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Congenital Diarrheal Disorders

What is a Congenital Diarrheal Disorder?

Some infants can be born having very loose, large volume stools that occur multiple times in a day. The diarrhea usually starts within the first two to four weeks of life. If this diarrhea persists, the child may become dehydrated and need to be admitted to the hospital for treatment.

There are some very rare problems that may cause this severe diarrhea. Most of these problems are with the lining of the intestine (tufting enteropathy, microvillus inclusion disease), or the way the intestine works (transport defects).

In these disorders, the lining of the intestine is different from the normal intestine. The shape and structure of the intestine leads to poor absorption / uptake of food. Most of these problems will continue to cause bad diarrhea for many years and require a specialist to help manage them.

Whenever a baby has such severe diarrhea in the first month of life that he or she needs to be hospitalized, a careful search for the cause is very important.

Causes / Type

  • Malabsorption syndromes: These are disorders in which the intestines are unable to absorb complex foods. The patient will have large volume stools after eating.
  • Congenital transport defects: Patients secrete and are unable to absorb large amounts of salt (sodium or chloride or potassium) from their intestines and have large volume, loose stools. The loose stools can occur at any time and not just when eating.
  • Structural defects: The lining of the intestines is abnormal and as a result does not function normally. This includes disorders like microvillus inclusion and tufting enteropathy.
  • Autoimmune enteropathy: This condition occurs when the body’s own immune system attacks itself and irritates or inflames the lining of the intestine.


  • Large volume, watery stools multiple times a day
  • Decrease in or no wet diapers
  • Dry mouth and crying without tears
  • Weight loss
  • Abdominal (belly) swelling


  • Each problem has its own treatment. Some problems may slowly get better with time and some don’t. Some get worse with eating, and some are always bad.
  • Fluids and nutrition through the veins are usually needed.
  • Small bowel / intestinal transplantation might be necessary in some of these patients.

When to Call the Doctor

Call your doctor if your infant is not growing and has watery diarrhea that does not go away with time.

Last Updated 07/2022

Reviewed By Jen Willoughby
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