TABLE 5

Semi-quantitative analysis of histological features of seven cases contrasted to age at biopsy and outcome

Case	Age at biopsy	Outcome	Alveolar simplification	Wide interstitium	PIG-like cells	Interstitial fibrosis^#	Back-to-back bronchioli^¶	Thickened PA muscular wall⁺	PA fibrointimal proliferation	Intrapulmonary bronchopulmonary anastomosis and bronchial vessel recruitment
1^§	4 months	Death at 5 months	Diffuse	Diffuse	Diffuse	None	Focal	Moderate	Absent	Absent
7^§	3 months	Death at 8 months	Diffuse	Diffuse	Patchy	Patchy	Absent	Moderate	Moderate	Absent
12^§	2 months	Lung transplant at 11 years	Diffuse	Patchy	Absent	Patchy	Multifocal	Moderate	Absent	Present
13^§	1.5 months	Severe ILD at 4 years	Diffuse	Patchy	Diffuse	Patchy	Absent	Moderate	Absent	Absent
10a^ƒ##	2 years	Lung transplant at 18 years	Diffuse	Absent	Absent	Absent	Absent	Moderate	Absent	Present
10b^ƒ##	2 years	Lung transplant at 18 years	Diffuse	Diffuse	Absent	Diffuse	Multifocal	Moderate	Absent	Present
18^ƒ	7 years	Moderate PH at 9 years	Diffuse	Diffuse	Absent	Patchy	Absent	Moderate	Absent	Present
19^ƒ	7 years	Moderate PH at 10 years	Diffuse	Absent	Absent	Absent	Absent	Moderate	Moderate	Present

PIG: pulmonary interstitial glycogenosis; PA: pulmonary artery; ILD: interstitial lung disease; PH: pulmonary hypertension. ^#: patchy, <50% of parenchyma; diffuse, >50% of parenchyma; ^¶: focal, three foci; multifocal, >3 foci; ⁺: moderate, PA muscle wall thickness estimated 40–70% of diameter; ^§: biopsies obtained during neonatal period and showed grimmer prognosis; ^ƒ: biopsies obtained during childhood and showed better outcomes; ^##: biopsy at age 2 and transplant at age 18, allowing longitudinal assessment of evolving histologic features (see details in text).