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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...

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Autores principales: Lazzareschi, Ilaria, Rossi, Elena, Curatola, Antonietta, Capozio, Giovanna, Benacquista, Luca, Iezzi, Ludovica, Rigante, Donato
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Università Cattolica del Sacro Cuore 2022
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.
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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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