Pulmonary interstitial glycogenosis

Lanfranchi, Michael; Allbery, Sandra M.; Wheelock, Lisa; Perry, Deborah

doi:10.1007/s00247-009-1455-7

Cited by 40 publications

(19 citation statements)

References 5 publications

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“…Sometimes ground-glass opacities are absent or have a different distribution (3,6,7,21). With regard to biopsy-proven PIG, the few published CT studies have shown mainly ground-glass opacities in a subsegmental, segmental, or diffuse distribution (11,15), very similar to what was found in NEHI (11): interlobar septal thickening or scarring, hyperinflation, air trapping, multifocal atelectasis (7), and multiple air-filled cystic changes of variable size (12).…”

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confidence: 86%

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Persistent Tachypnea of Infancy. Usual and Aberrant

Rauch¹,

Wetzke

Reu

et al. 2016

Am J Respir Crit Care Med

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RATIONALE: Persistent tachypnea of infancy (PTI) is a specific clinical entity of undefined etiology comprising the two diseases neuroendocrine cell hyperplasia of infancy (NEHI) and pulmonary interstitial glycogenosis. The outcome of typical NEHI is favorable. The outcome may be different for patients without a typical NEHI presentation, and thus a lung biopsy to differentiate the diseases is indicated. OBJECTIVES: To determine whether infants with the characteristic clinical presentation and computed tomographic (CT) imaging of NEHI (referred to as "usual PTI") have long-term outcome and biopsy findings similar to those of infants with an aberrant presentation and/or with additional localized minor CT findings (referred to as "aberrant PTI"). METHODS: In a retrospective cohort study, 89 infants with PTI were diagnosed on the basis of clinical symptoms and, if available, CT scans and lung biopsies. Long-term outcome in childhood was measured on the basis of current status. MEASUREMENTS AND MAIN RESULTS: Infants with usual PTI had the same respiratory and overall outcomes during follow-up of up to 12 years (mean, 3.8 yr) as infants who had some additional localized minor findings (aberrant PTI) visualized on CT images. Both usual and aberrant PTI had a relatively favorable prognosis, with 50% of the subjects fully recovered by age 2.6 years. None of the infants died during the study period. This was independent of the presence or absence of histological examination. CONCLUSIONS: PTI can be diagnosed on the basis of typical history taking, clinical findings, and a high-quality CT scan. Further diagnostic measures, including lung biopsies, may be limited to rare, complicated cases, reducing the need for an invasive and potentially harmful procedure. AbstractRationale: Persistent tachypnea of infancy (PTI) is a specific clinical entity of undefined etiology comprising the two diseases neuroendocrine cell hyperplasia of infancy (NEHI) and pulmonary interstitial glycogenosis. The outcome of typical NEHI is favorable. The outcome may be different for patients without a typical NEHI presentation, and thus a lung biopsy to differentiate the diseases is indicated.

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confidence: 86%

“…Histological examinations show oval, glycogen-rich mesenchymal cells expanding the interstitium. No fibrosis or inflammatory reaction is seen (11)(12)(13)(14)(15)(16)(17)(18)(19)(20). Of interest, NECs are also hyperplastic (10).…”

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confidence: 99%

Persistent Tachypnea of Infancy. Usual and Aberrant

Rauch¹,

Wetzke

Reu

et al. 2016

Am J Respir Crit Care Med

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“…architectural distortion with ground-glass opacities, thick perilobular opacities, and variably-sized pulmonary lobules, some of which exhibit cyst-like hyperlucency related to alveolar simplification Reported features of PIG on CXR include hyperinflation and interstitial opacities (Smets et al 2004;Canakis et al 2002;Onland et al 2005;Lanfranchi et al 2010). Reported features of PIG on CT include pulmonary architectural distortion, interstitial thickening, ground-glass opacities, and hyperlucent areas (Smets et al 2004;Onland et al 2005;Lanfranchi et al 2010;Castillo et al 2010) (Fig. 6).…”

Section: 3mentioning

confidence: 91%

“…Corticosteroid therapy may hasten the resolution of PIG, possibly due to acceleration of lung maturation rather than to suppression of inflammation (Deterding 2010;Deutsch and Young 2010;Canakis et al 2002;Onland et al 2005), but should be used judiciously in this self-limited disorder due to the risks of neuro-developmental impairment, immunosuppression, and poor wound healing associated with corticosteroids (Radman et al 2013). architectural distortion with ground-glass opacities, thick perilobular opacities, and variably-sized pulmonary lobules, some of which exhibit cyst-like hyperlucency related to alveolar simplification Reported features of PIG on CXR include hyperinflation and interstitial opacities (Smets et al 2004;Canakis et al 2002;Onland et al 2005;Lanfranchi et al 2010). Reported features of PIG on CT include pulmonary architectural distortion, interstitial thickening, ground-glass opacities, and hyperlucent areas (Smets et al 2004;Onland et al 2005;Lanfranchi et al 2010;Castillo et al 2010) (Fig.…”

Section: 3mentioning

confidence: 94%

Diffuse Lung Disease

Guillerman

2014

Pediatric Chest Imaging

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“…Imaging findings vary considerably based on whether PIG occurs as an isolated condition (diffuse PIG), or associated with an underlying lung growth disorder (patchy PIG). 52,53 Typical HRCT features include GGO, reticular changes, and hyperinflated areas in a predominantly subpleural distribution. 41 In patchy PIG, these abnormalities overlap with those of the underlying lung growth disorder.…”

Section: Nehimentioning

confidence: 99%

Interstitial Lung Disease in Children Younger Than 2 Years

Spagnolo

Bush

2016

Pediatrics

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Childhood interstitial lung disease (chILD) represents a highly heterogeneous group of rare disorders associated with substantial morbidity and mortality. Although our understanding of chILD remains limited, important advances have recently been made, the most important being probably the appreciation that disorders that present in early life are distinct from those occurring in older children and adults, albeit with some overlap. chILD manifests with diffuse pulmonary infiltrates and nonspecific respiratory signs and symptoms, making exclusion of common conditions presenting in a similar fashion an essential preliminary step. Subsequently, a systematic approach to diagnosis includes a careful history and physical examination, computed tomography of the chest, and some or all of bronchoscopy with bronchoalveolar lavage, genetic testing, and if diagnostic uncertainty persists, lung biopsy. This review focuses on chILD presenting in infants younger than 2 years of age and discusses recent advances in the classification, diagnostic approach, and management of chILD in this age range. We describe novel genetic entities, along with initiatives that aim at collecting clinical data and biologic samples from carefully characterized patients in a prospective and standardized fashion. Early referral to expert centers and timely diagnosis may have important implications for patient management and prognosis, but effective therapies are often lacking. Following massive efforts, international collaborations among the key stakeholders are finally starting to be in place. These have allowed the setting up and conducting of the first randomized controlled trial of therapeutic interventions in patients with chILD.

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Pulmonary interstitial glycogenosis

Cited by 40 publications

References 5 publications

Persistent Tachypnea of Infancy. Usual and Aberrant

Persistent Tachypnea of Infancy. Usual and Aberrant

Diffuse Lung Disease

Interstitial Lung Disease in Children Younger Than 2 Years

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