Related Experiment Videos
Enteropathy and renal involvement in an infant with evidence of widespread autoimmune disturbance
S G Mitton1, R Mirakion, V F Larcher
1Department of Child Health, Westminster Children's Hospital, London, U.K.
Insights
This study reports an infant with severe gut and kidney issues, likely caused by autoimmune disease. Autoantibody monitoring may help track this rare condition.
Area of Science:
- Pediatric Gastroenterology
- Autoimmune Disorders
- Nephrology
Background:
- An infant presented with prolonged diarrhea, vomiting, and weight loss.
- Small intestinal biopsy revealed a flat mucosa, unresponsive to dietary modifications.
Purpose of the Study:
- To investigate the potential autoimmune basis of a rare infant condition involving gut and renal dysfunction.
- To assess the utility of serial autoantibody testing in monitoring disease progression.
Main Methods:
- Serial autoantibody testing for gut epithelial cells, renal brush borders, and liver-kidney microsomal antibodies.
- Clinical observation and response to steroid treatment.
Main Results:
- Persistent autoantibodies against gut epithelial cells and renal brush borders were detected.
- Atypical liver-kidney microsomal antibodies were also found.
- Steroid treatment led to temporary clinical improvement, but the patient ultimately succumbed to combined gut and renal failure.
Conclusions:
- The widespread autoantibodies and multi-organ involvement (gut, liver, kidney) suggest an autoimmune pathogenesis.
- Serial autoantibody measurements may serve as a prognostic tool and guide therapeutic decisions in similar cases.
Abstract:
An 8-month-old infant presented with a 1 month history of protracted diarrhea, vomiting, and weight loss. Small intestinal biopsy showed a flat mucosa and there was no clinical improvement with gluten, cow's milk protein, and disaccharidase-free diet. Serial testing for autoantibodies revealed persistent autoantibodies to gut epithelial cells and to renal brush borders; on two occasions, atypical liver-kidney microsomal antibodies were detected. Treatment with steroids produced clinical improvement but the patient finally succumbed with a combination of gut and renal dysfunction. The widespread nature of the antibodies, with clinical involvement of gut, liver, and kidney, suggest an underlying autoimmune mechanism for the pathogenesis of the condition. Serial autoantibody measurements may provide a means to monitor the disease progress and may be a guide to treatment.