Table 1.

Characteristics of Patients with Pulmonary Interstitial Glycogenosis

	Case 1	Case 2	Case 3	Case 4	Case 5
Sex	Male	Male	Female	Female	Male
Gestational age, wk	38	37	41	35	36
Pre-/neonatal complications	Pneumothorax	Severe chylothorax	Total anomalous pulmonary venous return/vein stenosis	VACTERL/double outlet right ventricle	Preeclampsia, pulmonary hypertension
O2 or mechanical ventilation at birth	Yes	Yes	Yes	Yes	Yes
Age at lung biopsy	5 d	4 wk	7 wk	4 wk	3 d
Status at lung biopsy	Mechanical ventilation	Mechanical ventilation	Mechanical ventilation (ECMO postoperative)	Mechanical ventilation	Mechanical ventilation (ECMO postoperative)
Additional indication for surgery	n/a; only unknown lung disease	Thoracic duct ligation	Cardiac surgery	Cardiac surgery	n/a; only unknown lung disease
Other histologic findings	Deficient growth	Deficient growth	Pulmonary hypertension,* lymphangiectasia	Deficient growth, pulmonary hypertension	Deficient growth, pulmonary hypertension
Outcome	9 mo; tachypnea, cough	5.5 yr; asymptomatic	Died 9 wk; pulmonary hypertension	4 yr; intermittent asthma	4 mo; asymptomatic

Definition of abbreviations: ECMO = extracorporeal membrane oxygenation; n/a = not applicable; VACTERL = vetebral defects, anal atresia, cardiac malformations, tracheoesophageal fistula and/or esophageal atresia, renal dysplasia, limb anomalies.

^*Pulmonary hypertensive histologic findings were defined by presence of medial hypertrophy of pulmonary arteries ± intimal hyperplasia.