Question

In: Anatomy and Physiology

Find a digestive disease that you don't know about and write a report describing this disease....

Find a digestive disease that you don't know about and write a report describing this disease.

Make sure this report is in your own words.

Please add any personal comments/experiences.

Remember to cite where you got your information.

-Submit the report and post for discussion

Solutions

Expert Solution

HIRSCHSPRUNG'S DISEASE

- Congenital defect in colonic innervation, producing congenital aganglionic megacolon

- more common in males but severe in females

- 1:5000 live births

PATHOGENESIS:

• Failure of migration of neural crest cells into the bowel.

• Leads to absence of ganglion cells particularly nitric oxide releasing cells in bowel.

• Affected part of bowel is not able to relax.

• Proximal part of affected bowel gets dilated.

MORPHOLOGY:

Short Segment Hirschsprung's Disease: when part of large intestine is involved.

Long Segment Hirschsprung's Disease: When entire colon is involved.

MOST COMMON SITE: Rectum

CLINICAL FEATURES:

• Failure of passage of meconium.

• Abdominal Distension

• Constipation

• Dilation causes thinning if bowel wall which may lead to rupture (especially in colon).

DIAGNOSIS:

Confirmed by Rectal Suction Biopsy.

It reveals:

  1. Absence of ganglionic cells.
  2. Nerve fibres are hypertrophied.

Special stains are used.

• AchE: heavy staining (Normally, no staining)

• Calretinin: no staining (Normally, staining is present)

Reference: Robins Pathological Basis of Disease


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