L-arginine and no levels are diminished in Children of African Descent with Acute Vaso-Occlusive Sickle Cell Crisis in Sokoto, Nigeria
*Erhabor Osaro Department Of Haematology And Blood Transfusion Science, Usmanu Danfodiyo University, Nigeria
*Corresponding Author: Erhabor Osaro
Department Of Haematology And Blood Transfusion Science, Usmanu Danfodiyo University, Nigeria Email:email@example.com
Published on: 2018-11-21
Sickle cell disease is an autosomal recessive disorder and the most common genetic disease affecting Africans. Alterations in nitric oxide production may have an important role in the pathophysiology of SCD. The aim of this study was to evaluate L-arginine and nitric oxide levels among children of African descent presenting to the Children Emergency Unit of Usmanu Danfodiyo University Sokoto with sickle cell disease (SCD). Plasma levels of L- arginine and Nitric Oxide (NO) were measured among 90 children aged 1-6 years and mean age 4.01 ± 0.87 years with sickle cell disease and 50 apparently healthy age-matched controls. The NO and L-arginine levels were significantly higher among normal control children compared to the SCD children (p=0.05). The study subjects were also classified based on the presence or absence of vaso-occlusive crisis (VOC) at the time of sample collection. Plasma L-arginine and serum NO levels were examined among 43 children with SCD with episodes of VOC and 47 age -matched children with SCD at steady-state. L-arginine levels was lower among SCD children with VOC (40.7 ± 3.77) compared to those in steady state (48.92± 6.10) (p=0.05). Similarly, the NO levels were significantly decreased among SCD children with VOC (94.80 ± 14.50) compared to those in the steady state (425.70 ± 15.05) (p=0.01). The findings from this study suggest that there may be a relationship between the L-arginine-nitric oxide pathway and vaso-occlusive crisis in SCD. Low arginine levels during VOC may be a reflection of acute L-arginine substrate depletion that may have resulted in decreased nitric oxide production. It may be necessary to routinely monitor the L-arginine and NO particularly among children presenting with vaso-occlusive crisis. Finding from this study may be a justification for L-arginine supplementation of children of African descent with SCD.
Sickle cell disease (SCD) is the most common and clinically haemoglobinopathy particularly among children of African descent. It is a collective name for a group of conditions causing clinical symptoms that result from the formation of sickle red cells. Normal red blood cells are biconcave discs. Sickle shaped red cells have a characteristic crescent shape. It results from a substitution of a single amino acid called glutamic acid by valine at position 6 of the beta globin polypeptide chain. It is inherited as an autosomal recessive trait.