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Endocrine Involvement in Systemic Amyloidosis
Institution:3. From the Department of Biochemistry and Molecular Biology, Thomas Jefferson University, Philadelphia, Pennsylvania 19107,;4. the Department of Biochemistry & Biophysics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania 19104,;5. the Department of Microbiology, University of Alabama at Birmingham, Birmingham, Alabama 35294,;6. the Division of Microbiology and Immunology, Department of Pathology, University of Utah School of Medicine, Salt Lake City, Utah 84112,;12. the Institute of Biomembranes and Bioenergetics, National Research Council, 70126 Bari, Italy;2. Department of Pathology, Hospital Clinic of Barcelona, Barcelona, Spain;1. Leukemia Program, Department of Hematology and Medical Oncology, Cleveland Clinic Taussig Cancer Institute, Cleveland, Ohio;2. Medical Humanities Department, Davidson College, Davidson, North Carolina;3. Department of Hematologic Oncology and Blood Disorders, Levine Cancer Institute, Carolinas HealthCare System, Charlotte, North Carolina;1. Department of ENT, Zain and Al-Sabah Hospitals, Ministry of Health, State of Kuwait;2. Department of Surgery, Al-Adan Hospital, Ministry of Health, State of Kuwait;3. Chairman of Oral and Maxillofacial Surgery, Al-Amiri Hospital, Ministry of Health, State of Kuwait;4. Professor of Paediatric and Developmental Pathology, NIHR Senior Investigator, NIHR GOSH BRC Theme Lead Diagnostics and Imaging, Great Ormond Street Hospital and ICH (UCL), United Kingdom;5. Chairman of ENT College, Post-Graduate Training, Kuwait Institute of Medical Specialization (KIMS), ENT Department, Zain and Al-Sabah Hospitals, Ministry of Health, State of Kuwait;1. Dept. of Psychiatry and Behavioral Neuroscience, University of Cincinnati, 2120 E. Galbraith Road, Cincinnati, OH, USA;2. Dept. of Pathology and Laboratory Medicine, University of Cincinnati, 2120 E. Galbraith Road, Cincinnati, OH, USA
Abstract:ObjectiveTo present an overview of the published data on endocrine involvement and endocrine dysfunction in patients with systemic amyloidosis.MethodsWe conducted a review of the medical literature using MEDLINE data sources, including clinical trials, in vitro studies, and case reports on pituitary, thyroid, parathyroid, pancreatic, adrenal, and gonadal involvement in systemic amyloidosis.ResultsReports of endocrine involvement in systemic amyloidosis seem to consist primarily of small-samplesize clinical trials or case reports, probably because of the rarity of the disease itself. Systemic amyloidosis mainly involves and causes functional impairment in the thyroid and testes in the endocrine system. Evaluation of adrenal function necessitates special consideration because amyloid infiltration of the adrenal glands resulting in failure may be a life-threatening condition. Amyloid deposition commonly seen in the pituitary gland and the pancreas of patients with Alzheimer disease and type 2 diabetes mellitus, respectively, is generally classified as local amyloidosis and should not be confused with systemic involvement. Additionally, detection of amyloid deposition in the thyroid and testes may have a diagnostic role in patients with suspected systemic or renal amyloidosis.ConclusionPublished data suggest that systemic amyloidosis frequently involves the endocrine system, and endocrine dysfunction seems to be not as rare as previously thought. A rapidly growing goiter or symptoms and signs of adrenal or gonadal dysfunction should raise suspicion of amyloid infiltration. Involvement of pituitary, parathyroid, and pancreatic sites in systemic amyloidosis still remains to be clarified. Further studies with larger sample sizes are needed for complete characterization of the effect of systemic amyloidosis on the endocrine system. (Endocr Pract. 2010;16:1056-1063)
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