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pulmonary interstitial glycogenosis

Wednesday 16 September 2009

Definition: Pulmonary interstitial glycogenosis (PIG) is an enigmatic lung disorder of unknown etiology that presents with neonatal respiratory distress. The "pulmonary interstitial glycogenosis" of the neonate is a new entity to be differentiated from other forms of interstitial lung disease.

Despite its dramatic clinical presentation the diagnosis of PIG has a favorable prognosis with rare mortality in the absence of co-morbid conditions.

Because abundant glycogen is not normally found in pulmonary interstitial cells, we postulate an abnormality in lung cytodifferentiation involving interstitial mesenchymal cells.

Images

- Infant with respiratory distress - pulmonary interstitial glycogenosis, reactive to underlying injury.

Clinical synopsis

- first month of life
- neonatal onset
- respiratory failure
- pulmonary hypertension
- tachypnea
- hypoxemia
- diffuse interstitial infiltrates
- overinflated lungs

Pathology synopsis

- expansion of the interstitium by spindle-shaped cells containing periodic acid-Schiff positive diastase labile material consistent with glycogen.

Immunohistochemical staining

- spindle-shaped cells

  • vimentin positive
  • negative for leucocyte common antigen, lysozyme, and other macrophage markers.

Electron microscopy

- primitive interstitial mesenchymal cells with few cytoplasmic organelles and abundant monoparticulate glycogen.
- Minimal or no glycogen seen in the alveolar lining cells.
- diffuse expansion of the alveolar interstitium by glycogenated mesenchymal cells.

Prognosis

- significant resolution of the disorder (19320535)

References

- Pulmonary interstitial glycogenosis. Lanfranchi M, Allbery SM, Wheelock L, Perry D. Pediatr Radiol. 2010 Mar;40(3):361-5. PMID: 19902199

- HISTOLOGIC RESOLUTION OF PULMONARY INTERSTITIAL GLYCOGENOSIS. Deutsch GH, Young LR. Pediatr Dev Pathol. 2009 Mar 25:1. PMID: 19320535

- Diffuse lung disease in infancy: a proposed classification applied to 259 diagnostic biopsies. Langston C, Dishop MK. Pediatr Dev Pathol. 2009 Nov-Dec;12(6):421-37. PMID: 19323600

- Pulmonary interstitial glycogenosis: a new variant of neonatal interstitial lung disease. Canakis AM, Cutz E, Manson D, O’Brodovich H. Am J Respir Crit Care Med. 2002 Jun 1;165(11):1557-65. PMID: 12045133 (Free)