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Pulmonary Emphysema


Presentation

  • Here, we present a case of a 3-month-old former full term male infant with no history of RDS or mechanical ventilation who presented with focal cystic lung disease associated with spontaneous tension pneumothorax.[ncbi.nlm.nih.gov]
  • Localized PIPE usually presents as multiple cysts 0.3 to 3 cm in one or more lobes of the lung.[ncbi.nlm.nih.gov]
  • The patient presented with respiratory distress and heart murmur in early neonatal life.[ncbi.nlm.nih.gov]
  • Therefore, some patients with stage I and II disease will present with contra-indications to resection including a predicted postoperative FEV1 of less than 0.81 or a VO2max of less than 10 ml/kg/min.[ncbi.nlm.nih.gov]
  • This work presents a novel method for emphysema quantification, based on parametric modeling of intensity distributions and a hidden Markov measure field model to segment emphysematous regions.[ncbi.nlm.nih.gov]
Weight Loss
  • Eventually, these patients develop muscle wasting and weight loss and are identified as "pink puffers."[emedicine.com]
  • loss The symptoms of pulmonary emphysema may look like other lung conditions or health problems.[hopkinsmedicine.org]
Hypoxemia
  • This V/Q mismatch results in rapid circulation in a poorly ventilated lung, leading to hypoxemia and polycythemia. Eventually, hypercapnia and respiratory acidosis develop, leading to pulmonary artery vasoconstriction and cor pulmonale.[emedicine.com]
  • In more advanced cases, when the patient already has a persistent hypoxemia (low blood oxygen), the use of supplemental oxygen is indicated.[tabletsmanual.com]
  • Interventions include: smoking cessation oxygen therapy (in chronic hypoxemia) symptom and exacerbation control short and long-acting beta-2 agonists inhaled anticholinergics inhaled glucocorticoids antibiotics pulmonary rehabilitation In patients with[radiopaedia.org]
  • With the ensuing hypoxemia, polycythemia, and increased CO 2 retention, these patients have signs of right heart failure and are known as "blue bloaters."[emedicine.com]
Down Syndrome
  • Alpha-1-antitrypsin deficiency (AAT) is one of the three most common lethal genetic diseases in the caucasian population (together with cystic fibrosis and Down syndrome). Its primary manifestation is early-onset panacinar emphysema.[ncbi.nlm.nih.gov]
Splenectomy
  • Two of the three affected siblings died of septic shock after splenectomy, at the ages of 7 and 3 1/2 years, respectively. The remaining affected sibling was shown to have cutis laxa and severe pulmonary emphysema at 15 years of age.[ncbi.nlm.nih.gov]
Cough
  • We present the case of a 6-year-old white boy with chronic cough and dyspnea on exertion.[ncbi.nlm.nih.gov]
  • Slowly progressive dyspnea is the primary symptom, although some patients initially have symptoms of cough, sputum production, or wheezing. A minority of patients develops hepatic cirrhosis.[ncbi.nlm.nih.gov]
  • Typical signs are a tickle in the throat, cough or rhinitis. In adults it tends to occur once or twice a year, usually in autumn and winter.[pari.com]
  • Sputum culture This test is done on the material that is coughed up from the lungs and into the mouth. A sputum culture is often used to see if an infection is present.[hopkinsmedicine.org]
Dyspnea
  • Feasibility, safety, and efficacy were analyzed by means of pulmonary function testing, 6-min walk test, dyspnea score, BODE (body mass index, air-flow obstruction, dyspnea, and exercise capacity) index, and St George's Respiratory Questionnaire.[ncbi.nlm.nih.gov]
  • Chronic dyspnea was also scored by modified Medical Research Council (mMRC) scale. RESULTS: In sitting position 13 patients (33%) were flow limited (FL) and 27 were non-flow limited (NFL).[ncbi.nlm.nih.gov]
  • We present the case of a 6-year-old white boy with chronic cough and dyspnea on exertion.[ncbi.nlm.nih.gov]
  • His severe dyspnea on exertion improved, and he no longer requires supplementary oxygen.[ncbi.nlm.nih.gov]
  • Dyspnea and anxiety were pominent in the older individuals, although pneumothoraces were usually small. Because physical findings were often unreliable, roentgenograms were required.[ncbi.nlm.nih.gov]
Respiratory Distress
  • This patient was a normal full-term baby with no respiratory distress symptom and no experience of assisted mechanical ventilation.[ncbi.nlm.nih.gov]
  • Neonatal interstitial pulmonary emphysema (IPE) is a well-characterized lesion usually presenting in preterm newborns as a complication of respiratory distress syndrome and/or assisted ventilation.[ncbi.nlm.nih.gov]
  • The patient presented with respiratory distress and heart murmur in early neonatal life.[ncbi.nlm.nih.gov]
  • Radioisotope lung scans may provide valuable information regarding lung function in regional pulmonary emphysema associated with assisted ventilation in neonatal respiratory distress syndrome, and thus determine patient management.[ncbi.nlm.nih.gov]
  • Usually, it appears in preterm neonates with a history of assisted ventilation or respiratory distress.[ncbi.nlm.nih.gov]
Regurgitation
  • Cardiac evaluation revealed pulmonic stenosis, atrioventricular regurgitation, and a dilated aortic root that were controlled by balloon dilatation of the pulmonic stenosis and medications for congestive heart failure.[ncbi.nlm.nih.gov]
Cyanosis
  • This compares with the hypercapnoea and cyanosis of chronic bronchitis with patients referred to as "blue bloaters". In practice, features of these two syndromes coexist as chronic obstructive pulmonary disease .[radiopaedia.org]
  • Other symptoms of emphysema include the following: Cyanosis (bluish tint to the lips, fingertips, and skin; caused by low levels of oxygen [O2] in the blood) Edema (swelling; commonly in the feet and ankles) Fatigue Headaches (especially upon waking in[healthcommunities.com]
  • […] distress) in infancy, (2) an enlarged chest due to overinflation of at least one lobe of the lung, (3) compressed normal lung tissue in the section of the lung nearest to the diseased lobe, (4) bluish color of the skin due to a lack of oxygen in the blood (cyanosis[rarediseases.org]
  • […] later stages, the person may have: A chronic cough is one of the early signs of emphysema, alongside shortness of breath. frequent lung infections a lot of mucus wheezing reduced appetite and weight loss fatigue blue-tinged lips or fingernail beds, or cyanosis[medicalnewstoday.com]
Heart Murmur
  • The patient presented with respiratory distress and heart murmur in early neonatal life.[ncbi.nlm.nih.gov]

Workup

  • Developments in the 20th century included the widespread use of spirometry (see Workup ), recognition of airflow obstruction as a key factor in determining disability, and the improvement of pathologic methods to assess emphysema.[emedicine.com]
Foam Cell
  • EXPERIMENTAL STUDIES: Foam cells are of great importance in the development of these diseases.[ncbi.nlm.nih.gov]

Treatment

  • Lung volume reduction surgery (LVRS) and pulmonary rehabilitation are newer options available in the treatment of advanced emphysema. We describe the progress of our first local patient to have undergone these 2 new treatment modalities.[ncbi.nlm.nih.gov]
  • Furthermore, a number of CT measures have been tested for the personalization of treatment, according to imaging detected heterogeneity of parenchymal disease.[ncbi.nlm.nih.gov]
  • The independent lung respiratory treatment lasted 35 and 25 days, respectively: to our knowledge, these are among the longest-lasting independent respiratory treatments reported.[ncbi.nlm.nih.gov]
  • WILBUR SHUFF UNDERGOES TREATMENT FOR EMPHYSEMA. POLLUTED AIR IS AN ACUTE PROBLEM FOR HIM - NARA - 545478.jpg 2 016 3 000; 939 KB FORMER MACHINIST MR. WILBUR SHUFF UNDERGOES TREATMENT FOR EMPHYSEMA.[commons.wikimedia.org]
  • Modern techniques allow for lobebased quantification that facilitates treatment planning.[ncbi.nlm.nih.gov]

Prognosis

  • After discussing with his parents the grave prognosis for neonatal Marfan syndrome, he was discharged home with oxygen treatment and died at home at age 4.5 months.[ncbi.nlm.nih.gov]
  • Further studies regarding the biological links between the intratumoral and extratumoral microenvironment will help to explain why lung cancers originating in emphysematous lung tissues are associated with a poor prognosis.[ncbi.nlm.nih.gov]
  • Conclusion Three different phenotypes can be identified in IIP patients with PE, and IPF with PE is a distinct clinical phenotype with a poor prognosis. [Indexed for MEDLINE] Free full text[ncbi.nlm.nih.gov]
  • PURPOSES: The prognosis of patients with emphysema is poor as there is no truly effective treatment.[ncbi.nlm.nih.gov]
  • CPFE has a poor prognosis, with a 5-year survival of 55% [ 4 ].[err.ersjournals.com]

Etiology

  • The possible etiologies of emphysema in this syndrome are discussed.[ncbi.nlm.nih.gov]
  • […] progressive process manifested clinically by dyspnea, which most commonly makes its appearance in middle-aged or elderly males who have had no previous localized or diffuse chronic pulmonary disease and which has generally been regarded as of unknown etiology[nejm.org]
  • Etiology and pathogenesis  There are two groups of causes leading to the development of emphysema  The first group includes factors that violate the elasticity and strength of the structural elements of the lung: pathological microcirculation, changes[slideshare.net]
  • Environmental factors COPD does occur in individuals who have never smoked. [19] Although the role of air pollution in the etiology of COPD is unclear, the effect is small when compared with that of cigarette smoking.[emedicine.com]

Epidemiology

  • Glomerular filtration rates (eGFRs) were estimated using the method of the Chronic Kidney Disease Epidemiology Collaboration. Quantitative determinants of emphysema and airway dimension were measured from multidetector chest CT scans.[ncbi.nlm.nih.gov]
  • Author information 1 Department of Molecular Epidemiology, Medical Research Institute, Tokyo Medical and Dental University, Tokyo, Japan. myamada.epi@mri.tmd.ac.jp Abstract The fucosyltransferase 8 gene (FUT8) encodes an enzyme that transfers fucose to[ncbi.nlm.nih.gov]
  • Epidemiology Frequency United States The National Health Interview Survey reports the prevalence of emphysema at 18 cases per 1000 persons and chronic bronchitis at 34 cases per 1000 persons. [17] While the rate of emphysema has stayed largely unchanged[emedicine.medscape.com]
  • Epidemiology The National Health Interview Survey reports the prevalence of emphysema at 18 cases per 1000 persons and chronic bronchitis at 34 cases per 1000 persons. [23] While the rate of emphysema has stayed largely unchanged since 2000, the rate[emedicine.com]
Sex distribution
Age distribution

Pathophysiology

  • Functional information of the lungs is important to understand the pathophysiology of emphysema and that of chronic obstructive pulmonary disease.[ncbi.nlm.nih.gov]
  • Prothymosin α transgenic mice exhibit an emphysema phenotype, but the pathophysiological role of prothymosin α in emphysema remains unclear.[ncbi.nlm.nih.gov]
  • CONCLUSION: (123)I-MIBG SPECT allows evaluation of lung pathophysiology in PE independently of perfusion SPECT or morphologic CT, and impairment of lung (123)I-MIBG uptake may be more extensive than perfusion or morphologic abnormalities in PE.[ncbi.nlm.nih.gov]
  • Chronic obstructive pulmonary disease (COPD) is a mixture of three separate disease processes that together form the complete clinical and pathophysiological picture. These processes are chronic bronchitis, emphysema and, to a lesser extent, asthma.[emedicine.com]
  • Although tobacco smoking has been identified as a major cause, the exact pathophysiology of the CPFE syndrome is unclear [ 20, 21 ]. Possible additional risk factors have been identified, such as exposure to agrochemical compounds [ 22 ].[err.ersjournals.com]

Prevention

  • However, lung-specific inhibition of iNOS with a iNOS-specific inhibitor, N6-(1-iminoethyl)-L-lysine, dihydrochloride (L-NIL) solely restricts lung protein nitration but fails to prevent or reverse the major tobacco smoke-induced oxidative lung injury[ncbi.nlm.nih.gov]
  • The alveolar capillary network could initially prevent enlargement of the fenestrae, and the thick elastic fibers constituting the alveolar framework could secondarily prevent destruction of the alveolar wall structure. 2014 Wiley Periodicals, Inc.[ncbi.nlm.nih.gov]
  • In the presence of pulmonary emphysema, loss of retractility prevented total collapse of the underlying lung.[ncbi.nlm.nih.gov]
  • The deletion region of 2q24.2 includes the integrin beta6 gene (ITGB6), which may prevent acute lung injury and pulmonary emphysema. Many previously reported patients with deletions of 2q24.2 showed poor outcomes because of respiratory failure.[ncbi.nlm.nih.gov]
  • Treatments include inhalers, oxygen, medications and sometimes surgery to relieve symptoms and prevent complications.* *Courtesy: National Institutes of Health Find a Pulmonary Specialist[healthcare.utah.edu]

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