Korean J Leg Med.  2012 Nov;36(2):178-181. 10.7580/KoreanJLegMed.2012.36.2.178.

Death of a Severe Underweight Infant with Pulmonary Interstitial Glycogenosis

• Jang SJ
• Kim KR
• Kim HJ
• Kim M

• KMID: 2305478
• DOI: http://doi.org/10.7580/KoreanJLegMed.2012.36.2.178

Abstract

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 usually 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.

Keyword

infant; pulmonary interstitial glycogenosis; underweight; death

MeSH Terms

Autopsy
Fibrosis
Glycogen
Glycogen Storage Disease
Humans
Infant
Inflammation
Lung Diseases, Interstitial
Male
Prognosis
Thinness
Glycogen

Figure

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  Fig. 1 The both lungs were pale salmon in color and spongy like appearance in cross section.

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  Fig. 2 Diffuse interstitial thickening without alveolar epithelial hyperplasia or protenaceous material in alveolar space (H & E, ×100).

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  Fig. 3 Marked increased interstitial mesenchymal cells showing vacuolated cytoplasm and oval and bland nuclei (H & E, ×400).

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  Fig. 4 The cytoplasm of interstitial cells displaying weak PAS positive diastase sensitive material (PAS, ×400).

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