Neuroendocrine Cell Distribution and Frequency Distinguish Neuroendocrine Cell Hyperplasia of Infancy From Other Pulmonary Disorders

Young, Lisa R.; Brody, Alan S.; Inge, Thomas H.; Acton, James D.; Bokulic, Ronald E.; Langston, Claire; Deutsch, Gail H.

doi:10.1378/chest.10-1304

Cited by 115 publications

(134 citation statements)

References 42 publications

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“…Therefore, in the current era of clinically available genetic testing, we anticipate reductions in the numbers of surgical lung biopsies in this patient population. Lastly, in retrospect, 5 of the 8 NEHI cases met criteria for diagnosis based on clinical and radiographic findings, 19,22 and therefore lung biopsy would be unlikely to be performed in the current clinical practice pattern at our center and others. 23 We recognize the limitations inherent to this type of retrospective study.…”

Section: Figurementioning

confidence: 97%

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Childhood Interstitial Lung Diseases: An 18-year Retrospective Analysis

Soares

Deutsch

Moore³

et al. 2013

Pediatrics

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WHAT'S KNOWN ON THIS SUBJECT: Childhood interstitial lung diseases occur in a variety of clinical contexts and are associated with high morbidity and mortality. Advances in the understanding of disease pathogenesis and use of standardized terminology have facilitated increased case ascertainment. WHAT THIS STUDY ADDS:This study demonstrates that cases of newly described forms of childhood interstitial lung diseases likely occur at all children' s hospitals. With advances in genetic testing and recognition of imaging patterns, a significant portion of cases are identifiable with noninvasive evaluations. abstract OBJECTIVE: Childhood interstitial lung diseases (ILD) occur in a variety of clinical contexts. Advances in the understanding of disease pathogenesis and use of standardized terminology have facilitated increased case ascertainment. However, as all studies have been performed at specialized referral centers, the applicability of these findings to general pulmonary practice has been uncertain. The objective of this study was to determine the historical occurrence of childhood ILD to provide information reflecting general pediatric pulmonary practice patterns. METHODS:Childhood ILD cases seen at Vanderbilt Children' s Hospital from 1994 to 2011 were retrospectively reviewed and classified according to the current pediatric diffuse lung disease histopathologic classification system. RESULTS:A total of 93 cases were identified, of which 91.4% were classifiable. A total of 68.8% (64/93) of subjects underwent lung biopsy in their evaluations. The largest classification categories were disorders related to systemic disease processes (24.7%), disorders of the immunocompromised host (24.7%), and disorders more prevalent in infancy (22.6%). Eight cases of neuroendocrine cell hyperplasia of infancy (NEHI) were identified, including 5 that were previously unrecognized before this review.CONCLUSIONS: Our findings demonstrate the general scope of childhood ILD and that these cases present within a variety of pediatric subspecialties. Retrospective review was valuable in recognizing more recently described forms of childhood ILD. As a significant portion of cases were classifiable based on clinical, genetic, and/or radiographic criteria, we urge greater consideration to noninvasive diagnostic approaches and suggest modification to the current childhood ILD classification scheme to accommodate the increasing number of cases diagnosed without lung biopsy. Pediatrics 2013;132:684-691

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Section: Figurementioning

confidence: 97%

“…4,19,21,22 Of the 8 NEHI cases, 5 were recognized via the retrospective review process. Three of these 5 cases came to clinical attention before the original description of NEHI in the medical literature in 2005.…”

Section: Figurementioning

confidence: 99%

Childhood Interstitial Lung Diseases: An 18-year Retrospective Analysis

Soares

Deutsch

Moore³

et al. 2013

Pediatrics

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“…OLB has been the primary surgical approach in young children (120,121,132,133). However, VATS visualizes a greater percentage of the lung and permits the sampling of different lobes with the same incision sites (a consensus statement recommends multiple biopsy sites [134], even though there are limited data to support this approach [58]). Studies that have evaluated pediatric lung biopsy using VATS found less postoperative and long-term morbidity compared with OLB (16,135,136).…”

Section: Diagnostic Testsmentioning

confidence: 99%

“…As an examples, SP-B deficiency (9,156) and ACD-MPV (107) have a bleak prognosis, mutations in ABCA3 (10) and STFPC (147,173,177) lead to more variable disease, and NEHI has a more favorable prognosis (58,197).…”

Section: Prognosismentioning

confidence: 99%

An Official American Thoracic Society Clinical Practice Guideline: Classification, Evaluation, and Management of Childhood Interstitial Lung Disease in Infancy

Kurland¹,

Deterding²,

Hagood³

et al. 2013

Am J Respir Crit Care Med

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“…However, although hyperplasia of neuroendocrine cells within bronchioles (as demonstrated by immunohistochemistry), the only consistent pathologic finding, may be seen in a variety of disorders, including bronchopulmonary dysplasia, bronchiolitis, and PH, 14,44,45 neuroendocrine cell prominence with otherwise normal histopathology is a distinguishing feature of NEHI. 46 These other conditions all have specific features, which would preclude diagnostic confusion with NEHI. Reported familial cases suggest that NEHI may have a genetic basis, 47 although, to date, no mutation has been consistently identified.…”

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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Neuroendocrine Cell Distribution and Frequency Distinguish Neuroendocrine Cell Hyperplasia of Infancy From Other Pulmonary Disorders

Cited by 115 publications

References 42 publications

Childhood Interstitial Lung Diseases: An 18-year Retrospective Analysis

Childhood Interstitial Lung Diseases: An 18-year Retrospective Analysis

An Official American Thoracic Society Clinical Practice Guideline: Classification, Evaluation, and Management of Childhood Interstitial Lung Disease in Infancy

Interstitial Lung Disease in Children Younger Than 2 Years

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