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...
Saved in:
| Main Authors: | , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
PAGEPress Publications
2022-01-01
|
| Series: | Mediterranean Journal of Hematology and Infectious Diseases |
| Subjects: | |
| Online Access: | https://mjhid.org/index.php/mjhid/article/view/4781 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1846098513916592128 |
|---|---|
| author | Ilaria Lazzareschi Elena Rossi Antonietta Curatola Giovanna Capozio Luca Benacquista Ludovica Iezzi Donato Rigante |
| author_facet | Ilaria Lazzareschi Elena Rossi Antonietta Curatola Giovanna Capozio Luca Benacquista Ludovica Iezzi Donato Rigante |
| author_sort | Ilaria Lazzareschi |
| collection | DOAJ |
| 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 of the neutrophil count and periodically-recurring infections every 21 days. Diagnosis of these disorders is frequently obscured by the multiple causes of recurrent fevers in children. 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 such patients. |
| format | Article |
| id | doaj-art-c15558cc49a640c7a126143e3d2a0f23 |
| institution | Kabale University |
| issn | 2035-3006 |
| language | English |
| publishDate | 2022-01-01 |
| publisher | PAGEPress Publications |
| record_format | Article |
| series | Mediterranean Journal of Hematology and Infectious Diseases |
| spelling | doaj-art-c15558cc49a640c7a126143e3d2a0f232025-01-02T00:23:27ZengPAGEPress PublicationsMediterranean Journal of Hematology and Infectious Diseases2035-30062022-01-0114110.4084/MJHID.2022.008ASSESSMENT OF CONGENITAL NEUTROPENIA IN CHILDREN: COMMON CLINICAL SCENERIES AND CLUES FOR MANAGEMENTIlaria Lazzareschi0Elena Rossi1Antonietta Curatola2Giovanna Capozio3Luca Benacquista4Ludovica Iezzi5Donato Rigante6Department of Life Sciences and Global Health, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy. Università Cattolica Sacro Cuore, Rome, ItalyDiagnostic Imaging, Oncological Radiotherapy and Hematology Department, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy. Università Cattolica Sacro Cuore, Rome, ItalyDepartment of Life Sciences and Global Health, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy. Department of Life Sciences and Global Health, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy. Department of Life Sciences and Global Health, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy. Department of Life Sciences and Global Health, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy. Department of Life Sciences and Global Health, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy. Università Cattolica Sacro Cuore, Rome, Italy. 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 of the neutrophil count and periodically-recurring infections every 21 days. Diagnosis of these disorders is frequently obscured by the multiple causes of recurrent fevers in children. 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 such patients.https://mjhid.org/index.php/mjhid/article/view/4781Congenital neutropeniacyclic neutropeniamyelopoiesisneutrophil elastaseperiodic feverautoinflammation |
| spellingShingle | Ilaria Lazzareschi Elena Rossi Antonietta Curatola Giovanna Capozio Luca Benacquista Ludovica Iezzi Donato Rigante ASSESSMENT OF CONGENITAL NEUTROPENIA IN CHILDREN: COMMON CLINICAL SCENERIES AND CLUES FOR MANAGEMENT Mediterranean Journal of Hematology and Infectious Diseases Congenital neutropenia cyclic neutropenia myelopoiesis neutrophil elastase periodic fever autoinflammation |
| 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 | Congenital neutropenia cyclic neutropenia myelopoiesis neutrophil elastase periodic fever autoinflammation |
| url | https://mjhid.org/index.php/mjhid/article/view/4781 |
| work_keys_str_mv | AT ilarialazzareschi assessmentofcongenitalneutropeniainchildrencommonclinicalsceneriesandcluesformanagement AT elenarossi assessmentofcongenitalneutropeniainchildrencommonclinicalsceneriesandcluesformanagement AT antoniettacuratola assessmentofcongenitalneutropeniainchildrencommonclinicalsceneriesandcluesformanagement AT giovannacapozio assessmentofcongenitalneutropeniainchildrencommonclinicalsceneriesandcluesformanagement AT lucabenacquista assessmentofcongenitalneutropeniainchildrencommonclinicalsceneriesandcluesformanagement AT ludovicaiezzi assessmentofcongenitalneutropeniainchildrencommonclinicalsceneriesandcluesformanagement AT donatorigante assessmentofcongenitalneutropeniainchildrencommonclinicalsceneriesandcluesformanagement |