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Glucose-6-phosphate dehydrogenase (G6PD) deficiency - commonest red blood cell enzyme defect. 
Mediterranean and Africa people 
inherited in a X-linked recessive fashion. 
Many drugs can precipitate a crisis as well as infections and broad (fava) beans

''Pathophysiology''
    ↓ G6PD → ↓ glutathione → increased red cell susceptibility to oxidative stress

''Features''
    neonatal jaundice is often seen
    intravascular haemolysis
    gallstones are common
    splenomegaly may be present
    Heinz bodies on blood films

Diagnosis is made by using a G6PD enzyme assay

''drugs causing haemolysis''
    anti-malarials: primaquine
    ciprofloxacin
    sulph- group drugs: sulphonamides, sulphasalazine, sulfonylureas

''drugs thought to be safe''
    penicillins
    cephalosporins
    macrolides
    tetracyclines
    trimethoprim


Comparing G6PD deficiency to hereditary spherocytosis

||G6PD deficiency|Hereditary spherocytosis|
|Gender|Male (X-linked recessive)|Male + female (autosomal dominant)|
|Ethnicity|African + Mediterranean descent|Northern European descent|
|Typical history|Neonatal jaundice|Neonatal jaundice|
||Infection/drugs precipitate haemolysis|Chronic symptoms although haemolytic crises may be precipitated by infection|
||Gallstones|Gallstones|
|||Splenomegaly is common|
|Blood film|Heinz bodies|Spherocytes (round, lack of central pallor)|
|Diagnostic test|Measure enzyme activity of G6PD|Osmotic fragility test|