Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0034063 (pulmonary edema)
 10,665 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Extracorporeal membrane oxygenation (ECMO) has proven to be life-saving in cases of reversible lung injury. One potential application of ECMO in the field of lung transplantation is the support of the patient with acute pulmonary failure immediately after transplantation until the transplanted lung has resumed satisfactory gas transfer function. The authors have had experience with ECMO in three patients who have had acute pulmonary failure and inadequate oxygenation after bilateral single lung (BSLT) or heart-lung transplantation (HLT). Patient 1 is a 47-year-old woman with alpha-1 antitrypsin deficiency who underwent a HLT and experienced fulminant pulmonary edema secondary to an intraoperative coagulopathy that required massive transfusion. Patient 2 was a 45-year-old man with a patent ductus arteriosus (PDA) that resulted in Eisenmenger's complex. Patient 2 underwent an HLT and experienced acute pulmonary failure. Patient 3 is a 58-year-old woman with an atrial septal defect (ASD) and pulmonary hypertension who underwent repair of the ASD and BSLT. Patient 3 experienced complete atelectatic collapse of the right lung and pulmonary edema of the left lung. These three patients had PO2 measurements of 23, 39, and 23 mmHg, respectively, despite receiving 100% FiO2 and maximal ventilatory support. All three patients were subsequently placed on ECMO and had improvement of their oxygenation. Patients 1 and 3 were successfully weaned from ECMO and extubated on post-operative day (POD) 21 and 16, respectively. Patient 2 had significant improvement in oxygenation but died on POD 4 of persistent mediastinal hemorrhage.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Extracorporeal membrane oxygenation after lung or heart-lung transplantation. 826 77

Alpha-1 antitrypsin deficiency is one of the genetic diseases with a clear impact on the structure and function of the lung, rarely diagnosed and treated. We present the case of a 51-year-old female patient, heavy smoker, known with chronic obstructive pulmonary disease (COPD) for 12 years, untreated, who was hospitalized for the first time in our clinic having symptoms of a severe COPD exacerbation. She has significant cardiac disease (rheumatic mitral disease, with previous episodes of pulmonary edema and cardiac arrest) and hepatitis B. The patient is hypoxic, with severe mixed ventilatory dysfunction. During the hospitalisation she received treatment of the exacerbation and after that she received recommendation of chronic inhaled bronchodilator and corticosteroid treatment. The test for alpha-1 antitrypsin deficiency has detected a plasma of 63 mg/dl, SZ phenotype. The patient returns for a second evaluation. Functional tests are significantly improved (despite inconsistent treatment) with the impressive improvement of FEV7 values and identification by plethysmography of a restrictive syndrome. Echocardiography identifies mitral valve changes likely rheumatic, severe pulmonary hypertension. Computer tomography was performed, highlighting discrete interstitial changes and denying the existence of emphysema. Marked increase in FEV1 values supported adding bronchial asthma to the list of diagnosis and recommendation to continue inhaled corticosteroid combination bronchodilator as treatment. The particularity of the case is the rare phenotype, association of asthma and COPD as the clinical manifestation and the presence of comorbidities, which complicates the diagnosis and prognosis.
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PMID:Alpha-1 antitrypsin deficiency, SZ phenotype: a rare type of a rare disease. Case report. 2566 66