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Children
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Sickle Cell Disease in Children
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Sickle Cell Disease in Children

A special issue of Children (ISSN 2227-9067). This special issue belongs to the section "Oncology and Hematology".

Deadline for manuscript submissions: closed (1 January 2021) | Viewed by 4115

Special Issue Editor

Dr. Berengere Koehl

E-Mail Website
Guest Editor
Department of Hematology, Reference Center for Sickle Cell Disease, Hôpital Robert Debré, 75019 Paris, France; University of Paris, INSERM U1134, Institut National de la Transfusion Sanguine, 75015 Paris, France
Interests: sickle cell disease; red blood cells disorders; inflammation and vascular disorders; transfusion; pediatrics; pain management; health educational programs

Special Issue Information

Dear Colleagues,

It is my pleasure to serve as the Guest Editor for this Special Issue of Children, entitled “Sickle Cell Disease in Children”. Sickle cell disease (SCD) is a widespread and fatal congenital hemoglobinopathy that strikes millions of children around the world and represents a heavy burden for many countries, not only in Africa but also in Europe and the Americas.

Nevertheless, SCD remains a disease that is still frequently unrecognized and has been neglected for too long by public health policies. This is now changing.

Indeed, enormous advances have been made over the last decade, both in understanding the physiopathology of the disease and in the development of new therapeutic options. Many innovative programs are being promoted around the world and at all levels of care: genetic counseling and neonatal screening, access to primary care, therapeutic education, new drug combinations, bone marrow transplants, and soon curative gene therapy. The future of a SCD-child born today will hopefully be much less grim than that of their predecessors.

The aim of this Special Issue is to share new knowledge in the pathophysiology of the disease and in global management of SCD children worldwide.

We invite researchers and physicians to contribute to this Special Issue in the format of original research articles, as well as review articles that will provide readers with an overview of SCD in its current and future management.

We look forward to your participation.

Dr. Berengere Koehl
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Children is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2400 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • sickle cell disease
  • neonatal screening
  • gene therapy
  • bone marrow transplantation
  • therapeutic education
  • pain management
  • therapeutic advances

Published Papers (2 papers)

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Research

10 pages, 1310 KiB  
Article
Variability of Prognostic Results Based on Biological Parameters in Sickle Cell Disease Cohort Studies in Children: What Should Clinicians Know?
by Julie Sommet, Enora Le Roux, Bérengère Koehl, Zinedine Haouari, Damir Mohamed, André Baruchel, Malika Benkerrou and Corinne Alberti
Children 2021, 8(2), 143; https://0-doi-org.brum.beds.ac.uk/10.3390/children8020143 - 13 Feb 2021
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Abstract
Background: Many pediatric studies describe the association between biological parameters (BP) and severity of sickle cell disease (SCD) using different methods to collect or to analyze BP. This article assesses the methods used for collection and subsequent statistical analysis of BP, and how [...] Read more.
Background: Many pediatric studies describe the association between biological parameters (BP) and severity of sickle cell disease (SCD) using different methods to collect or to analyze BP. This article assesses the methods used for collection and subsequent statistical analysis of BP, and how these impact prognostic results in SCD children cohort studies. Methods: Firstly, we identified the collection and statistical methods used in published SCD cohort studies. Secondly, these methods were applied to our cohort of 375 SCD children, to evaluate the association of BP with cerebral vasculopathy (CV). Results: In 16 cohort studies, BP were collected either once or several times during follow-up. The identified methods in the statistical analysis were: (1) one baseline value per patient (2) last known value; (3) mean of all values; (4) modelling of all values in a two-stage approach. Applying these four different statistical methods to our cohort, the results and interpretation of the association between BP and CV were different depending on the method used. Conclusion: The BP prognostic value depends on the chosen statistical analysis method. Appropriate statistical analyses of prognostic factors in cohort studies should be considered and should enable valuable and reproducible conclusions. Full article
(This article belongs to the Special Issue Sickle Cell Disease in Children)
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9 pages, 430 KiB  
Article
Neuropathic Pain in Children with Sickle Cell Disease: The Hidden Side of the Vaso-Occlusive Crisis
by Jeanne Sigalla, Nathalie Duparc Alegria, Enora Le Roux, Artemis Toumazi, Anne-Françoise Thiollier, Laurent Holvoet, Malika Benkerrou, Sophie Dugue and Berengere Koehl
Children 2021, 8(2), 84; https://0-doi-org.brum.beds.ac.uk/10.3390/children8020084 - 26 Jan 2021
Cited by 3 | Viewed by 2046
Abstract
The majority of hospitalizations of patients with sickle cell disease (SCD) are related to painful vaso-occlusive crises (VOCs). Although the pain of VOC is classically nociceptive, neuropathic pain (NP) has also been demonstrated in SCD patients. The aim of our study is to [...] Read more.
The majority of hospitalizations of patients with sickle cell disease (SCD) are related to painful vaso-occlusive crises (VOCs). Although the pain of VOC is classically nociceptive, neuropathic pain (NP) has also been demonstrated in SCD patients. The aim of our study is to specify the prevalence of NP during VOCs in SCD children using a dedicated scale and to measure its characteristics. We performed a prospective study that included SCD children hospitalized for an acute VOC. The presence of NP was sought with the DN4 scale on the second and fourth days of hospitalization. A total of 54 SCD children were included in the study. Overall, 41% of the patients (n = 22) experienced neuropathic pain during the VOC, mostly at an early stage (Day 2). The median age, the sex ratio, the location of the pain, and the morphine consumption were similar for patients with and without NP. Our study shows that neuropathic pain is very common during VOCs in SCD children. The absence of identified risk factors should prompt us to be vigilant regardless of the patient’s age, sex, and clinical presentation. Full article
(This article belongs to the Special Issue Sickle Cell Disease in Children)
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