الفهرس 
Sickle Cell AnemiaOnly 14 pages are availabe for public view
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Abstract
Sickle cell disease or sickle cell anemia is a life -long blood
disorder characterized by red blood cells that assume an abnormal, rigid,
sickle shape. Sickling decreases the cells’ flexibility and results in a risk
of various complications. The sickling occurs because of a mutation in
the hemoglobin gene.
Kynurenine is the main metabolite of tryptophan degradation. The
rate-limiting step in the kynurenine pathway is catalyzed by indoleamine
2,3- dioxygenases (IDO1 and IDO2) and tryptophan 2,3- dioxygenase
(TDO). TDO is known to be induced by Trp and steroids, whereas IDO is
induced by proinflammatory stimuli and cytokines like IFN-γ, tumor
necrosis factor (TNF)-α, interleukin (IL)-6. Hence, IDO is responsible for
the immune mediated Trp degradation. Kynurenine carry out diverse
biological functions, including dilating blood vessels during inflammation
and regulating the immune response.
This case control study was conducted on 40 children from
hematology unit, pediatric department, Menoufia University Hospital in
the age group of 2-18 years.40 children with sickle cell disease as a
patient group and 40 apparently healthy children, age, sex, and
socioeconomic standard matched as a control group. Full history taking
and complete clinical examination was done. Complete blood count
(CBC), serum ferriti, Hb electrophoresis, Serum Kynurenine
concentrations will be determined by commercial enzyme-linked
immunosorbent assay (ELISA) kits.
In the present study Consanguinity was significantly higher in
patients group than control but there no significant difference between
both groups regarding age, sex, height and BMI.
Also in our study Clinical variabilities as pallor, jaundice,
splenomegally and criseses presented in patients in different ratios,
patients had multiple episodes of pain yearly.patients presented in
different ratios regarding intake of hydroxyurea, its dose, iron
chelation,its dose and transfusion index per year.patient group was
subdivided to sickle cell anemia and sickle thalassemia by about 67,5%
and 32, 5%respectively.