Pulmonary interstitial glycogenosis associated with a spectrum of neonatal pulmonary disorders

Cutz, Ernest; Chami, Rose; Dell, Sharon D.; Langer, Jacob C.; Manson, David

doi:10.1016/j.humpath.2017.06.026

Cited by 34 publications

(77 citation statements)

References 29 publications

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“…2,11 One patient in our series died. Unfortunately, this patient was lost to follow-up after initial discharge, so the cause of death is unknown.…”

Section: Discussionmentioning

confidence: 70%

“…have further defined P.I.G. 4,11 However, no in depth review has been published to define a phenotype that clinicians can use to consider this diagnosis. This pathologic diagnosis is poorly understood, and has only been seen in biopsies from infants.…”

Section: Discussionmentioning

confidence: 99%

“…may have been present and missed as has been presented in other series. 11 We do not have lung tissue on all patients with structural heart disease or at every gestational age to better understand the prevalence of P.I.G in populations such as congenital heart disease or prematurity. Understanding this phenotype will help clinicians evaluate infants with unexplained respiratory distress or failure, especially in late pre-term or term infants with comorbid cardiac disease.…”

Section: Discussionmentioning

confidence: 99%

“…In our experience, the CT findings of GGO and cystic changes are typical, but not pathognomonic for P.I.G., and not all patients had these findings; previous studies have not found unifying radiographic findings. 11 Biopsy, therefore, is still considered the gold standard for diagnosis.…”

Section: Discussionmentioning

confidence: 99%

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Pulmonary interstitial glycogenosis: Diagnostic evaluation and clinical course

Liptzin

Baker

Darst

et al. 2018

Pediatric Pulmonology

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The P.I.G. phenotype has not been comprehensively described, and poor recognition and misconceptions about P.I.G. persist. P.I.G. is a disease that presents in early infancy, requires significant medical intervention, and frequently is seen in association with alveolar simplification and/or cardiovascular disease. CT findings include ground glass opacities and cysts. Patients should be monitored for pulmonary hypertension. Without life-threatening comorbidities, many patients do well over time, although respiratory symptoms may persist into adolescence.

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“…2,11 One patient in our series died. Unfortunately, this patient was lost to follow-up after initial discharge, so the cause of death is unknown.…”

Section: Discussionmentioning

confidence: 70%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Pulmonary interstitial glycogenosis: Diagnostic evaluation and clinical course

Liptzin

Baker

Darst

et al. 2018

Pediatric Pulmonology

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“…In these cases, interstitial glycogenosis is usually patchy and does not constitute the main pathological abnormality. Although PIG contributes to the clinical presentation, the associated alterations influence the clinical evolution and prognosis of the disease, which might become fatal in some cases Patients with isolated PIG not associated with other disorders, in which PIG is the main abnormality.…”

Section: Introductionmentioning

confidence: 99%

Isolated pulmonary interstitial glycogenosis associated with alveolar growth abnormalities: A long‐term follow‐up study

Sardón

Torrent‐Vernetta

Rovira‐Amigo

et al. 2019

Pediatric Pulmonology

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Introduction Pulmonary interstitial glycogenosis (PIG) is a rare infant interstitial lung disease characterized by an increase in the number of interstitial mesenchymal cells, presenting as enhanced cytoplasmic glycogen, and is considered to represent the expression of an underlying lung development disorder. Methods This study describes the clinical, radiological, and functional characteristics and long‐term outcomes (median 12 years) of nine infants diagnosed with isolated PIG associated with alveolar simplification in the absence of other diseases. Results All patients presented with tachypnea. Additionally, seven patients had breathing difficulties and hypoxemia. Abnormalities in chest‐computerized tomography (CT) with a pattern of ground‐glass opacity, septal thickening, and air trapping were observed in all individuals, with images suggesting abnormal alveolar growth (parenchymal bands and architectural distortion). All lung biopsies showed alveolar simplification associated with an increased number of interstitial cells, which appeared as accumulated cytoplasmic glycogen. In the follow‐up, all patients were asymptomatic. The respiratory function test was normal in only two patients. Five children showed an obstructive pattern, and two children showed a restrictive pattern. Chest‐CT, performed after an average of 6.5 years since the initial investigation, revealed a partial improvement of the ground‐glass opacity pattern; however, relevant alterations persisted. Conclusion Although the patients with PIG in the absence of other associated pathologies had a good clinical outcome, significant radiographic alterations and sequelae in lung function were still observed after a median follow‐up of 12 years, suggesting that PIG is a marker of some other persistent abnormalities in lung growth, which have effects beyond the symptomatic period.

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