Frequency and clinical correlations of granulomas in children with Crohn disease.

BACKGROUND: Granulomas are pathognomonic findings of Crohn disease (CD); however, their occurrence and clinical significance are not well characterized. Our aim was to determine the frequency and distribution of granulomas in untreated and treated patients with CD and their relation to age and disease severity. PATIENTS AND METHODS: Records from patients with CD undergoing colonoscopy with terminal ileum biopsy over 7 years were reviewed. Clinical information and laboratory, pathology, and radiology results were recorded. The frequency and distribution of granulomas were determined. RESULTS: From 376 patients with CD, 75% underwent concurrent esophagogastroduodenoscopy and colonoscopy. Of those, 65% (184/282) were untreated. Granulomas were identified in 48% (136/282) of all patients and in 61% (112/184) of untreated patients and 24.5% (24/98) of treated patients (P < 0.0005). The upper tract and terminal ileum biopsies were essential to the identification of 42% of patients with granulomas. The presence of granulomas at diagnosis was related to anti-Saccharomyces cerevisiae antibodies, hypoalbuminemia, perianal disease, and gastritis at presentation (P = 0.03, P = 0.008, P = 0.03, and P = 0.001), respectively, and to perianal disease and infliximab treatment at the latest visit (P = 0.02 and P = 0.01), respectively. Granulomas were not related to age, sex, ethnicity, weight and height z scores, hemoglobin, C-reactive protein, erythrocyte sedimentation rate, CARD15/NOD2 mutations, abdominal surgery, or stricturing or fistulizing disease. CONCLUSIONS: Granulomas were identified in 61% of fully investigated pediatric patients with CD at diagnosis, including a substantial proportion of patients in whom colonoscopy to the cecum would have been insufficient for diagnosis. Granulomas were more frequent in untreated patients (P < 0.0005), and their prevalence was not affected by age. The presence of granulomas at diagnosis was associated with perianal disease, gastritis, hypoalbuminemia, anti-Saccharomyces cerevisiae antibodies, and infliximab treatment.

Histological features of ileitis differentiating pediatric Crohn disease from ulcerative colitis with backwash ileitis.

BACKGROUND/AIM: Pediatric ileocolonic Crohn disease (CD) may be difficult to distinguish from ulcerative colitis (UC) with backwash ileitis (BWI). The primary aim of the study was to determine the probability of CD in children with a confluent colitis and ileitis when newly diagnosed with inflammatory bowel disease (IBD). METHODS: A retrospective observational study of 100 newly diagnosed patients with IBD was performed. Two pathologists reviewed ileal biopsy specimens for 8 histological features. Biopsy and clinical features were evaluated for predictive ability of a final diagnosis of CD. RESULTS: The presence of crypt distortion, lamina propria (LP) expansion, and acute LP inflammation combined with 4 clinical variables in multivariate regression analysis had adequate discriminative validity when comparing the mean probability of a final CD diagnosis between CD and not-CD groups (0.90 vs. 0.59, p value <0.001). When crypt distortion, LP expansion, and acute LP inflammation are present in any combination, the sensitivity and specificity for presence of CD ranges 38.4-57% and 92.9-100%, respectively. CONCLUSIONS: Combining histological features of ileitis and clinical variables can adequately discriminate between the presence and absence of Crohn disease in children who present with confluent colitis and ileitis. Combined presence of certain histological features has high specificity for CD.

Does "cystic" biliary atresia represent a distinct clinical and etiological subgroup? A series of three cases.

We describe a unique series of 3 cases of biliary atresia (BA) associated with a choledochal cyst. All 3 children presented with jaundice at birth and had no other abnormalities. Although these children had a fetal form of BA, their presentation and outcome differed from those of biliary atresia splenic malformation syndrome (BASM), the well-described form of early onset BA. Unlike those with BASM, these children had no other associated malformations, had a normal birth weight, and did not yet require a liver transplant. We believe that the present series of patients and their associated pathology may represent a distinct phenotype with a common, prenatally acquired etiology that is different from other fetal forms of BA, such as BASM, and from patients who present with the classic perinatal form of BA.