| Autor: |
Sondheimer, Judith M, Szczepanik, Patricia, Perrault, Jean F, Richard, J, Watkins, John B |
| Zdroj: |
Pediatric Research; April 1978, Vol. 12 Issue: 1, Number 1 Supplement 4 p442-442, 1p |
| Abstrakt: |
We studied a 5 year old girl from early infancy with severe chronic diarrhea, steatorrhea and growth failure. Intestinal structure, pancreatic function, liver function and structure and a laparotomy all were normal. During the first 2 years of life BA levels in duodenal juice were consistently below the critical micellar concentration. In recent studies fecal BA were 7 times normal, serum BA by radioimmunassay failed to rise significantly after meals or after oral taurocholate and in duodenal juice, dihydroxy predominated over trihydroxy BA. Pool sizes of cholate and chenodeoxycholate, by isotope dilution were reduced to 6 and 23%, serum cholate T½ was 12% and chenodeoxycholate 7% of normal. Vitamin B12 absorption (Schilling test) at age 4 years was normal. These data lead us to conclude that our patient, who now thrives, suffers from a specific congenital defect of ileal transport of bile acids causing a depleted BA pool and steatorrhea. This defect has not been reported previously. |
| Databáze: |
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