PT  - JOURNAL ARTICLE
AU  - Olaia Sardon
AU  - Sandra Rovira
AU  - Paula Corcuera
AU  - Javier Korta
AU  - Joan Carles Ferreres
AU  - Elena Perez
AU  - Eduardo G. Perez-Yarza
AU  - Antonio Moreno
TI  - Primary pulmonary interstitial glycogenosis. Long term outcome
DP  - 2013 Sep 01
TA  - European Respiratory Journal
PG  - P2343
VI  - 42
IP  - Suppl 57
4099  - http://erj.ersjournals.com/content/42/Suppl_57/P2343.short
4100  - http://erj.ersjournals.com/content/42/Suppl_57/P2343.full
SO  - Eur Respir J2013 Sep 01; 42
AB  - Background:Pulmonary interstitial glycogenosis (PIG) or infantile cellular interstitial pneumonia (ICIP) is a rare and poorly understood entity of the infant lung.Objectives:Describe clinical presentation and long term clinical outcome of infants diagnosed with primary PGI or ICIP.Methods:Multicentre prospective study of patients diagnosed by lung biopsy between October 2003 and December 2012.Results: Eight patients were included; 6 term born infants and 2 preterm infants (GA 36-32+2 weeks).Three infants were male. Four had respiratory distress and two required mechanical ventilation.Two infants had prolonged hypoxemia and the others were studied because of persistent tachypnea (median respiratory rate 57.5; range 45-70).Hospital admission because of acute bronchiolitis or wheezing bronchitis was required in 5/8 cases.CT-scan showed ground glass attenuation (n=8), parenchymal bands (n= 7) and septal thickening (n=8). Median age at diagnosis was 5 months (range 2-9).Patients were treated with intravenous methylprednisolone (7/8), hydroxychloroquine (4/8), and home oxygen (3/8; range 11-36 months). Median follow-up was 6.5 years (1-9 years). All patients enhanced clinically with resolution of tachypnea and hypoxemia.All infants had wheezing bronchitis until the median age of 3 years (range 1.5-8). Spirometry results (6/8) showed a median FVC of -1.76 z-score (range 0.07 to -2.85) and a median FEV1 of -2.27 z-score (range -0.54 to -4.37) suggesting a restrictive pattern (5/6) or a mixed pattern (1/6).Plethysmographic study confirmed a restrictive pattern in 4/5 patients.Conclusions:Although patients with ICIP/PIG showed a good long term clinical outcome they have significant functional, mainly restrictive, defects.