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| {{DiseaseDisorder infobox |
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| Name = {{PAGENAME}} |
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| ICD10 = {{ICD10|D|44|8|d|37}} |
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| ICD9 = {{ICD9|258.0}} |
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| ICDO = 8360/1 |
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| Image = |
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| Caption = |
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| OMIM = 131100 |
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| MedlinePlus = 000398 |
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| eMedicineSubj = med |
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| eMedicineTopic = 2404 |
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| DiseasesDB = 7971 |
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| MeshID = D018761 |
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| }}
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| {{SI}}
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| {{CMG}}
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| ==Overview==
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| '''Multiple endocrine neoplasia type 1''' is part of a group of disorders that affect the [[endocrine system]].
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| == Explanation == | | ==[[Type page name here overview|Overview]]== |
| These disorders greatly increase the risk of developing multiple [[cancer]]ous and noncancerous [[tumor]]s in glands such as the [[parathyroid]], [[pituitary]], and [[pancreas]]. Multiple endocrine neoplasia occurs when tumors are found in at least two endocrine glands. Tumors can also develop in organs and tissues other than endocrine glands. If the tumors become cancerous, some cases can be life-threatening. The disorder affects 1 in 30,000 people.
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| Although many different types of hormone-producing tumors are associated with multiple endocrine neoplasia, tumors of the parathyroid gland, pituitary gland, and pancreas are most frequent in multiple endocrine neoplasia type 1. Tumors cause an overactivation of these hormone-producing glands, leading to serious health problems such as severe [[ulcer]]s. Overactivity of the parathyroid gland ([[hyperparathyroidism]]) is the most common sign of this disorder. Hyperparathyroidism disrupts the normal balance of [[calcium]] in the [[blood]], which can lead to [[kidney stones]], thinning of [[bone]]s, weakness, and fatigue.
| | ==[[Type page name here historical perspective|Historical Perspective]]== |
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| Neoplasia in the pituitary gland can manifest as [[prolactinoma|prolactinomas]] whereby too much [[prolactin]] is secreted, suppressing the release of [[gonadotropins]], causing a decrease in sex hormones such as [[testosterone]].
| | ==[[Type page name here classification|Classification]]== |
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| Pancreatic tumors usually form in the [[Islets of Langerhans|islet cells]], which over-secrete [[insulin]], causing a decrease in blood glucose levels.
| | ==[[Type page name here pathophysiology|Pathophysiology]]== |
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| == Major types == | | ==[[Type page name here causes|Causes]]== |
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| The two major types of multiple endocrine neoplasia, type 1 and [[Multiple endocrine neoplasia type 2|type 2]], are often confused because they have similar names. These types are distinguished by the genes involved, the hormones that are affected, and their characteristic signs and symptoms. They are also very different in their options for cancer.
| | ==[[Type page name here differential diagnosis|Differentiating Acute renal failure from other Diseases]]== |
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| Mutations in the ''[[MEN1]]'' [[gene]] cause multiple endocrine neoplasia type 1. The function of the ''MEN1'' gene is unknown.The '''MEN1''' gene is located in [[Chromosome 11]]q13 and the gene product is '''Menin''' (a cofactor for [[Transcription (genetics)|transcription]]).<ref>Fauci,et al ''Harrison's Principle of Internal Medicine'' 16th Ed. p 2453 </ref> Researchers believe that it acts as a tumor suppressor, which means it normally keeps [[cell (biology)|cell]]s from growing and dividing too rapidly or in an uncontrolled way. If mutations inactivate both copies of the ''MEN1'' gene, cells can grow and divide in a poorly controlled way to form tumors.
| | ==[[Type page name here epidemiology and demographics|Epidemiology and Demographics]]== |
| [[Image:autodominant2.jpg|thumb|left|Most cases of multiple endocrine neoplasia type 1 are inherited in an [[autosomal dominant]] pattern.]]
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| Most cases of multiple endocrine neoplasia type 1 are inherited in an [[autosomal dominant]] pattern, which means affected people may have affected siblings and relatives in successive generations (such as parents and children). An affected person usually has one parent with the condition. Some cases, however, result from new mutations in the ''MEN1'' gene. These cases occur in people with no history of the disorder in their family.
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| == Genetic effects == | | ==[[Type page name here risk factors|Risk Factors]]== |
| People with multiple endocrine neoplasia type 1 are born with one mutated copy of the ''MEN1'' gene in each cell. Then, during their lifetime, the other copy of the gene is mutated in a small number of cells. These genetic changes result in no functional copies of the ''MEN1'' gene in selected cells, allowing the cells to divide with little control and form tumors.
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| == See also == | | ==[[Type page name here screening|Screening]]== |
| * [[Multiple endocrine neoplasia]]
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| * [[Multiple endocrine neoplasia type 2]]
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| ==References== | | ==[[Type page name here natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
| {{reflist|2}}
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| ''This article incorporates public domain text from [http://ghr.nlm.nih.gov The U.S. National Library of Medicine]''
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| {{Endocrine pathology}}
| | ==Diagnosis== |
| | [[Type page name here history and symptoms|History and Symptoms]] | [[Type page name here physical examination|Physical Examination]] | [[Type page name here laboratory findings|Laboratory Findings]] | [[Type page name here electrocardiogram|Electrocardiogram]] |[[Type page name here chest x ray|Chest X Ray]] | [[Type page name here CT|CT]] | [[Type page name here MRI|MRI]] | [[Type page name here echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Type page name here other imaging findings|Other Imaging Findings]] | [[Type page name here other diagnostic studies|Other Diagnostic Studies]] |
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| [[Category:Genetic disorders]] | | ==Treatment== |
| [[Category:Endocrine system]] | | [[Type page name here medical therapy|Medical Therapy]] | [[Type page name here surgery|Surgery]] | [[Type page name here primary prevention|Primary Prevention]] | [[Type page name here secondary prevention|Secondary Prevention]] | [[Type page name here cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Type page name here future or investigational therapies|Future or Investigational Therapies]] |
| [[Category:Endocrinology]] | | |
| {{WikiDoc Help Menu}}
| | ==Case Studies== |
| {{WikiDoc Sources}}
| | [[Type page name here case study one|Case #1]] |