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Table 1 Treatment of the refractory adult PAP case (listed according to timeline)

From: Whole lung lavage combined with Granulocyte-macrophage colony stimulating factor inhalation for an adult case of refractory pulmonary alveolar proteinosis

Time

Treatment choice

Details

Patient response

SEP 16TH, 2010

Whole lung lavage

Left lung: 8 L saline flushed in, 7.5 L recycled. Right lung: 9 L saline flushed in, 8.88 L recycled.

Good response, improvement in symptoms and PA-a maintained throughout 1 year

OCT 13TH, 2011

Whole lung lavage

Left lung: 5.9 L saline flushed in, 5.68 L recycled. Right lung: 7 L saline flushed in, 6.8 L recycled.

Improved symptoms and PA-a;

NOV 16TH, 2011

Whole lung lavage

Left lung: 8.0 L saline flushed in, 7.7 L recycled. Right lung: 7 L saline flushed in, 6.75 L recycled.

Improved symptoms, PA-a, and pulmonary function; good control of symptoms through 3 months.

FEB 2ND, 2012

Whole lung lavage

Left lung: 8.0 L saline flushed in, 7.82 L recycled. Right lung: 8 L saline flushed in, 7.71 L recycled.

Symptoms and hypoxia not improved, still required high volume oxygen support;

FEB 8th-20th, 2012

5 sessions of plasmapheresis therapy

Exchange volume: 2.5 L, every 2–3 days during two weeks.

JUNE 1ST, 2012

Whole lung lavage

Left lung: 9.0 L saline flushed in, 9.33 L recycled. Right lung: 7.0 L saline flushed in, 6.98 L recycled.

Good symptomatic control, with no hypoxia and no further flare of PAP

JUNE 2nd, 2012

GM-CSF inhalation

At a dosage of 150 μg, twice daily from day 1 to day 8, with inhalation stopped from day 9 to day 14, every 2 weeks for 3 months;

Sep, 2012

GM-CSF inhalation

At a dosage of 150 μg, once daily from day 1 to day 8, every 2 weeks for 6 month;

  1. WLL, whole lung lavage; PAP, pulmonary alveolar proteinosis; GM-CSF, Granulocyte-macrophage colony-stimulating factor.
  2. During the WLL, we used aliquots of (600-800) ml.