Bombesin staining in neuroendocrine cell hyperplasia of infancy (<scp>NEHI</scp>) and other childhood interstitial lung diseases (ch<scp>ILD</scp>)

Yancheva, Slaveya; Velani, Asha; Rice, Alistair; Montero, Ángeles; Hansell, David M.; Koo, Sergio; Thia, Lina; Bush, Andrew; Nicholson, Andrew G.

doi:10.1111/his.12672

Cited by 37 publications

(36 citation statements)

References 15 publications

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“…Such NEC hyperplasia is well known in many other disorders among children with interstitial lung disease (10). Thus, we can conclude that lung biopsy does not appear to be helpful in the diagnosis of PTI when there is no clinical need to differentiate the condition further into NEHI or PIG.…”

Section: Discussionmentioning

confidence: 83%

“…Because this condition is associated with increased numbers of neuroendocrine cells (NECs) (3), lung biopsies became an important diagnostic procedure. However, it soon became clear that hyperplasia of those cells was not a specific feature of NEHI (i.e., PTI), as this presentation has been described in a variety of other rare pediatric lung diseases (3,10).…”

mentioning

confidence: 95%

“…No fibrosis or inflammatory reaction is seen (11)(12)(13)(14)(15)(16)(17)(18)(19)(20). Of interest, NECs are also hyperplastic (10).…”

mentioning

confidence: 99%

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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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Section: Discussionmentioning

confidence: 83%

mentioning

confidence: 95%

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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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“…There was no formal criterion for defining excess of pulmonary neuroendocrine cells, but it was proposed in previous study [24] that finding of PNEC within 70% of bronchioles in the lung biopsy specimen and >10% in an individual airway is consistent with diagnosis of NEHI in appropriate clinical setting. This criterion has been questioned in study by Yancheva et al [25] that children with various lung diseases would also meet this criterion and therefore while the criterion might be sensitive for diagnosis of NEHI, it may lack specificity. They proposed using the average percentage of NEC per airway to identify NEHI more accurately; however, there was still significant overlap in the number of NEC/airway between NEHI and other lung diseases including surfactant protein disorder.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Neuroendocrine Cell Hyperplasia Associated With Surfactant Protein C Gene Mutation

Jiramethee

Khoór

Erasmus

2016

Chest

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Familial interstitial lung disease (ILD) is defined as presence of ILD in 2 or more family members. Surfactant protein C (SFTPC) gene mutations are rare, but well-known cause of familial ILD. We reported a 20-year-old male, who was referred for lung transplantation. He was symptomatic at age 3 and underwent surgical lung biopsy at age 6, which revealed a nonspecific interstitial pneumonia (NSIP) pattern. Genetic workup revealed a novel SFTPC mutation in the first intron with a C to A transversion. At age 21, he underwent bilateral lung transplantation. Explanted lung histology suggested NSIP. In addition there was pulmonary neuroendocrine cell (PNEC) hyperplasia and carcinoid tumorlets. His mother had undergone lung transplantation several years earlier, and her explanted lung showed similar pathology. SFTPC mutations are inherited in an autosomal dominant pattern. Various types of ILD have been associated with SFTPC mutation including NSIP, usual interstitial pneumonia (UIP), and desquamative interstitial pneumonia (DIP). PNEC hyperplasia has been described to occur in association with lung inflammation but has not been previously described with familial ILD associated with SFTPC mutation.

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“…9,19,[26][27][28][29][30][31][32][33][34][35][36][37] A primary advance in this area is the growing recognition that NEHI can be diagnosed by using a chest CT scan without the need for lung biopsy. Brody et al 9 first fully described the radiologic features of NEHI in 2010.…”

Section: Neuroendocrine Cell Hyperplasia Of Infancymentioning

confidence: 99%