Cases of Trichohepatoenteric Syndrome (Syndromic Diarrhea) with Underlying Crohn’s Disease

Management of syndromic diarrhea/tricho-hepato-enteric syndrome: A review of the literature

“…google.fr) in the "cited by" article from Girault et al (4) and Verloes et al (5), produced 2 more articles (30)(31). A search performed on abstracts from the European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) and the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) meetings from 2012 to 2017, produced two abstracts with unpublished data (32,33). Finally, a medical thesis provided more complete clinical data from patients that were only briefly described in reference 11 (34).…”

“…Steroids were administered to 17 patients (4,17,23,26,27,30,(32)(33)(34). No effect was reported in 11 patients and in 5 patients only a partial amelioration was noted.…”

“…In one patient (33) there were no details and it was before HSCT. It should be noted that the patients with partial effect presented with some aspect of IBD-like SD/THE (17,26,27,32). In some cases steroids were given in combination with immunosuppressant drugs.…”

“…Seven drugs were used for a combined total of 24 times in 13 patients (4,17,26,27,30,32,33). Thus, some patients were given multiple drugs, either sequentially or at the same time.…”

“…Sirolimus was used in 2 patients without effect, Tacrolimus was used twice in 3 patients without any effect (17,26) and one before HSCT (33). Anti-TNF antibody was used in 7 patients; for one there was no description of outcome (33), for 2 (17) there was no improvement, for 3 there was a partial and inconsistant improvement (26,27,32).…”