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Chapter-071 Orthopedic Manifestations of Sickle Cell Hemoglobinopathy

BOOK TITLE: Textbook of Orthopedics & Trauma (4 Volumes)

Author
1. Babhulkar SS
ISBN
9789385891052
DOI
10.5005/jp/books/12869_72
Edition
3/e
Publishing Year
2016
Pages
6
Author Affiliations
1. Sushrut Hospital and Research Centre, Ramdaspeth, Nagpur, Sushrut Hospital Research Centre and Postgraduate Institute of Orthopedics, Nagpur, Maharashtra, India; Trauma Society of India; Centre for Joint Reconstruction and Trauma Surgery
Chapter keywords
orthopedic, sickle cell hemoglobinopathy, hemoglobin, hypoxia, acidosis, anoxia, musculoskeletal, hematology, drug therapy

Abstract

This chapter discusses orthopedic manifestations of sickle cell hemoglobinopathy. Sickle cell hemoglobinopathy is a common genetic disorder with a typical racial and topographic distribution. Skeletal manifestation is one of the presentations of this multisystem disorder. Sickle cell disease is a hereditary familial chronic disease which varies widely in its clinical manifestations from mild symptoms and normal life span to early onset of severe symptoms and consequently reduced life expectancy. Musculoskeletal pain prompts the patients of sickle cell disease to seek orthopedic assistance. Clinical features, diagnosis, and treatment of sickle cell hemoglobinopathy are also discussed in this chapter. The aim of drug therapy is prevent stress conditions. The patient is given adequate doses of buffer solution, soda bicarbonate, zinc and folic acid. Hypoxia, acidosis, and dehydration are avoided. Genetic Counseling is important for a patient in particular and the Sickler community at larger. Hemoglobin electrophoresis helps in differentiating patients with sickle cell disease and trait.

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