Dataset Information

Neuroendocrine Cell Hyperplasia of Infancy. Clinical Score and Comorbidities.

ABSTRACT: Rationale: Neuroendocrine cell hyperplasia of infancy (NEHI) is an important form of children's interstitial and diffuse lung disease for which the diagnostic strategy has evolved. The prevalence of comorbidities in NEHI that may influence treatment has not been previously assessed.Objectives: To evaluate a previously unpublished NEHI clinical score for assistance in diagnosis of NEHI and to assess comorbidities in NEHI.Methods: We performed a retrospective chart review of 199 deidentified patients with NEHI from 11 centers. Data were collected in a centralized Research Electronic Data Capture registry and we performed descriptive statistics.Results: The majority of patients with NEHI were male (66%). The sensitivity of the NEHI Clinical Score was 87% (95% confidence interval [CI], 0.82-0.91) for all patients from included centers and 93% (95% CI, 0.86-0.97) for those with complete scores (e.g., no missing data). Findings were similar when we limited the population to the 75 patients diagnosed by lung biopsy (87%; 95% CI, 0.77-0.93). Of those patients evaluated for comorbidities, 51% had gastroesophageal reflux, 35% had aspiration or were at risk for aspiration, and 17% had evidence of immune system abnormalities.Conclusions: The NEHI Clinical Score is a sensitive tool for clinically evaluating NEHI; however, its specificity has not yet been addressed. Clinicians should consider evaluating patients with NEHI for comorbidities, including gastroesophageal reflux, aspiration, and immune system abnormalities, because these can contribute to the child's clinical picture and may influence clinical course and treatment.

SUBMITTER: Liptzin DR

PROVIDER: S-EPMC7258416 | biostudies-literature | 2020 Jun

REPOSITORIES: biostudies-literature

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Neuroendocrine Cell Hyperplasia of Infancy. Clinical Score and Comorbidities.

Liptzin Deborah R DR Pickett Kaci K Brinton John T JT Agarwal Amit A Fishman Martha P MP Casey Alicia A Towe Christopher T CT Taylor Jane B JB Kurland Geoffrey G Hagood James S JS Wambach Jennifer J Srivastava Ruma R Al-Saleh Hani H Dell Sharon D SD Young Lisa R LR Deterding Robin R RR

Annals of the American Thoracic Society 20200601 6

Rationale: Neuroendocrine cell hyperplasia of infancy (NEHI) is an important form of children's interstitial and diffuse lung disease for which the diagnostic strategy has evolved. The prevalence of comorbidities in NEHI that may influence treatment has not been previously assessed.Objectives: To evaluate a previously unpublished NEHI clinical score for assistance in diagnosis of NEHI and to assess comorbidities in NEHI.Methods: We performed a retrospective chart review of 1 ...[more]

PMID: 32109152

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Project description:IntroductionDeformational plagiocephaly (DP) is one of the most common cranial shape disorders in infancy. It is characterised by unilateral flattening of the skull due to head preference to one side. The literature suggests that DP is associated with comorbidities such as developmental delay, but the nature and prevalence of the comorbid impairments are still unclear and controversial. Therefore, our scoping review (ScR) aims to explore systematically the extent and nature of literature by identifying, mapping and categorising the most relevant comorbidities of DP in children up to the age of 2 years.Methods and analysisThis protocol is based on the framework outlined by Arksey and O'Malley. A systematic search will be conducted to identify relevant full text studies from 1992 to 2021 using the databases of Cochrane, MEDLINE, Google Scholar, EMBASE, PubMed and University of Nicosia EBSCO. Two independent reviewers will screen abstracts and full articles in parallel, using specific inclusion and exclusion criteria. Specifically, this review will consider studies investigating DP and relevant comorbidities in children up to the age of 2 years of life without craniosynostosis, torticollis and any other diagnosed neurodevelopmental deficiency.The Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for ScR Checklist will be considered for results' analysis and reporting. The results will be described in a narrative form in relation to the research question and in the context of the overall study purpose.Ethics and disseminationResearch ethics approval is not required for this ScR since data will be retrieved from publicly available studies. Dissemination activities will include research findings' submission for publication in a relevant peer-reviewed journal and presentation of the results at relevant conferences.RegistrationOur protocol was registered prospectively with the Open Science Framework (https://osf.io/48am3/).

Project description:BackgroundSeveral risk factors predict clinical outcome in gastro-entero-pancreatic neuroendocrine neoplasms (GEP-NENs); however, the impact of their combination has not been investigated so far.Patients and methodsA retrospective analysis of stage IV GEP-NENs was performed. Multivariate analysis for progression of disease (PD) was performed by Cox proportional hazards method to obtain a risk score. Area under the curve obtained by receiver operating characteristic analysis was used to assess the score performance. Progression-free survival analysis was performed by Kaplan-Meier method.ResultsTwo hundred eighty-three stage IV GEP-NENs were evaluated, including 93 grade 1 neuroendocrine tumors (32.9%), 153 grade 2 neuroendocrine tumors (54%), and 37 grade 3 neuroendocrine carcinomas (13.1%). Independent risk factors for PD were Ki67, proportion of metastatic liver involvement, and presence of extra-abdominal metastases. The risk score was calculated as follows: (0.025 × Ki67) + [(0 if no liver metastases or liver involvement <25%) OR (0.405 if liver involvement 25%-50%) OR (0.462 if liver involvement >50%)] + [(0 if no extra-abdominal metastases) OR (0.528 if extra-abdominal metastases present)]. The risk score accuracy to predict PD was superior compared with the G grading system (area under the curve: 0.705 and 0.622, respectively). Three subgroups of patients with low, intermediate, and high risk of PD according to risk score were identified, median progression-free survival being 26 months, 19 months, and 12 months, respectively.ConclusionIn stage IV GEP-NENs, a risk score able to predict PD was obtained by combining Ki67, proportion of metastatic liver involvement, and presence of extra-abdominal metastases. The score may help to discriminate patients with different progression risk level to plan tailored therapeutic approaches and follow-up programs. The Oncologist 2017;22:409-415Implications for Practice: Clinical outcome of patients with advanced gastro-entero-pancreatic neuroendocrine neoplasms is affected by several risk factors, including the proliferative index Ki67, extension of liver metastases, and the presence of distant extra-abdominal lesions. A risk score that combines these variables may help physicians dealing with these diseases to plan the optimal therapeutic approach and follow-up program.

Dataset Information

Neuroendocrine Cell Hyperplasia of Infancy. Clinical Score and Comorbidities.

Publications

Neuroendocrine Cell Hyperplasia of Infancy. Clinical Score and Comorbidities.

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