An overriding aorta is a congenital heart defect where the aorta is positioned directly over a ventricular septal defect (VSD), instead of over the left ventricle. The result is that the aorta receives some blood from the right ventricle, causing mixing of oxygenated and deoxygenated blood, and thereby reducing the amount of oxygen delivered to the tissues. It is one of the four findings in the classic tetralogy of Fallot. The other three findings are right ventricular outflow tract (RVOT) obstruction (most often subpulmonary stenosis), right ventricular hypertrophy (RVH), and ventricular septal defect (VSD). References


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Mild glutathione synthetase deficiency usually results in the destruction of red blood cells (hemolytic anemia). Rarely, affected people also excrete large amounts of a compound called 5-oxoproline (also called pyroglutamic acid, or pyroglutamate) in their urine (5-oxoprolinuria). This compound builds up when glutathione is not processed correctly in cells. Individuals with moderate glutathione synthetase deficiency may experience symptoms beginning shortly after birth including hemolytic anemia, 5-oxoprolinuria, and elevated acidity in the blood and tissues (metabolic acidosis). In addition to the features present in moderate glutathione synthetase deficiency, individuals affected by the severe form of this disorder may experience neurological symptoms. These problems may include seizures; a generalized slowing down of physical reactions, movements, and speech (psychomotor retardation); intellectual disability; and a loss of coordination (ataxia). Some people with severe glutathione synthetase deficiency also develop recurrent bacterial infections. Treatment
As of 2018, there is no cure for GSD, and treatment is restricted to manage symptoms and associated problems. Thus, sodium bicarbonate is recommended to treat metabolic acidosis, and antioxidants, among them vitamins E and C, can reduce oxidative damage. References
Further reading
Glutathione synthetase deficiency at NLM Genetics Home Reference
Beutler, E; Gelbart, T; Pegelow, C (1986). "Erythrocyte glutathione synthetase deficiency leads not only to glutathione but also to glutathione-S-transferase deficiency". Journal of Clinical Investigation. 77 (1): 38–41. doi:10.1172/JCI112298. PMC 423305. PMID 3944259. == External links ==