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Most individuals with G6PD deficiency are asymptomatic.When it induces hemolysis, the effect is usually short-lived. [5]Most people who develop symptoms are male, due to the X-linked pattern of inheritance, but female carriers can be affected due to unfavorable lyonization or skewed X-inactivation, where random inactivation of an X-chromosome in certain cells creates a population of G6PD ...
The most important measure taken for treatment of 6-phosphoglucanate dehydrogenase is prevention. Avoidance of chemical exposures to drugs and foods that have the potential to cause hemolysis. Although some foods and supplements have antioxidant properties, their use does not decrease the severity of G6PD deficiency. [citation needed]
Those with a deficiency in antioxidant mechanisms such as those with G6PD deficiency or Hemoglobin H disease are particularly susceptible to oxidative injury from drugs. In G6PD deficiency, there is a deficiency of the glucose-6-phosphate dehydrogenase enzyme, which plays a crucial role in the generation of the key antioxidant [[Nicotinamide ...
Isobutyryl-coenzyme A dehydrogenase deficiency is a rare metabolic disorder in which the body is unable to process certain amino acids properly. [1]People with this disorder have inadequate levels of an enzyme that helps break down the amino acid valine, resulting in a buildup of valine in the urine, a symptom called valinuria.
Very long-chain acyl-coenzyme A dehydrogenase deficiency is a fatty-acid metabolism disorder which prevents the body from converting certain fats to energy, particularly during periods without food. [1] [2] [3] Those affected by this disorder have inadequate levels of an enzyme that breaks down a group of fats called very long-chain fatty acids.
Vitamin B 12 deficiency Pernicious anemia; Folate deficiency; Hemolytic anemias (destruction of red blood cells) Genetic disorders of RBC membrane Hereditary spherocytosis; Hereditary elliptocytosis; Congenital dyserythropoietic anemia; Genetic disorders of RBC metabolism Glucose-6-phosphate dehydrogenase deficiency (G6PD) Pyruvate kinase ...
In some cases, a drug can cause the immune system to mistakenly think the body's own red blood cells are dangerous, foreign substances. Antibodies then develop against the red blood cells. The antibodies attach to red blood cells and cause them to break down too early. It is known that more than 150 drugs can cause this type of hemolytic anemia ...
Patients must be monitored regularly for blood lactate levels, transaminase and plasma ketone levels. [18] There is some evidence that dichloroacetate reduces the inhibitory phosphorylation of pyruvate dehydrogenase complex and thereby activates any residual functioning complex. Resolution of lactic acidosis is observed in patients with E1 ...