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Table 1 Clinical characteristics of reported cases of hereditary pulmonary alveolar proteinosis

From: Elderly-onset hereditary pulmonary alveolar proteinosis and its cytokine profile

 

Present case

Tanaka et al.

Junne et al.

Characteristics

Adult onset

Adult onset

Juvenile (n = 20)

Sex

Female

Female

85% (Female)

Age at symptom onset (yr)

77

35

0.2–19

Prognosis

Good

Poor

Good

GM-CSF (pg/mL)

86.61

124.8

Elevated

GM-CSF antibody (μg/mL)

ND

ND

ND

Mutated gene

CSF2RA

CSF2RB

CSF2RA

WLL therapy

responded

Worsened

Improved 64%

   

Unchanged 14%

   

Worsened 0%

   

Deceased 7%

  1. GM-CSF granulocyte/macrophage colony-stimulating, ND not detected, CSF2RA, CSF2 receptor alpha, CSF2RB, CSF2 receptor beta, WLL whole lung lavage