Synonyms
Childhood enteropathy; Congenital enteropathy; Primary enteropathies; Severe (primary) enteropathies of infancy
Definition
Primary enteropathies of infancy comprise of epithelial defects including microvillus inclusion disease, tufting enteropathy, and enteroendocrine cell dysgenesis and autoimmune enteropathies. The diseases in this group cause severe chronic (>2–3 weeks) diarrhea starting in the first weeks of life and resulting in failure to thrive in the infant. The diarrhea is either “intractable,” that is, chronic, unexplained diarrhea, or “protracted” describing infants with loose and frequent stools which resolves despite initial severity. Both forms are usually associated with malabsorption. Causes of intractable or protracted diarrhea in infancy can be classified as entities showing a normal villus/crypt axis and those associated with villous atrophy. Congenital enteropathies are presented in Table 1. Congenital defects in the transport of sodium, chloride,...
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References and Further Reading
Cuenod, B., Brousse, N., Goulet, O., De Potter, S., Mougenot, J. F., Ricour, C., Guy-Grand, D., & Cerf-Bensussan, N. (1990). Classification of intractable diarrhea in infancy using clinical and immunohistological criteria. Gastroenterology, 99(4), 1037–1043.
Gambarara, M., Diamanti, A., Ferretti, F., Papadatou, B., Knafelz, D., Pietrobattista, A., & Castro, M. (2003). Intractable diarrhea of infancy with congenital intestinal mucosa abnormalities: Outcome of four cases. Transplantation Proceedings, 35(8), 3052–3053.
Girault, D., Goulet, O., Le Deist, F., Brousse, N., Colomb, V., Césarini, J. P., de Potter, S., Canioni, D., Griscelli, C., Fischer, A., et al. (1994). Intractable infant diarrhea associated with phenotypic abnormalities and immunodeficiency. The Journal of Pediatrics, 125(1), 36–42.
Goulet, O., Kedinger, M., Brousse, N., Cuenod, B., Colomb, V., Patey, N., de Potter, S., Mougenot, J. F., Canioni, D., Cerf-Bensussan, N., et al. (1995). Intractable diarrhea of infancy with epithelial and basement membrane abnormalities. The Journal of Pediatrics, 127(2), 212–219.
Sherman, P. M., Mitchell, D. J., & Cutz, E. (2004). Neonatal enteropathies: Defining the causes of protracted diarrhea of infancy. Journal of Pediatric Gastroenterology and Nutrition, 38, 16–26.
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Ensari, A. (2017). Enteropathy, Neonatal (Congenital). In: Carneiro, F., Chaves, P., Ensari, A. (eds) Pathology of the Gastrointestinal Tract. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-40560-5_1462
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DOI: https://doi.org/10.1007/978-3-319-40560-5_1462
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