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Aquinas University of LegazpiCollege of Nursing and Health SciencesRawis, Legazpi City
HIRSCHSPRUNG’S DISEASE withACUTE COLITIS:A CASE PRESENTATION
Agripa, Marvin J.Astrero, Ann Lauren M.Azada, Reagan S.Balaoro, Robert Patrick E.Base, FridaBayola, Joseph StevenBelleza, John Carlo R.Berango, Maeryl Ayn V.Bonos, Ailyn MaeBremen, Joyce A.Buenconsejo, RoyCabisora, Jhane Karen4BSN3Ray Andrew S. Del RosarioConcept Instructor
INTRODUCTION
 
Hirschsprung’s disease
is a disease of the
large intestine
. It is namedafter Harald Hirschprung, the Danish physician who first described the disease in1886, describing two infants who had died with swollen bellies. It is otherwiseknown as
congenital aganglionic megacolon
which usually occurs in children.It causes constipation, which means that bowel movements are difficult. Somechildren with HD can't have bowel movements at all. The stool creates a blockagein the intestine.If HD is not treated,
stool
can fill up the large intestine. This can causeserious problems like infection, bursting of the colon, and even death.Normally, muscles in the intestine push stool to the
anus
, where stoolleaves the body. Special
nerve cells
in the intestine, called
ganglion cells
makethe muscles push. A person with HD does not have these nerve cells in the lastpart of the large intestine.Healthy large intestine:Nerve cells are foundthroughout the intestine.HD large intestine: Nervecells are missing from thelast part of the intestine.In a person with HD, the healthy muscles of the intestine push the stooluntil it reaches the part without the nerve cells. At this point, the stool stopsmoving. New stool then begins to stack up behind it.Sometimes the ganglion cells are missing from the whole large intestineand even parts of the small intestine before it. When the diseased section reachesto or includes the small intestine, it is called long-segment disease. When thediseased section includes only part of the large intestine, it is called short-segment disease. HD develops before a child is born. Normally, nerve cells grow
 
in the baby's intestine soon after the baby begins to grow in the womb. Thesenerve cells grow down from the top of the intestine all the way to the anus. WithHD, the nerve cells stop growing before they reach the end.No one knows why the nerve cells stop growing. But we do know that it'snot the mother's fault. HD isn't caused by anything the mother did while she waspregnant.Some children with HD have other health problems, such as Downsyndrome and other rare disorders. This disease is named after Harald Hirschsprung, the Danish physician whofirst described the disease in 1886, describing two infants who had died withswollen bellies. "The autopsies showed identical pictures with a pronounceddilatation and hypertrophy of the colon as the dominant features.
SYMPTOMS
Symptoms of HD usually show up in very young children. But sometimesthey don't appear until the person is a teenager or an adult. The symptoms are alittle different for different ages.
Symptoms in Newborns
Newborns with HD don't have their first bowel movement when theyshould. These babies may also throw up a green liquid called bile after eating andtheir
abdomen
may swell. Discomfort from gas or constipation might make themfussy. Sometimes, babies with HD develop infections in their intestines.
Symptoms in Young Children
Most children with HD have always had severe problems with constipation.Some also have more
diarrhea
than usual. Children with HD might also have
anemia
, a shortage of red blood cells, because blood is lost in the stool. Also,many babies with HD grow and develop more slowly than they should.
DIAGNOSTICSBarium Enema X Ray
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