Sandbox MEN
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and keywords:
Overview
Historical Perspective
- In 1903 Erdheim described the case of an acromegalic patient with a pituitary adenoma and three enlarged parathyroid glands.
- In 1953 Underdahl et al. reported a case series of 8 patients with a syndrome of pituitary, parathyroid, and pancreatic islet adenomas.
- In 1954 Wermer noted that this syndrome was transmitted as a dominant trait.
- In 1959 Hazard et al. described medullary (solid) thyroid carcinoma.
- In 1961 Sipple described a combination of a pheochromocytoma, medullary thyroid carcinoma and parathyroid adenoma.
- In 1966 Williams et al. described the combination of mucosal neuromas, pheochromocytoma and medullary thyroid carcinoma.
- In 1968 Steiner et al. introduced the term "multiple endocrine neoplasia" (MEN) to describe disorders featuring combinations of endocrine tumors and proposed the terms 'Wermer syndrome' for MEN 1 and 'Sipple syndrome' for MEN 2.
- In 1974 Sizemore et al. showed that the MEN 2 category included two groups of patients with MTC and pheochromocytoma: one with parathyroid disease and a normal appearance (MEN 2A) and the other without parathyroid disease but with mucosal neuromas and mesodermal abnormalities (MEN 2B).
- In 1988 the MEN1 locus was assigned to Chromosome 11 (11q13).
- In 1993 mutations in the RET oncogene were shown to be the cause of MEN 2A by Lois Mulligan, working in the laboratory of Dr Bruce Ponder in Cambridge.[1]
- In 1998 the MEN1 gene was cloned[2]
Classification
Pathophysiology
- The term multiple endocrine neoplasia (MEN) encompasses several distinct syndromes featuring tumors of endocrine glands, each with its own characteristic pattern. In some cases, the tumors are malignant, in others, benign. Benign or malignant tumors of nonendocrine tissues occur as components of some of these tumor syndromes.
- MEN syndromes are inherited as autosomal dominant disorders.[3]
Genetics
Associated Conditions
Gross Pathology
Microscopic Pathology
Causes
Life Threatening Causes
Life-threatening causes include conditions which may result in death or permanent disability within 24 hours if left untreated.
Common Causes
Causes by Organ System
Cardiovascular | No underlying causes |
Chemical / poisoning | No underlying causes |
Dermatologic | No underlying causes |
Drug Side Effect | No underlying causes |
Ear Nose Throat | No underlying causes |
Endocrine | No underlying causes |
Environmental | No underlying causes |
Gastroenterologic | No underlying causes |
Genetic | No underlying causes |
Hematologic | No underlying causes |
Iatrogenic | No underlying causes |
Infectious Disease | No underlying causes |
Musculoskeletal / Ortho | No underlying causes |
Neurologic | No underlying causes |
Nutritional / Metabolic | No underlying causes |
Obstetric/Gynecologic | No underlying causes |
Oncologic | No underlying causes |
Opthalmologic | No underlying causes |
Overdose / Toxicity | No underlying causes |
Psychiatric | No underlying causes |
Pulmonary | No underlying causes |
Renal / Electrolyte | No underlying causes |
Rheum / Immune / Allergy | No underlying causes |
Sexual | No underlying causes |
Trauma | No underlying causes |
Urologic | No underlying causes |
Dental | No underlying causes |
Miscellaneous | No underlying causes |
Causes in Alphabetical Order
- A...
- Z...
Make sure that each diagnosis is linked to a page.
Differentiating type page name here from other Diseases
Epidemiology and Demographics
Age
Gender
Race
Developed Countries
Developing Countries
Risk Factors
Screening
Natural History, Complications and Prognosis
Diagnosis
Diagnostic Criteria
If available, the diagnostic criteria are provided here.
History
A directed history should be obtained to ascertain
Symptoms
"Type symptom here" is pathognomonic of the "type disease name here".
"Type non specific symptoms" may be present.
Past Medical History
Family History
Social History
Occupational
Alcohol
The frequency and amount of alcohol consumption should be characterized.
Drug Use
Smoking
Allergies
Physical Examination
Appearance of the Patient
Vital Signs
Skin
Head
Eyes
Ear
Nose
Mouth
Throat
Heart
Lungs
Abdomen
Extremities
Neurologic
Genitals
Other
Laboratory Findings
Electrolyte and Biomarker Studies
Electrocardiogram
Chest X Ray
CT
MRI
Echocardiography or Ultrasound
Other Imaging Findings
Other Diagnostic Studies
Treatment
Pharmacotherapy
Acute Pharmacotherapies
Chronic Pharmacotherapies
Surgery and Device Based Therapy
Indications for Surgery
Pre-Operative Assessment
Post-Operative Management
Transplantation
Primary Prevention
Secondary Prevention
Cost-Effectiveness of Therapy
Future or Investigational Therapies
References
- ↑ Germ-line mutations of the RET proto-oncogene in multiple endocrine neoplasia type 2A. Mulligan LM, Kwok JB, Healey CS, Elsdon MJ, Eng C, Gardner E, Love DR, Mole SE, Moore JK, Papi L, et al. Nature 1993 Jun 3;363(6428) 458-60 PMID 8099202
- ↑ Guru SC, Manickam P, Crabtree JS, Olufemi SE, Agarwal SK, Debelenko LV. Identification and characterization of the multiple endocrine neoplasia type 1 (MEN1) gene. J Intern Med 243(6) 433-9
- ↑ Template:DorlandsDict