G6PD Deficiency in Ghanaians: How to recognise it

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) [1], so what are the Ghanaian associations? (i) “Dorkita, I’m passing coca cola urine”. Mist alba, septrin, fansidar, chloramphenicol, APC, are the greatest offenders [2]. (ii) Typhoid disease [3 4] (iii) lobar pneumonia with jaundice [2 4] (iv) Renal failure [5](v) No enzyme-at-all in 5% G-6-P-D deficient males [6](vi) Greater delay in recovery from coma [7] (vii) Greater representation in Cirrhosis of the liver [7] (viii) Greater proportion of diabetics [7] (ix) Sickle cell disease patients fare worse [8 9 10] (x) Female homozygotes (XX) have more severe disease than hemizygotes (XY), 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” [11]. 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 [12] could have been in one with no enzyme. (xiii) Hepatomegaly with quick progression to cirrhosis [7]. (xiv) Viral Hepatitis is commoner in G6PD deficient patients, and characterised by intrahepatic cholestasis [13]. (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” [6] (xvi) There is an inexplicable north-south divide of incidence: 11% of males are deficient in the north [14] while 23% have G6PD deficiency in the south [15].  (xvii) Protection against malaria has not been proved in Ghana for hemizygotes and female homozygotes [7]. Indeed, blackwater fever is often related to G6PDdeficiency (xviii) “Sabolaa  Emanbii yi” is a truism in my Krobo tribe: “Onions and M&B disagree” [16]. 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” [17]. (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) [18] (xx) Voluntary family size limitation (VFSL) [19] is the advice I give hemizygotes who (in Africa) have a high male procreative superiority index (MPSI) [20] 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

 

felix@konotey-ahulu.com

 

10 Harley Street, London W1G 9PF, UK

 

1     Capellini MD, Fiorelli G. Glucose-6-phosphate dehydrogenase deficiency. Lancet 2008; 371: 64-74.

 

2     Owusu SK. Glucose-6-phosphate dehydrogenase (G-6PD) deficiency in the causation of disease in

       Ghana. Ghana  Med J 1974; 13: 168-170.

 

3        Owusu SK, Foli AK, Konotey-Ahulu FID, Janosi M. Frequency of Glucose-6-phosphate dehydrogenase deficiency in typhoid fever in Ghana. Lancet 1972; 1: 320.  

 

4    Adu D, Anim-Addo Y, Foli AK, Yeboah ED, Quartey JKM. Acute renal failure and typhoid fever. Ghana  

      Medical Journal 1975; 14: 172-174.

 

5     Owusu SK, Addy JH, Foli AK, Janosi M, Konotey-Ahulu FID, Larbi EB. Acute reversible renal failure

       associated with glucose-6-phosphate dehydrogenase deficiency. Lancet 1972; 1: 1255-1257 

 

6     Owusu SK. Absence of glucose-6-phosphate dehydrogenase in red cells of an African. BMJ  1972; 4: 

       25-26

 

7   Owusu SK. Clinical manifestations of glucose-6-phosphate dehydrogenase (G-6PD) deficiency      

       in Ghana. Ghana Med J 1978; 17: 235-39.  

 

8   Konotey-Ahulu FID. Glucose-6-phosphate dehydrogenase deficiency and sickle cell anaemia. New Eng J

     Med 1972: 287: 887-888.

 

9   Acquaye CTA, Gbedemah KA, Konotey-Ahulu FID. Glucose-6-pogosphate dehydrogenase deficiency

      incidence in sickle cell disease patients in Accra. Ghana Med J 1977; 16: 4-7

 

10      Konotey-Ahulu FID. The sickle cell disease patient: natural history from a clinico-epidemiological study   

        of 1550 patients of Korle Bu Hospital Sickle Cell Clinic. London: Macmillan 1992; Watford: Tetteh 

                A’Domeno Co 1996.   

       

        11   Konotey-Ahulu FID. Alpha thalassaemia nomenclature and abnormal haemoglobins. Lancet 1984; 1:

                1024-1025

       

        12   Bedu-Addo G. Chloroquine induced bilateral ptosis. Trans Roy Soc Trop Med Hyg 2006;

                100: 696-697. 

 

13      Morrow RH, Smetana HF, Sai FT, Edgecomb JH. Unusual features of viral hepatitis in Accra, Ghana. 

      Ann Intern Med 1968; 68: 1250-1264.

       

         14   Ringelhann B, Dodu SRA, Konotey-Ahulu FID, Lehmann H. A survey for haemoglobin variants,

    thalassaemia and Glucose-6-phosphate dehydrogenase deficiency in northern Ghana. Ghana Med J 

              1968; 7: 120-124.

 

15      Owusu SK, Opare-Mante A. Electrophoretic characterization of glucose-6-phosphate dehydrogenase (G6PD) enzyme in Ghana.  Ghana Medical Journal 1972; 11: 304.

 

        16   Konotey-Ahulu FID. Probing anecdotes in traditional African therapeutics. African Journal of Health  

               Sciences 1194; 1: 53-56.

 

17      Fenwick G, Hanley AB. The genus Allium – Part 3 Section X Medicinal Effects. CRC Critical Reviews

                  in Food Science and Nutrition 1985; 23: 1-73.

 

18      Konotey-Ahulu FID. Missing the wood for one genetic tree? In, The First International Symposium on

       the Role of Recombinant DNA in Genetics. Proceedings, Chanai, Crete – Greece, May 13 -16, 1985. Eds

       Loukopoulos D, Teplitz R. Athens, P Paschalidis 1986, pages 105-116.

 

19      Konotey-Ahulu FID.  The male procreative superiority index (MPSI): its relevance to genetical

                 counselling in Africa. In: Eds, Oliver Mayo, Carolyn Leach. Fifty Years of Human Genetics. A

                 Festschrift and liber amicorum to celebrate the life and work of George Robert Fraser. South Australia,

                 Wakefield Press, August 2007 pages 48-50.

        

         20    Konotey-Ahulu FID. Need for ethnic experts to tackle genetic public health. Lancet 2007; 370: 1826-27.   

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