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[Autoimmune enteropathy with onset in early infancy: clinico-morphologic and immunologic manifestations]
J Sýkora1, J Varvarovská, F Stozický
1Dĕtská klinika FN a LF UK, Plzen. sykorajo@fnplzen.cz
Insights
Autoimmune enteropathy in infants can cause severe diarrhea. Early diagnosis via autoantibody testing and treatment with immunosuppressants and specialized nutrition can lead to remission.
Area of Science:
- Pediatric Gastroenterology
- Immunology
- Autoimmune Diseases
Background:
- Autoimmune enteropathy is a rare cause of protracted diarrhea in infants.
- Early-onset severe diarrhea in infants requires thorough investigation.
Observation:
- A male infant presented with severe protracted diarrhea at 6 weeks of age.
- The condition was linked to small bowel villous atrophy and circulating antienterocyte antibodies.
Findings:
- Treatment with steroids and specialized nutrition (parenteral and enteral) led to clinical improvement.
- Resolution of symptoms, normalization of small bowel biopsy, and negative antienterocyte antibodies were observed post-treatment.
- The patient achieved clinical remission for over 18 months.
Implications:
- Autoantibody testing is recommended for infants with unexplained protracted diarrhea.
- Improved outcomes are possible with early diagnosis, immunosuppressive therapy, and nutritional support.
- Advances in treatment offer hope for previously fatal autoimmune gastrointestinal disorders.
Abstract:
This paper describes a severely affected male infant with serious protracted diarrhoea caused by a rare autoimmune enteropathy. The disease began at 6 weeks of age of the child and it was associated with small bowel villous atrophy and the presence of circulating antienterocyte antibodies. The child was treated with steroids and with parenteral and special enteral nutrition. The patient showed clinical improvement as documented by decreased stool output and possibility to terminate the parenteral nutrition. The small biopsy samples showed a return to normal. Antienterocyte antibodies were negative after the treatment. The patient has been followed up for at least 18 months and was in a clinical remission. We recommend that autoantibodies tests should be performed in all infants with unexplained protracted diarrhoea. The use of potent immunosuppressive drugs and the increasing experience with parenteral and enteral nutrition can improve the perspective of these previously fatal disorders.