SCREENING OF UGT1A1 GENE AND GENOTYPE-PHENOTYPE CORRELATIONSHIP IN NEONATAL JAUNDICE FROM A SAMPLE OF NEWBORNS IN KELANTAN

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Date
2012-04
Authors
NUR HASNAH, MA’AMOR
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Pusat Pengajian Sains Perubatan Universiti Sains Malaysia
Abstract
The rate limiting step of bilirubin excretion is the glucuronidation of bilirubin in the liver, a process that is catalyzed by an enzyme, Uridine glucuronyl transferase. This enzyme is encoded by the UGT1A1 gene. In several populations, mutations in this gene have been shown to cause neonatal jaundice. However data on the Malaysian Malay population are scanty at best. The objectives of this study included: to determine the frequency of variants in the exons of the UGT1A1 gene in a population of term Malay neonates with jaundice and without jaundice, and to correlate the genotype finding with some phenotypic data. A cross sectional study was performed in Kelantan, Malaysia. A group of term jaundiced neonate (jaundice group) and a group of term non-jaundiced neonate (control group) were included in the study. Blood was taken for genetic testing. DNA was extracted from blood before polymerase chain reaction (PCR) was performed. Denaturing high performance liquid chromatography (DHPLC) was performed to screen the whole exons in the UGT1A1 gene and for subjects that were identified to have a heteroduplex peak in DHPLC, sequencing was performed to confirm the mutation or SNP. Clinical data were collected as part of a larger study. Data were entered in SPSS and analyzed. Two hundred and eighteen six (286) neonates were included in each the jaundice and control groups. Nine variants have been identified in this study. The most common variant was the G71R variant in exon 1, which is common in other Asian populations as well. Only 4 of the other variants detected in this study had been reported as SNPs in another population. Other variants appeared to be novel mutations. Even though variants were found in a higher number in the jaundice group than in the control group, the difference between the groups was statistically not significant. There were also no significant differences in severity of neonatal jaundice and in haemolysis between jaundiced neonates with and without identified variants in the UGT1A1 gene. The G71R variant had been identified as the most common variant in the exons of the UGT1A1 gene. The results of this study did not clearly show that this was a risk factor for neonatal jaundice.
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Paediatrics
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