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Pulmonary interstitial glycogenosis (PIG) is a very rare interstitial lung disease in infants. It is poorly understood, but its pathological features are distinct; they include uniform alveolar septal thickening, caused by a group of oval to spindle-shaped cells containing abundant glycogen, without apparent inflammation or fibrosis. PIG is usual- ly associated with a good prognosis. However, in the present report, we describe the case of a 5-month-old male infant who died due to PIG; he was severely underweight and not administered proper treatment or care. The pathology of PIG was determined following a medico-legal autopsy.

Death of a Severe Underweight Infant with Pulmonary Interstitial Glycogenosis