Health  Vol.7 No.5 , May 2015
An Assessment of Frequent Haemolytic Crisis in G6PD Deficiency Subjects Linked with Chronic Disease States in Al Ahsa Community, Saudi Arabia
The main treatment strategy for glucose-6-phosphate dehydrogenase (G6PD) deficiency is averting the use of oxidative precipitants like drugs or foods that trigger haemolysis. However, subjects could experience acute crisis in a comorbidity state of diabetes, hypertension or infection. In addition, the condition could in turn predispose carriers to these chronic diseases. Aim of the study was to evaluate the frequency of haemolytic crisis in carriers with attendant chronic diseases, and also assess awareness and knowledge of aggravated reactions from these conditions. 282 subjects consented and participated in the study. A cross-sectional semi-structured interview questionnaire conducted from May to August 2013 was adopted. Questions focused on demographics, awareness and when disease was diagnosed, type and frequency of crisis, family history, screening and presence of other diseases and awareness of trigger from these conditions. A total of 69.9% were G6PD deficiency gene carriers and mostly fell within the age group of 31 - 40 (43.6%). 75.6% of the carriers were not aware that other disease conditions could exacerbate crisis. Non-drug related crisis was 85.3% compared with 14.7 drug related reactions. Out of the 69.9% carriers, 17.3% are diabetics, 18.3% of them are hypertensive and 25.9% are with bleeding disorders. Also, 29.1% had frequent episodes with bacterial infection. Symptoms experienced that were not drug related included, 44.8% attacks of pallor, 25.3% jaundice, 28.5% with shortness of breath, while 21.7% had dark urine frequently. No routine screening for other diseased conditions was carried out for these subjects despite experiencing frequent crisis that were not drug related. Carriers with other diseased conditions experienced more non-drug related crisis and were not aware of it.

Cite this paper
Emeka, P. , Hilal, H. , Asif, A. , Almukhalaf, A. and Khan, T. (2015) An Assessment of Frequent Haemolytic Crisis in G6PD Deficiency Subjects Linked with Chronic Disease States in Al Ahsa Community, Saudi Arabia. Health, 7, 625-632. doi: 10.4236/health.2015.75074.
[1]   Al-Jaouni, S.K., Jarullah, J., Azhar, E. and Moradkhani, K. (2011) Molecular Characterization of Glucose-6-Phosphate Dehydrogenase Deficiency in Jeddah, Kingdom of Saudi Arabia. BMC Research Notes, 4, 436.

[2]   Pathak, R.R., Chhaiya, S.B. and Mehta, D.S. (2013) Prevalence of G6PD Deficiency versus Oxidizing Drugs: A Survey in the Tertiary Care Hospital. International Journal of Basic Clinical Pharmacology, 2, 170-176.

[3]   Alabdulaali, M.K., Alayed, K.M., Alshaikh, A.F. and Almashhadani, S.A. (2010) Prevalence of Glucose-6-Phosphate Dehydrogenase Deficiency and Sickle Cell Trait among Blood Donors in Riyadh. Asian Journal of Transfusion Science, 4, 31-33.

[4]   Al-Sweedan, S.A., Jdaitawi, H., Khriesat, W.M., Khader, Y.Y. and Al-Rimawi, H.S. (2009) Predictors of Severe Hemolysis in Patients with Glucose-6-Phosphate Dehydrogenase Deficiency Following Exposure to Oxidant Stresses. Hematology/Oncology and Stem Cell Therapy, 2, 354-357.

[5]   WHO Working Group (1989) Glucose-6-Phosphate Dehydrogenase Deficiency. Bulletin of the World Health Organization, 67, 601-611.

[6]   Beutler, E. (2008) Glucose-6-Phosphate Dehydrogenase Deficiency: A Historical Perspective. Blood, 111, 16-24.

[7]   Santana, M.S., Monteiro, W.M., Costa, M.R., Sampaio, V.S., Brito, M.A., Lacerda, M.V. and Alecrim, M.G. (2014) High Frequency of Diabetes and Impaired Fasting Glucose in Patients with Glucose-6-Phosphate Dehydrogenase Deficiency in the Western Brazilian Amazon. American Journal of Tropical Medicine and Hygiene, 91, 74-76.

[8]   Nasserullah, Z., Al Jame, A., Abu Srair, H., Al Qatari, G., Al Naim, S., Al Aqib, A., et al. (1998) Neonatal Screening for Sickle Cell Disease, Glu-cose-6-Phosphate Dehydrogenase Deficiency and A-Thalassemia in Qatif and Al Hasa. Annals of Saudi Medicine, 18, 289-292.

[9]   Al-Ali, A.K., Al-Mustafa, Z.H., Al-Madan, M., Qaw, F. and Al-Ateeq, S. (2002) Molecular Characterization of Glucose-6-Phosphate Dehydrogenase Deficiency in the Eastern Province of Saudi Arabia. Clinical Chemistry and Laboratory Medicine, 40, 814-816.

[10]   Gelpi, A.P. (1965) Glucose-6-Phosphate Dehydrogenase Deficiency in Saudi Arabia. Blood, 25, 486-493.

[11]   Alhamdan, N.A., Almazrou, Y.Y., Alswaidi, F.M. and Choudhry, A.J. (2007) Premarital Screening for Thalassemia and Sickle Cell Disease in Saudi Arabia. Genetics in Medicine, 9, 372-377.

[12]   Elyassi, A.R. and Rowshan, H.H. (2009) Perioperative Management of the Glucose-6-Phosphate Dehydrogenase Deficient Patient: A Review of Literature. Anesthesia Progress, 56, 86-91.

[13]   Phillips, S.M. and Silvers, N.P. (1969) Glucose-Phosphate Dehydrogenase Deficiency, Infectious Hepatitis, Acute Hemolysis, and Renal Failure. Annals of Internal Medicine, 70, 99-104.

[14]   Heymann, A.D., Cohen, Y. and Chodick, G. (2012) Glucose-6-Phosphate Dehydrogenase Deficiency and Type 2 Diabetes. Diabetes Care, 35, e58.

[15]   Zhang, Z., Liew, C.W., Handy, D.E., Zhang, Y., Leopold, J.A., Hu, J., Guo, L., Kulkarni, R.N., Loscalzo, J. and Stanton, R.C. (2010) High Glucose Inhibits Glucose-6-Phosphate Dehydrogenase, Leading to Increased Oxidative Stress and Beta-Cell Apoptosis. The FASEB Journal, 24, 1497-1505.

[16]   Hecker, P.A., Leopold, J.A., Gupte, S.A., Recchia, F.A. and Stanley, W.C. (2013) Impact of Glucose-6-Phosphate Dehydrogenase Deficiency on the Pathophysiology of Cardiovascular Disease. AJP: Heart and Circulatory Physiology, 304, H491-H500.

[17]   Hecker, P.A., Lionetti, V., Ribeiro Jr., R.F., Rastogi, S., Brown, B.H., O’Connell, K.A., Cox, J.W., Shekar, K.C., Gamble, D.M., Sabbah, H.N., Leopold, J.A., Gupte, S.A., Recchia, F.A. and Stanley, W.C. (2013) Glucose 6-Phosphate Dehydrogenase Deficiency Increases Redox Stress and Moderately Accelerates the Development of Heart Failure. Circulation: Heart Failure, 6, 118-126.

[18]   Wiesenfeld, S.L., Petrakis, N.L., Sams, B.J., Collen, M.F. and Cutler, J.L. (1970) Elevated Blood Pressure, Pulse Rate and Serum Creatinine in Negro Males Deficient in Glucose-6-Phosphate Dehydrogenase. New England Journal of Medicine, 282, 1001-1002.

[19]   Clark, M. and Root, R.K. (1979) Glucose-6-Phosphate Dehy-drogenase Deficiency and Infection: A Study of Hospitalized Patients in Iran. Yale Journal of Biology and Medicine, 52, 169-179.

[20]   Hsieh, Y.-T., Lin, M.-H., Ho, H.-Y., Chen, L.-C., Chen, C.-C. and Shu, J.-C. (2013) Glucose-6-Phosphate Dehydrogenase (G6PD)-Deficient Epithelial Cells Are Less Tolerant to Infection by Staphylococcus aureus. PLoS ONE, 8, e79566.

[21]   Nowiński, A., Jablońska-Skwiecińska, E., Wojda, E., Bieleń, P., Ptak, J., Sliwinski, P. and Górecka, D. (2007) Community-Acquired Pneumonia Complications in a Patient with Hereditary Glucose-6-Phosphate Dehydrogenase Deficiency. Pneumonologia i Alergologia Polska, 75, 283-288.

[22]   Luzzatto, L., Mehta, A. and Vulliamy, T. (2001) Glucose 6-Phosphate Dehydrogenase Deficiency. In: Scriver, C.R., Beaudet, A.L., Sly, W.S. and Valle, D., Eds., The Metabolic and Molecular Bases of Inherited Disease, 8th Edition, McGraw-Hill, New York, 4517-4533.

[23]   Beutler, E. (1994) G6PD Deficiency. Blood, 84, 3613-3636.

[24]   Carette, C., Dubois-Laforgue, D., Gautier, J.F. and Timsit, J. (2011) Diabetes Mellitus and Glucose-6-Phosphate Dehydrogenase Deficiency: From one Crisis to Another. Diabetes & Metabolism, 37, 79-82.

[25]   Moore, G.S. and Calabrese, E.J. (1979) The Possible Role of Hypertension in Aggravating Hemolytic Episodes in G-6PD Deficient Persons. Medical Hypotheses, 5, 453-462.

[26]   Gu, X.J., Chen, S.P., Ge, S.J., Zheng, L.Q., Wang, D.W. and Shen, F.X. (2013) G6PD Deficiency-Induced Hemolysis in a Chinese Diabetic Patient: A Case Report with Clinical and Molecular Analysis. Acta Diabetologica, 50, 89-92.

[27]   Agarwal, A., Nayak, M.D., Patil, A. and Manohar, C. (2013) Glucose 6 Phosphate Dehydrogenase Deficiency Unmasked by Diabetic Ke-toacidosis: An Underrated Phenomenon. Journal of Clinical and Diagnostic Research, 7, 3012-3013.