About this item:

362 Views | 805 Downloads

Author Notes:

Corresponding Author: Samir K. Ballas Email: samir.ballas@jefferson.edu

Subjects:

Research Funding:

The authors greatly appreciate the creation of the figures by D. Scott McLeod, Wilmer Ophthalmological Institute, Johns Hopkins Hospital. Drs. Goldberg's and Lutty's recent research on sickle cell retinopathy was supported by NIH grant HL45922 (GL) and EY01765 (Wilmer) as well as unrestricted funds from Research to Prevent Blindness and the Reginald Lewis Foundation.

Keywords:

  • Science & Technology
  • Multidisciplinary Sciences
  • Science & Technology - Other Topics
  • MULTIDISCIPLINARY SCIENCES
  • ACUTE CHEST SYNDROME
  • ACUTE SPLENIC SEQUESTRATION
  • OPTICAL COHERENCE TOMOGRAPHY
  • ORBITAL COMPRESSION SYNDROME
  • RANDOMIZED CONTROLLED-TRIAL
  • PLACENTA GROWTH-FACTOR
  • BONE-MINERAL DENSITY
  • HEMOLYTIC TRANSFUSION REACTION
  • ACTIVITY LIMITATIONS INTERVIEW
  • ACUTE HEPATIC SEQUESTRATION

Beyond the Definitions of the Phenotypic Complications of Sickle Cell Disease: An Update on Management

Show all authors Show less authors

Tools:

Journal Title:

Scientific World Journal

Volume:

Volume 2012

Publisher:

, Pages 949535-949535

Type of Work:

Article | Final Publisher PDF

Abstract:

The sickle hemoglobin is an abnormal hemoglobin due to point mutation (GAG → GTG) in exon 1 of the globin gene resulting in the substitution of glutamic acid by valine at position 6 of the globin polypeptide chain. Although the molecular lesion is a single-point mutation, the sickle gene is pleiotropic in nature causing multiple phenotypic expressions that constitute the various complications of sickle cell disease in general and sickle cell anemia in particular. The disease itself is chronic in nature but many of its complications are acute such as the recurrent acute painful crises (its hallmark), acute chest syndrome, and priapism. These complications vary considerably among patients, in the same patient with time, among countries and with age and sex. To date, there is no well-established consensus among providers on the management of the complications of sickle cell disease due in part to lack of evidence and in part to differences in the experience of providers. It is the aim of this paper to review available current approaches to manage the major complications of sickle cell disease. We hope that this will establish another preliminary forum among providers that may eventually lead the way to better outcomes.

Copyright information:

© 2012 Samir K. Ballas et al.

This is an Open Access work distributed under the terms of the Creative Commons Attribution 3.0 Unported License (http://creativecommons.org/licenses/by/3.0/).

Creative Commons License

Export to EndNote