MCID: EMP001
MIFTS: 39

Empty Sella Syndrome malady

Categories: Rare diseases, Endocrine diseases

Aliases & Classifications for Empty Sella Syndrome

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Aliases & Descriptions for Empty Sella Syndrome:

Name: Empty Sella Syndrome 11 46 47 13 37 66
Empty Sella Turcica 46
 
Empty Sella 48

Classifications:



External Ids:

Disease Ontology11 DOID:3642
MeSH37 D004652
NCIt43 C84686

Summaries for Empty Sella Syndrome

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NIH Rare Diseases:46 Empty sella syndrome (ess) is a condition that involves the sella turcica, a bony structure at the base of the brain that protects the pituitary gland. there is a primary and secondary form of the condition. the primary form occurs when a structural defect above the pituitary gland increases pressure in the sella turcica and causes the gland to flatten. the secondary form occurs when the pituitary gland is damaged due to injury, a tumor, surgery or radiation therapy. some people with ess have no symptoms. people with secondary ess may have symptoms of decreased pituitary function such as absence of menstruation, infertility, fatigue, and intolerance to stress and infection. in children, ess may be associated with early onset of puberty, growth hormone deficiency, pituitary tumors, or pituitary gland dysfunction. treatment focuses on the symptoms present in each person. last updated: 7/14/2014

MalaCards based summary: Empty Sella Syndrome, also known as empty sella turcica, is related to diabetes insipidus and central retinal vein occlusion, and has symptoms including pituitary enlargement An important gene associated with Empty Sella Syndrome is PRL (Prolactin), and among its related pathways are Glucocorticoid receptor regulatory network and Development_Leptin signaling via JAK/STAT and MAPK cascades. Affiliated tissues include pituitary, brain and testes, and related mouse phenotypes are liver/biliary system and renal/urinary system.

NINDS:47 Empty Sella Syndrome (ESS) is a disorder that involves the , a bony structure at the base of the brain that surrounds and protects the pituitary gland. ESS is often discovered during radiological imaging tests for pituitary disorders. ESS occurs in up to 25 percent of the population. An individual with ESS may have no symptoms or may have symptoms resulting from partial or complete loss of pituitary function (including headaches, low sex drive, and impotence).

Wikipedia:69 Empty sella syndrome (abbreviated ESS) is where the pituitary gland shrinks or becomes flattened,... more...

Related Diseases for Empty Sella Syndrome

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Diseases related to Empty Sella Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50)    (show all 153)
idRelated DiseaseScoreTop Affiliating Genes
1diabetes insipidus28.8GNRH1, INS, POMC, PRL
2central retinal vein occlusion28.8INS, POMC, PRL, TRH
3obesity28.8INS, NPY, POMC, PRL
4pituitary deficiency due to empty sella turcica syndrome12.4
5lactic acidosis10.4POMC, PRL
6colloid carcinoma of the pancreas10.4POMC, PRL
7hypothryoidism, congenital, nongoitrous 410.4POMC, PRL
8immune system organ benign neoplasm10.4POMC, PRL
9osseous heteroplasia, progressive10.4POMC, PRL
10cold-induced sweating syndrome 210.4POMC, PRL
11congenital epulis10.4POMC, PRL
12acute myocarditis10.4POMC, PRL
13genetic lipodystrophy10.4PRL, TRH
14loeffler endocarditis10.4POMC, PRL
15craniofacial anomalies and anterior segment dysgenesis syndrome10.4
16nasopharyngeal disease10.4POMC, PRL
17malt worker's lung10.4NPY, PRL
18myoclonic cerebellar dyssynergia10.3POMC, PRL
19malignant granular cell skin tumor10.3POMC, TRH
20syncope10.3NPY, PRL
21molybdenum cofactor deficiency b10.3POMC, PRL
22echolalia10.3PRL, TRH
23chronic salpingo-oophoritis10.3POMC, PRL
24myxopapillary ependymoma10.2NPY, TRH
25adenoma10.2
26hemiplegia alterans10.2INS, PRL
27paranasal sinus cancer, adult10.2INS, PRL
28benign eccrine breast spiradenoma10.2INS, POMC
29acromegaly10.2
30pituitary adenoma10.2
31hypopituitarism10.2
32acth deficiency10.2
33scleredema adultorum10.2INS, PRL
34acinar cell carcinoma10.2NPY, PRL
35conjunctival vascular disease10.2INS, POMC
36intracranial hypertension, idiopathic10.2
37lymphosarcoma10.2NPY, POMC
38hyperprolactinemia10.2
39intracranial hypertension10.2
40neurilemmoma of the pleura10.1INS, POMC
41hypertelorism, preauricular sinus, punctal pits, and deafness10.1INS, TRH
42patellofemoral pain syndrome10.1INS, POMC
43constrictive pericarditis10.1INS, NPY
44central nervous system angiosarcoma10.1INS, POMC
45chancroid10.1INS, POMC
46laryngeal carcinoma10.1INS, POMC
47pituitary apoplexy10.1
48amenorrhea10.1
49hypothyroidism10.1
50thyroiditis10.1

Graphical network of the top 20 diseases related to Empty Sella Syndrome:



Diseases related to empty sella syndrome

Symptoms for Empty Sella Syndrome

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UMLS symptoms related to Empty Sella Syndrome:


pituitary enlargement

Drugs & Therapeutics for Empty Sella Syndrome

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Interventional clinical trials:

idNameStatusNCT IDPhase
1Growth Hormone Deficiency and Empty Sella Cardio-Metabolic Risk Factors in Obesity: a Cross-Sectional StudyCompletedNCT02092779
2Da Vinci Transoral Robotic-assisted Surgery of Pituitary GlandRecruitingNCT02743442

Search NIH Clinical Center for Empty Sella Syndrome


Cochrane evidence based reviews: empty sella syndrome

Genetic Tests for Empty Sella Syndrome

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Anatomical Context for Empty Sella Syndrome

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MalaCards organs/tissues related to Empty Sella Syndrome:

34
Pituitary, Brain, Testes, Thyroid, Bone

Animal Models for Empty Sella Syndrome or affiliated genes

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MGI Mouse Phenotypes related to Empty Sella Syndrome:

39
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053708.0GNRH1, INS, NPY, POMC, PRL
2MP:00053677.7GNRH1, INS, NPY, POMC
3MP:00053797.5GNRH1, INS, POMC, PRL, TRH
4MP:00036317.1GNRH1, INS, NPY, POMC, PRL, TRH
5MP:00053767.1GNRH1, INS, NPY, POMC, PRL, TRH

Publications for Empty Sella Syndrome

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Articles related to Empty Sella Syndrome:

(show top 50)    (show all 192)
idTitleAuthorsYear
1
Ectopic Acromegaly Arising from a Pituitary Adenoma within the Bony Intersphenoid Septum of a Patient with Empty Sella Syndrome. (27468406)
2016
2
A Case Report of Late Onset Mania Caused by Hyponatremia in a Patient With Empty Sella Syndrome. (26871784)
2016
3
Empty sella syndrome in a male child with failure to thrive. (25878744)
2015
4
Partial empty sella syndrome: a case report and review. (24757313)
2014
5
Empty sella syndrome - beyond being an incidental finding. (23565413)
2012
6
Empty sella syndrome. (23304808)
2012
7
Empty sella syndrome in thyroid cancer survivor. (21837937)
2011
8
Diagnostic dilemmas in a patient with anaemia. Empty sella syndrome--a case report. (20806186)
2010
9
Transsphenoidal treatment of secondary empty sella syndrome using low field strength intraoperative MRI: case report. (21132613)
2010
10
Empty sella syndrome associated with a hyperfunctioning microadenoma invading the clivus. (18071980)
2007
11
Cardiac tamponade under thyroid hormone replacement therapy in a patient with empty sella syndrome. (17143379)
2006
12
Treatment of empty sella syndrome with ventriculoperitoneal shunt. (15749434)
2005
13
Empty sella syndrome in nevoid basal cell carcinoma syndrome. (10838118)
2000
14
Prader-Labhart-Willi syndrome with central precocious puberty and empty sella syndrome. (10591439)
1999
15
Adrenal adenocarcinoma and empty sella syndrome in a 37-year-old woman. (10533165)
1999
16
Two cases of empty sella syndrome with variable dexamethasone-suppressibility of ACTH and cortisol. (8930530)
1996
17
Transsphenoidal extradural chiasmapexy in the management of the symptomatic primary empty sella syndrome. (8237166)
1993
18
Primary empty sella syndrome and hypogonadotropic hypogonadism in young male patients. (1502422)
1992
19
Empty sella syndrome associated with diabetes insipidus: case report and review of the literature. (2671113)
1989
20
Transsphenoidal chiasmopexy for long standing visual failure in the secondary empty sella syndrome. (3267311)
1988
21
Antipituitary antibodies in patients with the primary empty sella syndrome. (3417843)
1988
22
Empty sella syndrome associated with central nervous system cysticercosis. (3154189)
1988
23
Primary empty sella syndrome and benign intracranial hypertension. (3948665)
1986
24
Headache and the primary empty sella syndrome. (3718697)
1986
25
Adipsic hypernatremia in a patient with pseudotumor cerebri and the primary empty sella syndrome. (4067208)
1985
26
Primary empty sella syndrome with panhypopituitarism in a child. (6543857)
1984
27
The empty sella syndrome. (6575148)
1983
28
Empty sella syndrome with visual field disturbance. (6855016)
1983
29
Coexistent primary empty sella syndrome and hyperprolactinemia. Report of 11 cases. (6870411)
1983
30
Intracranial pressure in patients with the empty sella syndrome without benign intracranial hypertension. (7086441)
1982
31
Empty sella syndrome. (7338504)
1981
32
The primary empty sella syndrome. Diagnosis with metrizamide cisternography. (6895391)
1981
33
Primary empty sella syndrome and hypopituitarism associated with primary hypothyroidism. (6792264)
1981
34
Blood-cerebrospinal fluid barrier for prolactin in empty sella syndrome. (7291947)
1981
35
A case of empty sella syndrome with sufficient hypophyseal function and normal pregnancy. (7250078)
1981
36
CSF circulation in subjects with the empty sella syndrome. (7254514)
1981
37
Familial pseudotumor cerebri and the empty sella syndrome. (6927213)
1980
38
Primary empty sella syndrome with CSF rhinorrhea. (7420665)
1980
39
The Empty Sella syndrome secondary to Rathke's cleft cyst. (7435283)
1980
40
Diagnosis of primary empty sella syndrome by computed tomography. (517991)
1979
41
Primary empty sella syndrome with panhypopituitarism, diabetes insipidus, and visual field defects. (707005)
1978
42
The primary Empty Sella Syndrome in Hawaii. (914514)
1977
43
The primary empty sella syndrome: analysis of the clinical characteristics, radiographic features, pituitary function and cerebrospinal fluid adenohypophysial hormone concentrations. (851132)
1977
44
The primary empty sella syndrome in a child. (839337)
1977
45
The empty sella syndrome. (904521)
1977
46
Spontaneous fracture of the dorsum sellae associated with post-irradiation empty sella syndrome. (897994)
1977
47
Primary empty sella syndrome with visual field defects. (937363)
1976
48
The empty sella syndrome. (1013000)
1976
49
Empty sella syndrome secondary to intrasellar cyst in adolescence. (961646)
1976
50
Contiguous internal carotid arteries in empty sella syndrome. (935432)
1976

Variations for Empty Sella Syndrome

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Expression for genes affiliated with Empty Sella Syndrome

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Search GEO for disease gene expression data for Empty Sella Syndrome.

Pathways for genes affiliated with Empty Sella Syndrome

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GO Terms for genes affiliated with Empty Sella Syndrome

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Cellular components related to Empty Sella Syndrome according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1secretory granuleGO:003014110.1POMC, TRH
2secretory granule lumenGO:00347749.6INS, POMC
3extracellular spaceGO:00056158.0GNRH1, INS, NPY, POMC
4extracellular regionGO:00055766.8GNRH1, INS, NPY, POMC, PRL, TRH

Biological processes related to Empty Sella Syndrome according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1regulation of appetiteGO:003209810.0NPY, POMC
2neuropeptide signaling pathwayGO:00072189.6NPY, POMC
3negative regulation of feeding behaviorGO:20002529.5INS, TRH
4female pregnancyGO:00075659.4GNRH1, PRL
5regulation of blood pressureGO:00082179.3NPY, POMC
6cell-cell signalingGO:00072677.8GNRH1, INS, POMC, TRH

Molecular functions related to Empty Sella Syndrome according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1G-protein coupled receptor bindingGO:00016649.4NPY, POMC
2neuropeptide hormone activityGO:00051848.7NPY, POMC, TRH
3hormone activityGO:00051798.0GNRH1, INS, POMC, PRL

Sources for Empty Sella Syndrome

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
25GTR
26HGMD
27HMDB
28ICD10
29ICD10 via Orphanet
30ICD9CM
31IUPHAR
32KEGG
35MedGen
37MeSH
38MESH via Orphanet
39MGI
42NCI
43NCIt
44NDF-RT
47NINDS
48Novoseek
50OMIM
51OMIM via Orphanet
55PubMed
56QIAGEN
61SNOMED-CT via Orphanet
65Tumor Gene Family of Databases
66UMLS
67UMLS via Orphanet