Back to Search
Start Over
Secretory diarrhea with protein-losing enteropathy, enterocolitis cystica superficialis, intestinal lymphangiectasia, and congenital hepatic fibrosis: a new syndrome.
- Source :
-
The Journal of pediatrics [J Pediatr] 1986 Jan; Vol. 108 (1), pp. 61-5. - Publication Year :
- 1986
-
Abstract
- Four infants had noninfectious intractable diarrhea, vomiting, anasarca, hepatomegaly, hypoglycemia, and malnutrition within the first 3 months of life. Their parents originated from the same Northeastern part of Quebec, and consanguinity was found in two kindreds. Diarrhea was secretory in three infants (mean stool volume 87 ml/kg/day, Na+ 108 mEq/L, Cl- 85 mEq/L). Hypoalbuminemia (mean 2.0 gm/dl), present in all infants, appeared to be secondary to a protein-losing enteropathy, which was documented in two infants. Histologic examination of the upper small intestine showed only mild to moderate villous atrophy. The remarkable findings were those of cystic dilation of the crypts and acute inflammation of crypts and lamina propria, all of which were most prominent in the colon and terminal ileum; the changes were progressive over time. Mild lymphangiectasia was found in all of the patients. Congenital hepatic fibrosis, present in all, was associated in one patient with a nonfunctional multicystic kidney. Prolonged total parenteral nutrition, intravenously administered albumin, antisecretory agents, and antibiotics were unsuccessful in controlling the disease. Although a total colectomy was followed by a temporary decrease in stool output and normalization of serum albumin concentration in one infant, the patients died between 4 and 21 months of age.
- Subjects :
- Anti-Bacterial Agents therapeutic use
Colectomy
Colon pathology
Diarrhea, Infantile mortality
Diarrhea, Infantile therapy
Humans
Infant
Intestinal Mucosa pathology
Intestine, Small pathology
Lymphangiectasis, Intestinal complications
Lymphangiectasis, Intestinal pathology
Parenteral Nutrition, Total
Protein-Losing Enteropathies pathology
Syndrome
Diarrhea, Infantile complications
Liver Diseases congenital
Protein-Losing Enteropathies complications
Subjects
Details
- Language :
- English
- ISSN :
- 0022-3476
- Volume :
- 108
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- The Journal of pediatrics
- Publication Type :
- Academic Journal
- Accession number :
- 3080572
- Full Text :
- https://doi.org/10.1016/s0022-3476(86)80769-7