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Table 2 Clinical characteristics and disease outcome of PAP patients stratified according to MUC1 rs4072037 gentype

From: MUC1 gene polymorphisms are associated with serum KL-6 levels and pulmonary dysfunction in pulmonary alveolar proteinosis

Variables MUC1 rs4072037 genotype p value
A/A A/G
n = 11 n = 13
Age, years 46 ± 3 46 ± 4 n.s.
Gender, male/female, n 6/5 8/5 n.s.d
Smoking history, Current/Non, n 10/1 8/5 n.s.d
BMI (kg/m2) 25 ± 2 27 ± 1 n.s.
Outcome    
 - Disease progressiona, n (%) 9 (82) 3 (23) 0.006d
 - Death, n (%)b 1 (9) 2 (15) 0.90d
 - Remission, n (%) 1 (9) 8 (61) 0.07
Treatment, (yes/no)    
 - Repeated WLL (>2) during follow-up, n 3 0 0.055d
 - cumulative number of WLL 7.3 ± 3.1 4.3 ± 1.2 0.359
FVC (% pred) 79 ± 6 82 ± 4 n.s.
FEV1 (% pred) 73 ± 4 74 ± 4 n.s.
PaO2 (mmHg) 67 ± 6 72 ± 3 n.s.
TLC (% pred) 79 ± 6 83 ± 4 n.s.
SaO2 (%) 93 ± 1 94 ± 1 n.s.
A-aDO2 (mmHg) 45 ± 4 35 ± 3 0.027
DLco (% pred) 39 ± 6 60 ± 3 0.012
GM-CSF autoantibody (μg/mL)c 51 ± 11 53 ± 8 n.s.
KL-6 (U/mL)c 8084 ± 1673 2399 ± 462 0.007
LDH (IU/L)c 369 ± 38 263 ± 22 0.030
  1. Unless otherwise indicated, values are expressed as mean ± SE
  2. n.s. not significant
  3. aDisease progression was defined as necessity of whole lung lavage on the basis of deterioration of self-reported symptoms (worsening of dyspnea, cough, chest pain and weight loss) and/or lung function (decrease in FVC or DLco >10 % pred or increase in the A-aDO2 > 10 mmHg), and/or chest imaging (increase of the previous findings or appearance of new infiltrates characteristic of PAP) since the last follow-up visit
  4. bOne death followed whole lung lavage, one was related to alcoholic liver cirrhosis and one to lung cancer
  5. cThe cut-off of normality for each biomarker is reported in the methods
  6. dFischer’s exact test