General Medicine

Pathophysiology

• Enzyme defect: Glucose-6-phosphate dehydrogenase deficiency
• Result: Impaired NADPH production → reduced glutathione → oxidative damage
• Inheritance: X-linked recessive (affects males predominantly)

Key Clinical Features

• Heinz bodies: Characteristic finding (precipitated denatured hemoglobin)
• Hemolytic episodes: Triggered by oxidative stress
• Chronic hemolysis: Usually absent between episodes
• Neonatal jaundice: May be severe

Important Clinical Points

• Estrogen does NOT induce hemolysis (unlike other conditions)
• Favism: Hemolysis after eating fava beans
• Drug-induced: Most common trigger for hemolytic crisis

Oxidative Stressors to Avoid

Medications

• Antimalarials: Primaquine, chloroquine
• Antibiotics: Sulfonamides, nitrofurantoin, chloramphenicol
• Analgesics: Aspirin (high doses), phenazopyridine
• Others: Methylene blue, naphthalene (mothballs)

Infections

• Bacterial infections
• Viral hepatitis
• Pneumonia
• Typhoid fever

Other Triggers

• Fava beans (favism)
• Diabetic ketoacidosis
• Severe illness/stress