G6PD And Co

Question 1

G6PD - Glucose 6 phosphate dehydrogenase deficiency

Answer 1

Genetic
Affects Africans,Asians and southern Europe
More common in fen than women
Individuals are susceptible to developing acute haemolytic anaemia - where blood cells break down, when they take some medications of eat fava beans
- G6PD is genetically heterogenous (a drug safe in some G6PD patients may not be safe in others)
- manufacturers do not do routine tests for drugs and their effects on patients with G6PD
- risk of haemolytic is always dose related

Question 2

Drugs with definite risk of haemolytic on most G6PD deficient individuals:

Answer 2

• daspone and other sulfones
• nitrofuratoin
• fluoroquinolones (ciprofloxicin, ofloxacin)
• sulphonamides (eg. Co trimazole)
• methylthionium chloride
• primaquine
• quinolones (eg. Nalidixic acid - not on uk market)
• rasburicase

Question 3

Possible risk

Answer 3

Sulfonylureas
Aspirin
Chloroquine’s
Menadione, water soluable derivatives
Quinine
Napthalene
Toxicity in animal studies - avoid in pregnancy unless benefits outweigh risk

Question 4

Oral rehydration therapy - qualities of good rehydration therapy

Answer 4

• intestinal absorption of sodium and water is enhanced by glucose and other carbs
• replaces electrolyte deficit safely
• containing alkalising agent to counter acidosis
• slightly hypo o solar - prevents possible indication of osmotic diarrhoea
• simple to used in hospital and at home
• palatable, child friendly and readily available

Question 5

Hydroxocobalamin injections

Answer 5

Indications - prophylaxis of anaemias associated with b12 deficiency, pernicious anaemia

• has completely replaced cyanocobalamin as form of vit b12 of choice (because it is retained by the body longer)
• given at 3 month intervals

Question 6

Folic acid - megaloblastic anaemia

Answer 6

• most megaloblastic anaemias are due to a lack of either vitamin B12 or folic acid
• another cause of megoblastic anaemia in the UK is pemicious anaemia (lack of gastric intrinsic factor resulting from an autoimmune gastritis causes malabsorption of vitamin B12)
• Folic acid needed for around 4 months until replenish body stores

Question 7

Folic Acid

Answer 7

• given during pregnancy, poor nutrition, antiepleptic drugs and methotrexate
• used in folate deficienct megoblastic anaemia as well as prophylaxis
• taken before and during pregnancy to prevent neural defects
• women at low risk - 400mcg daily before conception and until 12 weeks
• women at high risk - 5mg daily until 12 weeks
• high risk couples - if one partner has had the defect or family history or other malabsorption issues (sickle cell, diabetes,antiepileptic meds)

Question 8

EPOETINS

Answer 8

Treat anaemia associated with erythropoietin deficiency in chronic kidney injury
- darbapoetin alfa has a longer half life, overdose can lead to increase in thrombosis

• hypertensive crisis and pure cell aphasia (decline of rbc)
• shortens period of symptomatic anaemia in patients receiving cytotoxic chemo
• EPO beta also used for prevention of anaemia in pre term neonates with low birth weight

Question 9

Sickle cell disease

Answer 9

Structural abnormality of the Hb - deformed and less flexible RBC

-reduced oxygen to organs and every pain
- increased susceptibility to infection and various vaccines required and prophylactic penicillin
- hydroxocarbamide can reduce frequency of crises and for blood transfusions
- chronic complications include skin ulcerations,renal failure and. Increased risk of infection

Question 10

Folinic Acid

Answer 10

Effective in the treatment of folate deficient megaloblastic anaemia but generally used with cytoxics

It’s given as calcium folinate