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The co-occurrence of Hirschsprung’s disease (HSCR) and multiple endocrine neoplasia type 2 (MEN2) is a relatively rare event. The basis for this association is the presence of a “Janus” mutation in the RET proto-oncogene––a mutation that acts simultaneously as both a gain-in-function and a loss-of-function mutation. To date, four mutations in the exon 10(More)
We aimed to evaluate the rate and examine potential predictors of subsequent anti-reflux procedures in a population undergoing percutaneous endoscopic gastrostomy (PEG) insertion. We retrospectively reviewed the pre- and post-operative clinical course of patients undergoing PEG insertion over a 10-year period with respect to indication, underlying(More)
PURPOSE Hirschsprung's disease-associated enterocolitis (HAEC) is the most common cause of morbidity and mortality in Hirschsprung's disease (HSCR). Altered intestinal epithelial barrier function has been suggested to play a role in the causation of HAEC. In rodent experimental models of colitis, a decreased expression of K(ATP) channels (Subunits:(More)
AIM To investigate whether the expression of platelet-derived growth factor receptor-α-positive (PDGFRα(+))-cells is altered in Hirschsprung's disease (HD). METHODS HD tissue specimens (n = 10) were collected at the time of pull-through surgery, while colonic control samples were obtained at the time of colostomy closure in patients with imperforate anus(More)
BACKGROUND Urethral catheter (UC) removal is often delayed following colorectal resection due to the perceived increased risk of post-operative urinary retention (POUR) in patients with post-operative epidural analgesia (POEA). We aimed to determine if UC removal at 48 h, irrespective of ongoing POEA use, altered the risk of POUR and other morbidities(More)
BACKGROUND Neuraxial analgesia is chosen by almost half of women who give birth in the United States. Unintentional dural puncture is the most common complication of this pain management technique, occurring in 0.4% to 6% of parturients. Severe positional headaches develop acutely in 70% to 80% of these parturients. Acute postdural puncture headaches are(More)
BACKGROUND The pathophysiology of Hirschsprung's disease (HSCR) is not fully understood. A significant proportion of patients have persisting bowel symptoms such as constipation, soiling, and enterocolitis despite correctly performed operations. Animal data suggest that stretch-activated 2-pore domain K+ channels play a critical role in the maintenance of(More)
Hirschsprung’s disease -associated enterocolitis (HAEC) is the most common cause of morbidity and mortality in Hirschsprung’s disease (HSCR). Altered intestinal epithelial barrier function is implicated in the pathogenesis of HAEC. IL-17 is a proinflammatory cytokine that plays a crucial role in host defense against microbial organisms in the development of(More)
PURPOSE Despite having optimal pull-through (PT) surgery for Hirschsprung's disease (HSCR), many patients experience persistent bowel symptoms with no mechanical/histopathological cause. Murine models of HSCR suggest that expression of key neurotransmitters is unbalanced proximal to the aganglionic colonic segment. We aimed to investigate expression of key(More)
The pathophysiology of Hirschsprung’s disease (HSCR) is not entirely understood. There is no clear explanation for the occurrence of the spastic or tonically contracted aganglionic segment of bowel. Kv11.1 (hERG) channels play a critical role in the regulation of the resting membrane potential as well as affecting either the force or frequency of(More)