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2007 ICD-9-CM Volume 1 Diagnosis Codes Home > Endocrine, Nutritional And Metabolic Diseases, And Immunity Disorders 240-279 > Diseases Of Other Endocrine Glands 250-259 > Polyglandular Dysfunction And Related Disorders 258.* > 2007 ICD-9-CM Diagnosis 258.0 Polyglandular activity in multiple endocrine adenomatosis This code may be outdated. View the most recent version of ICD-9-CM 258.0 A rare syndrome characterized by hyperplasia and/or neoplasms of the pituitary, parathyroid glands, and pancreatic islets. Hyperparathyroidism occurs in 90% of the cases and is usually the first manifestation of the syndrome. The most frequent pancreatic manifestation is gastrinoma typically leading to Zollinger-Ellison Syndrome. The appearance of this condition has been limited to the loss of allelic heterozygosity at the 11q13 locus on the long arm of chromosome 11. Patients overall exhibit long survival times. Chemotherapy is rare and surgical management is generally dependent on the genetic expression in individual patients. (From Holland et al., Cancer Medicine, 3d ed, pp1169-72) The MEN1 gene is a cloned tumor suppressor gene for Multiple Endocrine Neoplasia, a predisposition for endocrine tumors and carcinoid tumors. The gene encodes the tumor suppressor protein MEN1, which regulates transcription through direct interaction with, and inhibition of, transcription factor JunD. Menin also interacts with SMAD3 and appears to be required for SMAD3/4-DNA binding at transcriptional regulatory sites. The human gene is located at 11q13. 258.0 is a specific code that can be used to specify a diagnosis 258.0 contains 3 index entries View the ICD-9-CM Volume 1 258.* hierarchy 258.0 also known as: Wermer's syndrome Index entries containing 258.0: Disease, diseased - see also Syndrome Wermer's 258.0 Syndrome - see also Disease Wermer's (polyendocrine adenomatosis) 258.0 Wermer's syndrome or disease (polyendocrine adenomatosis) 258.0