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Prognostic criteria and management of sickle cell disease

Thesis

Last updated: 06 Feb 2023

Subjects

-

Tags

Pediatrics

Advisors

El-Beshlawi, Amal , Abdel-Raouf, Eiman , Bazaraa, Hafezh

Authors

Magdi, Ranya Esmaeil

Accessioned

2017-03-30 06:21:20

Available

2017-03-30 06:21:20

type

M.Sc. Thesis

Abstract

Sickle cell disease is a common hemoglobinopathy characterized by an autosomal co-dominant mutation in the B-globin gene in which a valine residue replaces a glutamic acid in number 6 position to form HbS (Perkins et al., 2001). Sickle cell disease is most common among people living in or originating from subsaharan africa,(Serjeant,. 1992). Sickle cell trait affects about 10-30% of Africa's tropical populations, (Ohene-Frempong & Nkrumah ,. 1994).Sickle cell disease affects an estimated 1-2%(120 000) of newborns in Africa, and about 60000 people in the USA(Hickman et al 1999). The term sickle cell disease refers to clinically and hematological significant sickling disorders, including sickle cell anemia(Hbss) and others as Hbs-b thalassemia, HbSC, HbSD Los Angeles, HbSO Arab and HbSE. Most of these produce less severe manifestations than HbS. The presenting symptoms of SCD involve: acute events e.g acute splenic sequestration, aplastic crisis, dactylitis (hand foot syndrome), acute chest syndrome, stroke, priapism and the most common clinical picture painful vaso-occlusive crisis. Chronic sequale e.g chronic anemia, chronic hypersplenism, renal failure, gallstones, avascular necrosis of the femoral head. (Serjeant. et al.,1999). Severe bacteria infections such as pneumonia, meningitis and septicaernia are common causes of morbidity and mortality, (Overtuff et al., 1977). Factors that precipitate or modulate the occurrence of sickle cell crisis are not fully understood, but infections, hypoxia, dehydration, acidosis, stress and cold are believed to play some role. (Ohene-Frempong & Nkrumah .1994). Different lines of treatment of sickle cell disease include:Blood transfusion, proper hydration, analgesics, pain control, monthly injection of depot penicillin, pneumococcal vaccine, Hib vaccine, exchange transfusion, oxygen in acute chest syndrome. New approaches to treatment include Hydroxyurea and BMT. About 10% of children with sickle cell anemia may develop a stroke, and more than half of these may suffer recurrent strokes(Cohen et al., 1996). Frequent episodes of crisis, infections, and organ damage reduce the quality of life of people with sickle cell disease. Life expectancy remains low, especially in communities with poor access to health services. In some parts of Africa, about 50% of children with sickle cell disease die before their first birthday(Ohene-Frempong & Nkrumah., 1994).The average life expectancy for men and women with sickle cell disease is about 42 and 48 years , respectively, (Platt et al 1994) . Objective of the thesis is to review the prognostic criteria and different lines of management of sickle cell disease.

Issued

1 Jan 2005

Details

Type

Thesis

Created At

05 Feb 2023