“Hirschsprung Disease” (N. Central WA)

Since 2009, The Samaritan Riders Okanogan have been raising public awareness of  childhood diseases affecting many local children in the North Central Washington Region. Their 2021 childhood illness awareness campaign begins with Hertzsprung Disease (HD).

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HIRSCHSPRUNG DISEASE (otherwise known as congenital aganglionic megacolon) is a motor disorder of the gut, which is caused by the failure of the neural crest cells (precursors of enteric ganglion cells)  to migrate completely during intestinal development during fetal life. The resulting aganglionic segment of the colon fails to relax, causing a functional obstruction. The emergency complications of HD include acute obstruction in the neonate (new borne), Hirschsprung-associated enterocolitis (HAEC), and volvulus.

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HD occurs in approximately 1 in 5000 live births with an overall male:female ratio of 3-4:1; when the entire colon is involved, the gender ratio is closer to 1:1.

The most accepted theory of the cause of HD is that there is a defect in carniocaudal migration of neuroblasts originating in the neural crest, a process that begins at 4 weeks of gestation and end at week 7 with the arrival of neural crest-derived cells at the distal end of the colon. Failure of the cells to reach the distal colon leaves that segment aganglionic (without ganglion cells) and therefore nonfunctional.

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Authors: David E Wesson, MD, Monica Esperenza Lopez, MD, MS. Printed from UpToDate 12/2020.

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The Samaritan Riders Okanogan has had a long term partnership with the Tonasket Commancheros and Tonasket Rodeo Grounds Annual Events where we raise monies and awareness for our organization. These Event Tables include:

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May – June the Tonasket Founders Day Rodeo (always the weekend after Memorial Day).

July the Tonasket Tractor Pulls.

September the Demolition Derby (always Labor Day Sunday).

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