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Robin R Deterding - One of the best experts on this subject based on the ideXlab platform.
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Lung and airway shape in Neuroendocrine Cell hyperplasia of infancy
Pediatric Radiology, 2018Co-Authors: Emily J. Mastej, Emily M. Deboer, Stephen M. Humphries, Marlijne C. Cook, Kendall S. Hunter, Deborah R. Liptzin, Jason P. Weinman, Robin R DeterdingAbstract:Background Neuroendocrine Cell hyperplasia of infancy (NEHI) is a rare lung disease associated with significant air trapping. Although chest CT is crucial in establishing a diagnosis, CT and biopsy findings do not reveal airway abnormalities to explain the air trapping. Objective We compared lung and airway morphology obtained from chest CT scans in children with NEHI and control children. In the children with NEHI, we explored relationships between lung and airway shape and lung function. Materials and methods We performed a retrospective review of children with NEHI who underwent clinical chest CT. We identified control children of similar size and age. We created lung masks and airway skeletons using semi-automated software and compared them using statistical shape modeling methods. Then we calculated a logistic regression model using lung and airway shape to differentiate NEHI from controls, and we compared shape model parameters to lung function measurements. Results Airway and lung shapes were statistically different between children with NEHI and controls. We noted a broad lung apex in the children with NEHI and a significantly increased apical anterior–posterior lung diameter. A logistic regression model including lung shape was 90% accurate in differentiating children with NEHI from controls. Correlation coefficients were significant between lung function values and lung and airway shape. Conclusion Lung and airway shapes were different between children with NEHI and control children in this cohort. Children with NEHI had an increased anteroposterior diameter of their lungs that might be useful in the diagnostic criteria.
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bronchoalveolar lavage fluid cytokine profiles in Neuroendocrine Cell hyperplasia of infancy and follicular bronchiolitis
Orphanet Journal of Rare Diseases, 2013Co-Authors: Jonathan Popler, Brandie D Wagner, Heidi Luckey Tarro, Frank J Accurso, Robin R DeterdingAbstract: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. 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. BALF Cellular profiles and ten cytokines were measured and values compared across groups using descriptive and nonparametric statistics. Significant BALF Cellular and cytokine differences were seen across all groups. NEHI subjects exhibited the lowest total absolute white blood Cell (WBC) levels with a higher percentage of BALF alveolar macrophages compared to controls. NEHI also had lower levels of IL-1β, MIP-1β and IL-8 and FB had higher levels of IL-1ra, G-CSF and VEGF compared to all groups. IL-6 was elevated in CF and FB. BALF cytokine and Cellular profiles differed between NEHI, FB, CF and DC subjects. This pilot data suggests different and distinguishing inflammatory responses in the airway, with the least inflammatory being NEHI. These data could have diagnostic implications.
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abnormal infant pulmonary function in young children with Neuroendocrine Cell hyperplasia of infancy
Pediatric Pulmonology, 2013Co-Authors: Gwendolyn S Kerby, Brandie D Wagner, Jonathan Popler, Frank J Accurso, Thomas C Hay, Carol Kopecky, Stephanie L Wilcox, Ralph Quinones, Roger Giller, Robin R DeterdingAbstract:Rationale Lung function in children with Neuroendocrine Cell hyperplasia of infancy (NEHI) and correlations with future clinical outcomes are needed to guide clinical management. Objective To compare results of infant pulmonary function tests (IPFTs) in children with NEHI to disease control (DC) subjects and to correlate NEHI IPFTs with future outcomes. Methods We performed a retrospective, single center study of IPFT in subjects diagnosed by lung biopsy (NEHI) or clinically (NEHI syndrome) and in DC subjects evaluated for cancer or pre-hematopoietic stem Cell transplantation (HSCT). Raised volume rapid thoracoabdominal compression (RVRTC) and plethysmography were performed on all infants and evaluated for quality. Standard spirometry measures, room air oxygen saturations (RA O2 sat), and weight percentiles were collected during follow up. Measurements and Main Results Fifty-seven IPFTs were performed in 15 NEHI, 22 NEHI syndrome, and 20 DC subjects. RVRTC and FRC measurements were obtained in 85% or more of subjects in all groups. Significant airflow limitation (FEV0.5 P-value ≤ 0.01) and air trapping (FRC P-value ≤ 0.01) were seen in NEHI and NEHI syndrome subjects compared to DCs. No significant correlations were found between IPFT, oxygen use, RA O2 sat, and weight at the time of the IPFTs. Initial FEV0.5 and FRC z-scores correlated with RA O2 sat (r = 0.60 and −0.49) at short-term follow up (6–12 months). Most measurements of RVRTC correlated with FEV1 (n = 5) measured 4–5 years later (r > 0.50). Conclusions IPFTs in NEHI subjects are feasible, demonstrate significant obstruction and air trapping, and correlate with future RA O2 sat and FEV1. IPFTs may provide valuable clinical information when caring for NEHI patients. Pediatr Pulmonol. 2013; 48:1008–1015. © 2012 Wiley Periodicals, Inc.
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Neuroendocrine Cell hyperplasia of infancy diagnosis with high resolution ct
American Journal of Roentgenology, 2010Co-Authors: Alan S Brody, Gail H. Deutsch, Paul R Guillerman, Brandie D Wagner, Lisa R Young, Robin R DeterdingAbstract:OBJECTIVE. Neuroendocrine Cell hyperplasia of infancy is a form of childhood interstitial lung disease originally reported as persistent tachypnea of infancy. Reports of small series of cases and anecdotal experience have suggested that this disorder may have a consistent CT pattern. The purpose of this study was to review the CT findings in children with Neuroendocrine Cell hyperplasia of infancy to determine the findings at high-resolution CT, the diagnostic accuracy of CT compared with biopsy, and interrater reliability.MATERIALS AND METHODS. Images from 23 CT examinations of children with biopsy-proven Neuroendocrine Cell hyperplasia of infancy and six CT examinations of children with other childhood interstitial lung diseases were reviewed by two pediatric radiologists with special expertise in thoracic imaging. Identifying digital data were removed, and images were reviewed without clinical data. A CT assessment form was completed for each patient.RESULTS. Ground-glass opacification was the most com...
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persistent tachypnea of infancy is associated with Neuroendocrine Cell hyperplasia
Pediatric Pulmonology, 2005Co-Authors: Robin R Deterding, Catherine Pye, Claire LangstoAbstract:We sought to determine the clinical course and histologic findings in lung biopsies from a group of children who presented with signs and symptoms of interstitial lung disease (ILD) without identified etiology. Patients were identified from the pathology files at the Texas Children's Hospital who presented below age 2 years with persistent tachypnea, hypoxia, retractions, or respiratory crackles, and with nonspecific and nondiagnostic lung biopsy findings. Age-matched lung biopsy controls were also identified. Their clinical courses were retrospectively reviewed. Biopsies were reviewed, and immunostaining with antibodies to Neuroendocrine Cells was done. Fifteen pediatric ILD patients and four control patients were identified for inclusion in the study. Clinically, the mean onset of symptoms was 3.8 months (range, 0-11 months). Radiographs demonstrated hyperinflation, interstitial markings, and ground-glass densities. Oxygen was initially required for prolonged periods, and medication trials did not eliminate symptoms. After a mean of 5 years, no deaths had occurred, and patients had improved. On review of the lung biopsies, all had a similar appearance, with few abnormalities noted. Immunostaining with antibodies to Neuroendocrine Cell products showed consistently increased bombesin staining. Subsequent morphometric analysis showed that immunoreactivity for bombesin and serotonin was significantly increased over age-matched controls. In conclusion, we believe this may represent a distinct group of pediatric patients defined by the absence of known lung diseases, clinical signs and symptoms of ILD, and idiopathic Neuroendocrine Cell hyperplasia of infancy. These findings may be important for the evaluation of ILD in young children.
Philipp U Heitz - One of the best experts on this subject based on the ideXlab platform.
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the gastroenteropancreatic Neuroendocrine Cell system and its tumors the who classification
Annals of the New York Academy of Sciences, 2004Co-Authors: Gunter Kloppel, Aurel Perren, Philipp U HeitzAbstract:Although well established in medical terminology, the term carcinoid is no longer adequate to cover the entire morphological and biological spectrum of neoplasms of the disseminated Neuroendocrine Cell system. Therefore, instead of carcinoid, the WHO classification published in 2000 uses the general terms Neuroendocrine tumor and Neuroendocrine carcinoma. In this review a classification of gastroenteropancreatic Neuroendocrine tumors based on the WHO criteria is described. We also classify and comment on the most important tumor entities. On the basis of localization and of various morphological and biological criteria, we distinguish between benign Neuroendocrine tumors, tumors with uncertain malignant potential, and tumors showing low-grade and high-grade malignancy.
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pathology and nomenclature of human gastrointestinal Neuroendocrine carcinoid tumors and related lesions
World Journal of Surgery, 1996Co-Authors: Gunter Kloppel, Philipp U Heitz, Carlo Capella, E SolciaAbstract:: The pathology and nomenclature of the Neuroendocrine Cell proliferations in the gut are reviewed. The neoplastic lesions are discussed within the light of a new classification system that attempts to consider the morphologic, functional, and biologic features of the tumors.
Giulio Rossi - One of the best experts on this subject based on the ideXlab platform.
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diffuse idiopathic pulmonary Neuroendocrine Cell hyperplasia dipnech syndrome and carcinoid tumors with without nech a clinicopathologic radiologic and immunomolecular comparison study
The American Journal of Surgical Pathology, 2018Co-Authors: Mc Mengoli, Em Silini, Giulio Rossi, Alberto Cavazza, Renato Franco, Federica Zito Marino, Mario Migaldi, Letizia Gnetti, Luca Ampollini, Marcello TiseoAbstract:The diagnostic criteria of diffuse idiopathic pulmonary Neuroendocrine Cell hyperplasia (DIPNECH) are not well defined, and DIPNECH can be mistaken for carcinoids associated with Neuroendocrine Cell hyperplasia (NECH). In this study, we compared clinical, radiologic, histologic, immunohistochemical,
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diffuse idiopathic pulmonary Neuroendocrine Cell hyperplasia dipnech syndrome and carcinoid tumors with without nech a clinicopathologic radiologic and immunomolecular comparison study
The American Journal of Surgical Pathology, 2018Co-Authors: Mc Mengoli, Em Silini, Giulio Rossi, Alberto Cavazza, Renato Franco, Federica Zito Marino, Mario Migaldi, Letizia Gnetti, Luca Ampollini, Marcello TiseoAbstract:The diagnostic criteria of diffuse idiopathic pulmonary Neuroendocrine Cell hyperplasia (DIPNECH) are not well defined, and DIPNECH can be mistaken for carcinoids associated with Neuroendocrine Cell hyperplasia (NECH). In this study, we compared clinical, radiologic, histologic, immunohistochemical, and molecular features of DIPNECH and isolated carcinoids with/without NECH. The study population included 151 cases (77 female patients and 74 male patients), 19 with DIPNECH and 132 with carcinoids with/without NECH. None of the cases displayed molecular alterations or anaplastic lymphoma kinase expression. Compared with individuals with carcinoids with/without NECH, patients with DIPNECH were more likely to be female individuals (P<0.0001), nonsmokers (P=0.021), and symptomatic, and to have an obstructive/mixed respiratory defect, peripheral location of the lesions, and air trapping (P<0.0001) on chest computed tomography, and constrictive bronchiolitis on histology (P<0.0001). Among immunohistochemical markers, DIPNECH was associated with higher expression of thyroid transcription factor-1, CD10, and gastrin-releasing peptide/bombesin-like peptide (P<0.0001). Yet, when a purely histopathologic definition of DIPNECH was applied, 40% of isolated carcinoids also met the diagnostic criteria for DIPNECH, even in the absence of symptoms and/or radiologic abnormalities. Therefore, as DIPNECH represents a distinct clinical syndrome, we suggest the term DIPNECH be limited to cases presenting with respiratory symptoms, functional and/or radiologic abnormalities, and constrictive bronchiolitis on histology.
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Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) Syndrome and Carcinoid Tumors With/Without NECH: A Clinicopathologic, Radiologic, and Immunomolecular Comparison Study
American Journal of Surgical Pathology, 2018Co-Authors: Mc Mengoli, Fz Marino, Em Silini, Giulio Rossi, Alberto Cavazza, Renato Franco, Mario Migaldi, Letizia Gnetti, Luca Ampollini, Marcello TiseoAbstract:The diagnostic criteria of diffuse idiopathic pulmonary Neuroendocrine Cell hyperplasia (DIPNECH) are not well defined, and DIPNECH can be mistaken for carcinoids associated with Neuroendocrine Cell hyperplasia (NECH). In this study, we compared clinical, radiologic, histologic, immunohistochemical, and molecular features of DIPNECH and isolated carcinoids with/without NECH. The study population included 151 cases (77 female patients and 74 male patients), 19 with DIPNECH and 132 with carcinoids with/without NECH. None of the cases displayed molecular alterations or anaplastic lymphoma kinase expression. Compared with individuals with carcinoids with/without NECH, patients with DIPNECH were more likely to be female individuals (P\\textless0.0001), nonsmokers (P=0.021), and symptomatic, and to have an obstructive/mixed respiratory defect, peripheral location of the lesions, and air trapping (P\\textless0.0001) on chest computed tomography, and constrictive bronchiolitis on histology (P\\textless0.0001). Among immunohistochemical markers, DIPNECH was associated with higher expression of thyroid transcription factor-1, CD10, and gastrin-releasing peptide/bombesin-like peptide (P\\textless0.0001). Yet, when a purely histopathologic definition of DIPNECH was applied, 40% of isolated carcinoids also met the diagnostic criteria for DIPNECH, even in the absence of symptoms and/or radiologic abnormalities. Therefore, as DIPNECH represents a distinct clinical syndrome, we suggest the term DIPNECH be limited to cases presenting with respiratory symptoms, functional and/or radiologic abnormalities, and constrictive bronchiolitis on histology.
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Diffuse idiopathic pulmonary Neuroendocrine Cell hyperplasia syndrome
European Respiratory Journal, 2016Co-Authors: Giulio Rossi, Alberto Cavazza, Paolo Spagnolo, Nicola Sverzellati, Lucia Longo, Agita Jukna, Gloria Montanari, Cristiano Carbonelli, Giada Vincenzi, Giuseppe BoginaAbstract:The term diffuse idiopathic pulmonary Neuroendocrine Cell hyperplasia (DIPNECH) may be used to describe a clinico-pathological syndrome, as well as an incidental finding on histological examination, although there are obvious differences between these two scenarios. According to the World Health Organization, the definition of DIPNECH is purely histological. However, DIPNECH encompasses symptomatic patients with airway disease, as well as asymptomatic patients with Neuroendocrine Cell hyperplasia associated with multiple tumourlets/carcinoid tumours. DIPNECH is also considered a pre-neoplastic lesion in the spectrum of pulmonary Neuroendocrine tumours, because it is commonly found in patients with peripheral carcinoid tumours. In this review, we summarise clinical, physiological, radiological and histological features of DIPNECH and critically discuss recently proposed diagnostic criteria. In addition, we propose that the term "DIPNECH syndrome" be used to indicate a sufficiently distinct patient subgroup characterised by respiratory symptoms, airflow obstruction, mosaic attenuation with air trapping on chest imaging and constrictive obliterative bronchiolitis, often with nodular proliferation of Neuroendocrine Cells with/without tumourlets/carcinoid tumours on histology. Surgical lung biopsy is the diagnostic gold standard. However, in the appropriate clinical and radiological setting, transbronchial lung biopsy may also allow a confident diagnosis of DIPNECH syndrome.
Gunter Kloppel - One of the best experts on this subject based on the ideXlab platform.
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the gastroenteropancreatic Neuroendocrine Cell system and its tumors the who classification
Annals of the New York Academy of Sciences, 2004Co-Authors: Gunter Kloppel, Aurel Perren, Philipp U HeitzAbstract:Although well established in medical terminology, the term carcinoid is no longer adequate to cover the entire morphological and biological spectrum of neoplasms of the disseminated Neuroendocrine Cell system. Therefore, instead of carcinoid, the WHO classification published in 2000 uses the general terms Neuroendocrine tumor and Neuroendocrine carcinoma. In this review a classification of gastroenteropancreatic Neuroendocrine tumors based on the WHO criteria is described. We also classify and comment on the most important tumor entities. On the basis of localization and of various morphological and biological criteria, we distinguish between benign Neuroendocrine tumors, tumors with uncertain malignant potential, and tumors showing low-grade and high-grade malignancy.
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pathology and nomenclature of human gastrointestinal Neuroendocrine carcinoid tumors and related lesions
World Journal of Surgery, 1996Co-Authors: Gunter Kloppel, Philipp U Heitz, Carlo Capella, E SolciaAbstract:: The pathology and nomenclature of the Neuroendocrine Cell proliferations in the gut are reviewed. The neoplastic lesions are discussed within the light of a new classification system that attempts to consider the morphologic, functional, and biologic features of the tumors.
Marcello Tiseo - One of the best experts on this subject based on the ideXlab platform.
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diffuse idiopathic pulmonary Neuroendocrine Cell hyperplasia dipnech syndrome and carcinoid tumors with without nech a clinicopathologic radiologic and immunomolecular comparison study
The American Journal of Surgical Pathology, 2018Co-Authors: Mc Mengoli, Em Silini, Giulio Rossi, Alberto Cavazza, Renato Franco, Federica Zito Marino, Mario Migaldi, Letizia Gnetti, Luca Ampollini, Marcello TiseoAbstract:The diagnostic criteria of diffuse idiopathic pulmonary Neuroendocrine Cell hyperplasia (DIPNECH) are not well defined, and DIPNECH can be mistaken for carcinoids associated with Neuroendocrine Cell hyperplasia (NECH). In this study, we compared clinical, radiologic, histologic, immunohistochemical,
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diffuse idiopathic pulmonary Neuroendocrine Cell hyperplasia dipnech syndrome and carcinoid tumors with without nech a clinicopathologic radiologic and immunomolecular comparison study
The American Journal of Surgical Pathology, 2018Co-Authors: Mc Mengoli, Em Silini, Giulio Rossi, Alberto Cavazza, Renato Franco, Federica Zito Marino, Mario Migaldi, Letizia Gnetti, Luca Ampollini, Marcello TiseoAbstract:The diagnostic criteria of diffuse idiopathic pulmonary Neuroendocrine Cell hyperplasia (DIPNECH) are not well defined, and DIPNECH can be mistaken for carcinoids associated with Neuroendocrine Cell hyperplasia (NECH). In this study, we compared clinical, radiologic, histologic, immunohistochemical, and molecular features of DIPNECH and isolated carcinoids with/without NECH. The study population included 151 cases (77 female patients and 74 male patients), 19 with DIPNECH and 132 with carcinoids with/without NECH. None of the cases displayed molecular alterations or anaplastic lymphoma kinase expression. Compared with individuals with carcinoids with/without NECH, patients with DIPNECH were more likely to be female individuals (P<0.0001), nonsmokers (P=0.021), and symptomatic, and to have an obstructive/mixed respiratory defect, peripheral location of the lesions, and air trapping (P<0.0001) on chest computed tomography, and constrictive bronchiolitis on histology (P<0.0001). Among immunohistochemical markers, DIPNECH was associated with higher expression of thyroid transcription factor-1, CD10, and gastrin-releasing peptide/bombesin-like peptide (P<0.0001). Yet, when a purely histopathologic definition of DIPNECH was applied, 40% of isolated carcinoids also met the diagnostic criteria for DIPNECH, even in the absence of symptoms and/or radiologic abnormalities. Therefore, as DIPNECH represents a distinct clinical syndrome, we suggest the term DIPNECH be limited to cases presenting with respiratory symptoms, functional and/or radiologic abnormalities, and constrictive bronchiolitis on histology.
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Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) Syndrome and Carcinoid Tumors With/Without NECH: A Clinicopathologic, Radiologic, and Immunomolecular Comparison Study
American Journal of Surgical Pathology, 2018Co-Authors: Mc Mengoli, Fz Marino, Em Silini, Giulio Rossi, Alberto Cavazza, Renato Franco, Mario Migaldi, Letizia Gnetti, Luca Ampollini, Marcello TiseoAbstract:The diagnostic criteria of diffuse idiopathic pulmonary Neuroendocrine Cell hyperplasia (DIPNECH) are not well defined, and DIPNECH can be mistaken for carcinoids associated with Neuroendocrine Cell hyperplasia (NECH). In this study, we compared clinical, radiologic, histologic, immunohistochemical, and molecular features of DIPNECH and isolated carcinoids with/without NECH. The study population included 151 cases (77 female patients and 74 male patients), 19 with DIPNECH and 132 with carcinoids with/without NECH. None of the cases displayed molecular alterations or anaplastic lymphoma kinase expression. Compared with individuals with carcinoids with/without NECH, patients with DIPNECH were more likely to be female individuals (P\\textless0.0001), nonsmokers (P=0.021), and symptomatic, and to have an obstructive/mixed respiratory defect, peripheral location of the lesions, and air trapping (P\\textless0.0001) on chest computed tomography, and constrictive bronchiolitis on histology (P\\textless0.0001). Among immunohistochemical markers, DIPNECH was associated with higher expression of thyroid transcription factor-1, CD10, and gastrin-releasing peptide/bombesin-like peptide (P\\textless0.0001). Yet, when a purely histopathologic definition of DIPNECH was applied, 40% of isolated carcinoids also met the diagnostic criteria for DIPNECH, even in the absence of symptoms and/or radiologic abnormalities. Therefore, as DIPNECH represents a distinct clinical syndrome, we suggest the term DIPNECH be limited to cases presenting with respiratory symptoms, functional and/or radiologic abnormalities, and constrictive bronchiolitis on histology.