Magnetic resonance enterography with oral mannitol solution: Diagnostic efficacy and image quality in Crohn disease.

PURPOSE: The aim of this study was to investigate the diagnostic efficacy and image quality of magnetic resonance enterography (MRE) using oral mannitol solution for the evaluation Crohn disease (CD). MATERIALS AND METHODS: We retrospectively evaluated MRE examinations of 153 patients with an assumed or definitive diagnosis of CD. There were 65 men and 88 women, with a mean age of 35.7 years (range: 6-73years). MRE findings of the patients were compared to histopathologic results obtained by surgery-fiberoptic endoscopy. The sensitivity, specificity and diagnostic efficacy rate were calculated. Additionally, image quality of MRE was evaluated using a four-point scale (1=excellent, 4=poor/non-diagnostic). RESULTS: Sensitivity, specificity and diagnostic efficacy were 92.5%, 93% and 92.8%, respectively. Six patients had false-positive and five patients had false-negative findings. Three falsely positive patients had ulcerative colitis and three had non-specific terminal ileitis. A total of 765 small bowel segments were analyzed; 475 (62%) had an image quality score of 1 and 15 (2%), an image quality score of 4. CONCLUSION: MRE using oral mannitol solution provides excellent image quality for MRE and has high degrees of diagnostic efficacy in CD patients.

Celiac disease and glycogenic acanthosis: a new association?

UNLABELLED: A 6-y-old boy and an 8-y-old girl were admitted to our clinic with anaemia and failure to thrive. Laboratory tests revealed iron deficiency anaemia and positive antigliadin antibodies in both of the patients. Slightly raised grey-white plaques were observed on oesophageal mucosa during endoscopical investigation of the patients. While intestinal mucosal samples confirmed diagnosis of celiac disease histologically, histopathological assessment of oesophageal lesions demonstrated glycogenic acanthosis. Since glycogenic acanthosis associated with celiac disease hasn't been reported in the literature previously to our knowledge, case reports of our patients were presented. CONCLUSION: We suggest that glycogenic acanthosis needs to be investigated as a possible new association of celiac disease in greater paediatric series.

Bernard-Soulier Syndrome Like Platelet Defect in a Patient with Noonan Syndrome; A Case Report.

Noonan's Syndrome (NS) is characterized by dismorphic facial features, short stature, short or webbed neck, congenital heart defects and testicular abnormalities. Various bleeding disorders in Noonan Syndrome have been reported. Bernard-Soulier Syndrome (BSS) is a rare congenital bleeding disorder characterized by thrombocytopenia and giant platelets. There is not any reported case of Noonan syndrome associated with BSS in literature. We report here a four-year-old male patient with Noonan Syndrome and BSS like platelet defect.

Juvenile rheumatoid arthritis presented with thrombocytopenia.

Leukopenia and thrombocytopenia are rare findings in systemic onset juvenile rheumatoid arthritis (S-JRA), and if present, bone marrow (BM) examination is necessary to exclude malignant diseases. We report here a 13.5-year-old boy with S-JRA who had severe thrombocytopenia and mild leukopenia, without arthritis, at the onset of the disease. BM was hypercellular with increased numbers of myeloid precursors and megakaryocytes. After treatment with acetylsalicylic acid, leukocyte and platelet counts returned to normal levels, and after two months chronic arthritis developed.