Table 2 Pathology of 11 biopsy specimens from children with ABCA3 mutations

Patients	Age at biopsy	Major histological pattern	Pneumocyte hyperplasia	Reactive interstitium	Proteinosis	Cholesterol clefts	Alveolar macrophages	Lobular remodelling	Lymphocytic inflammation	Lymphoid aggregates	Fibrosis	Iron	Lamellar dense bodies
1A	3 weeks	PAP	++	++	++	−	+	+	−	−	−	−	++
2	1 month	DIP	++	+++	+	−	+++	+	+	−	−	−	+++
3	3 months	PAP	++	++	+++	−	+	+	−	−	−	+	++
4	2 years	DIP	++	+	++	−	+++	++	+	+	+/−	++	+++
5	2 years	NSIP/ ELP	+	+	+	++	+	++	++	−	+/−	++	ND
1B	4 years	NSIP/ ELP	+	−	+	++	++	+++	+	−	−	+++	ND
6A	4 years	NSIP/ ELP	+	−	+	+++	++	+++	+++	++	+	++	ND
7	6 years	NSIP/ ELP	+	−	−	++	++	++	++	++	+	++	+
8	6 years	NSIP/ ELP	+	−	+	++	++	++	++	++	+	+++	ND
9	8 years	NSIP	+	−	+	−	++	+++	++	++	+	+	ND
6B	9 years	NSIP/ ELP	+	−	+	+++	++	+++	+++	++	+	+++	ND

PAP, pulmonary alveolar proteinosis; DIP, desquamative interstitial pneumonia; NSIP, non-specific interstitial pneumonia; ELP, endogenous lipoid pneumonia; ND, no data (not done or inadequate specimen).
+, rare/few foci/mild; ++, occasional/scattered foci/moderate; +++, frequent/many foci/severe; +/−, early fibroplasia without organised fibrosis.