Results 81 to 90 of about 419 (109)

Pulmonary interstitial glycogenosis associated with a spectrum of neonatal pulmonary disorders

Human Pathology, 2017
Primary or isolated pulmonary interstitial glycogenosis (PIG) is a rare disease presenting as tachypnea and hypoxemia during the perinatal period. A diffuse interstitial infiltrate with focal hyperinflation is visible on chest imaging. The biopsy findings include diffuse expansion of the interstitium by spindle-shaped cells with pale cytoplasm that, on
Ernest Cutz, Rose Chami, Sharon D Dell
exaly   +3 more sources

Pulmonary Interstitial Glycogenosis

World Journal for Pediatric & Congenital Heart Surgery, 2015
Transposition of the great arteries with intact ventricular septum and persistent pulmonary hypertension (TGA-IVS PPHN) is a rare association with a poor prognosis. We report the case of a term newborn with TGA-IVS PPHN successfully managed with perioperative extracorporeal membrane oxygenation (ECMO) and aggressive pulmonary vasodilation therapy that ...
Joan Sanchez-De-Toledo, Sebastiã González-Peris, FERRÁN Gran   +2 more
exaly   +3 more sources

Persistent Pulmonary Hypertension Without Underlying Cardiac Disease as a Presentation of Pulmonary Interstitial Glycogenosis

Fetal and Pediatric Pathology, 2018
Pulmonary interstitial glycogenosis (PIG) is an idiopathic lung condition that remains clinically underrecognized despite a growing body of literature.We present a case of PIG with pulmonary hypertension without underlying cardiac disease. This patient presented with respiratory distress and spontaneous pneumothorax at 6 months of age.
Shuo Li, Mark Wilson
exaly   +3 more sources

Pulmonary interstitial glycogenosis in the setting of lung growth abnormality: radiographic and pathologic correlation

Pediatric Radiology, 2010
Pulmonary interstitial glycogenosis (PIG) is a rare pediatric interstitial lung disease. We report a case of a term boy presenting with tachypnea at birth requiring supplemental oxygen. Chest radiographs followed by high-resolution CT (HRCT) demonstrated hyperinflation and diffuse interstitial markings interspersed with multiple cystic spaces.
Aruna Vade
exaly   +3 more sources

Pulmonary Interstitial Glycogenosis: A New Variant of Neonatal Interstitial Lung Disease

American Journal of Respiratory and Critical Care Medicine, 2002
Abstract We present the clinical, radiologic, and pathologic findings in lung biopsies from seven infants with atypical neonatal lung disease. All seven infants presented with tachypnea, hypoxemia, and diffuse interstitial infiltrates with overinflated lungs on chest radiographs in the first month of life. Lung biopsies from all cases
Anne-Marie, Canakis, Ernest, Cutz, David, Manson, Hugh, O'Brodovich   +3 more
openaire   +2 more sources

Pulmonary interstitial glycogenosis: Diagnosis and follow-up

7.5 Neonatology and Paediatric Intensive Care, 2016
Background: Pulmonary interstitial glycogenosis (PIG) is a form of Children9s Interstitial and Diffuse Lung Disease that is reportedly responsive to systemic corticosteroids and has good outcomes. Aims and objectives: We aimed to describe our patients with PIG to better understand presentation, evaluation, cardiac findings, computed tomography ...
Deborah Liptzin, Christopher Baker, Jeffrey Darst, Jason Weinman, Megan Dishop, Csaba Galambos, Miranda Kroehl, Robin Deterding   +7 more
openaire   +1 more source

Pulmonary interstitial glycogenosis cells express mesenchymal stem cell markers

European Respiratory Journal, 2020
Pulmonary interstitial glycogenosis (PIG) was first defined as a distinct neonatal interstitial lung disease of unknown etiology that presents in neonates and young infants with mild to severe hypoxic lung disease [1]. Characterised clinically by unexplained respiratory distress and cyanosis with an onset during early infancy, PIG was primarily defined
Csaba Galambos, Eric Wartchow, Jason P. Weinman, Steven H. Abman   +3 more
openaire   +2 more sources

Neonatal pulmonary interstitial glycogenosis in a patient with Hunter syndrome

European Journal of Pediatrics, 2011
In 2004, we reported on a neonate with pulmonary interstitial glycogenosis (PIG), with favorable outcome at the age of 19 months when the case report was published [8]. However, at the age of 3 years, hepatosplenomegaly and a mild limitation in joint mobility was detected by clinical examination.
Koenraad Smets, Sabine Van Daele
openaire   +1 more source

Primary pulmonary interstitial glycogenosis. Long term outcome

European Respiratory Journal, 2013
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 ...
Olaia Sardon, Sandra Rovira, Paula Corcuera, Javier Korta, Joan Carles Ferreres, Elena Perez, Eduardo G. Perez-Yarza, Antonio Moreno   +7 more
openaire   +1 more source

An Infant With Pulmonary Interstitial Glycogenosis: Clinical Improvement Is Associated With Improvement In The Pulmonary Diffusion Capacity

B62. PEDIATRIC CLINICAL CASES, 2012
AbstractPulmonary interstitial glycogenosis (PIG) is an idiopathic interstitial lung disease of infants. The underlying pulmonary pathophysiology of PIG has not been well characterized. Herein we report a term‐gestatation infant who presented with persistent tachypnea and hypoxia.
Zarmina, Ehsan, Gregory S, Montgomery, Christina, Tiller, Jeffrey, Kisling, Daniel V, Chang, Robert S, Tepper   +5 more
openaire   +2 more sources