Correspondence: Mamata Sivagnanam, Division of Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, University of California San Diego, 9500 Gilman Drive, MC 0669, La Jolla, CA 92093, USA, mengineer@ucsd.edu, phone 619-543-7544, fax 619-543-7537. Publisher's Disclaimer: This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. Conflicts of Interest: None NIH-PA Author ManuscriptNIH-PA Author Manuscript NIH-PA Author ManuscriptBackground and Aims-Congenital Tufting Enteropathy (CTE) is a rare autosomal recessive diarrheal disorder presenting in the neonatal period. CTE is characterized by intestinal epithelial cell dysplasia leading to severe malabsorption and significant morbidity and mortality. The pathogenesis and genetics of this disorder are not well understood. The objective of this study was to identify the gene responsible for CTE.
A wide spectrum of entities may give rise to soft-tissue masses in children, including benign and malignant tumors, pseudotumors, and both neoplastic and nonneoplastic vascular lesions. Because of its excellent tissue contrast, multiplanar capability, and lack of ionizing radiation, magnetic resonance (MR) imaging has become the modality of choice in the evaluation of deep and large soft-tissue masses in children. In the vast majority of cases, however, accurate interpretation of the MR imaging findings requires correlation with the clinical findings. For example, in most posttraumatic and inflammatory pseudotumors, the clinical history is fundamental to establishing the diagnosis. In the evaluation of periarticular cysts, the location of the mass and its relationship to a joint are crucial for diagnosis, whereas in the evaluation of vascular lesions, including hemangiomas and vascular malformations, clinical findings combined with MR imaging findings are needed for accurate diagnosis in most cases. The identification of fat within adipocytic tumors is useful, but tissue biopsy may be required for final diagnosis. Nevertheless, MR imaging is useful in determining the origin and character of pediatric soft-tissue masses, defining their extent and their relationship to adjacent structures, and performing posttherapy follow-up.
Administration of antileukemic chemotherapy at diagnosis of chloroma is associated with a significantly lower probability of developing acute myeloid leukemia and with longer survival.
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