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Assessment of Congenital Neutropenia in Children: Common Clinical Sceneries and Clues for Management
A disparate group of rare hematological diseases characterized by impaired maturation of neutrophil granulocytes defines congenital neutropenias. Neutropenic patients are prone to recurrent infections beginning in the first months of life. Of interest is “cyclic neutropenia,” an ultra-rare disorder...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Università Cattolica del Sacro Cuore
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8747088/ https://www.ncbi.nlm.nih.gov/pubmed/35070215 http://dx.doi.org/10.4084/MJHID.2022.008 |
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author | Lazzareschi, Ilaria Rossi, Elena Curatola, Antonietta Capozio, Giovanna Benacquista, Luca Iezzi, Ludovica Rigante, Donato |
author_facet | Lazzareschi, Ilaria Rossi, Elena Curatola, Antonietta Capozio, Giovanna Benacquista, Luca Iezzi, Ludovica Rigante, Donato |
author_sort | Lazzareschi, Ilaria |
collection | PubMed |
description | A disparate group of rare hematological diseases characterized by impaired maturation of neutrophil granulocytes defines congenital neutropenias. Neutropenic patients are prone to recurrent infections beginning in the first months of life. Of interest is “cyclic neutropenia,” an ultra-rare disorder revealed by sinusoidal variations in the neutrophil count and recurring infections every 21 days. Diagnosis of these disorders is frequently obscured by the multiple causes of recurrent fevers in children. The aim of this overview is to outline the physical assessment of children presenting with early-onset symptomatic neutropenia, identify the disease between the many medical conditions and even emergencies which should enter in differential diagnosis, hint at the potential management with granulocyte-colony stimulating factor, define the risk of evolution to hematologic malignancy, and summarize inter-professional team strategies for improving care coordination and outcomes of patients. |
format | Online Article Text |
id | pubmed-8747088 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Università Cattolica del Sacro Cuore |
record_format | MEDLINE/PubMed |
spelling | pubmed-87470882022-01-21 Assessment of Congenital Neutropenia in Children: Common Clinical Sceneries and Clues for Management Lazzareschi, Ilaria Rossi, Elena Curatola, Antonietta Capozio, Giovanna Benacquista, Luca Iezzi, Ludovica Rigante, Donato Mediterr J Hematol Infect Dis Review Article A disparate group of rare hematological diseases characterized by impaired maturation of neutrophil granulocytes defines congenital neutropenias. Neutropenic patients are prone to recurrent infections beginning in the first months of life. Of interest is “cyclic neutropenia,” an ultra-rare disorder revealed by sinusoidal variations in the neutrophil count and recurring infections every 21 days. Diagnosis of these disorders is frequently obscured by the multiple causes of recurrent fevers in children. The aim of this overview is to outline the physical assessment of children presenting with early-onset symptomatic neutropenia, identify the disease between the many medical conditions and even emergencies which should enter in differential diagnosis, hint at the potential management with granulocyte-colony stimulating factor, define the risk of evolution to hematologic malignancy, and summarize inter-professional team strategies for improving care coordination and outcomes of patients. Università Cattolica del Sacro Cuore 2022-01-01 /pmc/articles/PMC8747088/ /pubmed/35070215 http://dx.doi.org/10.4084/MJHID.2022.008 Text en https://creativecommons.org/licenses/by-nc/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by-nc/4.0 (https://creativecommons.org/licenses/by-nc/4.0/) ), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Review Article Lazzareschi, Ilaria Rossi, Elena Curatola, Antonietta Capozio, Giovanna Benacquista, Luca Iezzi, Ludovica Rigante, Donato Assessment of Congenital Neutropenia in Children: Common Clinical Sceneries and Clues for Management |
title | Assessment of Congenital Neutropenia in Children: Common Clinical Sceneries and Clues for Management |
title_full | Assessment of Congenital Neutropenia in Children: Common Clinical Sceneries and Clues for Management |
title_fullStr | Assessment of Congenital Neutropenia in Children: Common Clinical Sceneries and Clues for Management |
title_full_unstemmed | Assessment of Congenital Neutropenia in Children: Common Clinical Sceneries and Clues for Management |
title_short | Assessment of Congenital Neutropenia in Children: Common Clinical Sceneries and Clues for Management |
title_sort | assessment of congenital neutropenia in children: common clinical sceneries and clues for management |
topic | Review Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8747088/ https://www.ncbi.nlm.nih.gov/pubmed/35070215 http://dx.doi.org/10.4084/MJHID.2022.008 |
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