Therapeutic Advances in Respiratory Disease can be found at: Email Alerts: http://tar.sagepub.com/cgi/alerts Subscriptions: http://tar.sagepub.com/subscriptions Reprints: http://www.sagepub.com/journalsReprints.nav Permissions: http://www.sagepub.com/journalsPermissions.nav Citations: http://tar.sagepub.com/content/6/3/173.refs.html >> Version of Record - May 28, 2012 OnlineFirst Version of Record - Apr 30, 2012 What is This? Downloaded from tar.sagepub.com at NORTHWESTERN UNIV LIBRARY on January 3, 2014 TAR631753465812443094LC Grammer and M MahdaviniaTherapeutic Advances in Respiratory Disease 4430942012 Therapeutic Advances in Respiratory Disease Review Ther Adv Respir Dis Management of allergic bronchopulmonary (2012) 6(3) 173 –187 DOI: 10.1177/ aspergillosis: a review and update 1753465812443094 © The Author(s), 2012. Reprints and permissions: Mahboobeh Mahdavinia and Leslie C. Grammer http://www.sagepub.co.uk/ journalsPermissions.nav Abstract: Since the first description of allergic bronchopulmonary aspergillosis (ABPA) in the 1950s there have been numerous studies that have shed light on the characteristics and immunopathogenesis of this disease. The increased knowledge and awareness have resulted in earlier diagnosis and treatment of patients with this condition. This article aims to provide a summary and updates on ABPA by reviewing the results of recent studies on this disease with a focus on articles published within the last 5 years.
    [Show full text]
  • Three Cases of Idiopathic Bronchiolitis Obliterans with Organizing Pneumonia
    Eur Reaplr J CASE REPORT 1991 ' 4, 902-904 Three cases of idiopathic bronchiolitis obliterans with organizing pneumonia J. Alegre-Martin*, T. Fernandez de Sevilla*, F. Garcia**, V. Falc6*, J.M. Martinez-Vazquez* Three cases of idiopathic bronchiolitis obliterans with organizing pneumo­ Depts of •Internal Medicine and ••Pathology, nia. J. Alegre-Martin, T. Fernandez de Sevilla, F. Garcia, V. Falc6, J.M. Hospital General Valle Hebr6n and Medical School, Martinez-Vazquez. Universidad Autonoma, Barcelona, Spain. ABSTRACT: Three patients with bronchiolitis obliterans organizing Correspondence: I . Alegre-Martin, c/Juan de Garay pneumonia are described. Chest X·ray films showed peripheral densities 19-21 3G 1• 08026 Barcelona, Spain. and pulmonary function tests a restrictive pattern. In all three cases transbronchlal biopsy was not useful for diagnosis and we point out the Keywords: Idiopathic bronchiolitis obliterans; Importance of an open lung biopsy In order to make the differential organizing pneumonia. diagnosis with other infiltrative lung disease with a different prognosis and therapy. Received: April 18, 1990; accepted after revision Eur Respir J., 1991, 4, 902-904. March 27, 1991. Bronchiolitis obliterans with organizing pneumonia with methylprednisolone with a good clinical and (BOOP) is defined by the presence of masses of organ­ radiological response. Eight months after discharge he izing granulation tissue in the bronchiolar lumen with is asymptomatic. obliteration of alveolar spaces. It is caused by a diver­ sity of agents and may be associated with a number of different diseases (1]. The idiopathic form is a rela­ Case 2 tively rare disease, although the clinical and histologi­ A 49 yr old male with one pack-day of smoking was ca.l patterns are well characterized, that has a good admitted to hospital because of fever, cough with response to therapy with steroids [2, 3].
    [Show full text]
  • Anatomical Review Anatomical Review Physiology Review
    4/13/2016 Physiology Review Pressure Relationships: Intrapulmonary Pressure ~ within the alveoli, rises & falls with phase of breathing, equalizes itself with the atmospheric pressure outside of the body. 1 Intrapleural Pressure ~ within the pleural cavity, always about 4mm Hg less than the pressure inside the alveoli, therefore it is negative relative to both the intrapulmonary & atmospheric pressures. 1 Transpulmonary Pressure ~ difference between the intrapulmonary & intrapleural pressures. This is the force that keeps the airspace of the lungs open, preventing atelectasis , or collapsed lung. 1 Anatomical Review Pneumothorax Respiratory Tract: Presence of air in the pleural space. 2 2 Trachea Bronchi Bronchioles Alveoli Alveolar sacs 1 Classified as spontaneous or traumatic. Spontaneous ~ occurs without preceding trauma or obvious Each lung is suspended in its own cause. 2 cavity inside the pleural cavity and is Primary ~ otherwise healthy person without underlying connected to the mediastinum. 1 lung disease. 2 The pleura covers the entire external Secondary ~ complication of underlying lung disease, surface of each lung, secreting fluid that most commonly COPD. 2 allows the lungs to glide with the thorax Traumatic ~ occur as result of direct or indirect trauma to the during respiration. 1 chest. 2 Anatomical Review Pneumothorax Term first described in 1803. 3 1st modern description of PTX occurring in healthy people (PSP) in 1932. 3 Significant global health problem ~ 7.4/100k cases per year reported in men in US, 37/100k in UK. Less than half that in women. 4 Risk factors ~ Family Hx, Marfan, homocystinuria, thoracic endometriosis, & smoking. 4 4 studies that included 505 patients, smoking habit associated with 7 x higher risk of PSP in light smokers than non, & 21 x in moderate smokers, & 102 x in heavy smokers (>pack/day).
    [Show full text]