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Table 3 Lung transplant cohort and main disease categories leading to transplantation

From: The frequency of rare and monogenic diseases in pediatric organ transplant recipients in Italy

Disease macro-category

Pediatrics (n. 94)

Adults (n. 1,245)

Total (n. 1,339)

n. (%)

n. (%)

n. (%)

Cystic fibrosis

75 (79.8)

354 (28.4)

429 (32.0)

Primary pulmonary hypertension

7 (7.4)

51 (4.1)

58 (4.3)

Other pneumopathies

6 (6.4)

148 (11.9)

154 (11.5)

Congenital disorders

4 (4.3)

1 (0.1)

5 (0.4)

Emphysema/Chronic obstructive pulmonary disease

1 (1.1)

229 (18.4)

230 (17.2)

Idiopathic pulmonary fibrosis

1 (1.1)

447 (35.9)

448 (33.5)

Congenital heart disease

0 (0.0)

4 (0.3)

4 (0.3)

Alpha-1 antitripsin deficiency

0 (0.0)

11 (0.9)

11 (0.8)