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Neuroendocrine hyperplasia of infancy

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https://www.readbyqxmd.com/read/27629751/neuroendocrine-cell-hyperplasia-of-infancy-an-unusual-cause-of-hypoxemia-in-children
#1
Silvia Caimmi, Amelia Licari, Davide Caimmi, Anna Rispoli, Eugenio Baraldi, Fiorella Calabrese, Gian Luigi Marseglia
BACKGROUND: Childhood interstitial lung disease (chILD) is a heterogeneous group of rare disorders characterized by abnormal imaging findings, impaired gas exchange; and is associated with substantial morbidity and mortality. Neuroendocrine cell hyperplasia (NEHI) is a unique sub-group, which is more prevalent in infants and children younger than 2 years of age, and typically manifests with chronic tachypnea, retractions, hypoxemia and failure to thrive. NEHI insidiously appears in the first year of life, subtly masquerading as one of the more common lung diseases of childhood...
September 15, 2016: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/27514602/diffuse-idiopathic-pulmonary-neuroendocrine-cell-hyperplasia-and-neuroendocrine-hyperplasia-of-infancy
#2
REVIEW
Laurie L Carr, Jeffrey A Kern, Gail H Deutsch
Although incidental reactive pulmonary neuroendocrine cell hyperplasia (PNECH) is seen on biopsy specimens in adults with chronic lung disease, disorders characterized by marked PNECH are rare. Primary hyperplasia of neuroendocrine cells in the lung and obstructive lung disease related to remodeling or physiologic constriction of small airways define diffuse idiopathic neuroendocrine cell hyperplasia (DIPNECH) in the adult and neuroendocrine cell hyperplasia of infancy (NEHI) in children. DIPENCH and NEHI share a similar physiology, typical imaging appearance, and increased neuroendocrine cells on biopsy...
September 2016: Clinics in Chest Medicine
https://www.readbyqxmd.com/read/27187870/persistent-lung-disease-in-adults-with-nkx2-1-mutation-and-familial-neuroendocrine-cell-hyperplasia-of-infancy
#3
Rebekah J Nevel, Errine T Garnett, John A Worrell, Ronald L Morton, Lawrence M Nogee, Timothy S Blackwell, Lisa R Young
RATIONALE: Neuroendocrine cell hyperplasia of infancy (NEHI) is a diffuse lung disease that presents in infancy and improves during childhood. Long-term outcomes have not previously been described. In one familial cohort, we have reported that NEHI is associated with a heterozygous variant of NKX2.1/TTF1. OBJECTIVES: Our objective was to determine whether pulmonary abnormalities persist in adults with NEHI, to aid in elucidating the natural history of this disease...
August 2016: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/26839222/new-entities-and-diagnostic-challenges-in-pediatric-lung-disease
#4
REVIEW
Megan K Dishop
The differential diagnosis of diffuse lung disease in children differs considerably from adults, and analysis of pediatric lung biopsies may prove challenging for pathologists with more extensive exposure to adult lung biopsies. Biopsy diagnosis of pediatric lung disease continues to evolve as new pathologic entities are recognized and new genetic determinants of disease are discovered. This article describes the clinical characteristics, pathologic features, and differential diagnosis of challenging and recently described entities in pediatric lung disease...
September 2010: Surgical Pathology Clinics
https://www.readbyqxmd.com/read/26744669/the-role-of-high-resolution-chest-ct-in-the-diagnosis-of-neuroendocrine-cell-hyperplasia-of-infancy-a-rare-form-of-pediatric-interstitial-lung-disease
#5
Julia Lee, Thomas Ray Sanchez, Yanhong Zhang, Sanjay Jhawar
Interstitial lung disease (ILD) is rare in infancy or early childhood. Differentiating between the different types of ILD is important for reasons of treatment, monitoring of clinical course and prognosis. We present a case of a 5-month old female with tachypnea and hypoxemia. The clinical suspicion of neuroendocrine cell hyperplasia of infancy (NEHI) was confirmed by high-resolution chest CT and subsequent lung biopsy. We conclude that high-resolution chest CT has characteristics findings that can be used as a non-invasive test to support the clinical diagnosis of neuroendocrine cell hyperplasia of infancy...
2015: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/26642007/exacerbations-in-neuroendocrine-cell-hyperplasia-of-infancy-are-characterized-by-increased-air-trapping
#6
Paul R Houin, Robin R Deterding, Lisa R Young
Neuroendocrine cell Hyperplasia of Infancy (NEHI) presents with tachypnea, retractions, hypoxemia, and often failure to thrive. The radiologic and physiologic findings in infants with NEHI have been well described with a distinct geographic pattern of ground-glass opacities on chest computerized tomography imaging and profound air-trapping on infant pulmonary function testing. Despite gradual improvement over time, unexplained exacerbation has been observed but not well characterized. We present physiological and radiological changes of increased air-trapping during acute exacerbations in two older children with NEHI who had previously experienced significant clinical improvement...
March 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/26584876/hyperplasia-of-pulmonary-neuroendocrine-cells-in-infancy-and-childhood
#7
REVIEW
Ernest Cutz
Pulmonary neuroendocrine cells (PNEC) are widely distributed throughout the airway mucosa of mammalian lung as solitary cells and as distinctive innervated clusters, neuroepithelial bodies (NEB). These cells differentiate early during lung development and are more prominent in fetal/neonatal lungs compared to adults. PNEC/NEB cells produce biogenic amine (serotonin) and a variety of peptides (i.e., bombesin) involved in regulation of lung function. During the perinatal period, NEB are thought to function as airway O(2)/CO(2) sensors...
November 2015: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/26540427/rare-becomes-more-common-recognizing-neuroendocrine-cell-hyperplasia-of-infancy-in-everyday-pulmonary-consultations
#8
LETTER
Michael Glenn O'Connor, Mark Wurth, Lisa R Young
No abstract text is available yet for this article.
November 2015: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/26474448/persistent-tachypnea-of-infancy-usual-and-aberrant
#9
Daniela Rauch, Martin Wetzke, Simone Reu, Waltraud Wesselak, Andrea Schams, Meike Hengst, Birgit Kammer, Julia Ley-Zaporozhan, Matthias Kappler, Marijke Proesmans, Joanna Lange, Amparo Escribano, Eitan Kerem, Frank Ahrens, Frank Brasch, Nicolaus Schwerk, Matthias Griese
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")...
February 15, 2016: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/26303495/augmented-5-ht-secretion-in-pulmonary-neuroepithelial-bodies-from-phd1-null-mice
#10
Simon Livermore, Jie Pan, Herman Yeger, Peter Ratcliffe, Tammie Bishop, Ernest Cutz
Sustained exposure to low oxygen concentration leads to profound changes in gene expression to restore oxygen homeostasis. Hypoxia-inducible factors (HIFs) comprise a group of transcription factors which accumulate under hypoxia and contribute to the complex changes in gene expression. Under normoxic conditions HIFs are degraded by prolyl-hydroxylases (PHD), however during hypoxia this degradation is inhibited causing HIF accumulation and subsequent changes in gene expression. Pulmonary neuroepithelial bodies (NEB) are innervated serotonin (5-HT)-producing cells distributed throughout the airway epithelium...
2015: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/26234096/-modern-views-on-children-s-interstitial-lung-disease
#11
REVIEW
E V Boĭtsova, M A Beliashova, D Iu Ovsiannikov
Interstitial lung diseases (ILD, diffuse lung diseases) are a heterogeneous group of diseases in which a pathological process primarily involved alveoli and perialveolar interstitium, resulting in impaired gas exchange, restrictive changes of lung ventilation function and diffuse interstitial changes detectable by X-ray. Children's interstitial lung diseases is an topical problem ofpediatricpulmonoogy. The article presents current information about classification, epidemiology, clinical presentation, diagnostics, treatment and prognosis of these rare diseases...
2015: Vestnik Rossiĭskoĭ Akademii Meditsinskikh Nauk
https://www.readbyqxmd.com/read/25684686/bombesin-staining-in-neuroendocrine-cell-hyperplasia-of-infancy-nehi-and-other-childhood-interstitial-lung-diseases-child
#12
Slaveya G Yancheva, Asha Velani, Alexandra Rice, Angeles Montero, David M Hansell, Sergio Koo, Lina Thia, Andrew Bush, Andrew G Nicholson
AIMS: We have analysed levels of bombesin-positive neuroendocrine cells (NECs) in neuroendocrine cell hyperplasia of infancy (NEHI) and other childhood interstitial lung diseases (chILDs) in order to validate proposed histological criteria for NEHI and investigate its aetiology. METHODS AND RESULTS: The extent of bombesin-positive cells within airway epithelium was analysed in lung biopsies from seven patients diagnosed with NEHI, including two classified previously as non-diagnostic, and other chILDs (n = 64) with age ranges of 1 month-18 years...
October 2015: Histopathology
https://www.readbyqxmd.com/read/25569780/-a-remarkable-experience-of-god-shaping-us-as-a-family-parents-use-of-faith-following-child-s-rare-disease-diagnosis
#13
Hillary N Purcell, Allison Whisenhunt, Joy Cheng, Sophia Dimitriou, Lisa R Young, Daniel H Grossoehme
A child's chronic illness can lead parents to utilize different types of coping, including religious beliefs and practices. Previous studies have generally focused on life-shortening diagnoses. The present study explored parental use of faith when the diagnosis was not life-shortening, using grounded-theory qualitative methodology. Data were collected using semi-structured telephone interviews with N = 12 parents of children diagnosed with Neuroendocrine Hyperplasia of Infancy (NEHI); approximately 50% of the diagnosed population in the United States at the time of the interview...
2015: Journal of Health Care Chaplaincy
https://www.readbyqxmd.com/read/24964239/-neuroendocrine-cell-hyperplasia-of-infancy-case-study
#14
Joanna Lange, Megan Dishop, Katarzyna Krenke
Neuroendocrine cell hyperplasia of infancy is a rare form of children's interstitial lung disease recognised usually in infancy and in children younger than two years old. The typical clinical scenario, such as chest retractions, tachypnoea, hypoxaemia, crackles, characteristic changes in high-resolution computed tomography and histological examination of the lung parenchyma, is the cornerstone for diagnosis. In the article, the authors describe clinical manifestation of neuroendocrine cell hyperplasia and a present case of an infant with this rare interstitial lung disease...
2014: Pneumonologia i Alergologia Polska
https://www.readbyqxmd.com/read/24915923/-a-case-of-neuroendocrine-cell-hyperplasia-of-infancy-nehi
#15
Liyuan Lyu, Xiuyun Liu, Zaifang Jiang
No abstract text is available yet for this article.
April 2014: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/24752172/interstitial-lung-disease-in-children
#16
REVIEW
Christin S Kuo, Lisa R Young
PURPOSE OF REVIEW: There has been tremendous progress in the approach to childhood interstitial lung diseases (chILD), with particular recognition that interstitial lung disease (ILD) in infants is often distinct from the forms that occur in older children and adults. Diagnosis is challenging because of the rarity of ILD and the fact that the presenting symptoms of ILD often overlap those of common respiratory disorders. This review summarizes the newly published recommendations for diagnosis and management, and highlights the recent scientific advances in several specific forms of chILD...
June 2014: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/24310630/diagnostic-criteria-and-follow-up-in-neuroendocrine-cell-hyperplasia-of-infancy-a-case-series
#17
Vivianne Calheiros Chaves Gomes, Mara Cristina Coelho Silva, José Holanda Maia Filho, Pedro Daltro, Simone Gusmão Ramos, Alan S Brody, Edson Marchiori
OBJECTIVE: Neuroendocrine cell hyperplasia of infancy (NEHI) is a form of childhood interstitial lung disease characterized by tachypnea, retractions, crackles, and hypoxia. The aim of this study was to report and discuss the clinical, imaging, and histopathological findings in a series of NEHI cases at a tertiary pediatric hospital, with an emphasis on diagnostic criteria and clinical outcomes. METHODS: Between 2003 and 2011, 12 full-term infants were diagnosed with NEHI, based on clinical and tomographic findings...
September 2013: Jornal Brasileiro de Pneumologia: Publicaça̋o Oficial da Sociedade Brasileira de Pneumologia e Tisilogia
https://www.readbyqxmd.com/read/24216293/bronchoalveolar-lavage-fluid-cytokine-profiles-in-neuroendocrine-cell-hyperplasia-of-infancy-and-follicular-bronchiolitis
#18
Jonathan Popler, Brandie D Wagner, Heidi Luckey Tarro, Frank J Accurso, Robin R Deterding
BACKGROUND: Neuroendocrine Cell Hyperplasia of Infancy (NEHI) and Follicular Bronchiolitis (FB) are rare pediatric diffuse lung diseases with poorly understood pathogenesis and similar clinical presentations. We sought to determine if cellular and cytokine profiles in bronchoalveolar lavage fluid (BALF) from subjects with NEHI and FB would differ from pediatric disease controls. METHODS: BALF was obtained from forty-one subjects classified into four disease groups: NEHI, Cystic Fibrosis (CF), other airway disease controls (DC), and FB during clinically indicated procedures...
2013: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/24081995/childhood-interstitial-lung-diseases-an-18-year-retrospective-analysis
#19
REVIEW
Jennifer J Soares, Gail H Deutsch, Paul E Moore, Mohammad F Fazili, Eric D Austin, Rebekah F Brown, Andrew G Sokolow, Melissa A Hilmes, Lisa R Young
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...
October 2013: Pediatrics
https://www.readbyqxmd.com/read/23973003/interstitial-lung-disease-in-children
#20
REVIEW
Salvatore Cazzato, Emanuela di Palmo, Vincenzo Ragazzo, Silvia Ghione
Children's interstitial lung disease (ILD) includes a wide range of rare respiratory disorders associated with high morbidity and mortality. Genetic factors, systemic disease processes, nonspecific inflammatory or fibrotic patterns of repair seen in a number of clinical settings are involved in the ILD pathogenesis. Specific disorders more prevalent in young children include diffuse developmental disorders, alveolar growth abnormalities, genetic surfactant disorders, pulmonary interstitial glycogenosis and neuroendocrine cell hyperplasia of infancy...
October 2013: Early Human Development
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