Abstract
BackgroundSome Hirschsprung’s disease (HSCR) patients showed persistent bowel symptoms following an appropriately performed pull-through procedure. The mechanism is presumed to be down-regulated small-conductance calcium-activated potassium channel 3 (SK3) expression in the HSCR ganglionic intestines. We aimed to investigate the SK3 expression’s impact in HSCR patients after a properly performed pull-through surgery in an Indonesian population, a genetically distinct group within Asia.MethodsWe assessed SK3 gene expression in both the ganglionic and aganglionic colon of HSCR patients and controls colon by quantitative real-time polymerase chain reaction (RT-PCR).ResultsWe ascertained fourteen sporadic HSCR patients and six anorectal malformation patients as controls. Quantitative RT-PCR showed that the SK3 expression was significantly lower (23-fold) in the ganglionic colon group compared to the control group (9.9 ± 4.6 vs. 5.4 ± 3.4; p = 0.044). The expression of SK3 in the aganglionic colon group was also significantly lower (43-fold) compared to the control group (10.8 ± 4.4 vs. 5.4 ± 3.4; p = 0.015).ConclusionOur study shows that the down-regulated SK3 expression in ganglionic intestines might contribute to the persistent bowel symptoms following a properly performed pull-through surgery in Indonesian HSCR patients. Furthermore, this study is the first report of SK3 expression in a sample population of Asian ancestry.
Highlights
Some Hirschsprung’s disease (HSCR) patients showed persistent bowel symptoms following an appropriately performed pull-through procedure
Quantitative real-time polymerase chain reaction (RT-PCR) showed that the small-conductance calcium-activated potassium channel 3 (SK3) expression was significantly lower (23-fold) in the ganglionic colon group compared to the control group (9.9 ± 4.6 vs. 5.4 ± 3.4; p = 0.044) (Fig. 1)
We describe new data on Indonesian Hirschsprung disease (HSCR) patients which reveal a similar frequency of short-segment aganglionosis as reported in the literature [17, 18]
Summary
Some Hirschsprung’s disease (HSCR) patients showed persistent bowel symptoms following an appropriately performed pull-through procedure. The mechanism is presumed to be down-regulated small-conductance calcium-activated potassium channel 3 (SK3) expression in the HSCR ganglionic intestines. The prevailing hypothesis is the down-regulated small-conductance calcium-activated potassium channel 3 (SK3) expression in ganglionic bowel in HSCR patients [9]. The SK3 channels have been shown to be involved in the outward currents activated by purines in the intact muscles in response to enteric inhibitory neurotransmission [10]. It is highly expressed in the platelet-derived growth factor receptor alpha-positive
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