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Table 4 AAT characteristics of PI*Mmalton (moderate and severe) and PI*ZZ patients

From: Characterization of the Mmalton carrier’s cohort within the EARCO (European Alpha- 1 Antitrypsin Research Collaboration) registry

 

Moderate

PI*Mmalton

N = 26

Severe

PI*Mmalton

N = 33

PI*ZZ

N = 827

P-value1

Age of onset of symptoms, mean ± SD

43.9

 ± 19.7

47.6

 ± 14.8

43.3

 ± 15.6

0.406

Age of diagnosis (AATD), mean ± SD

46.3

 ± 18.7

48.6

 ± 15.7

45.9

 ± 16.9

0.662

Alpha- 1 antitrypsin levels (mg/dl), Median (IQR)

54

(48–2277)

25

(20–29)

24

(18–97)

0.191

Patients under Augmentation Therapy, n (%)

4*

(15.4%)

14β

(45.2%)

288

(35.2%)

0.054

Age at the beginning of Augmentation Therapy, mean \(\pm\) SD

65.3

 ± 11.5

53.7

 ± 13.8

55.3

 ± 11.1

0.185

Number of years after diagnosis, median (IQR)

3

(1–8)

1

(0–2)

2

(1–8)

0.152

  1. Abbreviations: AATD Alpha- 1 antitrypsin deficiency, IQR Interquartile range, SD Standard deviation
  2. *4 Pi*Mmalton/S patients
  3. β10 Pi*Mmalton/Z patients; 3 Pi*Mmalton/Mmalton patients and 1 Pi*Mmalton/MHerleen patient
  4. 1P-value for the global comparison between moderate PI*Mmalton, severe PI*Mmalton and PI*ZZ patients