1/4. Colonic manifestations of multiple endocrine neoplasia type 2b: report of four cases.multiple endocrine neoplasia type 2b is best known for its endocrine manifestations and typical phenotype. The gastrointestinal manifestations, however, are also an important and commonly unrecognized component of the syndrome. We present four cases that demonstrate the varied presentation of patients with colonic manifestations of multiple endocrine neoplasia type 2b. We discuss the cause, diagnostic significance, and management of the colonic disease that is a component of multiple endocrine neoplasia type 2b.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
2/4. Multiple endocrine neoplasia (men) syndrome type IIB: gastrointestinal manifestations.Multiple endocrine neoplasia syndrome IIB is an inherited autosomal dominant disorder with variable penetrance. Multiple organ systems are involved with a characteristic triad of medullary thyroid carcinoma, pheochromocytoma, and alimentary tract ganglioneuromas. Most patients have characteristic facies with patulous lips and thickened tarsal plates of the eyelids. The entire gastrointestinal tract can be involved from tongue to anus. tongue nodules are common. Altered intestinal motility, dilation, diverticula, and mucosal abnormality may be seen radiographically. Gastrointestinal symptoms are common, may be the presenting manifestation of the syndrome early in life, and allow the radiologist to suggest the proper diagnosis. Five patients are described. All had megacolon.- - - - - - - - - - ranking = 0.625keywords = endocrine (Clic here for more details about this article) |
3/4. Multiple endocrine neoplasia, type 2b, as a cause of megacolon.A case of multiple endocrine neoplasia, type 2b, is reported presenting with weight loss, neck masses, and constipation. A medullary thyroid carcinoma was found to be present together with a megacolon. In this syndrome, the diagnosis of the thyroid tumor is rarely made before it is well established; hence an appreciation of the accompanying gastrointestinal symptoms and signs may lead to early diagnosis. The case had many classical features, such as ganglioneuromata involving the tongue, lips, and eyelids and typical biochemical findings. However, several unusual findings were present, such as the absence of any skeletal abnormalities, an acute onset of constipation, and the presence of bony metastases and the case is reported to illustrate the complexity of this uncommon entity.- - - - - - - - - - ranking = 0.625keywords = endocrine (Clic here for more details about this article) |
4/4. hyperplasia of the myenteric plexus. Its association with early infantile megacolon and neurofibromatosis.An infant with cutaneous criteria for neurofibromatosis had hyperplasia of the intestinal myenteric plexus and a clinical presentation mimicking Hirschsprung's disease. Many of his phenotypic features are also found in multiple endocrine neoplasia type IIb, a condition in which hyperplasia of the myenteric plexus is common. This case illustrates the overlapping nature of neural crest-derived conditions and the variable presentation of megacolon (ganglionic or aganglionic) in infancy.- - - - - - - - - - ranking = 0.125keywords = endocrine (Clic here for more details about this article) |