Multiple endocrine neoplasia type 1: Difference between revisions

Jump to navigation Jump to search
m (Robot: Automated text replacement (-{{WikiDoc Cardiology Network Infobox}} +, -<references /> +{{reflist|2}}, -{{reflist}} +{{reflist|2}}))
(Mahshid)
 
(30 intermediate revisions by 7 users not shown)
Line 1: Line 1:
{{DiseaseDisorder infobox |
__NOTOC__
  Name          = {{PAGENAME}} |
{{Multiple endocrine neoplasia type 1}}
  ICD10          = {{ICD10|D|44|8|d|37}} |
{{CMG}}; {{AE}} {{Ammu}} {{Ajay}} {{ARK}}
  ICD9          = {{ICD9|258.0}} |
  ICDO          = 8360/1 |
  Image          = |
  Caption        = |
  OMIM          = 131100 |
  MedlinePlus    = 000398 |
  eMedicineSubj  = med |
  eMedicineTopic = 2404 |
  DiseasesDB    = 7971 |
  MeshID        = D018761 |
}}
{{SI}}
{{GS}}


'''For patient information, click [[{{PAGENAME}} (patient information)|here]].'''


{{SK}} Multiple endocrine adenomatosis, type 1; Multiple endocrine neoplasia syndrome type 1; Wermer syndrome; MEN, type 1; MEN 1 syndrome; MEN 1 - Multiple endocrine neoplasia syndrome type 1; MEN type I; Multiple endocrine neoplasia type 1


==Overview==
==[[Multiple endocrine neoplasia type 1 overview|Overview]]==
'''Multiple endocrine neoplasia type 1''' is part of a group of disorders that affect the [[endocrine system]].


== Explanation ==
==[[Multiple endocrine neoplasia type 1 historical perspective|Historical Perspective]]==
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.


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.
==[[Multiple endocrine neoplasia type 1 classification|Classification]]==


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]].
==[[Multiple endocrine neoplasia type 1 pathophysiology|Pathophysiology]]==


Pancreatic tumors usually form in the [[Islets of Langerhans|islet cells]], which over-secrete [[insulin]], causing a decrease in blood glucose levels.
==[[Multiple endocrine neoplasia type 1 causes|Causes]]==


== Major types ==
==[[Multiple endocrine neoplasia type 1 differential diagnosis|Differentiating Multiple Endocrine Neoplasia Type 1 From Other Diseases]]==


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.
==[[Multiple endocrine neoplasia type 1 epidemiology and demographics|Epidemiology and Demographics]]==


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.
==[[Multiple endocrine neoplasia type 1 risk factors|Risk Factors]]==
[[Image:autodominant2.jpg|thumb|left|Most cases of multiple endocrine neoplasia type 1 are inherited in an [[autosomal dominant]] pattern.]]
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.


== Genetic effects ==
==[[Multiple endocrine neoplasia type 1 screening|Screening]]==
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.


== See also ==
==[[Multiple endocrine neoplasia type 1 natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
* [[Multiple endocrine neoplasia]]
* [[Multiple endocrine neoplasia type 2]]


==References==
==Diagnosis==
{{reflist|2}}
[[Multiple endocrine neoplasia type 1 history and symptoms|History and Symptoms]] | [[Multiple endocrine neoplasia type 1 physical examination|Physical Examination]] | [[Multiple endocrine neoplasia type 1 laboratory findings|Laboratory Findings]] | [[Multiple endocrine neoplasia type 1 electrocardiogram|Electrocardiogram]] | [[Multiple endocrine neoplasia type 1 x ray|X Ray]] | [[Multiple endocrine neoplasia type 1 CT|CT]] | [[Multiple endocrine neoplasia type 1 MRI|MRI]] | [[Multiple endocrine neoplasia type 1 ultrasound|Ultrasound]] | [[Multiple endocrine neoplasia type 1 other imaging findings|Other Imaging Findings]] | [[Multiple endocrine neoplasia type 1 other diagnostic studies|Other Diagnostic Studies]]
''This article incorporates public domain text from [http://ghr.nlm.nih.gov The U.S. National Library of Medicine]''


{{Endocrine pathology}}
==Treatment==
[[Multiple endocrine neoplasia type 1 medical therapy|Medical Therapy]] | [[Multiple endocrine neoplasia type 1 surgery|Surgery]] | [[Multiple endocrine neoplasia type 1 primary prevention|Primary Prevention]] | [[Multiple endocrine neoplasia type 1 secondary prevention|Secondary Prevention]] | [[Multiple endocrine neoplasia type 1 cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Multiple endocrine neoplasia type 1 future or investigational therapies|Future or Investigational Therapies]]


[[Category:Genetic disorders]]
==Case Studies==
[[Category:Endocrine system]]
[[Multiple endocrine neoplasia type 1 case study one|Case #1]]
 
[[Category:Oncology]]
[[Category: Endocrinology]]
[[Category:Up-To-Date]]
[[Category:Oncology]]
[[Category:Medicine]]
[[Category:Endocrinology]]
[[Category:Endocrinology]]
{{WikiDoc Help Menu}}
[[Category:Surgery]]
{{WikiDoc Sources}}

Latest revision as of 02:44, 27 November 2017

Multiple endocrine neoplasia type 1 Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Multiple endocrine neoplasia type 1 from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X Ray

CT

MRI

Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Multiple endocrine neoplasia type 1 On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Multiple endocrine neoplasia type 1

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Multiple endocrine neoplasia type 1

CDC on Multiple endocrine neoplasia type 1

Multiple endocrine neoplasia type 1 in the news

Blogs on Multiple endocrine neoplasia type 1

Directions to Hospitals Treating Multiple endocrine neoplasia type 1

Risk calculators and risk factors for Multiple endocrine neoplasia type 1

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ammu Susheela, M.D. [2] Ajay Gade MD[3]] Aravind Reddy Kothagadi M.B.B.S[4]

For patient information, click here.

Synonyms and keywords: Multiple endocrine adenomatosis, type 1; Multiple endocrine neoplasia syndrome type 1; Wermer syndrome; MEN, type 1; MEN 1 syndrome; MEN 1 - Multiple endocrine neoplasia syndrome type 1; MEN type I; Multiple endocrine neoplasia type 1

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Multiple Endocrine Neoplasia Type 1 From Other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | X Ray | CT | MRI | Ultrasound | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1