Sickle Cell Disease

A special issue of Medical Sciences (ISSN 2076-3271).

Deadline for manuscript submissions: 31 December 2026 | Viewed by 1355

Editor


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Guest Editor
Department of Pediatrics, The University of Tennessee Health Science Center, Memphis, TN 38163, USA
Interests: spectrin membrane skeleton; sickle cell disease; cell and molecular biology; proteomics

Special Issue Information

Dear Colleagues,

Sickle cell disease (SCD) is known as the first "molecular disease" based on Linus Pauling's 1949 demonstration of an abnormal hemoglobin protein. Currently, FDA-approved pharmacological treatments and gene editing therapies are utilized to improve the health and quality of life of patients. This Special Issue will include manuscripts on all translational stages of SCD research, covering, but not limited to, the following topics: the basic science that led to an understanding of how the defective beta globin molecule results in sickling, pain and organ damage; the use of omics to identify biomarkers of sickle cell severity; SCD animal models that provide a better understanding of the pathophysiology while allowing the preclinical testing of therapeutics; and the pharmacologic and gene therapy clinical trials that have provided newly approved treatments leading to improved health outcomes and cures for the first molecular disease.

Prof. Dr. Steven R. Goodman
Guest Editor

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Keywords

  • sickle cell disease
  • hemoglobin
  • vaso-occlusion
  • anemia
  • genomics
  • epigenomics
  • proteomics
  • metabolomics
  • gene therapy
  • clinical trials

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Published Papers (1 paper)

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Research

15 pages, 601 KB  
Article
Associations Between Sickle Cell Disease, Pica, and Enuresis in Pediatric Neurodevelopmental Disorders
by Kit Neikirk, Aliyah Allick, Christopher J. Gamper, Alicia D. Cannon, Wilfreda Lindsey, Bridget G. Gibbons and Eboni I. Lance
Med. Sci. 2026, 14(2), 186; https://doi.org/10.3390/medsci14020186 - 7 Apr 2026
Viewed by 874
Abstract
Background: Sickle cell disease (SCD) is a hereditary disorder affecting red blood cells’ shape and functional capacity. Individuals with SCD report relatively high co-occurrence of neurodevelopmental disorders (NDDs). In addition, these children also have higher rates of enuresis (incontinence) and pica, disorders [...] Read more.
Background: Sickle cell disease (SCD) is a hereditary disorder affecting red blood cells’ shape and functional capacity. Individuals with SCD report relatively high co-occurrence of neurodevelopmental disorders (NDDs). In addition, these children also have higher rates of enuresis (incontinence) and pica, disorders prevalent in children with developmental delays. Both enuresis and pica can have negative effects on mental health, but their pathophysiology, especially in SCD, remains unclear. Objectives: The objective of this study was to determine the rates of pica and enuresis in a pediatric SCD clinic to compare the co-occurrence of NDDs and enuresis/pica. Methods: To do so, we performed a cross-sectional explanatory retrospective chart review of 275 pediatric SCD patients. Results: Our SCD cohort had a 27% prevalence of enuresis, 9% prevalence of pica, and 24% prevalence of one or more NDDs. We noted significant inter-group overlap between pica/enuresis and other risk SCD severity factors. NDDs were approximately twice as frequent in SCD patients with pica or enuresis compared to those without. While pica was associated with HbSβ+, it was not linked to disease severity indicators. Enuresis was associated with hydroxyurea usage (66.7% vs. 42.6%, p = 0.001) and reticulocyte counts, indicative of higher disease severity. Conclusions: Clinically, these results are the first to show co-occurrence between pica, enuresis, and NDDs in SCD. We suggest that the occurrence of pica or enuresis may serve as an indicator for previously unknown NDD risk. Together, these results underscore the need for targeted screenings of pica and enuresis in SCD populations. Full article
(This article belongs to the Special Issue Sickle Cell Disease)
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