G6PD Deficiency and Chronic Kidney Disease
Glucose – 6 – phosphate dehydrogenase deficiency (G6PD deficiency) is the most common enzymopathy in human. This enzymopathy affects more than 400 million people worldwide4-5. It has a wide geographical distribution, reaching higher prevalence in certain parts of Africa, the Mediterranean and Asia. The incidence of G6PD deficiency over 1% is distributed in the Mediterranean regions, across the Middle East, India, Indochina, South China as well as middle Africa. This distribution is similar to that of the thalassemia and endemic malaria infection in the past. More than 140 different mutations of the G6PD gene have been identified4.
Glucose – 6 – phosphate dehydrogenase enzyme catalyzes the first step of pentose phosphate pathway where coverts NADP to its reduced state NADPH preserving the membrane structure of Red Blood Cells. G6PD Deficiency is an X – linked recessive inherited disorder, which only affect the males in the population while females are heterozygotes. Individuals with G6PD deficiency can present with a spectrum of disorders including acute massive hemolysis, neonatal hyperbilirubinemia, chronic nonspherocytic hemolytic anemia and acute renal failure. G6PD deficiency can cause hemolytic anemia, usually after exposure to drugs, viral hepatitis, Favisam, malaria, bacterial sepsis, chemicals and herbal medicine.
There are significant differences in the prevalence of G6PD deficiency among different nations, and even within the same nation, due to migrations, geographical characteristics, and various religious and cultural factors.4
The literature shows that G6PD Deficiency is so far related to acute renal failure and no association with CKD3. Local researchers investigated that low G6PD levels show among CKD patients in North Central province3 of Sri Lanka. There is a higher prevalence of the G6PD deficiency in patients with CKD of unknown origin and indicates the possibility that G6PD deficiency plays an important role in the pathogenesis of the CKD of unknown origin3.
Chronic Kidney Disease (CKD) is increasingly recognized as world health problem. It causes premature morbidity and mortality and lowers the quality life. In the US, 9.6% of adults are estimated to have CKD. Studies from Europe, Australia and Asia also confirm high prevalence of CKD1. During last three decades, incidence and prevalence of CKD and End Stage Kidney Disease (ESKD) of unknown etiology have risen progressively in Sri Lanka1. Their prevalence is very high (10%) in North Central Province of Sri Lanka2. Recent studies show that approximately 2000 patients are annually being added to the CKD burden in North Central Province. According to health sector statistics, more than 6,000 cases of CKD have been reported for the last 5 years in Sri Lanka. Approximately 300 – 600 deaths annually occur in hospitals due to CKD of unknown etiology2.
In North Central province of Sri Lanka, over half of the population is engaged in agriculture. Young people are predominantly affected and CKD of unknown etiology is common among people residing in paddy cultivating areas2. Almost all those affected are men from farming families without pre-existing conditions than can lead to renal disease, such as hypertension or diabetes6. The absence of clinical symptoms until the late stages of renal failure is also puzzling researchers and making early diagnosis difficult, leading to many deaths3. The affected male: affected female ratio is 4:12.
The disease onset is in adolescence and number of cases Reported increases with increasing age. These patients typically present with non-specific signs and symptoms, lethargy, weakness, moderate anemia and mild hypertension. Western medical studies conducted so far suggest that a common although yet unknown etiology, leading to marked Tubulointerstitial fibrosis of the kidney that finally causes Irreversible kidney failure. Renal Biopsies showed TubuloInterstitial disease in more than 80% of the cases3.
1.Palihawadana P; Chronic Kidney Disease – A Global Public Health Problem; WER Sri Lanka 2009, 36:48.
2.Gunasekera A; Help Treat Chronic Renal Failure in Sri-Lanka – presentation; Embassy of Sri Lanka Washington DC, USA;2009
3.Abeysekera DTDJ; Dissanayake AMSDM; Jayasekera JMKB and Wazeel AWM; Low G6PD activity in Patients with Chronic Kidney Disease of Unknown Etiology in Sri Lanka.
4.Vjekoslav Krželj; High Incidence of Glucose-6-phosphate Dehydrogenase Deficiency in Croatian Island Isolate: Example from Vis Island, Croatia; Croat Med J 2006; 47,566-570.
5.Muzaffer MA; Neonatal screening of glucose-6-phosphate dehydrogenase de?ciency in Yanbu, Saudi Arabia; Journal of Medical Screening 2005, 12 :4.