Glicogenosis intersticial pulmonar. presentación de un caso y revisión de la literatura

Abstract

Pulmonary interstitial glycogenosis (PIG) is a rare form of Childhood Interstitial Lung Disease (chILD), typical of neonates and young infants. Clinical manifestations range from mild tachypnea and hypoxia to unexplained respiratory failure and pulmonary hypertension (PHT). Chest computed tomo­graphy (CT) images show ground glass images, interstitial infiltrates and cysts. Histopathologically, interstitial thickening is found and fused and ovoid mesenchimal cells loaded with glycogen, called lipofibroblasts, which are essential for diagnosis. Some patients only require oxygen therapy, while others require invasive mechanical ventilation or noninvasive mechanical ventilation, treatment of PHT and comorbidities. The use of glucocorticosteroids should be evaluated, they are indicated according to clinical severity. Mortality is low, being higher when associated with lung growth or development anomalies or respiratory syncytial virus pneumonia. These patients should be followed up, because persistent alterations in lung function and chest CT have been reported. We present a young infant ca­rrying PIG, suspected by clinical signs and imaging, diagnosed by biopsy and immunohistochemistry, with successful response to treatment with corticosteroids.