G6PD Deficiency in Ghanaians: How to recognise it
In their instructive seminar Professors Capellini and Fiorelli put Africa first in the list of areas with the “highest frequencies of G6PD deficiency” (Jan 5, p 64) , so what are the Ghanaian associations? (i) “Dorkita, I’m passing coca cola urine”. Mist alba, septrin, fansidar, chloramphenicol, APC, are the greatest offenders . (ii) Typhoid disease [3 4] (iii) lobar pneumonia with jaundice [2 4] (iv) Renal failure (v) No enzyme-at-all in 5% G-6-P-D deficient males (vi) Greater delay in recovery from coma  (vii) Greater representation in Cirrhosis of the liver  (viii) Greater proportion of diabetics  (ix) Sickle cell disease patients fare worse [8 9 10] (x) Female homozygotes (X–X–) have more severe disease than hemizygotes (X–Y), making me wonder “how that can be reconciled with the Lyon hypothesis of inactivation of one X chromosome” [10, page 105] (xi) Periodic intravascular haemolysis from interaction between “alpha thalassaemia type 1 equivalent to African homozygous alpha thalassaemia type 2, with G-6PD Total deficiency” . Exercise acts as a trigger. The combination per se does not appear to account for the hyperbilirubinaemia in babies [All cases of ‘march haemoglobinuria’ must be screened for both alpha thalassaemia and G-6PD deficiency]. (xii) The enzyme is found in liver, brain, kidney, adrenals, skin, pancreas, nerve and muscle, hence the extra-erythrocytic manifestations of G6PD deficiency. Bedu-Addo’s description of chloroquine induced bilateral ptosis  could have been in one with no enzyme. (xiii) Hepatomegaly with quick progression to cirrhosis . (xiv) Viral Hepatitis is commoner in G6PD deficient patients, and characterised by intrahepatic cholestasis . (xv) Terminology of A-minus (attributed to Africans) and B-minus (Mediterranean)is meaningless, as in A-minus Ghanaians “absence of enzyme in new red cells” produces cases “similar to the Mediterranean type of total deficiency”  (xvi) There is an inexplicable north-south divide of incidence: 11% of males are deficient in the north  while 23% have G6PD deficiency in the south . (xvii) Protection against malaria has not been proved in Ghana for hemizygotes and female homozygotes . Indeed, blackwater fever is often related to G6PDdeficiency (xviii) “Sabolaa kε Emanbii yi” is a truism in my Krobo tribe: “Onions and M&B disagree” . In the Colonial days some who took sulphonamides (‘M&B’) for infections fell gravely ill on eating onions. Dipropyl disulphide in onions “alters G6PD in the metabolic chain within the erythrocytes, which causes denaturing and precipitation of haemoglobin” . (xix) Genetic Counselling goes beyond haemoglobinopathy to erythrocytopathy. A G6PD deficient sickle cell trait mother (AS) has two healthy daughters with her sickle cell trait (AS) husband, and they seek advice for a third pregnancy hoping to get a boy. She is told that although neither daughter has inherited her deficiency, and both avoided sickle cell anaemia (SS), her next child could be SS with G6PD deficiency (severe if a boy)  (xx) Voluntary family size limitation (VFSL)  is the advice I give hemizygotes who (in Africa) have a high male procreative superiority index (MPSI)  in that males have more children than females, with consequent greater donation of abnormal genes to the next generation.
I declare that I have no conflict of interest
Felix I D Konotey-Ahulu
10 Harley Street, London W1G 9PF, UK
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