TABLE 2.

AGE AT BIOPSY AND OUTCOME BY CATEGORY

Category	Age at Biopsy, mo Mean ± SEM (range)	% Mortality	Age at Death, mo Mean ± SEM (range)	Age at Follow-up of Survivors, mo Mean ± SEM (range)
Diffuse developmental disorders	0.7 ± 0.2 (0.3–1.2)	100%	0.7 ± 0.1 (0.3–1.2)	n/a
Lung growth abnormalities	5.35 ± 0.8 (0.3–22)	34%	9.5 ± 2.7 (0.3–31)	25.3 ± 3.1 (3–64)
Pulmonary interstitial glycogenosis	1.3 ± 0.4 (0.3–3.0)	0%	n/a	20.3 ± 6.1 (2–43)
Neuroendocrine cell hyperplasia of infancy	13.9 ± 1.7 (2.7-24)*	0%	n/a	37.9 ± 5.3 (15–71)
Surfactant dysfunction (all)	5.8 ± 1.6 (0.2–22)	41.2%	1.9 ± 0.6 (0.4–4.5)	36.9 ± 7.9 (10–73)
SP-C mutations	8.9 ± 3.0 (2.0 –22)	0%	n/a	36.8 ± 8.4 (10–61)
ABCA-3 mutations	1.3 ± 0.5 (0.2 –3.0)†	100%	1.9 ± 0.7 (0.4–4.5)	n/a
Disorders of the normal host	13.1 ± 1.7 (1.2–24)	5%	15.0	39.7 ± 4.8 (11–72)
Disorders resulting from systemic disease processes	10.5 ± 3.6 (1–22)	20%	1.4	23.5 ± 3.8 (16–33)
Disorders of the immunocompromised host	11.6 ± 1.3 (1.5–24)	30.8%	12.4 ± 2.9 (2.5–28)	36.1 ± 3.3 (10–61)
Disorders masquerading as ILD	7.3 ± 2.3 (0.2–24)	28.6%	11.0 ± 5.0 (6–16)	17.8 ± 5.5 (7–33)

Definition of abbreviations: ILD = interstitial lung disease; NEHI = neuroendocrine cell hyperplasia of infancy; SP-C = surfactant protein C.

^*P < 0.05 for NEHI versus other disorders of infancy.

^†P < 0.01 for ABCA-3 versus SP-C.