A Case Report and Review Pulmonary alveolar proteinosis is characterized at the accumulation of proteinaceous material in the alveoli leading to varying measures of impairment in gas exchange.
A Case Report and Review
Pulmonary alveolar proteinosis is characterized at the accumulation of proteinaceous material in the alveoli leading to varying measures of impairment in gas exchange. Generally, the extent of hypoxemia is mild, and it is rare to have respiratory failure requir ing mechanical ventilation. We near a 53-year-old woman with the most numerous severe degree of hypoxemia associated with alveolar proteinosis reported in the English-language adult literature. Her therapy of sequential whole-lung lavage performed while receiving venovenous extracorporeal membrane oxygenation in undivided operative session is the first reported happy use of this approach. (CHEST 2001; 120:1024-1026)
guide words: extracorporeal membrane oxygenation; hypoxemia; lung lavage; pulmonary alveolar proteinosis
Abbreviations: ECMO = extracorporeal membrane oxygenation; [FIO.sub.2] = fraction of inspired oxygen; PAS = periodic acid Shiff; WLL = whole-lung lavage
Pulmonary alveolar proteinosis give an account ofs a rare syndrome characterized by the agency of the accumulation of large amounts of proteinaceous phospholipid-laden material in the alveoli. This leads to impaired gas exchange and arterial hypoxemia of varying ranks Whole-lung lavage (WLL), first described in 1963[1] remains the chiefly effective treatment. However, this technique can be difficult and dangerous to perform in the chiefly severely hypoxemic patients.
We report the case of a woman with, to our knowledge, the in the greatest degree severe degree of refractory hypoxemia associated with alveolar proteinosis reported in the literature. WLL was performed sequentially while she received venovenous extracorporeal membrane oxygenation (ECMO) in individual operative session. In previously reported cases utilizing venovenous ECMO no other than one lung was lavaged during an operative session, and the patients remained onward ECMO for [is greater than] 24 h[2-4]
CASE REPORT
A 53-year-old woman at handed to an outside hospital with progressive dyspnea, general malaise, and a decreased appetite through the whole extent of a duration of approximately 3 weeks. Chest radiography revealed bilateral alveolar infiltrates. ECG findings were remarkable for no other than sinus tachycardia. Abnormal laboratory findings at presentation included the following arterial progeny gas measuremen(s: pH, 7.,5; [PCOsub2] 30 mm Hg; Pa[O.sub.2], 30 mm Hg in succession room air; hemoglobin, 18.7 g/dL; and hematocrit, 55 % An initial diagnosis of congestive heart failure and/or pneumonia was made; the patient was treated accordingly with IV diuretics and broadspectrum antibiotics, and was admitted to the ICU. Although she had a virtuous response to diuretics, her dyspnea and hypoxemia tidied to improve and she was subsequently intubated and administered mechanical ventilation, with fraction of inspired oxygen [(FIO.sub.2)] of 10
Her medical history included previous atrial fibrillation treated with procainamide, and gastroesophageal ebb and osteoarthritis. Medications at presentation were lansoprazole, conjugated estrogen and alprazolam. She had a 60-pack-year smoking history and was a recovering alcoholic.
The patient underwent bronchoscopy with BAL and transbrontrial biopsies without complication. Milky fluid was obtained from lavage of the right middle lobe, and pathologic examination showed pink staining periodic acid-Schiff (PAS)-positive material. Biopsy specimens showed preserv alveolar architecture, again with a PAS-positive staining extracellular substance consistent with pulmonary alveolar proteinosis (Fig 1) The slides were sent to a consulting pathologist who concurr with the initial reading. There was no vegetation of bacteria or fungi from the lavage specimen and biopsy, the one and the other initially and at 6 weeks. The patient was extubated after the conduct but remained on 100% oxygen by dint of mask. She was sent to our institution for WLL
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The patient arrived at our institution 7 days after initial presentation. Arterial relations gas analysis measurements at that time were as follows: pH 75; [PCOsub2] 39 mm Hg; and Pa[O.sub.2], 68 mm Hg forward a 100% nonrebreather face mask. Physical examination revealed bilateral, fine inspiratory rales in every part all lung fields. There was no digital clubbing. A high-resolution CT scan of the chest was obtained and showed the classic findings of widespread airspace consolidation with thickened interlobular septa producing a "crazy paving" pattern (Fig 2)
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proper to the patient's severe hypoxemia, WLL was performed 36 h after transfer using venovenous ECMO The patient was reintubated upon the evening prior to the transaction because of worsened hypoxemia: while receiving 100% oxygen via nonrebreather face mask, her Pa[O.sub.2] was 48 mm Hg The following morning, immediately prior to the manner of proceeding results of arterial blood gas analysis while receiving mechanical ventilation at Floe of 10 and positive end-expiratory influence of 8 mm Hg were as follows: pH 736; [PCOsub2] 51 mm Hg; and Pa[O.sub.2], 54 mm Hg
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